10-17-02CraniofacialAnomaliesPart2

CRANIOFACIAL EMBRYOLOGY

To understand craniofacial anomalies and the con-text in which the anomalies occur, a completeunderstanding of embryogenesis of this region isimperative. Sperber1 presents a complete descrip-tion of the processes of embryogenesis of the cranio-facial skeleton, excerpted here.

The primary germ layers of the embryo, the ecto-derm and endoderm, form in the inner cell mass ofthe blastocyst. The ectoderm differentiates intocutaneous and neural portions by about day 20.Neural crest cells make up the most important struc-tures in craniofacial biology. Neural crest ectomesen-chyme has great migratory propensity and is the majorsource of connective tissue throughout the body.Neural crest cells differentiate into cartilage, bone,ligaments, muscles, and arteries. Any disruption inthe orderly migration and differentiation of thesecells can have severe consequences, manifested ascongenital defects.2

The crucial period of organogenesis takes place inthe first 12 weeks of gestation, and it is during thistime that the majority of congenital craniofacialanomalies occur.1,2 The earliest signs of the futureface make their appearance at approximately day23–24 of embryonic life as paired mandibular pro-cesses of the first branchial arch. Neural crest cellsmake up the most important structures in the head.Next, the medial nasal processes combine with theintervening forebrain to form the frontonasal pro-

cess, which is destined to become the future fore-head and the dorsum of the nose. The lateral nasalfolds separate the olfactory pits from the graduallydeveloping eye region. By the end of embryonicweek 5, the maxillary and mandibular processes havebegun to increase in size but have not yet fused. It isnot until week 6 that definitive jaws are formed.

By the end of week 8, the face assumes most ofthe characteristics that make it recognizable ashuman. The face derives from five prominencesthat surround the future mouth, the single frontonasaland the paired maxillary and mandibular processes(Fig 1).

The grooves between these facial prominencesusually disappear by day 46–47 of gestation. A per-sisting groove will result in a congenital facial cleft.

ETIOLOGY OF CRANIOFACIAL ANOMALIES

Table 1 represents suspected contributions tothe development of congenital craniofacial abnor-malities.3,4

The possibility of identifying distinct genetic aber-rations in craniofacial dysmorphology has improvedsignificantly in recent times. Specific genetic abnor-malities resulting in syndromic craniosynostoses andfacial dysostoses will be presented later in this issue.

In addition to the genetic component, distinctenvironmental causes have been identified, includ-

CRANIOFACIAL ANOMALIES II:SYNDROMES AND SURGERY

Delora L Mount MD

Fig 1. Embryonic development of the human face at (a) 5, (b) 6, and (c) 7 weeks. BG, first branchial groove; FNP, frontonasal prominence;LNP, lateral nasal prominence; MDP, mandibular prominence; MNP, medial nasal prominence; MXP, maxillary prominence. (Reprintedwith permission from Sperber GH: Craniofacial Embryology, 4th ed. London, Wright, 1989.)

SRPS Volume 10, Number 17, Part 2

2

ing radiation, infection, maternal factors, and chemi-cal exposures.

Radiation. Large doses of radiation have beenassociated with microcephaly.

Infection. The children of mothers affected withtoxoplasmosis, rubella, or cytomegalovirus showincreased frequency of facial clefts as well as con-comitant hand and ocular abnormalities.

Maternal idiosyncrasies. Mothers of cleft lip andpalate children have been noted to have a higher-than-normal incidence of the phenylketonuria disor-der. The oculoauriculovertebral (OAV) spectrum hasbeen seen with unusual frequency in infants of dia-betic mothers.5 Many studies associate maternalfactors such as age, weight, and general health aspotential causes of malformation.

Chemicals. Vitamin deficiency states are associ-ated with an increased incidence of cleft lip and

palate; which may be reduced with vitamin-supplementation diets for the mothers. Numerousstudies have demonstrated a reduced incidence offacial clefts after maternal supplementation with folicacid.6

Vitamin A, its derivatives, and related compoundssuch as isotretinoin (Accutane) have been implicatedin the development of facial clefting and hemifacialmicrosomia. Maternal smoking is associated withcraniosynostosis7 and facial clefts.8

Additional substances are implicated in increasedrisk of craniofacial anomalies, such as chlorphen-iramine, chlordiazepoxide, and nitrofurantoin expo-sure and craniosynostosis.9

CLASSIFICATION

To date no single classification system has beendevised that accurately describes all congenital cran-iofacial anomalies. In 1981 the Committee onNomenclature and Classification of CraniofacialAnomalies of the American Cleft Palate Association10

grouped craniofacial disorders according to theirdiverse etiology, anatomy, and treatment. They pro-pose a practical and simple classification system inwhich five categories of deformity are identified, asfollows:

I Clefts (Tessier classification)II SynostosesIII Atrophy/hypoplasiaIV Neoplasia/hyperplasiaV Unclassified

I — CLEFTS, DYSPLASIAS, AND DYSOSTOSES

Anatomic Classification

In 1976 Tessier11 described an anatomic classifi-cation system whereby a number is assigned to eachof the malformations according to its position rela-tive to the sagittal midline (Fig 2).

For orientation, the orbit is divided into two hemi-spheres: The lower lid with cheek and lip demon-strate facial clefts, the upper lid demonstrates cranialclefts. Two Tessier classification schemes exist, onefor the skeleton and one for soft tissue. Numerousinstances may occur where there is discrepancybetween the two classification systems—for example,a subject with a bony cleft but no soft-tissue cleft, or

TABLE 1Causes of Congenital Deformities in Man

(Reprinted with permission from Slavkin HC: DevelopmentalCraniofacial Biology. Philadelphia, Lea & Febiger, 1979.)


3

where skeletal cleft and soft-tissue cleft are not in thesame position. Nevertheless, Tessier’s scheme remainsin wide use today because it is relatively easy to learnfor communicating with other clinicians. David etal12 illustrate a complete series of these craniofacialclefts in 3D CT scans.

The tissue-deficiency disorders—arhinencephalyand holoprosencephaly—are secondary to failure ofcleavage of the embryonic holoprosencephalon andof the normal longitudinal split into cerebral hemi-spheres. The tissue-excess deformities range from aslight midline notch of the upper lip to severe orbitalhypertelorism.

The holoprosencephaly malformation representsa hypoplastic No. 14 cleft in association with a tissuedeficiency or a tissue excess.13,14 Cohen and Sulik15

present a modern analytic review of the holo-prosencephalic disorders. Central nervous systemfindings and craniofacial anatomy are discussed, syn-dromes and associated anomalies are updated, andthe differential diagnosis is reviewed.

Elias, Kawamoto, and Wilson16 reviewed holo-prosencephaly and midline facial anomalies in anattempt to redefine their classification and manage-ment. They note that true holoprosencephalyencompasses a series of midline defects of the brainand face, and in most cases is associated with severemalformations of the brain which are incompatiblewith life. At the other end of the spectrum arepatients with midline facial defects and normal ornear-normal brain development.

Embryologic Classification

Van der Meulen and coworkers13 tried to correlateclinical features of the disorders with embryologicevents.

Site of dysplasia/dysostosis and associated cleft:Frontosphenoidal = Tessier 9Frontal dysplasia = Tessier 10 and 11Interfrontal dysplasia = Tessier 0 and 14Treacher Collins = 6, 7, and 8 cleftsTemporoauromandibular dysplasia = Hemifacial

microsomia (craniofacial microsomia)

Pathogenesis of Clefts

There are two leading theories of facial cleft for-mation. The classic theory holds that clefts are causedby failure of fusion of the facial processes.17-19 In thistheory the medial face unites by fusion of the pairedfacial processes beneath the nasal pits. Epithelialcontact is established and mesenchymal penetrationcompletes the fusion of the lip and palate. If thesequence is disturbed, a cleft forms.

CENTRICCorresponding Cranial

Facial Clefts Extension of Facial CleftsNo. 0 No. 14No. 1 No. 13No. 2 No. 12No. 3 No. 11

ACENTRICCorresponding Cranial

Facial Clefts Extension of Facial CleftsNo. 4 No. 10No. 5 No. 9No. 6No. 7No. 8

Fig 2. Tessier’s classification of craniofacial clefts. Localization onthe soft tissues (above) and skeleton (below). (Reprinted withpermission from Tessier P: Anatomical classification of facial,cranio-facial, and latero-facial clefts. J Maxillofac Surg 4:69,1976; list from Whitaker LA, Pashayan H, and Reichman J: Aproposed new classification of craniofacial anomalies. CleftPalate J 18:161, 1981.)


4

The mesodermal penetration theory states thatthe edges of the facial processes consist of a bilaminarmembrane of ectoderm with epithelial seamsdemarcating the major process.20-23 Mesenchymalcells then penetrate the layers and smooth out theseams. If the mesenchymal penetration fails, theepithelium dehisces and a cleft results. The severityof the cleft is proportional to the amount of meso-dermal penetration.

Management of Facial Clefts

By far the most common craniofacial anomaly iscleft of the lip/palate, followed by isolated cleft pal-ate as a distant second. These topics have beendiscussed in detail in Selected Readings in PlasticSurgery volume 10, number 16,24 and will not beaddressed further here.

The incidence of rare clefts is estimated at 1.4–4.9per 100,000 live births.25 Reconstruction focusesinitially on soft-tissue closure25 with excision of thefree borders of the cleft to normal tissue, followed bymeticulous layered soft-tissue closure.

Van der Meulen26 offers a thorough review of thepathology, etiology, and reconstruction of obliquefacial clefts. He agrees with Tessier’s principle ofcombining skeletal and soft-tissue realignment in onemajor surgical procedure. Resnick and Kawamoto,27

Galante and Dado,28 and Fuente del Campo29 givespecific recommendations for the treatment ofunusual facial clefts on the basis of their respectiveexperiences with Tessier’s Nos. 4, 5, and 8 clefts.

Menard30 describes the application of tissueexpansion in the soft-tissue closure of facial clefts in 8patients. Due to underlying bony hypoplasia, skel-etal reconstruction is often necessary when the childis older.25

There are multiple approaches to cleft repair, tim-ing of repair, and technique of reconstruction.Although several protocols have been suggested, theseshould be viewed as guidelines, not absolute direc-tives for care. The variability in approaches mirrorsthe extreme variability in congenital clefts, dysplasias,and dysostoses. A few in-depth details and recon-structive algorithms will be presented for the morecommon abnormalities, including craniofacialmicrosomia, Goldenhar syndrome, Treacher Collinssyndrome, Nager syndrome, Binder syndrome, PierreRobin sequence, and encephaloceles.

CRANIOFACIAL MICROSOMIA

Craniofacial or hemifacial microsomia is the termmost frequently used to describe the first and secondbranchial arch syndrome, which is defined as a Tessier7 atypical facial cleft. Thomson31 was the first tosuggest in 1843 that the malformation was due toimperfect development of the first two anterior bran-chial arches. Clinical manifestations are underdevel-opment of the external and middle ear, mandible,zygoma, maxilla, temporal bone, facial muscles,muscles of mastication, palatal muscles, tongue, andparotid gland; macrostomia; a first branchial cleftsinus;31–33 and possible involvement of any or allcranial nerves34,35 (Fig 3).

Fig 3. Craniofacial microsomia in a 7-year-old child. (Reprintedwith permission from McCarthy JG, Grayson BH: Reconstruc-tion: Craniofacial Microsomia. In Mathis SJ (ed), PlasticSurgery, 2nd ed. Philadelphia, Elsevier, 2006. Vol IV, Ch 103,pp 521-554.)

The birth incidence of craniofacial microsomia isapproximately 1 in 4000.35 Approximately 10% ofcases are bilateral.31,36 The etiology is thought torelate to occlusion or thrombosis of the stapedialartery with injury to the developing first and secondbranchial arches.35,37 In its fullest expression, thecraniofacial microsomia syndrome is made up of aconstellation of congenitally malformed facial struc-tures arising from the embryonic first and second


5

visceral arches, the intervening first pharyngeal pouchand first branchial cleft, and the primordia of thetemporal bone.38

Originally craniofacial microsomia was thought torepresent a progressive skeletal and soft-tissue defor-mity that worsens over time,39 but subsequentlyPolley40 assessed longitudinal cephalometric data from26 patients with unoperated hemifacial microsomiaand demonstrated that the condition is notprogressive. These findings were further confirmedby Kearns et al41 in 67 subjects. The disorder varieswidely in presentation and may range from simplepreauricular skin tags to composite mandibular andmaxillary hypoplasia. Its management depends onthe severity of the defect and the functional andaesthetic reconstructive needs.42-46

Pruzansky44 described three types of mandibulardeficiency in craniofacial microsomia according tothe anatomical area affected (Table 2).

This classification was modified by Mulliken andKaban,47 who subdivided Type II into Type IIA, inwhich the glenoid fossa-condyle relationship is main-tained and the TMJ is functional, and Type IIB, inwhich the glenoid fossa-condyle relationship is notmaintained and the TMJ is nonfunctional.

Munro’s42,44 classification extended the skeletalanomaly to include the orbit. This system aims atproviding a basis for surgical reconstruction and con-sists of five types denoting increasingly severe hypo-plasia of the facial bones (Table 3). Isolated microtiais considered to be a microform of craniofacialmicrosomia.48

Meurman46 recognizes three grades of auriculardeformity, as follows: Grade I: distinctly smaller,

malformed auricle but all components are present;Grade II: only a vertical remnant of cartilage andskin is present, with atresia of the external meatus;Grade III: complete or nearly complete absence ofthe auricle.

David and colleagues49 proposed a multisystemclassification of hemifacial microsomia in the TNMstyle. The physical manifestations of hemifacialmicrosomia are graded according to five levels ofskeletal deformity (S1–S5) equivalent to the Pruzanskyclassification for S1-S3, plus S4 representing orbitalinvolvement and S5 representing orbital dystopia.Auricular deformity (A0–A3) is similar to the classifi-cation described by Meurman. Tissue deficiency(T1–T3) is graded as mild, moderate, or severe. TheSAT sytem allows a comprehensive and stagedapproach to skeletal and soft-tissue reconstruction.

Early macrostomia repair (1–2 months of age)yields excellent functional and cosmetic results.50

More extensive reconstruction, including compositecorrection in moderate to severe deformity, is reservedfor early childhood (age 5–6) but should not waituntil facial growth is complete.39,42–44,51–54 The man-dible is usually corrected first in the hope that reposi-tioning the jaw will unlock the growth potential ofthe functional matrix to allow normal growth of themandible55,56 and release abnormal growth tenden-cies of the maxilla.

In mild cases, Posnick prefers to wait for skel-etal maturity, and employs traditional orthognathicsurgery to achieve favorable aesthetic results.50 Dis-traction osteogenesis provides excellent correctionin cases of mandibular deformity up to Type IIB.57,58

In cases of Type III deformity, a costochondral

TABLE 2Mandibular Deficiency in Craniofacial Microsomia (Pruzansky)


6

graft is usually indicated, though many authors havenoted unpredictable overgrowth of costochondralgrafts.34,42–44,53,59

Ross60 reviewed 55 cases of severe craniofacialmicrosomia treated with costochondral grafts atThe Hospital for Sick Children. The success rateswere higher when the children were operated ear-lier (85% for age 3–7y vs 50% for age >14y).Growth equal to the other side was seen in 46%,undergrowth in 15%, and overgrowth in 39%.Given the option of early or delayed surgery, theauthor favors early surgery at age 4–5, citing ahigher graft success rate, the psychosocial advan-tage of attaining facial symmetry at a younger age,and the additional benefit that erupting teeth willassume a more normal position, making futureorthodontic treatment less difficult.

Munro42,44,53 usually advocates much moreextensive surgery, operating on the maxilla at thesame time as the mandible. For mild cases, man-dibular surgery may involve contour surgery with orwithout genioplasty once skeletal maturity isreached. In case of orbitozygomatic hypoplasia,Posnick50 uses split cranial bone grafts at age 5–7,stating that at age 7 the cranioorbitozygomatic com-plex is nearly mature so that an adult-size vault,orbit, and cheekbone can be fashioned. Also thethickness of the calvarium at that age makes for aneasier harvest of split calvarial bone grafts. Refine-ments to the mature skeleton may be needed even-tually to achieve a symmetrical and aestheticresult,50and may take the form of sagittal splitosteotomy, Le Fort I osteotomy, or genioplasty.

At times the soft-tissue deformity in craniofacialmicrosomia must also be addressed, and usually fol-lows skeletal reconstruction. Various methods of

soft-tissue augmentation with microvascular free trans-fers are described by La Rossa61 and Upton.62 Thissubject is further discussed in Selected Readings inPlastic Surgery volume 10, number 5, part 1.63

GOLDENHAR SYNDROME(Oculoauriculovertebral Dysplasia)

Goldenhar syndrome is characteristically bilat-eral. Features include prominent frontal bossing,a low hairline, mandibular hypoplasia, low-set ears,colobomas of the upper eyelid, epibulbar dermoids,accessory auricular appendages that are bilateraland anterior to the ears, and vertebral anomalies.5

Occurrence is believed to be sporadic, with only aweak genetic component. The presence ofepibulbar dermoid is required for a diagnosis ofGoldenhar syndrome. The reconstruction followsthe principles of craniofacial microsomia presentedabove.

TREACHER COLLINS SYNDROME(Mandibulofacial Dysostosis)

The first reference to mandibulofacial dysostosisin the medical literature was made by Berry in 1889.Berry described the physical symptoms and specu-lated about the inherited character of the deformity.His treatise was certainly much more detailed thanthe two cases reported by E Treacher Collins 11years later, after whom the syndrome was named.In Europe the deformity is known as the Franceschetti-Zwahlen-Klein syndrome on the basis of their 1949monograph summarizing the world literature.64 Theincomplete form should be designated as TreacherCollins syndrome65 and the complete form asFranceschetti’s syndrome.

TABLE 3Mandibular and Orbital Deficiency in Craniofacial Microsomia (Munro)


7

The Treacher Collins syndrome66 represents amanifestation of the Tessier Nos. 6, 7, and 8 cleft. Itis inherited as an autosomal dominant trait with anincidence of 1/10000 live births.67 Bilaterality is thenorm and phenotypic expression is variable. Thegene for Treacher Collins syndrome was identifiedin 1991, when it was mapped to chromosome 5 byDixon and colleagues68 from a study of 12 families.Later that year the genetic locus was refined to bands5q31.3?q33.3.69 In 1996, transcription mappinglocalized the critical region to a specific locus, TCOF1,which produces an abnormal protein named Treacle.This nucleolar protein is under intense study andappears to function in microtubule formation andcell migration.70,71

The pathogenesis of Treacher Collins syndromeremains unknown. Sulik and colleagues72 were ableto produce the facial abnormalities of Treacher Collinsin mice by administering isotretinoin, suggesting thatthe syndrome can be triggered by disruption of vita-min A metabolism. There is an apparent correlationbetween frequency of mutation and advancedpaternal age.

The typical features of the Treacher Collins syn-drome include the following (Fig 4):

• palpebral fissures sloping downward laterally(antimongoloid slant), with coloboma of the outerportion of the lower lid and rarely the upper lid

• hypoplasia (aplasia) of the facial bones, especiallythe malar bones and mandible

• malformation of the external ear and occasion-ally the middle and inner ear

• macrostomia, high palate, abnormal position andmalocclusion of the teeth

• blind fistula between the angles of the mouthand the ears

• atypical hair growth in the form of tongue-shapedprocesses of the hairline extending toward thecheeks

• absence of eyelashes in at least the medial thirdof the lower eyelid

In affected newborns the first priority is airwaymanagement. Shprintzen et al73 noted that somepatients have marked narrowing of the airway (pha-ryngeal diameter <1cm in some cases). Behrents,McNamara, and Avery74 described extreme shorten-ing of the mandible with severe lower face retrusion.Combined, these malformations can account for theobstructive sleep apnea and reports of neonatal deathassociated with cases of Treacher Collins. Tracheo-stomy may be necessary, although distraction osteo-genesis of the mandible in the neonatal period cansometimes avert it.

The coloboma is addressed by Tessier with a Z-plasty.67 Jackson75 uses a full-thickness skin-cartilageflap from the upper lid for reconstruction of thelower lid. Despite these measures, an “operatedlook” is hard to avoid.

Reconstructive surgery in the Treacher Collins syn-drome is directed to the maxilla, mandible, andzygoma and to the soft-tissue deficiencies of the eye,ear, and malar region.76 Pruzansky Type I and TypeII mandibular defects can be corrected with dis-

Fig 4. Treacher-Collins Syndrome: Soft-tissue and skeletal deformities. (Reprintedwith permission from Bartlett SP, Losee JE,Baker SB: Reconstruction: Craniofacial Syn-dromes. In Mathis SJ (ed), Plastic Surgery,2nd ed. Philadelphia, Elsevier, 2006. Vol IV,Ch 102, pp 495-519.)


8

traction osteogenesis in children <10y. Type IIIdefects may require chostochondral rib grafts, whichare best done during the mixed dentition phase(age 6–10).77 Posnick78 augments hypoplasticzygomas with onlay nonvascularized bone graftsand reports no significant graft resorption or changein contour over time. Others prefer vascularizedcalvarial bone flaps79,80 or osteotomy and advance-ment.81

Van der Meulen and associates82 prefer a tempo-ral osteoperiosteal flap, and Psillakis83 describes anouter table fascial flap vascularized by the temporalaponeurosis. Autogenous cartilage is the preferredform of auricular reconstruction.84 Soft-tissue aug-mentation can be performed with dermal grafts85 orfree-tissue transfer.61,62

Orthognathic surgery and refinement procedureswith onlay grafts and soft-tissue augmentation canbe beneficial to children ages 10–19. Freihofer’s81

protocol begins treatment early in the second decade.In the first session the main components are a chinadvancement and concomitant malar osteotomies.At the second operation the chin is moved fartherforward by simultaneous vertical movement of themaxilla, sagittal split osteotomy, and body osteotomyof the mandible.

NAGER SYNDROME (Acrofacial Dysostosis)

The Nager anomaly is very similar to TreacherCollins syndrome but much rarer. It is inherited asan autosomal recessive trait. Patients have much thesame craniofacial features as in mandibulofacial dys-ostosis, coupled with preaxial reduction defects ofthe upper and sometimes the lower limbs. In theupper limbs there is hypoplasia or agenesis of thethumbs and radius and of one or more metacar-pals.86 Unlike the condition in Treacher Collins,lower eyelid colobomas are not as frequent but cleftpalate is practically universal. Affected individualsare also typically of short stature and have subnor-mal intelligence.87

BINDER SYNDROME (Maxillonasal Dysplasia)

In 1882 Zuckerland described an anomaly in whichthe normal crest that separates the nasal floor fromthe anterior surface of the maxilla is absent. A smallpit, the fossa prenasalis, marks the inferior margin ofthe piriform aperture.88 In 1939 Noyes89 described

a patient with a flat nasal tip sitting on a retrudedmaxillonasal base. In 1962 von Binder90 described asyndrome consisting of a short nose with a flat bridge,absent frontonasal angle, absent anterior nasal spine,limited nasal mucosa, short columella and acutenasolabial angle, perialar flatness, convex upper lip,and a tendency to Class III occlusion. Occasionallythere may be hypoplastic frontal sinuses. Von Binderpostulated these defects were due to rhinocephalicdysplasia, which he called “maxillonasal dysostosis.”Since that time the condition has been known asmaxillonasal dysplasia or Binder syndrome.91

Posnick and Tompson92 note that the physical find-ings of Binder syndrome are the result of hypoplasia(depression) of the anterior nasal floor (fossaprenasalis) and localized, symmetric, maxillary hypo-plasia of the alar rim regions. From the worm’s eyeview, typical variations from normal include aretracted columella–lip junction, a lack of normaltriangular flare at the nasal base, a perpendicularala–cheek junction, a convex upper nasal tip with awide shallow philtrum, crescent-shaped nostrils with-out a sill, a low-set and flat nasal tip, and a stretchedand shallow Cupid’s bow (Fig 5).93 The most strikingexternal characteristics of the nose are vertical short-ening, lack of tip projection, perialar flattening, andan acute nasolabial angle.

Holmstrom93 studied 50 patients with Binder syn-drome and found a hereditary connection in 16%.Inheritance may be autosomal recessive with incom-plete penetrance.94,95

Surgical correction is complex.92,96 Techniquesfor nasal reconstruction have been described byPosnick,92 Holmstrom,96 Jackson,97 and Banks andTanner.98 Reconstructive options include Le Fort IIosteotomy, Le Fort I osteotomy, a combination of LeFort II and I osteotomies, compensatory orthodonticalignment of the teeth, Durafoam, and infraorbitalrim augmentation.91,96,97,99–105 Nasal reconstructionmay involve both autogenous and homogenous boneand cartilage grafts extending up the columella andover the dorsum, from the radix to the tip (Fig 6).

Rune and Aberg106 questioned the supposedly dis-appointing long-term results that were attributed tobone graft resorption. In a follow-up of 11 patientsfor 40 months, they found a mean reduction in graftlength of 28%, although this did not alter the achievedimprovement in nasal length or tip projection.

Banks and Tanner98 approach the nasal deformityin Binder syndrome by lifting the facial mask through


9

a coronal incision and reaching the nasal floor throughan incision in the upper buccal sulcus. The nasal softtissues and alar cartilages are mobilized. The nose islengthened and tip projection is achieved with a can-tilever graft of lyophilized cartilage.

Wolfe107 describes a technique of nasofrontalosteotomy to lengthen the nose in cases of post-traumatic shortening and Binder syndrome.McCollum108 reviews the literature and provides longterm follow up of 2 patients, one treated with tradi-tional orthognathic surgery and the other with agrowth center implant to the nose.

PIERRE ROBIN SEQUENCE

In 1923 Pierre Robin, a French stomatologist, noteda triad of characteristics of the upper airway which isnow known as the Pierre Robin sequence.109 Thecharacteristic features consist of micrognathia,glossoptosis, and airway obstruction.8,109 An associatedhigh arched midline cleft of the soft palate and occa-sionally of the hard palate is present in approximately50% of cases.110,111 The sequence shows great etiologicheterogeneity, with as many as 18 associated syndromes.

The glossoptosis in Pierre Robin can begin a vicioussequence of events: airway obstruction, increased

energy expenditure, and decreased caloric intakefrom impaired feeding. Afflicted infants typically failto thrive because of respiratory and feeding difficul-ties. If these problems are ignored, respiratory fail-ure, cardiac failure, and death may result.

Fig 5. (Above) The nose and upper lip in maxillonasal dysplasia.1 - Retracted columella-lip junction and lack of triangular flare atthe base. 2 - Perpendicular alar-cheek junction. 3 - Upper lipconvex with wide, shallow philtrum. 4 - Crescent-shaped nostrilwithout nostril sill. 5 - Low set and flat nasal tip. 6 - Cupid’s bowstretched and shallow. (Below) Surgical correction is by medialrotation of tissues on either side of the nasal midline. (Reprintedwith permission from Holmstrom H: Clinical and pathologicfeatures of maxillonasal dysplasia (Binder’s syndrome): signifi-cance of the prenasal fossa on etiology. Plast Reconstr Surg78:559, 1986.)

Fig 6. Patient with Binder syndrome. A, B, at age 10. C, E,preoperatively at age 17. D, F, after orthodontic treatment (maxillaryfirst bicuspid extractions), orthognathic surgery (Le Fort I osteotomywith horizontal advancement), and nasal reconstruction(corticocancellous iliac graft). (Reprinted with permission fromPosnick JC, Tompson B: Binder syndrome: staging of reconstructionand skeletal stability and relapse patterns after Le Fort I osteotomyusing miniplate fixation. Plast Reconstr Surg 99:967, 1997.)


10

In 1946 Douglas112 reported >50% mortality withconservative treatment of Pierre Robin. It is nowclear that the key to successful medical treatment ofinfants with Pierre Robin is to hold the infant proneto relieve the glossoptosis and open the airway. Insome cases this position must be maintained for 24hours a day, even during feeding, baths, and diaperchanging.113

While most infants can be successfully managedconservatively, a few will require surgical interven-tion. If medical treatment fails to relieve the symp-toms of airway obstruction, the baby would formerlybe considered for tongue-lip adhesion or tracheo-stomy.112,114,115 The advent of distraction osteogen-esis provides an effective alternative for addressingairway obstruction. Denny116 describes a series of10 patients with airway obstruction from severe cran-iofacial syndromes who were treated with distractionosteogenesis of the mandible. All children were clini-cally improved, and 2 of 3 patients with tracheosto-mies were successfully decannulated within 6 weeks.The mean functional airway increase after distrac-tion was 67.5% (Fig 7).

ENCEPHALOCELES

An encephalocele is a protrusion of part of thecranial contents through a defect in the skull. Themass may contain meninges (meningocele), meningesand brain (meningoencephalocele), or meninges,brain, and ventricle (meningoencephalocystocele).117

Encephaloceles categorized by their position in theskull can be basal, sincipital, or convexity.118 Thesincipital group can be further divided intofrontoethmoidal, interfrontal, and those associatedwith clefts. The frontoethmoidal group can be sub-divided into nasofrontal, nosoethmoidal andnasoorbital types.119

The presence of an encephalocele may be detectedon fetal ultrasound or by an elevated alpha fetopro-tein level.118 The differential diagnosis of frontal mid-line masses includes encephaloceles, teratomas, glio-mas, and dermoids.120,121 High-resolution CT scanscan establish the intracranial component ofencephaloceles.121 In a frontoethmoidal or nasalencephalocele, the cranial defect is in the anteriormidline between the frontal bone preformed in mem-brane and the ethmoid preformed in cartilage. Thecraniofacial deformity consists of hypertelorism, orbitaldystopia, elongation of the face, and dental maloc-

clusion, reflecting the distorting influences on facialbone growth by the extruded intracranial contents(Fig 8).

The pathogenesis of frontoethmoidoencephalo-celes is as follows. Early in embryogenesis, diver-ticula of dura project anteriorly through the fonticulusnasofrontalis (a small fontanelle between the devel-oping nasal and frontal bones) or inferiorly throughthe developing frontal bone into the prenasal space.These diverticula may come in contact with skin andadhere to it. Normally the diverticulum regressesand the bone closes, creating both the normalnasofrontal suture anteriorly and, passing throughthe skull base just anteriorly to the crista galli, the

Fig 7. Two patients with Nager syndrome and tracheostomy before(left) and after (right) mandibular distraction. (Reprinted withpermission from Denny AD, Talisman R, Hanson PR, Recinos RF:Mandibular distraction osteogenesis in very young patients tocorrect airway obstruction. Plast Reconstr Surg 108:302, 2001.)


11

foramen cecum. In a frontoethmoidal encephalo-cele the diverticulum does not recede and the bonedoes not close. The etiology of encephalocele isunknown but includes racial, genetic, environmen-tal, and paternal factors.122

Encephaloceles occur with a number of craniofa-cial syndromes.123 The world wide incidence ofencephalocele is 1/5000.124 In Western Europe, NorthAmerica, Australia, and Japan, occipital encephalo-celes predominate. In Southeast Asia and Russia,anterior encephaloceles outnumber posterior onesby a 9.5:1 ratio.124,125 The reason for this discrep-ancy is unknown.

The principles of treatment are incision of the sac,amputation of excess tissue to the level of the sur-rounding skull, closure of the dura, and closure ofthe skin.126 David,127 Forcada et al,128 and Smit andcolleagues129 review the spectrum of cranial andcerebral malformations that may be present infrontoethmoidal encephaloceles and discuss thediagnosis and management of these deformities.David127 analyzed the experience with fronto-ethmoidal encephaloceles by the Australian Cranio-facial Unit from 1975 to 1993 and reached the fol-lowing conclusions:

• Early complete surgery is indicated to allow thedeveloping brain and eyes to remodel the facialdeformity.

• Intracranial abnormalities are common.

• Frontoethmoidal encephaloceles differ from otherneural tube defects in their lack of a familial pat-tern and peculiar geographic distribution.

• Treatment by craniofacial technique is best.

• The established deformity can be effectively man-aged by craniofacial osteotomies.

• Most patients have abnormal intercanthal dis-tances but normal interpupillary and lateral can-thal measurements.

• The frontal sinus region often needs repeat bonegrafting, and nasal bone grafts commonly needto be replaced as patients age.

• Treatment for craniofacial clefts should be post-poned until after growth is complete.

• Early treatment of patients with basal encephalo-celes is indicated to prevent further damage andinfection.

• Surgery for extensive basal encephaloceles is com-plex and probably should be done through afacial hemisection approach.

Holmes et al130 offer their experience with 35cases of frontoethmoidal encephalocele. The goalsof treatment are as follows (Fig 9):

• urgent closure of open skin defects to preventinfection and desiccation of brain

• removal or invagination of nonfunctional extra-cranial tissue

• watertight dural closure

• total craniofacial reconstruction with special careto avoid the “long nose deformity”

To correct the deformity caused by hypertelorismand a long midface, Holmes and coworkers130 lowerthe supraorbital bar by rotating it medially, posteri-orly and downward in the midline, while laterally it iswidened to correct the trigonocephalic deformity.

Successful correction depends on an understand-ing of the pathologic anatomy; careful planning ofosteotomies and bone movements to correct thewhole deformity, including trigonocephaly and thelong nose deformity; nasal reconstruction with canti-lever graft to avoid the long nose deformity; skinclosure removing abnormal skin and careful placingof scars; transnasal canthoplasty to reposition the

Fig 8. Left, child with asymmetrical nasoethmoidal, nasoorbitalencephalocele. Right, same child at age 5, after removal of theencephalocele and correction of the trigonocephaly, orbitaldystopia, and hypertelorism in one procedure at 8 months of age.(Reprinted with permission from Holmes AD, Meara JG, KolkerAR, et al: Frontoethmoidal encephaloceles: reconstruction andrefinements. J Craniofac Surg 12:6, 2001.)


12

medial canthi; and single-stage surgery displaying cran-iofacial and neurosurgical expertise.

II — CRANIOSYNOSTOSIS

Virchow (1851) was the first to use the term cran-iosynostosis, although abnormal head shapes relatedto cranial sutures were noted by Hippocrates as earlyas 100 BC. Virchow noted that growth restrictionoccurred perpendicular to the fused cranial sutureand compensatory growth occurred parallel to theaffected suture131 (Fig 10). This combination of growthrestriction in one dimension and compensatorygrowth at right angles results in consistent patterns ofabnormal head shape.132

The incidence of craniosynostosis is estimated at 1in 2000 live births.133 Retrospective studies usingradiographic criteria alone underestimate the occur-rence of craniosynostosis.134,135 Craniosynostosis mayoccur as a sporadic, isolated abnormality or as a fea-ture of a congenital syndrome. It can be isolatednonsyndromic or syndromal. The best way to classifyisolated craniosynostosis is to name the suture(s)involved—eg, left unicoronal synostosis, metopic synos-tosis, bicoronal synostosis—but it is also common torefer to the resulting head shape123,136–138 (Fig 11).

Trigonocephaly results from premature fusion ofthe metopic suture. The spectrum of deformitiesincludes a vertical midline forehead ridge, triangularor “keel-shaped” contour, bitemporal narrowing, andhypotelorism.

Fig 9. Three-dimensional models of a frontoethmoidal encephalocele and its correction. A, B, note the external cranial opening, interorbitalhypertelorism, and depressed cribriform plate. The supraorbital osteotomies and central metopic area of bone are marked, as well as thebony Z-plasties on the lateral orbital rims. C, diagram of the proposed osteotomies and bone movements. D, after repositioning the medialand lateral walls of the orbit and supraorbital bar and fixation of the bone graft for nasal reconstruction. (Reprinted with permission fromHolmes AD, Meara JG, Kolker AR, et al: Frontoethmoidal encephaloceles: reconstruction and refinements. J Craniofac Surg 12:6, 2001.)


13

Scaphocephaly (dolichocephaly) is due to prema-ture fusion of the sagittal suture. Features of sagittalsynostosis include a palpable ridge overlying the sag-ittal suture, decreased biparietal diameter, and elon-gation of the skull in the anteroposterior dimension.Significant frontal and occipital bossing are commonlynoted. The appearance resembles a boat or “scaphe.”

Plagiocephaly stems from the Greek word mean-ing “crooked head”, and is an asymmetrical defor-mity. It may be anterior or posterior. Two etiologi-cal variants must be distinguished – deformationaland synostotic.

Posterior plagiocephaly is usually positional and aresult of the child lying predominately on his/herback. It produces a classic parallelogram skulldeformity.139 The incidence has increased with therecommendation by the American Academy ofPediatrics to lay infants on their backs to reduce therisk of sudden infant death syndrome (SIDS).140,141

Treatment of positional plagiocephaly depends on

the severity of the deformity and often requires hel-met therapy for correction in severe cases. How-ever, large studies have shown little difference inoutcome between helmet therapy and consistentrepositioning of the infant off the flat spot in mild tomoderately severe cases.141,142

Posterior synostotic plagiocephaly can also resultfrom unilambdoid synostosis. Unilambdoid synosto-sis is a very rare entity.

Brachycephaly is the result of bicoronal synosto-sis and is characterized by anteroposterior shorten-ing of the skull. The lower part of the forehead andsupraorbital bar are retropositioned. This is thecharacteristic head shape deformity that accompa-nies Apert and Crouzon syndrome, and in thesecases it is believed to be due to abnormalities thatextend into the cranial base, causing the associatedfacial deformities of exorbitism and maxillary retru-sion. Posterior brachycephaly is unusual but canbe the external manifestation of bilateral lambdoidsuture synostosis.

Turricephaly (towering head deformity) is charac-terized by excessive skull height and a vertical fore-head. This deformity is typically an untreatedbrachycephaly where compensatory expansion leadsto an increasing vertical height to the cranium. Chil-dren with Apert syndrome have a particular ten-dency toward turricephaly.

Oxycephaly is a pointed head. The forehead isretroverted and tilted back in continuity with thenasal dorsum. Oxycephaly is usually due to fusion ofmultiple sutures.

When multiple sutures are involved, head shapesare more variable and the distinctions blur. In thesecases the specific sutures involved should be namedwhen describing the deformity. The kleeblattschadelor cloverleaf skull deformity results from pansuturalsynostosis, and usually requires early, aggressive treat-ment to prevent cerebral compromise.

Craniofacial Growth

The cranium is made up of the neurocranium,which includes the chondrocranium of the skull baseand the membranous bone of the calvarium, andthe viscerocranium, which forms the membranousbones of the face. The various areas of thecraniomaxillofacial skeleton grow by very differentmethods. The cranial sutures are skeletal joints ofthe syndesmosis type as well as being sites of osteo-

Fig 10. Cranial sutures in the human fetus. Premature closureproduces growth restriction perpendicular to the line of thesuture and compensatory overgrowth parallel to it.


14

genesis and skeletal adjustments. The interlocking,peg-and-socket arrangement, allows osteogenesis tooccur mainly at the bottom of the socket and pointof the peg, for simultaneous jointing and growth ofthe bone against the suture. In contrast, the facialsutures, especially the zygomatic, maxillary, andpalatine, are simply overlapping or sliding joints wherethe direction of bone growth tends to parallel theplane of the suture. This arrangement provides foradaptive adjustments to pressure in utero and earlyinfancy.

The intrinsic maxillary growth concept recognizesspecific bone growth sites in the maxilla, with growthoccurring mainly in a backward direction by osteo-genesis on the retromaxillary surface. The nasal sep-tum exerts forward pull on the maxilla at the insertionof the septopremaxillary ligament.143,144 Mandibulargrowth is primarily through growth and elongation atthe level of the ramus and subcondylar segments.Experimental studies indicate that condylar cartilage

growth is probably secondary to traction by the softtissues within and attached to the mandible, such asthe tongue and muscles of mastication.55

Pathogenesis of CraniosynostosisThe pathogenesis of craniosynostosis is complex

and probably multifactorial. Moss145 theorized thatabnormal tensile forces are transmitted to the duracovering the brain from an anomalous cranial basethrough key ligamentous attachments, and this leadsto craniosynostosis. This hypothesis fails to explainthe coexistence of nonsyndromic craniosynostosis witha normal cranial base configuration.

Cohen123,146 suggests craniosynostosis is either pri-mary due to suture biology147 or secondary toanother event such as in-utero compression of thecranium,148,149 decompression of a hydroceph-alus,150–152 inadequate intrinsic growth forces of thebrain (microcephaly), hyperthyroidism,153 ricketts,or shunted hydrocephalus.

Fig 11. Skull shapes and affected sutures in craniosynostosis. See text for details. (Reprinted with permission from Cohen MM Jr, MacLeanRE: Anatomic, Genetic, Nosologic, Diagnostic, and Psychosocial Considerations. In: Cohen MM Jr, MacLean RE (eds), Craniosynos-tosis. Diagnosis, Evaluation, and Management, 2nd ed. New York, Oxford Univ Press, 2000; Ch 11, pp 119-143.)


15

Approximately 2% of cases of isolated craniosynos-tosis are inherited.154 Cohen, Dauser, and Gorski155

documented three close relatives with delayed onsetof exorbitism and midfacial retrusion thought to beconsistent with a diagnosis of familial, nonsyndromiccraniosynostosis. In contrast, a hereditary compo-nent has been identified in as many as 50% ofsyndromal craniosynostosis patients.154

The Role of the Dura in Craniosynostosis

In cases of primary craniosynostosis the underly-ing dura mater acts locally to supply the overlyingsuture with osteogenic growth factors. Opperman in1993 showed the role of the dura in determining thefate of suture fusion,156 and that these dural factorswere soluble.157 Hobar158,159 noted the importanceof the dura in the regeneration of cranial bones ininfants. Greenwald160,161 went further, finding thatimmature dura mater contained a subpopulation ofosteoblast-like cells. Levine162 showed that the regionof the dura was as important as the interactionbetween the suture and the dura. The overlyingpericranium does not play a role in suture biology,according to Opperman.163 Clearly the dura under-lying the suture drives the timing of closure throughosteoinductive growth factors

Molecular Genetics in Craniosynostosis

Elevated FGF-R2 was identified by Delezoide164

as the first real marker of prechondrogenic conden-sations. Mangasarian165 identified a tyrosine for cys-teine substitution on the mutated FGF-R2 that cre-ates an activated form, resulting in uncontrolled FGFsignaling and premature suture closure.

Most166 and Mehrara167 found that expressionof basic fibroblast growth factor (bFGF) wasincreased in the dura beneath the suture prior tofusion and increased in the osteoblasts at the sutureduring fusion. Other growth factors have beenfound to be increased in prematurely fusingsutures: transforming growth factor B (TGF-B)166,168–171 and bone morphogenic proteins(BMPs).171 Research is now directed at inducingcraniosynostosis in animal models by the applica-tion of exogenous FGFs to confirm their role insuture closure.172 Genes whose mutations resultin craniosynostosis syndromes are listed in Table4.173,174

In 1993 a mutation in the homeobox gene MSX2was identified in a single family with autosomal domi-nant craniosynostosis, now known as Boston-typecraniosynostosis.175 Recent evidence suggests thatthis mutation may exert its effect through BMP path-ways176,177 and that MSX2 mutations may functiontogether with FGF-R2.178

Crouzon syndrome has been extensively studiedgenetically and a mapped abnormality is noted onthe long arm of chromosome 10.179 Further analysislocalized an FGF-R2 genetic mutation within thischromosome in subjects with Crouzon.180

In 1994 Muenke and colleagues181 described acommon mutation in the FGF-R1 gene as the causeof Pfeiffer’s syndrome. In 1995 Apert and Pfeiffersyndromes were also found to be related to FGF-R2mutations.182–184 Mutations in the FGF-R2 gene nowhaving been shown to be the cause of Jackson-Weisssyndrome, Crouzon syndrome, Pfeiffer syndrome, andApert syndrome, these conditions should be seen asdefined points along a spectrum of disease (Fig 12).

FGF-R3 mutations are also implicated in isolatedunicoronal craniosynostosis.185 Current understand-ing allows a molecular diagnosis in all phenotypicalcases of bicoronal synostosis.186 Other genetic muta-tions have been associated with synostosis. Thehedgehog family of homologs, including sonic hedge-hog, play a role in vertebral embryogenesis. Recently

TABLE 4Genes Bearing Known Mutations

for Craniosynostosis

(Reprinted with permission from Cohen MM Jr: Discussion of“Differential expression of fibroblast growth factor receptors inhuman digital development suggests common pathogenesis incomplex acrosyndactyly and craniosynostosis”, by Britto JA,Chan JCT, Evans RD, et al. Plast Reconstr Surg 107:1339, 2001.)


16

an association was made between craniofacial anomalyand sonic hedgehog target genes.187–189 The TWISTgene locus regulates osteoblast differentiation190 andmutations are associated with Saethre-Chotzen syn-drome,191–193 causing an up-regulation in FGF-Rexpression leading to premature oseoblast differentia-tion and cranial suture fusion. Rice194 proposes anintegration between FGF and TWIST mutations in suturedevelopment and suggests that different mutations maywork on the same pathway at different stages.

Ting195,196 examined the molecular differencebetween fused and nonfused human coronal suturesand identified overexpresion of the Nell-1 gene, againrelated to premature osteoblast differentiation.

It is not adherent tensile forces or an intrinsic prop-erty of the suture that determines suture biology, butrather a combination of dura-related genetic,molecular, and cellular factors that act individuallyor jointly in the development of craniosynostosis.Longaker197 provides a comprehensive review of cur-rent cranial suture research, concluding that genetherapy may offer targeted interventions that mayalter synostosis onset and progression:

Looking forward into the new millennium, onecan imagine a time when major reconstructivesurgery for craniosynostosis will no longer benecessary.

Longaker (2001)

Indications for Surgery

Indications to proceed with surgical reconstruc-tion of craniosynostosis include significant cranial andfacial asymmetry, elevated intracranial pressure (ICP),and neuropsychologic disorders. In most cases ofsingle suture craniosynostosis, “functional” indications(elevated ICP and treatable neuropsychologic distur-bance) are not present, and the indication for sur-gery is the cranial/facial asymmetry, its degree andseverity.

The clinical symptoms of intracranial hyperten-sion include headaches, irritability, and difficultysleeping. Radiographic signs may include corticalthinning or a luckenschadel (beaten metal) appear-ance of the inner table of the skull. Unfortunately,clinical and radiographic signs are relatively latedevelopments, and increased intracranial pressuremay be present for some time before these signsarise. Because of this, most craniofacial surgeonsadvocate early treatment, especially of infants withmultiple suture synostoses.198

Marchac and Renier136,199 found that intracranialpressure may be elevated in those with single suturesynostosis, although it is more common with multiplesuture involvement (13% and 42%, respectively). Themean ICP has been shown to decrease after cranialvault remodeling. Young children with craniosynos-

Fig 12. Fibroblast growth factormutations and their associated cran-iofacial anomalies. (Reprinted withpermission from Cohen MM Jr: Dis-cussion of “Differential expression offibroblast growth factor receptors inhuman digital development suggestscommon pathogenesis in complexacrosyndactyly and craniosynosto-sis”, by Britto JA, Chan JCT, EvansRD, et al. Plast Reconstr Surg107:1339, 2001.)


17

tosis usually have normal mental development, butthe proportion of normal children decreases withage, particularly for children with multisuture synos-tosis displaying brachycephaly and oxycephaly.200 Onthe basis of their studies, the authors recommendearly surgery, as there is no reliable way to distin-guish which infants will not have problems from cran-iosynostosis.201,202

Kapp-Simon and colleagues,203 on the other hand,longitudinally examined the mental development ofinfants before and after cranial release, and com-pared it with that of infants who were not surgicallytreated. The authors concluded that cranial releaseand reconstruction did not affect mental develop-ment either positively or negatively. Renier andMarchac,204 in a commentary of the above paper,strongly dispute this finding and state that the num-ber of patients in Kapp-Simon’s study was too smallto warrant any conclusions.

Subsequently Kapp-Simon205 published her analysisof a series of 84 patients with single suture synostosisfollowed longitudinally for >1y and reported a men-tal retardation rate of 6.5%, which is 2–3X the nor-mal. Almost half the children who were of schoolage displayed some type of learning disorder. Moreimportantly, these results were independent of earlysurgical correction, discounting the hypothesis thatearly correction of craniosynostosis would improvemental function. Similarly, Virtanen206 found thatthe neurocognitive performance of children with cran-iosynostosis did not reach that of matched normalcontrols, suggesting the impairment of brain functionhad already taken place in utero.

Gault et al207 attempted to correlate elevations ofintracranial pressure with decreases in intracranialvolume as measured by CT. They found no directrelationship between the two on measurement of104 children with craniosynostosis.202 It appears thatdecreased intracranial volume alone is not adequatejustification for surgery in cranisynostosis.208

Posnick and colleagues209 demonstrated that truemeasurements of intracranial volume can be obtainedindirectly using CT scans. Premature closure of eitherthe sagittal or metopic suture does not result indiminished intracranial volume. This was confirmedin long-term follow-up by Polley,210 who showedthat craniofacial procedures can be relied upon toincrease the intracranial volume. Polley also foundthat long-term normative intracranial volume wasmaintained postoperatively, and hypothesized that

although normal volumes are seen pre- and postop-eratively in craniosynostosis, reconfiguration of theskull dimensions in the region of the synostosed suturemay be beneficial.

David et al211 adds further support to this theoryin a study of cerebral perfusion pre- and postopera-tively, where they found cerebral perfusion defectspreoperatively in the area of the fused suture thatwere corrected following surgery. In cases of com-plex craniosynostosis, abnormalities of venous drain-age at the level of the skull base produce venoushypertension and subsequent raised ICP.212 Mostsurgeons therefore operate on infants at age 6–9months, and even earlier in severe cases.

Cohen213 reviews the evidence and concludes thatdifferences in methods of pressure measurement,patient selection, and the lack of normative datamake interpretation of existing studies difficult. Hesuggests that a clinical awareness of the signs of raisedICP is essential, though in cases of moderate defor-mity and no signs of raised ICP, close follow-up isapplicable.

Neuropsychiatric disorders range from mildbehavioral disturbances to overt mental retardationpossibly secondary to cerebral compression. Theabnormally shaped skull also imposes psychologicalconsiderations that can be severe and should not beunderestimated. Barritt and associates214 report thatchildren with untreated scaphocephaly are teasedand taunted at school for their head shape, whichcompounds their slow learning and poor motor skills.

Arndt and others215 and Pertschuk and Whitaker216

studied the psychosocial adjustments of children tothe correction of a deformity by craniofacial surgery.The authors noted increased self-esteem and adap-tive functioning along with a decrease in hyperactivebehavior and inhibited attitude, peaking at 1 yearpostoperatively. Despite cosmetic improvements andlower anxiety levels, however, social interactions werenot helped by the surgery. Likewise, Ousterhoutand Vargervik217 noted normalization of anthropo-metric points on CT scan of children who underwentLe Fort III and genioplasty because of craniosynosto-sis, but the degree of postoperative change did notequate with attractiveness.

In another study, Barden and colleagues218,219

looked at changes in physical attractiveness as well asemotional and behavioral reactions of children beforeand after craniofacial surgery. Their findings suggest


18

a positive effect of the surgery in terms of socialinteractions and the development of cognitive andemotional competence.

ISOLATED CRANIOSYNOSTOSES

Trigonocephaly

Trigonocephaly accounts for 10–20% of all cran-iosynostosis cases, occurring in 1 in 2500–15000 new-borns.220 Lajeunie221 found 237 cases of metopicsynostosis for an incidence of 1 in 15000. Themale:female ratio was 3.3:1 and 5.6% were familial.

Infants with trigonocephaly have an easily visibleand palpable midline frontal ridge that extends fromthe anterior fontanelle to the glabella. When viewedfrom above, the forehead resembles the keel of aboat. Sadove and coworkers222 note that dependingon the timing and extent of premature suture clo-sure, metopic synostosis can manifest as a spectrumof deformities ranging from an isolated midline fore-head ridge to a keel-shaped frontal bony pro-truberance. The forehead deformity is accompa-nied by variable degrees of orbital hypotelorism, eth-moidal hypoplasia, and bitemporal narrowing. Theintracranial volume of both frontal areas is oftendecreased, which results in compensatory overgrowthof both parietal areas. The frequency of elevatedintracranial pressure is estimated to be approximately10%,223 though it is likely that pressure is only anissue in the frontal lobes of those with severe defor-mity. Cognitive impairment is some series reaches33%224 and correlates with severity of deformity,225

though it is likely to be an associated factor ratherthan a causative one.

Posnick and associates226 evaluated the results ofsurgical intervention in metopic synostosis. They usedCT scans to quantitatively record the deformities ofuncorrected and corrected metopic synostosis,namely orbital hypotelorism, retruded lateral orbitalrims, and narrow bitemporal width. Postoperativeassessment confirmed that the anterior cranial vaultand lateral orbital wall positions, which were initiallydysmorphic, were corrected successfully andremained in good position despite subsequent calva-rial growth (Fig 13). The orbital hypotelorism wasimproved but not totally corrected.

Havlik et al227 examined 10 patients with severetrigonocephaly as defined by the central angle—theconvergence of the two hemisupraorbital segments.

Preoperative angles were consistently in the vicinityof 110 °. In these cases they recommend expandingthe supraorbital bar with interpositional bone graftsmeasuring 10–20mm, expanding the angle toapproximately 145°, and thus widening the bitem-poral narrowing (Fig 14). In milder cases they sug-gest that more conventional methods such as con-tour reduction or the floating forehead techniquewill be adequate.

McCarthy and others228 reviewed a 20-year expe-rience with early surgery for isolated craniosynosto-sis. Of the 104 patients in their study, 29 had metopicsuture synostosis. Orbital hypotelorism, initiallyapparent in 19, was significantly reduced postopera-tively in 17 patients. In general, surgical correctionof metopic synostosis was associated with the bestaesthetic results of all the isolated craniosynostoses;only one patient required a second craniofacial pro-cedure. McCarthy also followed 24 patients withmetopic synostosis for whom surgical correction wasnot recommended. None of these patients demon-

Fig 13. Left, a 10-month old boy with metopic synostosis andtrigonocephaly. Right, after surgical correction with cranial vaultand three-quarter orbital osteotomies. (Reprinted with permis-sion from Posnick JC, Lin KY, Chen P, Armstrong D: Metopicsynostosis: quantitative assessment of presenting deformity andsurgical results based on CT scans. Plast Reconstr Surg 93:16,1994.)


19

strated progression of the deformity, and 15 showedsignificant improvement in frontoorbital form. Theauthors believe that surgical correction is not justifiedin cases of metopic synostosis associated with onlymild change in morphology and without evidence offunctional disturbance. The risk of surgically inducedcomplications, including failure of frontal sinusdevelopment,229 outweighs the potential benefits ofsurgery.

Scaphocephaly

Sagittal synostosis, with its characteristic oblongcalvarial shape (scaphocephaly), is the most com-mon type of craniosynostosis.230 The severity of thecalvarial deformity varies from slight cranial elonga-tion with sagittal ridging to extreme elongation with alarge occipital shelf and pronounced frontal boss-ing.231 Persing, Jane, and Edgerton232 suggested thatthe deformity is not limited to the sagittal suture butalso involves the base of the skull, which in partcontributes to the degree of cranial dysmorphology.

The optimal operative management of scapho-cephaly is still debated. Multiple procedures aredescribed in the literature. Strip craniectomy (Fig

15) as described by Ingraham233 and its modifica-tions234 are employed in cases of early diagnosis, buthave produced unreliable results.235–239 This may bedue in part to recurrence of the synostosis240 ordeformation where rigid fixation is not employed.

The “pi technique” as described by Jane241 and itsmodifications242 have been associated with elevationin intracranial pressure.243 Techniques that employwider stripping, such as total vertex craniectomy,244

produce a superior result198 compared with stripcraniectomy.245 Kaiser198 compared the postopera-tive results of midline craniectomy, bilateralparasagittal craniectomy, and total vertex craniec-tomy. Cases of vertex craniectomy of Epstein244 allhad normalization of the cephalic index, while theother two groups were successful only one-fourth toone-half of the time.

The trend to more radical and extensive correc-tion of the entire cranial vault236,246–248 has led to thegoal of actively correcting the cranial index andattaining an ideal cranial shape at the time of sur-gery. Postoperative passive correction is not expected.For severe cases of sagittal synostosis, Marchac andcoworkers249 perform frontocranial remodeling in onestage. The supraorbital bar is usually left in placeand the upper forehead is reconstructed just aboveit. Posteriorly the occipital bone is moved forwardand the vault is constructed of three or four bonesegments cut transversely like parts of a barrel. Thesesegments are rearranged to shorten the anterior-posterior distance, expand the transverse diameter,and lift up the retrocoronal depression.

Older affected children who have not been oper-ated on and children with failed previous attempts atscaphocephalic correction probably warrant a moreaggressive approach consisting of total calvarialremodeling with anterior craniofacial reconstruction,temporoparietal widening, posterior remodeling, andanterior-posterior shortening if required.250

Plagiocephaly

Deformational Plagiocephaly

Mulliken251 and Huang and colleagues252 reviewthe morphologic findings in posterior plagiocephalyand the differential diagnosis between positional andsynostotic (Fig 16). In Huang’s prospective series of115 infants with posterior plagiocephaly, only onehad lambdoid synostosis.252

Fig 14. Technique for correction of trigonocephaly showing twoperspectives on the osteotomy sites (A & B, left) and afteranterolateral transposition of the hemisupraorbital segment andbone grafting (A & B, right). (Reprinted with permission fromHavlik RJ, Azurin DJ, Bartlett SP, Whitaker LA: Analysis andtreatment of severe trigonocephaly. Plast Reconstr Surg 103:381,1999.)


20

The importance of differentiating betweendeformational and synostotic plagiocephaly251 is thatconservative therapy will achieve results equivalentto those of surgery.253,254 Deformational plagioceph-aly is best treated with positioning, frequent headturning, and helmet therapy.141,255 Vies,142 reportingon a series of 105 patients treated with either headpositioning or helmet therapy, noted faster and bet-ter results in the helmet group.

Synostotic Plagiocephaly

Posterior synostotic plagiocephaly is due to lamb-doid synostosis and accounts for only 1% of occipitalplagiocephaly cases.256 In analyzing their series,Huang and colleagues139,252 found that in true lamb-doid synostosis the contralateral posterior bossingoccurred more laterally and superiorly in the parietalregion; frontal bossing was not a striking feature, but

when it occurred it was contralateral rather thanipsilateral; and ipsilateral occipitomastoid bossing wasconsistently present in lambdoid synostosis, whereasit was conspicuously absent in deformational poste-rior plagiocephaly.

The treatment implications are clear: Few patientswith positional plagiocephaly actually require opera-tive correction. Their skulls will remold satisfactorilywith conservative measures (positioning of the child inthe crib or helmet therapy). The surgical correction ofposterior plagiocephaly caused by unilateral lambdoidsynostosis is via posterior craniofacial reconstructionand remodeling, as recommended by Persing.257

Anterior synostostic plagiocephaly is due to unilat-eral coronal suture synostosis. Coronal synostosismay be either unilateral or bilateral. In a 21 yearexperience consisting of 116 patients with coronalsynostosis, 47% were unicoronal, 9% were bicoronalwithout associated syndromes, 34% were bicoronal

Fig 15. Child with sagittal synostosis before (A-C) and after (D-F) surgical correction. (Reprinted with permission from Persing JA, JaneJA, Edgerton MT: Surgical Treatment of Craniosynostosis. In: Persing JA, Edgerton MT, Jane JA (eds), Scientific Foundations andSurgical Treatment of Craniosynostosis. Baltimore, Williams & Wilkins, 1989, p 191.)


21

associated with a syndrome, and the remaining 20%were associated with multiple suture synostosis.258

Unicoronal synostosis causes regional growthrestriction and compensatory expansion of the neigh-boring tissues, producing overt frontoorbitaldysmorphology. Characteristic deformities ipsilateralto the synostosis include flattening of the frontal boneand ipsilateral forehead, ipsilateral elevation-recessionof the supraorbital rim, and narrowing and lateraldeviation of the orbit, deviation of the nasal roottowards the flattened side, and elevation of the ipsi-lateral ear.259 On the contralateral side there is bulg-ing of the frontal bone.260,261 An AP radiograph usu-ally demonstrates the characteristic harlequin eyedeformity. Bruneteau and Mulliken262 believe thatphysical examination focusing on the supraorbital

rims, nasal root, ears, and malar eminences can eas-ily distinguish between synostotic and deformationalplagiocephaly. This distinction has obvious clinicalimplications, as synostotic plagiocephaly is the onlygroup with strong surgical implications.

Lo and colleagues263 described the endocranial con-figuration in unilateral coronal synostosis as follows:• constriction of the ipsilateral anterior cranial fossa• deviation of the anterior fossa midline• elevation of the ipsilateral floor• straightening of the lesser sphenoid wing

These features of the cranial base correlate withthe orbital dysmorphology. The authors reviewed28 patients with unicoronal synostosis and noted more

Fig 16. Posterior plagiocephaly from positional molding (above) and unilambdoid synostosis (below). Arrows indicate vectors ofcompensatory growth. (Reprinted with permission from Huang MHS, Mouradian WE, Cohen SR, Gruss JS: The differential diagnosis ofabnormal head shapes: separating craniosynostosis from positional deformities and normal variants. Cleft Palate Craniofac J 35:204, 1998.)


22

symmetrical bony orbits and orbital contents duringthe first year after cranioorbital surgery. They con-clude that surgery for unicoronal synostosis (Fig 17)in infancy does not inhibit growth of orbital hard orsoft tissues and seems to allow normalization of pre-viously impaired growth.265

Fig 17. Anterior plagiocephaly and its surgical correction. A, siteof anterior cranial vault and three-quarter orbital osteotomies. B,reshaping and fixation of bone segments after osteotomies.(Reprinted with permission from Posnick JC: Craniosynostosis:Surgical Management in Infancy. In: Bell WH (ed), Orthognathicand Reconstructive Surgery. Philadelphia, WB Saunders, 1992;vol 3, p 1837.)

Bicoronal Synostosis

Bicoronal synostosis produces an abnormal skullshape—brachycephaly. The calvarium is shortanteroposteriorly, widened mediolaterally, and elon-gated vertically compared with normal subjects. Theorbital rims are hypoplastic, the occipital region is flat-tened, the frontal and temporal bones are protruberant,and the anterior cranial base is foreshortened.266

Although the brachycephalic deformity is characteris-tic of bicoronal synostosis, morphologic variants havebeen recognized.267

Marchac’s268 series of 35 children included 20patients with ‘simple’ and ‘familial’ brachycephalytreated by his “floating forehead” technique. Onlyone patient (5%) required secondary frontal advance-ment.

In his series of 22 consecutive patients withnonsyndromic bicoronal synostosis, Wagner266 reports62% relapse in patients operated on at age 5 monthsor younger. These infants subsequently needed totalreoperation. In contrast, only 11% of children oper-ated on at age >6mo showed recurrence of thedeformity. This observation is contrary to contem-porary theory and the experimental and clinicalimpression of others, which indicate that normaliza-tion of calvarial shape is more likely after early sur-gery.269,270

SYNDROMES WITH CRANIOSYNOSTOSIS

Craniosynostosis is not a syndrome in itself but asign of at least 150 different syndromes.271 Amultidisciplinary team involving craniofacial, ophthal-mologic, and ear-nose-throat surgeons, geneticists,neuropsychologists, nutritionists, and social workerswill allow total patient care. Priorities regardingintracranial pressure, airway obstruction, and globeexposure will dictate surgical priorities. Some of themore common syndromes with craniosynostosis astheir predominant physical finding are describedbelow (Table 5).

Apert Syndrome

Originally described by Wheaton in 1894, thecondition is named after Apert, who in 1906described 4 cases.272 The incidence of Apert syn-drome is reported to be 1:100,000 to 1:160,000births.273 Although transmission is autosomal domi-nant, occurrence is sporadic as new mutations withnormal karyotype.

The skull in Apert syndrome is brachycephalic.274

Facial features incude hypoplasia of the midface,hypertelorism,275 strabismus, ocular muscle palsies,276

and slanting palpebral fissures. There is moderate tosevere exorbitism, short zygomatic arches and, in theeuryprosopic type of Apert, a prominent bregmaticbump. The fontanelles may be large and late inclosing. The palate is narrow and either has a mediangroove or is cleft with a bifid uvula (Fig 18). Thelimbs show bony syndactyly (secondary to the FGF-R


23

mutations implicated in the craniosynostosis173) withcomplete fusion of the fingers and a free thumb.The distal phalanx of the thumb is often broad.Cutaneous syndactyly of all toes may be either simpleor complex. There is moderate to severe facial andupper extremity acne.

Mental retardation is variously reported in asso-ciation with Apert syndrome, but it is unclear howmuch of it is secondary to environmental influences.Certainly many patients with Apert syndrome attaina high level of mental function.

Although head shape in Apert syndrome suggestsbicoronal synostosis, a postmortem study of the cran-iofacial changes in the syndrome concluded that thepathology is not primarily due to craniosynostosis butrather stems from reduced growth potential of thecranial base, leading to premature fusion of the mid-line sutures from the occiput to the anterior nasalspine273 and resulting in severe calvarial, maxillary,nasal, and mandibular abnormalities. Other autopsyreports277 suggest that basisphenoid synchondrosis

TABLE 5Syndromic Craniosynostoses

(Reprinted with permission from Whitaker LA, Bartlett SP: Craniofacial Anomalies. In: Jurkiewicz MJ, Krizek TJ, Mathes SJ, Ariyan S (eds),Plastic Surgery. Principles and Practice. St Louis, CV Mosby, 1990. Vol 1, Ch 7.)


24

may be the main factor in facial deformities throughsynostoses posteriorly and anteriorly to the vomer.

For a comprehensive discussion of Apert syndrome,the reader is referred to the April 1991 issue of Clin-ics in Plastic Surgery,278 which is devoted entirely tovarious aspects of the syndrome.

Saethre-Chotzen Syndrome

Saethre-Chotzen syndrome was first recognizedas a discrete entity in 1931-32 by the physicianswho gave their name to the syndrome. Saethre-Chotzen syndrome is characterized by a broadand variable pattern of craniofacial anomalies, fre-quently asymmetrical. Bicoronal synostosis, lowhairline and upper eyelid ptosis characterize thesyndrome. Intellect is normal. The midface isusually normal, which led Tessier273 to call it “up-per Apert.” There is partial cutaneous or simplesyndactyly, usually between the second and thirdfingers but sometimes involving the small fingertoo. Inheritance is autosomal dominant.

Clauser279 reviewed the literature and found anincidence of 1 in 25000 to 1 in 50000. A mutationof the TWIST gene is identified in approximately68% of cases, and mutations of FGF-R2 and FGF-R3have been described.

Pfeiffer Syndrome(Acrocephalosyndactyly Type V)

Pfeiffer syndrome is characterized by broad thumbsand great toes. The severe midfacial hypoplasia

characteristic of Apert syndrome is present, but cra-nial vault malformations are not as extreme. Intel-lect is normal. Tessier273 refers to Pfeiffer syndromeas a “low Apert.”

A 1993 report280 identified subgroups of Pfeiffersyndrome according to the apparently consistent pat-terns of phenotypic expression, including head shape.The classic syndrome (Cohen type I) consists of sym-metrical bicoronal synostosis; all other sutures arenormal. Cohen type II or cloverleaf skull is anexpression of early, extensive multisuture synostosis.All affected subjects have multiple constricting ringsof prematurely fused sutures, grossly foreshortenedanterior and posterior cranial bases, and coincidenthydrocephalus. Moore281 states: “This early, exten-sive fusion of multiple suture rings in association withan intrinsic, or secondary, cranial base distortion pro-motes the development of hydrocephalus, raisedintracranial pressure, calvarial vault thinning, and cra-nial lacunae. Despite early radical craniectomiesand cranial vault reshaping in these patients, recur-ring fusion of the ‘neosutures’ is often rapid.”

Cohen type III is intermediate in the pattern ofextensive sutural involvement. In addition to thebicoronal synostosis, isolated fusion of other suturesis evident early. This becomes progressively morewidespread with time. Cohen280 describes patientswith type III Pfeiffer syndrome as having severe ocu-lar proptosis, an extremely short anterior cranial base,neurological compromise (hydrocephalus is com-mon), and a poor prognosis with early death. Allreported cases of type III Pfeiffer syndrome havebeen sporadic.282

Fig 18. Child with Apert syndrome. A, B, clinical presentation. C, findings on 3-D reconstruction of CT scan. Note the synostotic coronalsuture, the open fontanelle, and the midfacial hypoplasia. (Reprinted with permission from Buchman SR, Muraszko KM: SyndromicCraniosynostosis. In: Lin KY, Ogle RC, Jane JA (eds), Craniofacial Surgery. Science and Surgical Technique. Philadelphia, WBSaunders, 2002; Ch 18, pp 252-271.)


25

Pfeiffer syndrome is inherited as an autosomaldominant disorder. Muenke et al283 and Kerr andcolleagues284 report an affected family in which someindividuals have normal thumbs although several rela-tives do have hand anomalies (symphalangism). Notonly is there phenotypic variability in Pfeiffer syn-drome and other acrocephalic syndactylies, but recentmolecular data indicate variable phenotypic expres-sion for patients who have identical mutations. Pointmutations in FGFR2 have been identified in Crouzon,Jackson-Weiss, and Apert syndromes in addition tosporadic cases of Pfeiffer syndrome.285 In addition, asubset of Pfeiffer syndrome families have a commonmutation in the FGFR1 gene.

Carpenter Syndrome

Carpenter syndrome was originally described in1901 by the man who lent it his name. Acro-cephalopolysyndactyly or Carpenter syndrome con-sists of craniosynostosis, short fingers, soft tissue syn-dactyly, preaxial polydactyly, congenital heart dis-ease, hypogenitalism, obesity, and umbilical hernia.As many as 75% of patients have some degree ofintellectual impairment.286

The craniofacial anomalies are those of brachy-cephaly with variably severe synostosis of the coro-nal, sagittal, and lambdoid sutures, such that thecalvarium is usually distorted and grossly asymmetri-cal, approaching the kleeblattschadel anomaly.Midfacial retrusion and dental malocclusion are lesspronounced than in Apert, and syndactyly is onlypartial and usually simple.273 This is one of the fewcraniofacial malformation syndromes that is inher-ited as an autosomal recessive.

Poole287 cautions surgeons contemplating surgeryon patients with Carpenter syndrome that they mustbeware of the venous and bony abnormalities atten-dant with this syndrome. The vascular anomaliesmay lead to excessive and rapid blood loss.

Crouzon Syndrome

The French neurosurgeon Crouzon described thedisease that bears his name in 1912, at which timehe listed four essential characteristics: exorbitism,retromaxillism, inframaxillism, and paradoxicalretrogenia288 (Fig 19). The estimated incidence is 1in 25000 live births.289 Inheritance is autosomal

dominant and occurrence is both sporadic andfamilial.

Fig 19. Clinical presentation of a child with Crouzon syndrome.Note exorbitism and hypertelorism, retromaxillism and midfacialhypoplasia, pseudoprognathic mandible, and paradoxical retrogenia.(Reprinted with permission from Buchman SR, Muraszko KM:Syndromic Craniosynostosis. In: Lin KY, Ogle RC, Jane JA (eds),Craniofacial Surgery. Science and Surgical Technique. Phila-delphia, WB Saunders, 2002; Ch 18, pp 252-271.)

There are many clinical variations of Crouzon dis-ease, often with one or more of the “typical” signsmissing.290 The shape of the skull alone does notdifferentiate Apert syndrome from Crouzon disease,although Crouzon shows less severe deformity.Crouzon is characterized by recession of the frontalbone and supraorbital rim, retrusion of the facialmass, exorbitism with proptosis, and hypoplasia ofthe infraorbital rim. Unlike Apert syndrome,hypertelorism and a bregmatic bump may not beevident, the nose does not always deviate, and theorbital deformities tend to be symmetrical. The orbitsare quite shallow, with hypoplastic and flat infraor-bital rims, distention of the eyelids, palpebral fissuresslanted downward, exophoria, and divergent strabis-mus; 50% of patients with Crouzon disease havehyperactivity of the inferior oblique muscles. Crouzonpatients usually develop normal intelligence and limbanomalies are absent. The mandible in Crouzondisease, as in Apert, is considerably shorter than nor-mal, producing a distinctive ramus/body length ratio,particularly in older patients.291


26

Cephalometric analysis of patients with Crouzondisease shows a direct correlation between the alteredfacial morphology and abnormalities of the cranialbase. The exorbitism is a reflection of vertical slopingof the anterior cranial fossa, while the midfacial retru-sion can be traced to angulation of the cranial baseflexure.292

Proudman and colleagues293 reviewed 59 patientswith Crouzon disease to determine the non-craniofacial manifestations. They conclude thatCrouzon syndrome is not just a craniofacial defor-mity but “a dysplastic condition that can affect otherareas of the bone and chondral growth, such as thespine and elbows. Ligaments and soft tissues mayalso be involved.” Cervical spine anomalies (40%),stylohyoid calcification (50%), elbow anomalies (18%),minor hand deformities (10%), other musculoskel-etal anomalies (7%), and visceral anomalies (7%) wereall present.

Kreiborg294 traces the craniofacial growth, clinicalfindings, craniofacial morphology, occlusion, andother aspects of dysmorphology in 61 patients withCrouzon syndrome before treatment. Extensive sta-tistical data on adult skeletal dimensions are given, aswell as a comprehensive bibliography. Later, Kreiborgand colleagues295 used 3D CT scans to compare theanatomy of the calvarial cranial base in Apert andCrouzon syndromes. Their findings suggest that theconditions are very different in cranial development.Cartilage abnormalities, particularly those of theanterior cranial base, play a major role in cranialdevelopment in Apert syndrome, whereas the pri-mary abnormality in Crouzon appears to be prema-ture fusion of sutures and synchondrosis. The authorsadvocate early surgery in both groups of patients,but for different reasons: In Apert syndrome, earlysurgery is indicated to reduce further dysmorphicgrowth changes in the calvarial cranial base; inCrouzon syndrome, early surgery can prevent orrelieve elevated intracranial pressure.

Posnick and colleagues296 reviewed their experi-ence in 14 children with Crouzon syndrome whopresented with bicoronal synostosis. Measurementsof the cranioorbitozygomatic region taken from CTscans done pre- and postoperatively served to docu-ment the results of surgical correction. Early surgicalattempts to decompress and reshape the cranioorbitalregions may limit the effects of increased intracranialpressure but do not correct the deformity, as judgedby CT scans. Although the Crouzon deformity did

not worsen after surgery, the measurements remainedfar from normal. Perhaps more importantly, themaxillary and mandibular deformities were notaddressed and the vertical dimension of the face wasunchanged.

In their review of syndromic craniosynostosestreated at NYU, McCarthy and associates269 notedisappointing aesthetic and functional results of sur-gery compared with cases of isolated craniosynosto-sis. The incidence of major secondary procedures,peri- and postoperative complications, hydroceph-alus, shunts, and seizures was significantly increasedin syndromic patients. Frequent problems includedincreased intracranial pressure, airway obstruction,and recurrent turricephaly or cranial vault maldevel-opment. Moreover, early frontoorbital advancement-remodeling “failed to promote midface develop-ment.”

Craniofrontonasal Dysplasia

Craniofrontonasal dysplasia (CFND) is a rarefamilial craniofacial disorder first described byCohen.297 The syndrome combines coronal cranio-synostosis and frontonasal dysplasia with a variety ofextracranial abnormalities. The coronal synostosisresults in brachycephaly (usually asymmetrical) andfrontal bossing, while the frontonasal “dysplasia”manifests as hypertelorism and a broad nose, fre-quently with a bifid nasal tip. Other features arecurly hair, grooved nails, cleft lip and palate, higharched palate, strabismus, shoulder and hip girdleanomalies, soft-tissue syndactyly of fingers and toes,and a broad great toe.298

Orr and colleagues299 reviewed their experiencewith craniofrontonasal dysplasia in 10 female patients.Male patients seem to have a milder phenotype.The precise mode of genetic transmission is unclear,but maternal transmission to daughters and sons hasbeen reported. Affected males have passed the con-dition to 100% of daughters in some published pedi-grees, but male to male transmission is not known tooccur. Treatment is in two stages, one for correctionof the craniosynostosis and the other to deal with thehypertelorism. Six patients had facial bipartition and3 patients had combined intra- and extracranialperiorbital osteotomies, resection of excess midlineor paramedian bony tissue and ethmoid sinuses, andmedial translocation of the orbits.


27

Associated Anomalies

Although cervical spine anomalies are not usuallymentioned in association with craniosynostosis,intervertebral fusion has been documented in 71%of children with Apert syndrome, in 38% of thosewith Crouzon disease, and in 30% of those withPfeiffer syndrome.300 Fusions are most often isolatedand involve complex C5-6 lesions in Apert syndromeand the upper-level cervical vertebrae in Pfeiffer andCrouzon patients. Affected children may have lim-ited range of cervical motion, which has implicationsfor airway management during surgery.300

III — ATROPHY/HYPOPLASIA

Romberg Disease(Progressive Hemifacial Atrophy)

Romberg disease was first described by Parry301 in1825 and later by Romberg302 in 1946. Eulenberg303

coined the term “progressive facial hemiatrophy” in1871. The disease commences usually in the first orsecond decade of life and is more common in girlsthan boys by a 1.5:1 ratio.304 The atrophy is unilat-eral in 95% of cases and affects either side of theface with equal frequency.

The etiology of the disorder is unknown, althoughmany theories for its pathogenesis have been pro-posed. Foremost among these are infection,305

trigeminal peripheral neuritis,306 scleroderma,307 andcervical sympathetic loss.308

The condition manifests as progressive hemifacialatrophy of skin, soft tissue, and bone. Pensler andcolleagues308 evaluated 41 patients and noted that allatrophic changes began in a localized area and pro-gressed at a variable rate within the dermatome ofone or more branches of the ipsilateral fifth cranialnerve. The average age at inception of the diseasewas 8.8 years. The main period of progression was8.9 ± 6 years. In 26 patients with skeletal involve-ment, the mean age of onset was 5.4 years vs 15.4years for 15 patients without skeletal involvement.No correlation could be established between theseverity of soft-tissue deformity and the age of onset.

Tissue from 6 patients who had ultrastructuralanalysis revealed a lymphocytic neurovasculitis withstriking abnormalities of the vascular endotheliumand basement membrane. The alterations of thevascular basal lamina in lymphocytic neurovasculitis

appears to reflect chronic vascular damage with repeatattempts at endothelial cell regeneration.

Moore and colleagues309 noted 50% of patientswith Romberg disease had the classic early sign ofcoup de sabre, reflecting soft-tissue involvement inthe upper face (frontal and maxillary dermatomes).In the presence of prolonged active disease, the soft-tissue atrophy extended to involve the whole hemiface.Late-onset disease appears to be characterized bysoft-tissue atrophy in the lower face. Bony hypopla-sia in the mid and lower face was most common.Involvement of the frontal region was relatively infre-quent.

The derangement of the craniofacial skeleton isunlikely to be solely due to an isolated intrinsic pro-cess. Moore et al309 surmise that restriction of theabnormal soft-tissue envelope undoubtedly com-pounds any primary skeletal growth disturbance. Ifthe disease involves bone, it likely exerts its effect onthe craniofacial skeleton only during periods of facialgrowth acceleration.

Treatment involves 3D reconstruction of all soft-tissue and skeletal disturbances. Surgery is usuallyundertaken at least 1 year after photographic recordsshow no further loss of volume. Greater omentumfree flaps have been described by Jurkiewicz andNahai,310 who note problems with lack of structuralstrength and gravitational descent.

Inigo and colleagues311 review their experiencewith dermis-fat free flaps in 35 patients with Rom-berg disease. They used the groin free flap in 33patients and a scapular flap in 3 patients in a two-stage procedure consisting of transfer of the freeflap and defatting and repositioning 6 months later.Adjuvant procedures included temporal fascial flapsfor the frontal regions and cartilage grafts in thepiriform fossa. The chin was corrected by eithersliding osteotomies for projections of >1cm andby alloplastic implants for projections of <1cm.Severe cases with bone involvement requiredorthognathic surgery in addition to all the aboveprocedures. A Le Fort I osteotomy was performedto lengthen the maxilla and improve the verticaldimension of the face on the affected side. Asagittal split osteotomy completes the requiredrotation-projection of the mandible.

Dunkley and Stevenson312 prefer a groin flapbecause of its less conspicuous donor defect andlower potential morbidity. Tweed et al313 suggestthat a smoother cheek contour can be achieved by


28

placing the dermis outward. Harashina and Fujino314

argue that placing the dermis side down should reducegravitational sagging, one of the major problems withthe reconstruction in this situation. The abdominalflap, a variation of the groin flap based on the infe-rior epigastric vessels, has also been reported.310,315

Koshima et al316 describe their experience with thedeep inferior epigastric perforator flap (DIEP).

Mordick and colleagues317 compared the outcomeof reconstruction with dermal fat grafts (8) vs vascu-larized tissue transfers (8) and and note that vascu-larized transfers provide better augmentation,although dermal fat grafts gave a satisfactory result inmild to moderate defects. Dermal fat grafting is ashorter procedure requiring less anesthetic time, lesstechnical expertise and support, and shorter hospitalstays than microsurgical transfers. Free flaps contain-ing adipose tissue appear to increase in volume dur-ing growth and may also increase with weight gain.As their series progressed, the authors opted increas-ingly for augmentation with the scapular flap becauseof its large-caliber vessels and long pedicle. Theirpatients averaged 3.3 procedures for final correc-tion.

Upton and colleagues318 report their experiencewith scapular flaps for cheek reconstruction in 28patients, 5 of whom had Romberg disease. Themajor advantages of the scapular flap were a con-stant proximal vascular anatomy, long vascular pediclewith large-caliber vessels, large amount of availabletissue (including bone), relatively hairless skin in mostpeople, and minimal or no functional problems atthe donor site. Disadvantages included the lack ofsensation, poor external cheek skin color match, anda predictably widened scar at the donor site. Thestudy produced the following observations:

• deficiency of the malar prominence cannot alwaysbe corrected with soft tissue alone and often needsbone grafting or alloplastic implant;

• when long fascial extensions are used for correc-tion, they must be transferred across the midlineof the upper and lower lips;

• the area most consistently undercorrected is themedial portion of the deficient upper and lower lip;

• the area most consistently overcorrected overliesthe mandibular body and ramus.

Longaker and Siebert319 reported their experiencewith 15 cases of Romberg disease representing 16

free tissue transfers. Deepithelialized, extendedparascapular flaps with large fascial extensions of thedorsal thoracic fascia were used for reconstruction.The fascia can be folded into variable thicknesses tocorrect subtle contour defects of the upper lip, medialcanthus, eyelids, and other facial features traditionallydifficult to reconstruct. These extensions can be placedeasily across the midline to interdigitate with normaltissues at the boundary of the facial deformity.

Past treatment methods have included silicone fluidinjections,320 which are contraindicated because oflong term complications, and injections of lipo-aspirated fat, which have met with mixed results.321

Autologous fat injections certainly have a place inthe correction of mild contour irregularities. Muscu-lar atrophy of pedicled or free muscle flaps present aproblem in calculating the final volume needed forthe repair.322

IV — NEOPLASIA/HYPERPLASIA

Craniofacial Tumors

Intracranial neoplasms may be of many differ-ent types, such as fibrous dysplasia, simple osteoma,benign angiofibroma, neural tumor—specificallymeningioma with extracranial erosion, encapsu-lated neurofibroma, schwannoma, or neurilem-moma323–325—cutaneous carcinoma, osteogenicsarcoma, and rhabdomyosarcoma.

The first major offshoot from craniofacial surgerywas the application of craniofacial techniques to theablation of intracranial tumors. As clinical experi-ence mounted, the following principles for the surgi-cal management of craniofacial neoplasms evolved:

• It is now possible to resect lesions that were previ-ously considered unresectable.326

• Tumor position should be related to the base ofthe skull.327

• Tumor resection demands strict adherence toguidelines designed for tumor location, cell type,and stage, as well as the concept of complete “enbloc” excision. This latter is possible throughregional orbitotomies for confined orbital tumors,bifrontal craniotomy, and facial incisions (eg,Weber-Ferguson) where necessary.

• There is significant risk of losing the frontal boneflap to infection.328,329


29

• The transfacial approach330 may reduce the fre-quency of infectious complications.

• It is essential to prevent communication betweenthe sinuses or nasal cavity and the meninges orintracranial dead space. The best way to achievethis is by interposing thin, vascularized tissue, suchas a pedicled paracranial flap for very small defects,a galea frontalis flap for anterior defects,331 and atemporalis muscle332 or temporoparietalis fascialflap for lateral and central defects.

• Free flaps may be used not only to isolate andprotect vital structures, but also to bring largeamounts of soft-tissue bulk for contouring. Freeflaps may be transferred secondarily to deal withcomplications, but probably should be raised pro-phylactically at the time of the ablative proce-dure.

FIBROUS DYSPLASIA

The most common osseous craniofacial tumorencountered by plastic surgeons is fibrous dyspla-sia.335 Fibrous dysplasia is an uncommon, non-neoplastic, benign disease of bone that was was firstdescribed by von Recklinghausen in 1891.336 Thepathogenesis of fibrous dysplasia involves abnormalactivity of the bone-forming mesenchyme with anarrest of bone maturation in the woven bone stage,forming irregularly shaped trabecula. Mutations ofsignaling protein and increased IL-6 levels have beenimplicated in the process.337 The condition is usuallyprogressive until age 30, and reports of progressionwell into adulthood are not uncommon.338

Fibrous dysplasia in the cranioorbital area tends tobe more osseous than fibrous dysplasia of other sites.Two patterns of presentation predominate: themonostotic form, with single-bone involvement, andthe polyostotic variety, which may be associated withabnormal skin pigmentation, premature sexualdevelopment, and hyperthyroidism (Albright syn-drome). The monostotic form is approximately 4Xmore common than the polyostotic form andapproximately 30X more frequent than the com-plete Albright syndrome. The monostotic form com-monly involves the ribs, femur, tibia, cranium, max-illa, and mandible. The most commonly affectedbones in the cranium are the frontal and sphenoidbone; in the face, the maxilla338,339 (Fig 20).

Posnick339 lists the keys to management of fibrousdysplasia, namely

• accurate diagnosis

• radiographic assessment

• clinical evaluation

• planning of interventions

• long term follow-up

Deformation caused by fibrous dysplasia of theanterior skull base can affect the architecture ofthe orbits and paranasal sinus, causing orbital dis-placement and exophthalmos.340,341 In craniofa-cial fibrous dysplasia, the symptoms are varied andrelated to tumor mass, and may include facial painand swelling, headaches, anosmia, deafness, blind-ness, malocclusion with displacement of teeth,diplopia, proptosis, and orbital dystopia. Cranialnerve palsies including optic nerve compressionare not uncommon.342 Eye symptoms may includeextraocular muscle palsy and trigeminal neuralgiasecondary to compression of the third and fifthcranial nerves. Orbital apex compression can ini-tiate the cascade of ophthalmic venous engorge-ment, optic nerve atrophy, and loss of vision. Sphe-noid body involvement can cause blindness as aresult of compression of the optic nerve betweenthe chiasm and optic foramen. Other minor oph-thalmic symptoms include epiphora produced byocclusion of the lacrimal duct and visible externallid deformities.343

Malignant degeneration occurs in approximately0.5% of cases.344 Clinical signs of malignancy consistof a rapid increase in size, pain, intralesional necro-sis, bleeding, and elevation of serum alkaline phos-phatase levels. The most common transformation isto osteogenic sarcoma, but fibrosarcoma and chon-drosarcoma are also seen. The mean interval fromdiagnosis of fibrous dysplasia to evidence of malig-nancy is 13.5 years.343 The polyostotic form of thedisease has a higher incidence of malignant degen-eration, although in the craniofacial region themonostotic form is more common. Malignantdegeneration can occur spontaneously or followingradiotherapy.

Cherubism is a genetic disorder of the mandibleand maxilla of the giant-cell type. It is strictly a self-limiting disease of children that regresses without sur-gery and leaves no deformity.345


30

In the past, conservative treatment of fibrous dys-plasia was preferred, occasionally with bone-contouring procedures. Spontaneous involution willnot occur, however, and early surgical interventionwhen symptoms are just beginning may avoid exten-sive resection later.346 When clinically feasible, themainstay of therapy is complete or near-completeresection and reconstruction with normal autogenousbone.

In 1972 Derome347 pioneered the concept of totalexcision and immediate reconstruction of bonetumors, including 4 cases of fibrous dysplasia. Chenand colleagues348 discussed the benefits and risks ofprophylactic optic nerve decompression before symp-toms develop. The decision for surgery in thesecases is made solely on the basis of CT findings ofencroachment of the optic canal by fibrous dyspla-

sia. The primary justification for prophylacticdecompression is the speed with which visual dete-rioration can occur and the brief interval before itbecomes permanent. Among reports of sudden lossof vision there are several that attributed the cause ofblindness to hemorrhage or mucocele secondary tofibrous dysplasia.349 In their own study of 18 patientswith clinical or radiological evidence of optic canalinvolvement, Chen and associates348 report 6 patients(33%) had loss of effective vision in the involved eye.From this experience and a review of the literature,the authors developed an algorithm for decompres-sion of the optic nerve in fibrous dysplasia. Absoluteindications for decompression are

• progressive gradual visual loss

• within 1 week of sudden visual loss

Fig 24. A 5-year-old girl with polyostotic fibrous dysplasia and massive involvement of the maxilla and mandible bilaterally. A, C, D, beforeoperation. B, 10 days after radical maxillary and mandibular debulking. (Reprinted with permission from Posnick JC, Hughes CA, MilmoeG, et al: Polyostotic fibrous dysplasia: an unusual presentation in childhood. J Oral Maxillofac Surg 54:1458,1996.)


31

Relative indications for decompression are

• patients presenting within 2–3 weeks of rapid visualloss

• children or adolescents with no visual loss butradiographic evidence of optic canal reduction,since they are likely to have progressive growth offibrous dysplasia

• patients with no visual loss who have radiographicevidence of optic canal reduction and continu-ing, active fibrous dysplasia.

Reconstruction after resection is typically immedi-ate. Edgerton and colleagues336 described the use ofthe dysplastic bone as grafts, which following resec-tion were contoured, thinned, and replaced. Thesegrafts of dysplastic bone seem to function similarly tonormal autogenous grafts and lack the potential forgradual recurrence of bone thickening frequently seenwith in situ bone contouring. Autoclaving350 and cryo-therapy351,352 have been proposed to destroy the cel-lular elements and yet preserve the mineral matrixprior to reinsertion.

Chen and Noordhoff353 analyzed their experiencewith the treatment of 28 patients with fibrous dyspla-sia. The authors outline a protocol for surgery (Table6) that includes1. total excision of dysplastic bone of the fronto-

orbital, zygomatic, and upper maxillary regionand primary reconstruction with bone grafts;

2. conservative excision of bone from under the hair-bearing skull, central cranial base, and tooth-bearing regions; and

3. optic canal decompression in patients with orbitalinvolvement and decreasing visual acuity.

In a follow-up averaging 5.3 years, they find norecurrence or invasion of fibrous dysplasia into thegrafted bone, but 5 of 19 patients treated withreduction of alveolar dysplasia had recurrence of thedeformity that necessitated reshaping. One patientwith recurrent mandibular fibrous dysplasia was suc-cessfully treated with hemimandibulectomy and man-dibular reconstruction with vascularized bone graft.

Hansen-Knarhoy and Poole354 remark on the dif-ficulty differentiating fibrous dysplasia fromintraosseous meningioma around the orbital apex.From a review of their files, they were able to sum-marize the differences as follows:• Fibrous dysplasia commences in childhood and

early adolescence while meningiomas are diseasesof middleage.

• Visual symptoms are prominent in meningiomacases and uncommon in fibrous dysplasia.

• Proptosis is much more marked than orbital dys-topia in the meningioma group while the reverseis true in fibrous dysplasia.

• Fibrous dysplasia patients have extensive frontalbone involvement that is clinically obvious. Thereis no evidence of this in meningioma patients.

• Pain is present in both groups and is not a usefuldiscriminating feature.

Most fibrous dysplasia lesions can be only partlyexcised and some are unresectable, arguing for treat-

TABLE 6Treatment Protocol Based on Location, as Proposed by Chen and Noordhoff

(Reprinted with permission from Chen Y-R, Noordhoff MS: Treatment of craniomaxillofacial fibrous dysplasia: How early and howextensive? Plast Reconstr Surg 86:835, 1990.)


32

ment modalities other than surgery. No medicaltreatment is available to cure or definitively halt theprogression of fibrous dysplasia. Reports of promis-ing responses to systemic treatments with biphospho-nate pamidronate await further studies before con-clusions can be drawn.355 Clark and Hobar356

implanted fibrous dysplasia cells in a nude mousemodel and successfully decreased the size of thetumor with tamoxifen administration.

NEUROFIBROMATOSIS

Neurofibromatosis is a hereditary conditionoccurring in 1 in 3000 live births. Approximately50% of patients have a positive family history of neu-rofibromatosis. The term neurofibromatosis is appliedto two clinical genetic disorders. The first, neurofi-bromatosis type 1 (NF1), also known as vonRecklinghausen disease, is more common. The sec-ond, neurofibromatosis type 2 (NF2), is a rarer con-dition and is known as bilateral acoustic neurofibro-matosis. The two disorders have very distinctive clini-cal manifestations and some overlapping features.Mutation of genes on two different chromosomesare at the origin of the two disorders. The gene forNF1 is located on the long arm of chromosome 17,while the gene for NF2 is located on the long arm ofchromosome 22.357 Transmission is autosomal domi-nant, with variable penetrance.

Neurofibromatosis is a benign tumor of neuroec-todermal origin with diffusion to skin, subcutaneoustissue, and bone (Fig 21). Tumor growth is slow andirregular and not accelerated by surgical interven-tion. Sarcomatous degeneration is rare.

Surgical care often produces less than completecorrection, with some further progression overtime.358,359 Poole,358 in a series of 11 patients, stressesthe high complication rate and modest improvementin appearance that should be expected. The bestcosmetic results are obtained in patients whose eyecan be removed and a satisfactory bed created foran orbital prosthesis. Patients who have a seeing eyeand wish to retain it should undergo a two-stageprocedure with conservative debulking of intraor-bital soft-tissue.

Krastanova-Lolov and Hamza357 reviewed theirexperience with 14 cases of cranioorbital neurofi-bromatosis. They note the partial or completeabsence of the greater wing of the sphenoid isresponsible for enlargement of the sphenoidal fis-

sure, with a consequent defect in the posterior wallof the orbit. Brain tissue, generally the temporallobe, may herniate into the orbit, further increasingexorbitism and causing pulsation of the eye. Theorbit is enlarged, with hypoplasia of the supraorbitaland infraorbital rims and the zygomatic arch. Whenthe globe is involved with the neurofibroma theremay be buphthalmos, severely diminished visual acu-ity, and even blindness. Associated symptoms areirritation, pain in the eye, and moderate to severeepiphora. Enophthalmos may occur from enlarge-ment of the inferior orbital fissure, which allows theorbital contents to prolapse into the infratemporalfossa.

Snyder360 describes the experience of the Austra-lian Craniofacial Unit in correcting 14 cases of orbitalneurofibromatosis. Of note is the finding of resorp-tion of the bone graft used to reconstruct the greaterwing of the sphenoid in four cases, which led to recur-rence of globe pulsation. Subsequently the authorbegan using titanium mesh in the reconstruction.

Jackson361 presents his experience with orbito-temporal neurofibromatosis in 24 patients and pro-vides a classification and treatment scheme. Hegroups the condition into three categories, each ofwhich requires a different treatment: (1) orbital soft-tissue involvement with a seeing eye; (2) orbital soft-tissue and significant bone involvement with a seeingeye; and (3) orbital soft-tissue and significant boneinvolvement with a blind or absent eye. The author

Fig 21. Patient showing clinical evidence of advanced neurofi-broma. Photo courtesy of PC Hobar MD.


33

traces the patients’ course over a maximum of 12years of recurrence-free follow-up.

V — UNCLASSIFIED

A few rare anomalies do not fit into the first fourcategories and are best placed in this last category.They should be described based an the organ ororgans involved. Examples include aglossia or mac-roglossia, anotia,and ocular anomalies such asepicanthal folds.10

CRANIOMAXILLOFACIAL SURGERY

HISTORY

The origin of craniofacial surgery can be traced asfar back as 1890, when Lannelongue and Lane per-formed the first craniotomies,362,363 but it was notuntil the First and Second World Wars that numer-ous battle casualties stimulated the development oftechniques for the replacement of missing bone andsoft-tissue of the face, and set the stage for attemptsin the 1940s and ’50s to correct congenital facialdeformities.

Waterhouse364 reviews the history of craniofacialsurgery with particular emphasis on the contribu-tions by Le Fort, Virchow, Gillies, and Tessier. In1951 Gillies and Harrison365 reported the first suc-cessful Le Fort III advancement osteotomy for cor-rection of midfacial retrusion in a patient withCrouzon syndrome. The operation was technicallyvery complex and only partially successful in correct-ing the exorbitism, because it carried the osteotomyin front of the orbital rim, lacrimal sac, and medialcanthal ligament. In 1962 Converse and Smith366

described an operation to correct hypertelorism basedon their experience with malunited nasoorbital frac-tures with telecanthus.

Paul Tessier is regarded by most clinicians as thefather of craniofacial surgery. After years of carefulstudy with many of the most innovative maxillofacialsurgeons of his time and numerous hours spent inthe laboratory doing cadaver dissections, Tessier pro-posed a novel method of facial osteotomies andwholesale mobilization of bone for operating onpatients with deformities of the craniofacial skeleton.Tessier pioneered the intracranial approach for thecorrection of hypertelorism, worked closely withneurosurgeons to resect difficult craniofacial tumors,

was first to manipulate cranial bones in the treat-ment of patients with craniosynostoses, and per-formed successful Le Fort III operations in difficultcases of Apert and Crouzon syndrome. In 1967Tessier367 presented this work at the Fourth Interna-tional Congress of Plastic and Reconstructive Surgeryin Rome, and the new field of craniofacial surgerywas launched.

Two principles fundamental to the practice ofcraniomaxillofacial surgery emerged from his discus-sion: (1) large segments of the facial and cranial skel-eton can be completely denuded of their blood sup-ply, repositioned, and yet survive completely; and(2) the eyes can be translocated horizontally or verti-cally over a considerable distance without impairingthe vision.

Tessier’s results in the late ’60s and early ’70sproved conclusively that most skeletal deformities ofthe face and calvarium can be corrected or at leastsignificantly improved by appropriate surgicalmaneuvers. The importance of his work to theintracranial correction of hypertelorism368–370 cannotbe overemphasized. Along with Converse’s one-stage procedure, it is the backbone of present-daytechniques for hypertelorism correction.

Basing his approach on Le Fort’s anatomic researchwith cadaver skulls,371 Tessier developed the Le FortIII osteotomy for facial advancement and presentedhis results in 1971. His experience encompassed151 patients representing almost 500 individual mal-formations of the cranial and facial region. Tessier’scontributions to craniofacial surgery were reviewedin Rome in 1982 on the occasion of the 15th anni-versary of Tessier’s original presentation.372

The advent of plate-and-screw fixation aftermaxillary and mandibular osteotomies has beenof tremendous benefit to the management of con-genital as well as traumatic cases of cranio-maxillofacial deformity. Rigid fixation has dramati-cally enhanced the stability of bony fragments,improved primary bone healing, and eliminatedthe need for prolonged maxillomandibular fixa-tion in the older patient.373–378

The fate of microfixation hardware during rapidcalvarial growth in infants and young children hasbeen questioned. Munro and colleagues379 acknowl-edge intracranial “migration” of microplates andscrews. Goldberg and colleagues380 report cases ofchildren undergoing revisional surgery who werefound to have microscrews and plates lying beneath


34

the inner calvarial lamina. Subsequently Gold-berg381 sought to clarify the incidence of markerplate translocation and tried to identify potentialclinical implications. In a retrospective review of27 pediatric patients, CT imaging demonstratedinternalization of marker fixation in 14 children.The younger the patient at the time of surgery, thehigher the likelihood of translocation. Patientswith syndromic craniosynostosis seemed to have agreater risk of translocation than those with iso-lated synostosis. Longer plates had a higher pro-pensity for movement than shorter plates. Despitethese observations, the authors note no complica-tions related to the translocation of marker plates.

Papay et al382 state that plating osteosynthesis pro-vides a three-dimensional, stable fixation of the cra-nial skeleton and anatomical recontouring of thecranial vault. In their series of 20 patients who hadsecondary cranial remodeling within 2 years of thefirst surgery, “false migration” of microplates wasnoted in 7. Neither the sharp edges of the screwsnor the ends of the stainless steel wires pierced thedura mater in any patient. Despite the lack of neu-rological sequelae from these fixation systems, it istheir opinion that marker plate fixation for cranialvault reconstruction should be limited to those regionswhere additional 3D structural support is essential.

Berryhill et al383 reviewed the fate of rigid platefixation in 96 children. There were 375 titaniumplates and 1944 screws placed among the patients inthe series. They found 5 cases of delayed growth,one instance of restricted growth, 9 palpable platescausing pain, 3 fluid accumulations over plates, and2 cases of meningitis. Plates were removed in 8patients. The overall complication rate was 23%.

A series by Orringer reported 55 instances of plateremoval.384 The most common reason was palpableor prominent hardware (34.5%). Other reasonsincluded loose plates (25.5%), infection (23.6%), andexposure of hardware (20%).

Resorbable plating systems have enjoyed wideacceptance in craniofacial surgery. The Lactosorb(Poly-Medics, Warsaw IN) plating material is acopolymer of polylactic and polyglycolic acid.Polyglycolic acid biodegrades more rapidly thanpolylactic acid, and thus these combination platesresorb faster than plates made entirely of polylacticacid.385

Eppley and Sadove386 reviewed the effects of bio-degradable plates on 10 juvenile rabbits. The fixa-

tion was placed across the left coronal suture. Sixmonths after implantation, the authors noted sym-metrical frontal bone development, unaffectedgrowth across the coronal suture, and a histologicallynormal underlying suture. Apparently the fixationplate stretches to accommodate growth along theunderlying suture. The change in shape of theresorbable device seems to be more important thancomplete degradation of the material in rapidly grow-ing bone sites.

Subsequently Eppley and Sadove387 analyzedtheir results with resorbable coupling fixation in20 infants with calvarial deformities. Thin, straight,resorbable plates were used for skeletal fixationafter osteotomies and repositioning. A total of231 fixation devices were implanted without com-plications and remained trouble-free after 12 post-operative months. Wiltfang et al388 noted that theresorbable plates were prone to intraosseousmigration (as are titanium plates), but their resorp-tion over 12–18 months obviates this problem.Multiple other clinical series with long follow-upshow the Lactosorb plating system to be associatedwith minimal or no complications.389–391

Other areas of significant advance in the field ofcraniofacial surgery include radiographic improve-ments such as MRI scans and 3D CT scans,392 dis-traction osteogenesis, increased use of microvasculartechniques and tissue expansion, and better under-standing of alloplastic materials.

SURGERY ON THE CRANIAL VAULT AND FOREHEAD

Early Treatment

The rationale for early treatment in craniosynos-tosis is to minimize the extent of primary and sec-ondary calvarial deformity, to allow cranial expan-sion and prevent the sequelae of raised intracranialpressure, and to minimize operative morbidity.

The history of neurosurgical craniectomy for thecorrection of craniosynostosis can be divided intotwo basic operative approaches. In the past thestandard treatment was a linear craniectomy alongthe course of the stenosed suture line. This treat-ment was associated with a high rate of recurrenceof craniosynostosis, and attempts at preventing relapseby the use of polyethylene film placed around thebone edges met with little success.393 More recentlythe trend has been towards a more active ana-


35

tomical correction of the deformitiy at the time ofsurgery, with less reliance on passive correctionpostoperatively, although many authors still advo-cate vertex craniectomy for young infants with sag-ittal synostosis.394

In 1971 Tessier275 mobilized the lower part of thefrontal bone in one piece while performing maxillaryadvancement in adult patients with Crouzon or Apertsyndromes. Hoffmann and Mohr395 adapted Tessier’ssupraorbital advancement techniques to infants, per-forming a unilateral coronal supraorbital advance-ment.

Marchac396 later devised a technique that involved(1) rocking and advancement of the supraorbital barwith a lateral temporal spur in the form of a Z-plastyfor stability and retention—the floating forehead—and (2) mobilization and rearrangement of the ante-rior cranial vault by means of bone grafts. Marchacoriginally used the floating forehead technique withfrontocranial remodeling in the treatment ofbrachycephaly, Crouzon or Apert syndromes, andoxycephaly.397 Marchac had originally hoped thatthe floating forehead technique would allow nor-malization of midfacial growth, but unfortunately thisdid not materialize.

Other techniques utilize the mathematics of pro-jection, geometry, and stress relief to produce anappropriate forehead contour.398 Other methodstreat the forehead as a totally misshapen unit thatmust be adjusted in all three dimensions with mul-tiple osteotomies and cranial bone grafts to recreatethe forehead/bandeau unit in jigsaw-puzzle fashion(Fig 22).399

Munro and coworkers400 advocate 180° reversalof the entire upper cranium, while Jackson, Hide,and Barker401 prefer frontal transposition cranioplastyfor correction of frontal contour. Ortiz Monasterioand colleagues402 combine frontal mobilization witha one-piece orbitofacial advancement in Crouzonsyndrome.

Operations for frontocranial remodeling havefacilitated the approach to cranial as well as facialdeformities and have considerably improved the over-all surgical results. The vast number and variety ofmethods to reshape the orbital rim and cranial vaultare proof that no technique is ideal in all cases. Thebasic concept should be to reshape all bone that isabnormal and to rearrange bone to arrive at the

most aesthetic contour, and the degree to whicheach of these maneuvers is applied differs amongpatients. For example, at times it may be preferableto shape the forehead by recontouring the existingfrontal bone, while at other times this goal is bestaccomplished by replacing the frontal bone withparietal bone. Early frontal remodeling is facilitatedby easy malleability of bone; rapid reossification;outward push by the growing brain; and the benefi-cial effect on adjacent structures of releasing thestenosed areas.136,199 On the other hand, the earlier

Fig 22. Bilateral coronal synostosis and its surgical correction. A,site of anterior cranial vault and three-quarter orbital osteoto-mies. B, after osteotomies and reshaping and fixation of thecranioorbital region. (Reprinted with permission from PosnickJC: Craniosynostosis: Surgical Management in Infancy. In: BellWH (ed), Orthognathic and Reconstructive Surgery. Philadel-phia, WB Saunders, 1992; vol 3, p 1859.)


36

in infancy the cranium is reshaped, the more it canbe potentially affected by growth.403,404

McCarthy et al405 reported an adverse effect onfrontal sinus development and forehead aestheticsof early frontoorbital advancement. Of 8 patientswho underwent bilateral frontoorbital advancementin infancy who had been followed for at least 10½years, only one developed a frontal sinus and all hada flattened brow. Of 3 patients with unilateraladvancement, one developed a frontal sinus on theoperated side and the others had an obvious defor-mity due to discrepancy in brow projection betweenthe operated and nonoperated sides. The authors405

advocate bilateral frontoorbital advancement for cor-rection of plagiocephaly because of the possible asym-metry that may result from unilateral frontal sinusdevelopment.

In contrast, Bartlett, Whitaker, and Marchac406

evaluated a study population of 48 children oper-ated for plagiocephaly in infancy using both bilateraland unilateral approaches. After following the patientsfor a minimum of 3 years, the authors concludedthat either unilateral or bilateral frontoorbitaladvancement produced good results, and there wasno advantage to using one technique over the other.The study obviously did not follow all these infants tothe time of frontal sinus development.

David and Sheen407 analyzed their results in thesurgical treatment of 39 patients with Crouzon syn-drome. They found that early frontoorbital advance-ment was universally successful in relieving elevatedintracranial pressure and reducing ocular proptosisbut did not prevent midfacial hypoplasia or providedefinitive cosmetic correction of the deformity.Although frontofacial advancement in adults gavegood long-term results, it was attended by more com-plications than other corrective procedures.

Marchac and Renier403,404,408 note normal facialgrowth after early craniofacial surgery in manynonsyndromic patients. McCarthy,409 on the otherhand, reports premature refusion of the sutures ordevelopment of turricephaly and other calvarial con-tour irregularities in 50 children who had early sur-gery for craniofacial synostosis. The mild casesreceived extensive strip craniectomy and the moresevere or syndromic patients had strip craniectomywith frontal bone advancement. A mean 8 yearsafter craniofacial remodeling, 20% had to be reop-erated because of recurrent deformity. The authors

conclude that early surgery did not result in satisfac-tory occlusal relationships or midfacial form.

Dufresne and associates410 used 3D CT analysis tocalculate the intracranial/ventricular increases in vol-ume that were produced by craniofacial surgical pro-cedures. Marsh411 studied with 3D CT imaging theendo- and exocranial bases of patients who had earlycorrective surgery for craniosynostosis. He concludedthat surgery during infancy induces normalization ofendocranial symmetry over the first postoperativeyear in patients with nonsyndromic solitary andbicoronal synostosis, but not in those with multiplesynostoses. Other analyses of long-term results412

find less improvement in endocranial base symmetryor normalization of intracranial growth after earlysurgery.

The optimal age of the patient at surgery remainscontroversial. Some authors strongly favor early treat-ment of craniosynostosis during infancy (which is vari-ously interpreted as the period between the first weekof life up to 4–6mo393) to allow brain growth toremodel the calvarium.413

Ocampo and Persing237 feel that the quality of theresult is inversely proportional to age of the child atoperation. Most craniofacial surgeons would agreethat early surgery is indicated in the event of multiplesuture synostosis414 and in sagittal synostosis when anextended vertex craniectomy is performed. Certainlysurgery should be done as early as possible for thesevere craniosynostosis syndromes such as kleeblatt-schadel.396,404 Depending on the number and loca-tion of prematurely fused sutures, therefore, earlycranial vault decompression may be indicated to pre-vent restrictions on the developing brain.403,411,415 Itis impossible to know which infants will require earlyrelease to prevent neural complications.

By age 18 months, children have attainedapproximately 75% of their brain growth136,411 (Table7). Reossification is more extensive at a youngerage416 and can be reliable up until age 2.

When single sutures are involved, many craniofa-cial surgeons delay surgery until the child is 6–12months, at which time the risk of intracranial hyper-tension is thought to be low.404,411 Wall417 notes ahigher reoperation rate when patients are operatedon at an early age (3mo) than if frontoorbitaladvancement is delayed (6–12mo). Because thechanges brought about by surgery will be diluted bypostoperative growth, others415 wait even longer incases of Apert syndrome and the more severe sym-


37

metrical deformities to lessen the need for majorrevisions at a later date.404

Certainly unilateral cases can be expected toachieve better results than bilateral cases. McCarthydemonstrated good to excellent results in 86% ofpatients undergoing unilateral procedures and in 49–70% of bilateral procedures.418,419

SURGERY FOR COMBINED CRANIOSYNOSTOSIS AND

HYPOPLASIA OF THE MIDFACE

There have been few changes in the treatment ofCrouzon disease since Tessier first described his clas-sic facial osteotomy technique.275,288,367,420,421 Modifi-cations have progressed from the Tessier I—asubcranial Le Fort III—in 1958, through the moreradical Tessier VIII of 1976, which split and bent afrontofacial monobloc segment, derotated the orbits,and brought the maxilla forward. In Tessier’s handsthis extensive procedure yielded nearly normal results.

In 1976 and again in 1979, Van der Meulen422,423

described a similar operation that involves simulta-neous total osteotomy of the two halves of the faceand correction of the orbitofrontal and maxillarydeformities. The coronal approach to osteotomy of

the pterygomaxillary fissure obviates intraoral surgerywith its potential contamination.

Ortiz Monasterio and associates402 first advocatedmonobloc advancement of the entire forehead andface (Fig 23) in 1977. Anderl and coworkers424 pro-posed a modification of the frontoorbital bar withpreservation of an intact anterior cranial base to sepa-rate the cranial and nasal spaces when indicated forrelief of respiratory or orbital distress accompanyingsevere craniosynostosis. Although many surgeonsbelieve that monobloc advancements are hazardousbecause of the high number of associated infectioussequelae, the authors feel their technique is safe evenat a young age.

Fig 23. Cranial vault reshaping for correction of Crouzonsyndrome with bilateral coronal synostosis. The procedureinvolves osteotomies of the anterior cranial vault, monobloc, LeFort I, and chin. (Reprinted with permission from Posnick JC:Craniosynostosis: Surgical Management of the Midface Defor-mity. In: Bell WH (ed), Orthognathic and ReconstructiveSurgery. Philadelphia, WB Saunders, 1992; vol 3, p 1888.)

Wolfe425 reviewed his experience with 32 patientswho underwent transcranial monobloc frontofacialadvancement +/– simultaneous facial bipartition.The author cautions that the procedure “carries withit substantial risks, [but] with careful consideration ofairway control, the anterior cranial base dura, andretrofrontal dead space, the procedure is recom-mended for carefully selected patients.” Wolfe listshis indications and contraindications for the proce-dure in various age groups.

Treatment for combined deformities from cran-iosynostosis of the anterior cranial vault and

TABLE 7Brain Growth During the First 20 Years of Life

(Data from Blinkov SM, Glezer II: The Human Brain in Figuresand Tables: A Quantitative Handbook. New York, PlenumPress, 1968. Reprinted with permission from Marchac D, RenierD: Craniofacial Surgery for Craniosynostosis. Boston, LittleBrown, 1982, p 36.)


38

midfacial retrusion typically involves two operativestages, initially advancing the forehead and subse-quently the face. This results in fewer infectionsthan monobloc advancement.426 Marchac andRenier397 advocate Tessier’s frontal horizontaladvancement with lateral fixation by tongue-in-groove for intracranial forehead correction. Theresultant exaggeration in facial retrusion is cor-rected at a later date.397,404

Fearon and Whitaker426 reviewed their 15-year,29-patient experience with midfacial andfrontofacial advancement for craniosynostosis. Ofthe 30 procedures performed, 20 were Le Fort IIIsand 10 were monobloc advancements. All majorinfections occurred in the monobloc group. Theaesthetic results were the same regardless of thetype of facial advancement performed. Given thehigh infection rate associated with monoblocadvancement and the similar outcomes, theauthors believe the surgical correction of cranio-synostosis should be staged.

SURGERY ON THE ORBITS

Surgical techniques for the correction of exorbitism,ocular dystopia, enophthalmos and hypertelorism arebased on principles of craniofacial surgery.

Dystopia

Ocular dystopia can be congenital or traumaticand is corrected by unilateral orbital repositioning.427

Edgerton and Jane428 review various eye-leveling tech-niques, ranging from simple bone grafts to complexC-type osteotomies429 and Tessier’s marginalosteotomy, which removes the orbital rim, levels it,and repositions it.

The surgical treatment of ocular dystopia variesaccording to the age of the patient and the causeof the orbital anomaly. In young patients who areactively growing, it is preferable to use thefrontoorbital bandeau technique so as not to dam-age the dental buds. In adult patients, Tessier’s430

orbital quadrant technique is used. At birth theorbital volume is about 70% of its adult volume.At 18 months, it is about 75–80%, and at 3 yearsof age, it is about 90%. The orbital volume iscompletely developed by age 10 to 11.

Hypertelorism

The severity of hypertelorism is rated according tothe interorbital distance (IOD),370 as follows:

1° — 30–35mm IOD2° — 35–39mm IOD with normal orientation

and shape of the orbits3° — 40+mm IOD with defects in the

cribriform plate, orbital region, and lateral canthus

The normal adult IOD is 25mm for women and28mm for men. The usual growth progression431 isas follows:

age 1 year 18.5mm3 years 21mm7 years 23mm12 years 26mm

The interpupillary distance (IPD) is not a true indica-tion of hypertelorism because abnormal rotation ordivergent strabismus of the eyes commonly accompaniesthe syndromic craniofacial anomalies. Similarly, theintercanthal distance or ICD can be skewed by exces-sive soft tissue bulk or traumatic telecanthus.

Evereklioglu et al432 propose a clinically applicableand practical definition of hypertelorism as anincrease in the interpupillary distance in the absenceof strabismus (Fig 24). An isolated measurement ofhypertelorism is not clinically applicable unless it isnoted in the context of overall head size. The authorspropose an interpupillary index which they believe isa more accurate projection of hypo- and hyper-telorism related to head size. The index is calculatedby the formula

The authors also provide normative values in healthychildren.

Hypertelorism is etiologically heterogeneous.Nontraumatic hypertelorism is always secondary toanother deformity, either craniosynostosis or a medianor paramedian facial cleft. Munro427 notes four typesof ethmoid and medial orbital wall deformity inhypertelorism (Fig 25). Types C and D are the rarestand most difficult to correct.

Correction of hypertelorism can be subcranial orintracranial. The subcranial approach offers twoalternatives: medial orbital wall migration or U-shaped


39

osteotomy mobilizing the lateral, inferior, and medialorbital walls as a single bloc. The maximum correc-tion that is possible with medial orbital wall mobilizationis 10mm; with a U-shaped osteotomy, 15mm.

The intracranial approach as described byTessier368,369 with Converse’s modification433 is pre-ferred in all severe cases of hypertelorism or whenthe cribriform plate lies below the level of thefrontonasal suture (Fig 26). The entire orbit is mobi-lized as a box, providing total control of the intracra-nial contents.

McCarthy and colleagues434 report correction oforbital hypertelorism on 20 children age <5 (aver-age age at operation 3.9y) who were followed for a

mean of 5y. The authors conclude that the Tessierprocedure can be safely performed at this age and isaesthetically desirable.

Ortiz Monasterio435 outlines a surgical protocolfor the correction of orbital hypertelorism that takesadvantage of the ability to section the face into halves.The procedure simultaneously approximates theorbits, elongates the midface to correct a short nose,and lowers the dental arch to minimize an anterioropen bite.

Van der Meulen and Vaandrager436 discuss haz-ards and complications of hypertelorism correction,including canthal drift, enophthalmos, ptosis, tem-poral depression, and abnormalities in orbital con-figuration. The authors speculate about the causesof less than optimal results and measures for theirprevention.

Fig 24. Interocular distance measured from inner canthus toinner canthus. A, normal. B, primary telecanthus. C, true ocularhypertelorism. D, ocular hypertelorism with secondarytelecanthus. (Reprinted with permission from Cohen MM Jr,Richieri-Costa A, Guion-Almeida ML, Saavedra D: Hypertelorism:interorbital growth, measurements, and pathogenetic consider-ations. Int J Oral Maxillofac Surg 24:387, 1995.)

Fig 25. Types of medial orbital wall deformity in patients withorbital hypertelorism. A, parallel. B, wedge-shaped posteriorly.C, oval-shaped. D, wedge-shaped anteriorly. (Reprinted withpermission from Munro IR, Das SK: Improving results in orbitalhypertelorism correction. Ann Plast Surg 2:499, 1979.)


40

Anophthalmia and Microphthalmia

Anophthalmos and microphthalmos represent fail-ure of optic development. Absence or diminutivesize of the eyes fails to stimulate orbital growth andresults in orbital hypoplasia. Kennedy437 elicitedhypoplasia of the orbit by ocular enucleation duringembryogenesis in cats.

The classic treatment for orbital hypoplasia sec-ondary to anophthalmia or microphthalmos has beenthe insertion of progressively larger silicone orbitalimplants covered by scleral shells. This treatmenthas met with only moderate success due to difficultyin maintaining the implants and the need for fre-quent readjustments.

Tessier438 enlarged the orbit by a lateralosteotomy that allowed expansion of the orbitalvolume inferiorly and laterally. Marchac439 lateradded a supraorbital craniectomy and frontal cran-iotomy that relocated the orbital roof and allowedexpansion of the orbital volume in three direc-tions. Elisevich and colleagues440 subsequently

described a technique for 3D expansion of theorbit without craniotomy using a single burr-holecraniectomy of the frontosphenoid bone.

Working from the basis that orbital hypoplasia isthe consequence of an inadequate stimulating matrix,Lo et al441 demonstrated the effectiveness of subperi-osteal tissue expanders in the bony orbit to substitutefor the missing stimulus. They enucleated one eye in12 6-week-old kittens and placed subperiosteal tis-sue expanders in half of the orbits; the other 6 servedas controls. All the orbits in which tissue expanderswere placed demonstrated equal or greater orbitalvolumetric measurements than normal orbits, com-pared to enucleated orbits that did not receive tissueexpanders, whose measurements were significantlylower than the normal.

Rodallec and coworkers442 report the use of sili-cone expanders in 17 patients. The expanders wereplaced within the intramuscular cone to expand notonly the bony orbit but also the soft tissues.

SURGERY ON THE MAXILLA

Le Fort I Osteotomy

The Le Fort I osteotomy is a horizontal osteotomyof the upper jaw at the level of the (Le Fort I) fractureline. It was initially described by von Langenbeck in1859 and used widely in the second half of the 19thcentury to temporarily mobilize the maxilla for bet-ter access to tumors and polyps. Not until the 1960s,however, did mobilization of the maxilla becomegenerally accepted, due in large measure to the pre-cise operative technique and superb results reportedby Obwegeser,373 who demonstrated that it was pos-sible to fully mobilize the maxillary segments andbring them into the desired position without soft-tissue resistance for primary stabilization.443

Obwegeser also described a higher maxillaryosteotomy (Le Fort I½) for use in patients with pro-nounced midfacial recession to advance the retrudedportion of the maxilla lateral to the piriform aper-ture. Other modifications of the Le Fort I osteotomyhave been described, including a self-stabilizingosteotomy suggested by Munro—a “self-retained LeFort I”. 444

Many authors have confirmed tooth viability sub-sequent to Le Fort I osteotomy, with reinnervation ofthe pulp over a period of several months.445,446

Fig 26. Above, one-stage medial orbital osteotomy with preser-vation of the cribriform plate. Below, subtotal orbital osteotomywith preservation of the cribriform plate. (Reprinted with permis-sion from Converse JM, Wood-Smith D: An atlas and classifica-tion of mid-facial and craniofacial osteotomies. In: Transac-tions of the Fifth International Congress of Plastic andReconstructive Surgery. Melbourne, Feb 1971, p 931.)


41

Le Fort II Osteotomy

The Le Fort II osteotomy was popularized byHenderson and Jackson447 in cleft patients with asso-ciated paranasal and nasal shortening. Several varia-tions have been described, including the tripartiteosteotomy of Converse.448

Le Fort III Osteotomy

The mainstay of total maxillary advancement isthe Le Fort III osteotomy.421 The procedure can becombined with other osteotomies, specifically a LeFort I, for definitive treatment of severe midfacialretrusion.288 Interpositional bone grafts and amaxillomandibular fixation appliance373–377 are use-ful adjuncts. If decreasing the interorbital distance orwidening of the palate is necessary, a facial biparti-tion is indicated (Fig 27).

A long-term study by Bachmayer et al449 of maxil-lary growth after Le Fort III osteotomy in syndromiccraniosynostosis showed minimal (mean 5%) verticalrelapse with rigid stabilization of the midface, butsevere anteroposterior growth retardation . Althoughthe average forward movement of the maxilla at thetime of surgery was 12.4mm, horizontal maxillarygrowth was minimal after surgery, and subsequentmaxillary advancement was invariably required atthe completion of growth.

In a retrospective study of patients who under-went Le Fort III advancement for craniofacial dysos-tosis, Kaban and colleagues450 note early skeletalrelapse in the sagittal plane in approximately 33% ofpatients. The magnitude of relapse was greater in

patients with Apert syndrome than in those withCrouzon disease. The small amount of relapse dur-ing the first year was followed by progressive down-ward and forward movement of the midface until, 2years after surgery, the midface was either anterioror inferior to the immediate postoperative positionin 15 of 19 patients. Three patients required anadditional Le Fort I osteotomy to correct Class IIImalocclusion.

A prospective analysis of 12 “growing” patients byMcCarthy and colleagues451 disclosed a remarkabledegree of postoperative skeletal stability. They fol-lowed this study with a clinical and cephalometriclongitudinal analysis of 12 children with craniofacialsynostosis syndromes who underwent Le Fort IIIadvancement at age <7y who were followed for amean of 5 years.452 Although vertical growth of themidface occurred postoperatively, there was mini-mal, if any, horizontal growth. The authors con-clude that a Le Fort III osteotomy is justified in earlychildhood for psychological and physiological rea-sons and can be performed at this early age withrelative safety and acceptable morbidity. Because ofthe deficient anterior maxillary growth and normalmandibular growth, most patients will requireorthognathic surgery when they reach adolescence.

COMPLICATIONS OF CRANIOFACIAL SURGERY

A survey of problems and complications ofcraniomaxillofacial surgery from six centers453 revealedan overall complication rate of 16.5%, including 4.4%infections and 1.6% mortality. Factors that reduce

Fig 27. Facial bipartition technique. A, nasoglabellar resection and splitting of palate. B, the orbitofrontal complex is bent backward tofurther reduce width of face. C, the orbits are approximated and the maxillae are wired between incisors to limit expansion. (Reprintedwith permission from Tessier P: Craniofacial Surgery in Syndromic Craniosynostosis. In: Cohen MM Jr, MacLean RE (eds),Craniosynostosis. Diagnosis, Evaluation, and Management, 2nd ed. New York, Oxford Univ Press, 2000; Ch 19, pp 228-268.)


42

morbidity and improve results include hypotensiveanesthesia, shorter operative time, rigid stabilizationof the mobilized bones at the end of the operation,fewer incisions, extensive antibiotic therapy, andgreater surgeon’s experience.

Munro and Sabatier454 analyzed the results ofcraniofacial surgery in 1092 patients representing2019 procedures. Complications of surgeryaccounted for 0.18% of deaths. Major complica-tions occurred in 14.3% of patients but not all hadpermanent sequelae. Infection was the greatestproblem, occurring in 5.3%. As the surgeons’experience increased, the complication ratedecreased.

David455 reviewed his 10-year experience withcraniofacial surgery in Australia. The overall infec-tion rate in 170 patients was 6.5%, with a markedage differential: 23.5% in adults and 2.2% in chil-dren. The highest infection rates were seen inpatients who required tracheostomy and had longeroperative times, and the most common pathogenwas Pseudomonas. His recommendations to pre-vent infectious complications include operativeintervention at an early age, short preoperative hos-pital stay, antibiotic prophylaxis to include gram-negative cover, isolating the dead space, and stricttracheostomy care.

Poole456 analyzed the postoperative course of 200consecutive patients treated at the Oxford Craniofa-cial Unit. Their overall complication rate was 22%,including 1% mortality and 1% infections. In thisseries as well as in those mentioned above, the fre-quency of infection was greater in adults than inchildren and intracranial procedures carried a higherrisk than extracranial procedures.

Whitaker and colleagues415 retrospectively ana-lyzed the records of 164 patients with craniosynos-tosis operated on over a 12-year period. Theauthors state that excellent results can be expectedin the asymmetrical deformities treated in infancyby a unilateral approach. Similarly, early surgery inthe form of bilateral orbital advancement gives sat-isfactory results in most patients with mild symmetri-cal deformities. In contrast, cases of severe bilateraldeformity or Apert syndrome often required a sec-ond, frequently extensive, operation. There wereno deaths, blindness, or brain dysfunctions as aresult of surgery.

DISTRACTION OSTEOGENESIS

History

Distraction osteogenesis (DO) is the process bywhich new bone is generated in the gap betweentwo bone segments in response to the application ofgraduated tensile stress across the gap. DO was firstdescribed by Codivilla in 1905.457 Beginning in theearly 1950s, Ilizarov458 introduced the concept ofdistraction osteogenesis for acquired and congenitaldeformities of enchondral bone.459 In 1973 Snyder460

reported distraction of the mandible in dogs, and 20years later McCarthy et al57 described lengthening ofthe mandible in 4 patients with hemifacial microso-mia by application of DO.

In 1998 Molina and Ortiz-Monasterio publishedtheir series of mandibular distraction in 274 patients,58

and the following year McCarthy reviewed his 10-year experience with distraction of the mandible.461

Habal462 reported midfacial distraction using anexternal distractor in 1995. That same year Polley463

described monobloc craniomaxillofacial distraction.Chin and Toth464 developed an internal device formidface advancement at the Le Fort III level in 1997.

Biology of Distraction

The process of distraction allows for the genera-tion of bone—osteogenesis—as well as soft-tissue—histogenesis. In a gap between two bone segmentsinitially a hematoma forms, which then develops intoa soft callus. The application of tensile stress pre-vents progression to hard callus and subsequent frac-ture union. Instead, intramembranous bone forma-tion occurs within the ever increasing gap. Withinthe bony gap there are three zones. The fibrousinterzone intervenes between the two primary min-eralizing fronts adjacent to the osteotomy. At cessa-tion of distraction the mineralizing fronts meet andunite to create union during the consolidation phase.The resultant bony union is dependent on the latency,rate, rhythm, and consolidation period of the dis-traction process.

Latency. Latency is the time period afterosteotomy/corticotomy and before commencing dis-traction. In an ovine model Tavakoli et al465 foundno difference in mechanical strength or bone densityof the callus when the latency period varied between


43

0 and 7 days. This would suggest that the traditionalpractice of allowing a nontreatment interval may beunnecessary and may only prolong treatment time.Cedars466 uses no latency period during Le Fort IIIdistraction.

Rate. Rate is the number of millimeters the bonegap is widened each day. Farhadieh andGianoutsos467 used a sheep model comparing dis-traction rates of 1mm, 2mm, 3mm, and 4mm perday. They then measured biomechanical proper-ties, mineralization, and histology of the regener-ate. They found a distraction rate of 1mm per daywas superior to all others. A rate of 1mm/d alsogave the most uniform bone formation radiologi-cally and histologically in a porcine model studiedby Troulis.468 When distracting at the Le Fort IIIlevel, Cedars et al466 uses a torque wrench to deter-mine the rate of distraction, believing this reflectsthe tolerance of the soft-tissue envelope and is moreappropriate than an arbitrary measurement.

Rhythm. Rhythm is the number of times daily thedistractor is activated. Rhythm varies from once perday to 4 times per day.

Consolidation. A long consolidation period willlead to weakening of the regenerate as a result ofdisuse atrophy,469 whereas too short a consolidationphase will lead to fibrous nonunion, buckling or frac-ture of the regenerate.470 Rachmiel et al471 showed24% mineralization after 3 weeks of consolidationand 77.8% by the end of 6 weeks.

To date little clinical research has been done intothe appropriate length of the consolidation period.Smith et al472 used computed tomography to suggest6 weeks was the minimum amount of time theregenerate should be allowed to mineralize prior todevice removal. Felemovicius473 used bone scintigra-phy with technetium-99 diphosphonate to assess theduration of the consolidation period. They foundconsolidation underway at 5 weeks in infants under12 months old, while in older children it was ongo-ing at 10 weeks and in adolescents and adults, at 10-14 weeks.

Distraction Treatment Planning

Compared with the relatively simple distraction oflong bones, distraction of the mandible in the hypo-

plastic deformity is decidedly a challenge. A treat-ment plan is essential to design the 3D vectors neces-sary to correct mandibular deficiency. Molina474 statesthat the most critical factor in mandibular DO isproper planning of the distraction vector on thecephalogram and its replication in the operating room.The hypoplastic ramus must be elongated, bringingthe affected side of the jaw downward, forward, andtoward the midline.

Tharanon and Sinn475 describe 3D treatmentplanning of mandibular hypoplasia using a pan-oramic, postero-anterior (PA), and lateralcephalogram. From the panoramic cephalogram,the site for the osteotomy is chosen to avoid dam-age to the inferior alvelolar nerve and the toothbuds. The necessary vertical lengthening is deter-mined from the PA cephalogram, to bring theangles of the mandible onto an equal plane. Fromthe lateral cephalogram, the required horizontallengthening is determined based on correction ofthe central incisors and chin to the desired posi-tion (Fig 28). The authors advocate this methodon the basis that operative time is saved, thepatient’s family can be informed as to the dura-tion of the distraction phase, and the technique issimple and inexpensive. Orientation of the vectorof distraction parallel to the sagittal plane avoidslateral displacement and bending forces on thebone.472

Gateno et al476 described a technique of 3D mod-eling and animation they employed in 7 patients tosimulate distraction osteogenesis in virtual reality. Thetechnique requires familiarity with advanced anima-tion software, is time consuming, and a surgical tem-plate must be constructed and used intraoperativelyto ensure correct pin placement.477

Distraction Devices

The initial devices developed for distractionosteogenesis were external and unidirectional. Morerecent devices operate in multiple planes and mul-tiple vectors, from Pensler’s478 ball and socket jointto McCarthy’s479 multiplanar distractor that worksin the sagittal, vertical, and coronal planes simulta-neously (Fig 29). McCarthy479 emphasizes the fol-lowing technical points in the application ofmultiplanar distraction:


44

• linear distraction continues at all times to avoidpremature consolidation

• linear distraction is begun first to generateadequate bony regenerate

• both limbs of the device are available for lineardistraction and can be varied appropriately

• linear distraction is by far the most important andoften the only vector required

The development of intraoral devices was spurredby a desire to minimize external scarring. The intraoral

devices can be tooth/bone borne480 and locatedintraorally (Guerrero481 reports more than 200 dis-tractions using this type of device), or bone borneand located submucosally or buried.482

Rachmiel et al483 compared the results of inter-nal and external devices in mandibular DO. In apoulation of 22 patients with hemifacial microso-mia, 12 had DO by an external device and 10 byan intraoral device. The external device allowedgreater elongation and better extraoral vector con-trol, and conserved the gonial angle. The maindisadvantages of the external device were the

Fig 28. Mandibular distraction with an extraoral device in hemifacial microsomia. A, location of the osteotomy. B, C, pin positions. D,amount of vertical mandibular distraction on the affected side. E, overbite relationship between the maxillary and mandibular centralincisors. F, center of rotation. The distal segment is moved horizontally until the overjet and chin are in the best position. The horizontalmovement determines the actual distance of AP distraction. (Reprinted with permission from Tharanon W, Sinn DP: Mandibulardistraction osteogenesis with multidirectional extraoral distraction device in hemifacial microsomia patients: three-dimensionaltreatment planning, prediction tracings, and case outcomes. J Craniofac Surg 10:202, 1999.)

Fig 29. Multiplanar mandibular distraction device to achieve simultaneous yet independent linear distraction (sagittal plane), angulardistraction (vertical plane), and transverse distraction (coronal plane). (Reprinted with permission from McCarthy JG, Williams JK,Grayson BH, Crombie JS: Controlled multiplanar distraction of the mandible: device development and clinical application. J CraniofacSurg 9:322, 1998.)


45

inconvenience to the patient and the cutaneous scars.The intraoral device was more socially acceptableand left no visible scars, but there was loss of vectorcontrol and a second procedure was required toremove the device.

Polley484 reported on an external halo-mounteddevice for midfacial monobloc DO in a newborn.Chin and Toth464 and Cohen486 developed internaldevices for midface advancement (Fig 30), citingthe advantage of patient convenience;Cohen’s486,487 device was resorbable to eliminatethe need for a removal procedure. Motor-drivendistraction devices have also been reported.488

External devices are most commonly used in themidface.489

Craniofacial Distraction

Mandible

McCarthy’s protocol for mandibular distraction isbased on patient age and type of mandibular defor-mity, as follows:461

Under 2 years – DO of the mandible is not indicatedAge 2-6 years –

Pruzansky Type I – orthognathic surgery includingDO are deferred until adolescence490,491

Pruzansky Type I (severe) and Type II –DO is indicated

Pruzansky Type III – Stage I: costochondral ribgraft at age 3-4 yrs; if absent, the glenoid isreconstructed with a rib graft and fixed tothe zygoma. Stage II: DO of the rib graft at6 months postinsertion of costochondral ribgraft

Age 6-teenager – primary DO is considered if noprevious treatment. Secondary DO isconsidered in patients with significant deformityafter rib grafting.

Teenager – surgery is postponed until skeletal matu-rity is reached.

Post skeletal maturation – minimal deformities arebest treated with classic orthognathic surgery.492

Mandibular distraction should be considered in patients with moderate to severe deformity andbilateral disease.493

McCormick and colleagues494,495 investigated theeffect of osteodistraction on the temporomandibularjoint (TMJ), first in a canine model and then in humansubjects. Compression on the cartilaginous portionof the condylar head has been shown to be detri-mental to subsequent TMJ morphology and func-tion,496–498 yet in this series initial condylar flatteningwas transient and completely reversible. In fact, bonedistraction appears to improve the temporomandibu-lar joint.499 When properly applied, the distractionforces pull the mandible in a downward and for-ward direction, leading to new bone deposition alongthe posterior aspect of the mandible and resorptionalong the anterior ramus region.500 The overall con-clusion of these studies was that distraction was ben-eficial to the TMJ complex.

Speech before and after mandibular DO wasassessed by Guyette et al,501 who report worsearticulation and nasal resonance post-distraction.These changes were temporary, however, and in

Fig 30. Patient with repaired cleft lip and severe midfacialhypoplasia. A, B, before distraction. Middle, patient fitted withrigid external distraction apparatus for correction of midfacialdeficiency. C, D, after maxillary distraction. (Reprinted withpermission from Polley JW, Figueroa AA, Kidd M: Priciples ofDistraction Osteogenesis in Craniofacial Surgery. In: Lin KY,Ogle RC, Jane JA (eds), Craniofacial Surgery. Science andSurgical Technique. Philadelphia, WB Saunders, 2002; Ch 11,pp 163-171.)


46

time all patients returned to their pre-distractionspeech levels.

The actual vector of distraction achieved may varyfrom that planned. This may be due to technicalerror or to the deforming forces of the soft-tissueenvelope. Maloclusion, an anterior open bite, or anunaesthetic result can be adjusted by manipulationof the callus prior to mineralization. Hoffmeister etal502 introduced the “floating bone” concept of elas-tics to reshape the regenerate and improve occlu-sion. Kunz503 uses elastics during and after distrac-tion to correct minor deviations of the distractionvector, a technique he calls “a lifeboat.” Pensler478

employs direct manual reshaping of the callus toachieve the same end. Early removal of the device(at 3 weeks) and orthodontic elastics can close anopen lateral bite when an undesirable vector isobtained.483

In the neonatal population, mandibular distrac-tion has been used to help manage the airway.Denny116 reports a series of 10 patients with man-dibular hypoplasia who were successfully treated withDO. After distraction was completed, 2 of 3 patientswho had tracheostomies were successfully decan-nulated. Denny504 later demonstrated that mandibu-lar advancement via distraction osteogenesis couldalso be used to avert tracheostomy in neonates withcraniofacial anomalies that impinge on the airway.

Le Fort I

Before the pubertal growth spurt, DO of the man-dible will allow descent of the maxilla orthodonticallyto a more normal position. After the pubertal growthspurt, combined DO of the maxilla and mandiblewill be necessary because of the cessation of maxil-lary growth. Molina and Ortiz Monasterio58,505

describe a technique involving Le Fort I corticotomyat the time of mandibular osteotomy and distractorplacement. After a 5-day latency, intermaxillary wir-ing of the jaws is performed and distraction begun.Consolidation extends for 8 weeks.

Molina and Ortiz Monasterio506 also report theirexperience with distraction of the maxilla in isolationin 36 patients, 18 with unilateral cleft lip and palate,12 with bilateral cleft lip and palate, 2 withnasomaxillary dysplasias, and 4 with mandibularprognathism. All patients were between the ages of3 and 11 years at the time of operation. The distrac-tion technique involved a vestibular incision, subpe-

riosteal dissection of the maxillae, and incompleteosteotomy above the canines and molar roots. Theosteotomy must respect the integrity of the maxillaryantral mucoperiosteum and should extend into themaxillary buttress. Pterygomaxillary dysjunction anddissection along the nasal floor and base of the sep-tum are not done. On the fifth postoperative day,the authors hook a maxillary orthopedic applianceto a facial mask with rubber bands and 950g ofmechanical force is applied to each side. Each weekthe maxilla is advanced about 3-4mm. Once half ofthe total projected maxillary advancement (8-12mm)and a Class II molar relationship have been obtained,the force is decreased to one rubber band on eachside to promote new bone formation at the osteotomysite and pterygomaxillary junction. The overallimprovement was reported as excellent.

Cohen485 applied the MIDS internal device for LeFort I advancement in 2 patients and reports 15mmand 17mm of advancement (Fig 31).

Ko et al507 examined the soft-tissue response toDO of the maxilla in 16 patients. They found thatthe concave facial profile became convex, theadvancement of soft-tissue to hard-tissue ratio at Apoint was 0.96:1, and the soft-tissue to hard-tissueratio for the nasal tip was 0.53:1. In a separatestudy, Ko et al508 reported on the effects of maxillaryDO on the speech of 22 patients. The averagemaxillary advancement was 8.9mm and resulted in14% worsening of hypernasality. The hypernasalitywas apparently related to the degree of advance-ment and unaffected by the presence of a pharyn-geal flap. Despite worsening of nasal air escape,57% of patients had improved articulation.

Le Fort III/Monobloc

Midface distraction was first performed in 1993.509

In 1995 Polley484 used an external distraction deviceto perform a monobloc distraction in a newborn,and in 1997 the same author described applicationsof the external device for midface distraction in child-hood and adolescence. In 1996 Chin and Toth464

reported on Le Fort III distraction with an internalburied device. Distraction advancement of up to25mm has been described.510

In younger patients who require frontoorbitaladvancement as well as advancement of the midface,monobloc DO is appropriate. In older patients whohave undergone previous frontorbital advancement


47

DO at the Le Fort III level, this may be all that isrequired. Both internal and external devices exist formidfacial DO. In younger patients the hypoplasticmalar complex may not withstand the forces of dis-traction511 and an external appliance attached to themaxillary dental arch may be more appropriate.

Cedars et al466 reported a series of 14 patientswho underwent Le Fort III DO with the internaldevice developed by Chin and Toth.464 Followingosteotomy and application of the device, the distrac-tion pins exit percutaneously through the cheek, anda torque wrench is used to begin distraction intraop-eratively. Distraction is continued immediately post-operatively using the torque wrench twice a day to aload of 14–18N-cm. DO is continued until theporion–orbitale distance is achieved as plannedcephalometrically. The average distraction was 18mm(range 12.5–27mm; 11mm achieved intraopera-tively). Cephalometric follow-up for more than 1year in 6 patients showed excellent stability of theadvancement at the occlusal level, with some relapseat the level of the orbitale. All patients had improve-ment in symptoms of sleep apnea. One of 2 patients

with tracheostomy was decannulated. The authorsnow concentrate the distraction process on achiev-ing superior maxillary position, reserving heightincrease for Le Fort I osteotomy to be performedsubsequently.

Fearon512 compares the results of Le Fort IIIadvancement in a pediatric series of 22 patients. Tenpatients underwent standard Le Fort III advance-ment and 12 underwent advancement by DO (2internal buried device, 10 external halo device). Inthe standard Le Fort III group, the average advance-ment was 6mm; in the distracted group, it was 19mm.Complications were evenly distributed between thetwo groups. The conclusion was that the advance-ment achieved with distraction is superior to that ofstandard Le Fort III. A halo distractor was preferredover the internal device because of better vectorcontrol. Focusing the vector on the facial midlineallowed better correction of the concave facial pro-file to a convex profile.

Cohen’s485 series of 11 patients treated with theModular Internal Distraction System (MIDS)(Howmedica-Leibinger) included 4 Le Fort III cases

Fig 31. Modular internal distraction system applied in left, monobloc osteotomy and right, Le Fort III osteotomy. (Reprinted withpermission from Cohen SR: Craniofacial distraction with a modular internal distraction system: evolution of design and surgicaltechniques. Plast Reconstr Surg 103:1592, 1999.)


48

and 3 monobloc advancements. Midface retrusionand exorbitism were corrected in all patients andthere were no infections in the monobloc group.Nadal513 reported similar results in 8 patients whohad monobloc advancement by distraction. Cohen514

also published a case report of a patient with facialbipartition treated with the MIDS.

To avoid extensive surgery for removal of thedevice, Cohen and Holmes487 used resorbableMacropore mesh as a substitute for the metallic fixa-tion plate and achieved 20mm of distraction at theLe Fort III level in a 4-year-old girl with Crouzonsyndrome.

Distraction has also been described in Le Fort IIadvancement,515 nasal bone lengthening,516

zygoma,487,517 alveolar clefts,518 and craniosynosto-sis.463,519,520

Reviews and Long-Term Follow-Up

Swennen and colleagues,489 in an attempt to quan-tify clinical indications and treatment protocols,reviewed the literature on DO from 1966 toDecember 1999. They found 285 articles, including109 clinical studies involving treatment of 828patients. This impressive review provides an insightinto the most widely employed indications and pro-tocols.

Mandibular distraction: Of 579 patients, 74.3%underwent mandibular lengthening. A latency periodof 5–7 days was most common, as were a distractionrate of 1mm/day, with rhythms between 1 and 4times per day and a 6–8 week consolidation period.Unidirectional devices were used in 80.2% of cases,bidirectional in 15.6%, and multidirectional in 4.2%.External devices were used in 72% of cases andinternal in 18%. Lengthening varied from 1–95mm.

Maxillary distraction: Of 129 patients, 122underwent maxillary advancement. An externaldevice was used in 95% of cases and an internaldevice in 5%. The average latency was 4–5 days, therate was 1mm/day, and consolidation of 2–3 monthswas most common. Distraction lengths were 1–17mm.

Simultaneous mandibular and maxillary distrac-tion: This group included 24 patients who under-went mandibular DO with simultaneous maxillary

DO though IMF, most often though unilateral man-dibular DO and an associated Le Fort I osteotomy.Mandibular distraction was accomplished with anexternal device in 87.5%, an internal device in theremaining 12.5%. Mandibular lengthening variedfrom 12–18mm.

Midface and cranium: Of 96 cases, 64.6% hadLe Fort III advancement and 35.4% had monoblocadvancement. In patients younger than 4 years,monobloc was the most common procedure.

The researchers’ conclusions were as follows:1. The issue of overcorrection remains controver-

sial.2. A rate of 1mm/day is average.3. The standard latency period of 5–7 days could

possibly be shortened, given the differencebetween the long bones and the membranousbones of the cranium and face.

4. The complication rate was 22%, though most ofthese were mechanical problems associated withthe device or minor pin site infections.

5. There is a lack of long term data, especiallyregarding relapses.

6. More objective data are necessary to refine pro-tocols.

7. The future of DO probably involves the develop-ment of miniaturized, multidirectional, andmotorized devices.

Mofid and colleagues521 reviewed 3278 cases ofcraniofacial DO by means of a 4-page survey sent to2476 craniofacial and oral/maxillofacial surgeonsworldwide. The response rate was 11.4%. Relapsewas recognized by 50.4% of respondents; of these,64.8% had mandibular relapse and 60.6% hadmidface relapse. Relapse occurred within 6 monthsof completion of distraction in more than 66%. Incases of mandibular distraction, a higher use of inter-nal devices (24.3%) and a higher rate (33.9%) ofmultivector devices was seen than reported bySwennen.489 This most likely reflects advances indevice design and surgeon experience. As noted bySwennen, the average latency was 4.9 days, the com-monest rate was 1mm/day, and the average consoli-dation period was 7 weeks: 6 days in the mandibleand 8 weeks and 6 days in the midface. Average


49

lengthening was 16.8mm in the mandible and14.5mm in the midface.

Other complications of craniofacial distractionosteogenesis are tabulated in Table 8.521

AUGMENTATION AND CONTOURING PROCEDURES

There are many indications for autogenous bonegrafts in craniofacial surgery.522 Most craniofacialsurgeons believe that autogenous grafts of bone orcartilage are the materials of choice in craniofacialreconstruction because of their resistance to infec-tion and low morbidity compared with alloplasticimplants.523 For an in-depth review of the subject,the reader is referred to Selected Readings in PlasticSurgery volume 10, number 2.524

The calvarium is usually the preferred source ofdonor autogenous bone for grafting in the craniofa-cial skeleton.79,80,525,526 Cutting525 delineated the vas-cular supply to the cranium and introduced the con-cept of using vascularized calvarial bone in craniofa-

cial procedures. He determined that the mostimportant source of perfusion to the cranium is themiddle meningeal artery, which of course is not use-ful as a pedicle for bone transfer. Branches of theanterior and posterior deep temporal arteries, whichalso supply the temporalis muscle, constitute a lesservascular supply to the cranium. Finally there is thevascular plexus fed by the supraorbital, supratrochlear,superficial temporal, and occipital arteries. Thetemporoparietalis fascia (superficial temporal fascia)contains the superficial temporal artery and providesa clinically useful pedicle to support a vascularizedcalvarial bone graft.525,527

McCarthy and colleagues79,80 suggest leaving thegalea and overlying vascular network broadlyattached to the bone when transferring a vascular-ized calvarium bone flap because of the poor vascu-lar connections within the periosteum.

A review of vascularized calvarial inlay grafts basedon a temporalis myoosseous design528 notes preserva-tion of the growth potential and a patent suture.

TABLE 8Complication Rates

(Reprinted with permission from Mofid MM, Manson PN, Robertson BC, et al: Craniofacial distraction osteogenesis: a review of 3278cases. Plast Reconstr Surg 108:1103, 2001.)


50

Transsutural growth in the vascularized grafts continuesfor the duration of maxillary development despite ces-sation of skeletal growth at the calvarial donor site.

Byrd and Hobar529 described the use of poroushydroxyapatite (HA) granules in craniofacial aug-mentation procedures when structural support isnot the main consideration. The HA granules con-tain pores approximately 200µ in diameter whichallow fibrovascular ingrowth, so the implants arevascularized within a very few weeks. The HAgranules are mixed with blood and microfibrillarcollagen to make them easier to work with, andare then injected into a subperiosteal pocket wher-ever augmentation is desired. Onlays of hydroxya-patite do not resorb over time, are very seldomassociated with infection, and seem to provoke notissue reaction. The authors’ experience nowextends to more than 200 patients operated onover an 8-year period, and they remain enthusias-tic about the technique.

A powdered form of hydroxyapatite marketedunder the name of Bone Source (Leibinger Corp,Carrollton, Texas) has found application in craniofa-cial contour refinement. Burnstein and coworkers530

describe their experience with 61 patients, 56 ofthem children, treated with Bone Source for second-ary craniofacial reconstruction over a 3- year period.The authors note excellent contour and volumeretention and no interference with craniofacialgrowth.

Jackson531 remarks that the smaller pore size ofthe powdered HA compared with HA granules lendsitself to a higher rate of bony replacement. Theyachieved excellent results in 20 patients, yet cautionthat long-term follow-up is necessary to establish thesafety and reliability of its use.

SURGERY FOR RADIATION-INDUCED DEFORMITY

Jackson and colleagues532 studied 14 children whoreceived therapeutic radiation for malignant tumorsin the orbital area and who subsequently showedwidespread craniofacial deformities. The originaltumors were retinoblastoma, rhabdomyosarcoma,and embryonic cell carcinoma. Years later, malde-velopment of the skull, orbit, maxilla, and mandiblebecame apparent. The authors recommend correc-tion of four levels of skeletal deformity in a singleprocedure, as follows:

1. frontotemporal expansion with repositioning ofthe cranial base

2. orbital expansion and repositioning3. maxillary surgery with bone grafts to the zygoma

as required4. mandibular lengthening and repositioning

Bone grafts should be inlay rather than onlay, andsoft tissue should be supplied by free-tissue transfer.At a second operation, the eye socket and eyelidsare reconstructed to allow more satisfactory rehabili-tation with an ocular prosthesis. On the basis ofobservations made during extensive skull base andorbital dissections, Jackson and colleagues532 believethe radiation impaired growth of the sphenoid, whichin turn locked the upper face and prevented normaldevelopment of the facial skeleton. In addition, thefrontal, ethmoid, and maxillary sinuses failed toexpand, resulting in further decrease of craniofacialdimensions. Craniotomy is essential to position theskull base correctly in order to accurately seat andremodel the orbit and maxilla. For further readingon this subject, one should refer to the articles byNwoku,533 Larson,534 Marx,535 Guyuron,536,537 andKawamoto.538

Cohen, Bartlett, and Whitaker335 described theirexperience with reconstruction of late craniofacialdeformities after therapeutic radiation of the headand face during childhood. High-dose radiation is astandard form of treatment for some pediatric cran-iofacial tumors, including retinoblastoma, rhabdomyo-sarcoma, Ewing’s sarcoma, and neurofibrosarcoma.The patients subsequently develop soft-tissue andbony hypoplasia of the irradiated areas, which canbe partially corrected by onlay bone grafting andsoft-tissue reconstruction with local pedicled flaps anddermal-fat grafts in multiple stages.335 Forty percentof patients in their series required secondary proce-dures for improvement. The authors anticipateincreasing usage of free-tissue transfer in these cases.

Individuals who as children were irradiated forhemangiomas of the face are also presenting to plas-tic surgery clinics with severe craniofacial deformi-ties. At one time high-beam radiotherapy was advo-cated for capillary hemangioma of the cheek, andthe sequelae of this treatment in the growing child isnow painfully evident as terrible facial deformities inthe adult. Reconstruction should follow the prin-ciples outlined by Jackson and colleagues532 above.


51

1. Sperber GH: Craniofacial Embryology, 4th Ed. London,Wright, 1989.

2. Johnston MC: Embryology of the Head and Neck. In:McCarthy JG (ed), Plastic Surgery. Philadelphia, WBSaunders, 1990. Vol 4, Ch 46, pp 2451-2495.

3. Slavkin HC et al: Early embryonic mouse mandibularmorphogenesis and cytodifferentiation in serumless, chemi-cally defined medium: a model for studies of autocrineand/or paracrine regulatory factors. J Craniofac Genet DevBiol 9:185, 1989.

4. Slavkin HC: Developmental Craniofacial Biology. Phila-delphia, Lea & Febiger, 1979.

5. Cohen MM Jr, Rollnick BR, and Kaye CI: Oculo-auriculovertebral spectrum: An updated critique. CleftPalate J 26:276, 1989.

6. Prescott NJ, Malcolm S: Folate and the face: evaluatingthe evidence for the influence of folate genes on cranio-facial development. Cleft Palate Craniofac J 39:327,2002.

7. Kallen K: Maternal smoking and craniosynostosis. Teratol-ogy 60:146, 1999.

8. Honein MA, Rasmussen SA, Reefhuis J, et al: Maternalsmoking and environmental tobacco smoke exposureand the risk of orofacial clefts. Epidemiology 18:226,2007.

9. Gardner JS, Guyard-Boileau B, Alderman BW, et al: Ma-ternal exposure to prescription and non-prescription phar-maceuticals or drugs of abuse and risk of craniosynostosis.Int J Epidemiol 27:64, 1998.

10. Whitaker LA, Pashayan H, Reichman J: A proposed newclassification of craniofacial anomalies. Cleft Palate J18:161, 1981.

11. Tessier P: Anatomical classification of facial, craniofacialand latero-facial clefts. J Maxillofac Surg 4:69, 1976.

12. David DJ, Moore MH, Cooter RD: Tessier clefts revisitedwith a third dimension. Cleft Palate J 26:163, 1989.

13. Van der Meulen JC et al: A morphogenetic classification ofcraniofacial malformations. Plast Reconstr Surg 71:560,1983.

14. DeMyer W, Zeman W, Palmer CG: The face predicts thebrain: Diagnostic significance of median facial anomaliesfor holoprosencephaly (arhinencephaly). Pediatrics 34:256,1964.

15. Cohen MM Jr, Sulik KK: Perspectives on holoprosencephaly:Part II. Central nervous system, craniofacial anatomy,syndrome commentary, diagnostic approach, and experi-mental studies. J Craniofac Gen Dev Biol 12(4):196, 1992.

16. Elias DL, Kawamoto HK Jr, Wilson LF: Holoprosencephalyand midline facial anomalies: Redefining classification andmanagement. Plast Reconstr Surg 90:951, 1992.

17. Dursy E: Zur Entwicklungsgeschichte des Kopfes desMenschen und der hoeheren Wirbelthiere. Tubingen,Verlag der H Lauppschen- Buchhandlung, 1869, p 99.

18. His W: Die Entwickelung der menschlichen und t.ierischerPhysiognomen. Arch Anat Entwicklungsgesch, 1892, p384.

19. Kawamoto HK: The kaleidoscopic world of rare craniofa-cial clefts: order out of chaos (Tessier classification). ClinPlast Surg 3:529, 1976.

20. Pohlmann EH: Die embryonale Metamorphose derPhysiognomie und der Mundhoehle des Katzenkopfes.Morphol Jahrb Leipzig 41:617, 1910.

21. Veau V: Hasencharten menschlicher Keimlinge auf derStufe 21-23 mm SSL. Z Anat Entwicklungsgesch 108:459,1938.

22. Stark RB: The pathogenesis of harelip and cleft palate. PlastReconstr Surg 13:20, 1954.

23. Stark RB, Saunders DE: The first branchial syndrome: Theoral-mandibular-auricular syndrome. Plast Reconstr Surg29:299, 1962.

24. Pantaloni M and Byrd HS, Salomon J, and Hollier L: CleftLip I: Primary Deformities; Cleft Lip II: Secondary Defor-mities; and Cleft Palate and Velopharyngeal Incompe-tence. Selected Read Plast Surg 9(21-23), 2001.

25. Ozaki W, Kawamoto HK Jr: Craniofacial Clefting. In: LinKY, Ogle RC, Jane JA (eds), Craniofacial Surgery. Scienceand Surgical Technique. Philadelphia, Saunders, 2002,Ch 21.

26. Van der Meulen JCH: Oblique facial clefts: Pathology,etiology, and reconstruction. Plast Reconstr Surg 76:212,1985.

27. Resnick JI, Kawamoto HK Jr: Rare craniofacial clefts:Tessier No. 4 clefts. Plast Reconstr Surg 85:843, 1990.

28. Galante G, Dado DV: The Tessier number 5 cleft: A reportof two cases and a review of the literature. Plast ReconstrSurg 88:131, 1991.

29. Fuente del Campo A: Surgical correction of Tessier num-ber 8 cleft. Plast Reconstr Surg 86:658, 1990.

30. Menard RM, Moore MH, David DJ: Tissue expansion inthe reconstruction of Tessier craniofacial clefts: a series of17 patients. Plast Reconstr Surg 103:779, 1999.

31. Thomson A: As cited in Grabb WC: The first and secondbranchial arch syndrome. Plast Reconstr Surg 36:485,1965.

32. Posnick JC: Hemifacial microsomia: evaluation andstaging of reconstruction. J Oral Maxillofac Surg 56:642,1998.

33. Posnick JC: Craniofacial and Maxillofacial Surgery inChildren and Young Adults. Philadelphia, WB Saunders,2000.

34. Bassila M, Goldberg R: The association of facial palsy andsensorineural hearing loss in patients with hemifacial mi-crosomia. Cleft Palate J 26:287, 1989.

35. Poswillo DE: The pathogenesis of the first and secondbranchial arch syndrome. Oral Surg 35:302, 1973.

36. Converse JM et al: Bilateral facial microsomia: Diagnosis,classification, and treatment. Plast Reconstr Surg 59:653,1977.

37. McKenzie J, Craig J: Mandibulofacial dysostosis. Arch DisChild 30:391, 1952.

38. Johnston MC, Sulik KK: Embryology of the head and neck.In: Serafin D and Georgiade NG (eds), Pediatric PlasticSurgery. St Louis, CV Mosby, 1984. Vol 1, Ch 13, pp 184-215.

39. Murray JE, Kaban LB, Mulliken JB, Evans CA: Analysis andtreatment of hemifacial microsomia. In: Caronni EP (ed),Craniofacial Surgery. Boston, Little Brown, 1982. Ch 33,pp 377- 390.

BIBLIOGRAPHY


52

40. Polley JW, Figueroa AA, LIOU EJW, Cohen M: Longitudi-nal analysis of mandibular asymmetry in hemifacial mi-crosomia. Plast Reconstr Surg 99:328, 1997.

41. Kearns GJ, Padwa BL, Mulliken JB, Kaban LB: Progressionof facial asymmetry in hemifacial microsomia. Plast ReconstrSurg 105:492, 2000.

42. Munro IR: Treatment of craniofacial microsomia. ClinPlast Surg 14:177, 1987.

43. Murray JE, Kaban LB, Mulliken JB: Analysis and treatmentof hemifacial microsomia. Plast Reconstr Surg 74:186,1984.

44. Munro IR: One-stage reconstruction of the temporoman-dibular joint in hemifacial microsomia. Plast Reconstr Surg66:699, 1980.

45. Pruzansky S: Not all dwarfed mandibles are alike. BirthDefects 5:120, 1969.

46. Meurman Y: Congenital microtia and meatal atresia. ArchOtolaryngol 66:443, 1957.

47. Mulliken JB, Kaban LB: Analysis and treatment of hemifa-cial microsomia in childhood. Clin Plast Surg 14:91, 1987.

48. Bennun RD et al: Microtia: A microform of hemifacialmicrosomia. Plast Reconstr Surg 76:859, 1985.

49. David DJ, Mahatumarat C, Cooter RD: Hemifacial mi-crosomia: a multisystem classification. Plast Reconstr Surg80:525, 1987.

50. Posnick JC: Surgical correction of mandibular hypoplasiain hemifacial microsomia: a personal perspective. J OralMaxillofac Surg 56:639, 1998.

51. Converse JM et al: The corrective treatment of the skeletalasymmetry in hemifacial microsomia. Plast Reconstr Surg52:221, 1973.

52. Ortiz-Monasterio F: Early mandibular and maxillary os-teotomies for the correction of hemifacial microsomia: Apreliminary report. Clin Plast Surg 9:509, 1982.

53. Munro IR, Lauritzen CGK: Classification and treatment ofhemifacial microsomia. In: Caronni EP (ed), CraniofacialSurgery. Boston, Little Brown, 1984. Ch 34, pp 391-400.

54. Ortiz-Monasterio F, Fuente del Campo A: Early skeletalcorrection of hemifacial microsomia. In: Caronni EP (ed),Craniofacial Surgery. Boston, Little Brown, 1984. Ch 35,pp 401- 410.

55. Moss ML: The functional matrix. In: Kraus BS, Reidel RA(eds), Vistas in Orthodontics. Philadelphia, Lea & Febiger,1962.

56. Moss ML et al: The passive role of nasal septal cartilage inmid-facial growth. Plast Reconstr Surg 41:536, 1968.

57. McCarthy JG et al: Lengthening the human mandible bygradual distraction. Plast Reconstr Surg 89:1, 1992.

58. Molina F, Ortiz Monasterio F: Mandibular elongation andremodeling by distraction: a farewell for major osteoto-mies. Plast Reconstr Surg 96:825, 1995.

59. McCarthy JG et al: Craniofacial Microsomia. In: McCarthyJG (ed), Plastic Surgery. Philadelphia, WB Saunders,1990. Vol 4, Ch 62, pp 3054-3100.

60. Ross RB: Costochondral grafts replacing the mandibularcondyle. Cleft Palate Craniofac J 36:334, 1999.

61. La Rossa D, Whitaker L, Dabb R, Mellissinos E: The use ofmicrovascular free flaps for soft tissue augmentation of theface in children with hemifacial microsomia. Cleft PalateJ 17:138, 1980.

62. Upton J et al: Restoration of facial contour using freevascularized omental transfer. Plast Reconstr Surg 66:560,1980.

63. Janis JE, Leedy JE: Lip, Cheek and Scalp Reconstruction.Selected Read Plast Surg 10(13), 2006.

64. Rogers BO: Berry-Treacher Collins syndrome: A reviewof 200 cases. Br J Plast Surg 17:109, 1964.

65. Munro IR et al: Craniofacial Syndromes. In: McCarthy JG(ed), Plastic Surgery. Philadelphia, WB Saunders, 1990.Vol 4, Ch 63, pp 3101-3123.

66. McCarthy JG, Zide BM: Rare craniofacial clefts. In: SerafinD and Georgiade NG (eds), Pediatric Plastic Surgery. StLouis, CV Mosby, 1984. Vol 1, Ch 25, p 390.

67. Tessier P: Surgical correction of Treacher Collins syn-drome. In: Bell WH (ed), Modern Practice in Orthognathicand Reconstructive Surgery. Philadelphia, Saunders,1992. Vol 2, p 1601.

68. Dixon M, Read A, Donnai D, et al: The gene for TreacherCollins syndrome maps to the long arm of chromosome 5.Am J Hum Genet 49:17, 1991.

69. Jabs E, Li X, Coss C, et al: Mapping the Treacher Collinssyndrome locus to 5q31.3®q33.3 Genomics 11:193,1991.

70. Marsh KL, Dixon J, Dixon MJ: Mutations in the TreacherCollins syndrome gene lead to mislocalization of the nucle-olar protein treacle. Hum Mol Genet 7:1795, 1998.

71. Marszalek B, Wojcicki P, Kobus K, Trzeciak WH: Clinicalfeatures, treatment and genetic background of TreacherCollins syndrome. J Appl Genet 43:223, 2002.

72. Sulik KK, JOhnston MC, Smiley SJ, et al: Mandibulofacialdysostosis (Treacher Collins syndrome): a new proposal forits pathogenesis. Am J Med Genet 27:245, 1987.

73. Shprintzen RJ et al: Pharyngeal hypoplasia in TreacherCollins syndrome. Arch Otolaryngol 105:127, 1979.

74. Behrents RG, McNamara JA, Avery JK: Prenatalmandibulofacial dysostosis (Treacher Collins syndrome).Cleft Palate J 14:13, 1977.

75. Jackson IT: Reconstruction of the lower eyelid defect inTreacher Collins syndrome. Plast Reconstr Surg 67:365,1981.

76. Raulo Y: Treacher Collins syndrome: Analysis and prin-ciples of surgery. In: Caronni EP (ed), CraniofacialSurgery. Boston, Little Brown, 1984. Ch 32, pp 371-376.

77. Posnick JC: Treacher Collins Syndrome. In: Aston S (ed),Grabb and Smith’s Plastic Surgery, 5th ed. Philadelphia,Lippincott Raven, 1997.

78. Posnick JC, Goldstein JA, Waitzman AA: Surgical correc-tion of the Treacher Collins malar deficiency: QuantitativeCT scan analysis of long-term results. Plast Reconstr Surg92:12, 1993.

79. McCarthy JG, Zide BM: The spectrum of calvarial bonegrafting: Introduction of the vascularized calvarial boneflap. Plast Reconstr Surg 74:10, 1984.

80. McCarthy JG, Cutting CB, Shaw WW: Vascularized calva-rial flaps. Clin Plast Surg 14:37, 1987.

81. Freihofer HPM: Variations in the correction of TreacherCollins syndrome. Plast Reconstr Surg 99:647, 1997.

82. Van der Meulen JCH et al: The use of a temporalosteoperiosteal flap for the reconstruction of malar hypo-plasia in Treacher Collins syndrome. Plast Reconstr Surg74:687, 1984.

83. Psillakis JM et al: Vascularized outer-table calvarial boneflaps. Plast Reconstr Surg 78:309, 1986.

84. Brent B: Auricular repair with autogenous rib cartilagegrafts: two decades of experience with 600 cases. PlastReconstr Surg 90:355, 1992.


53

85. Dufresne C: Treacher Collins Syndrome. In: Dufresne C(ed), Complex Craniofacial Problems: A Guide toAnalysis and Treatment. New York, Churchill Livingstone,1992; p 281.

86. Munro IR et al: Craniofacial Syndromes. In: McCarthy JG(ed), Plastic Surgery. Philadelphia, WB Saunders, 1990.Vol 4, Ch 63, pp 3101-3160.

87. Jackson I et al: A significant feature of Nager’s syndrome:Palatal agenesis. Plast Reconstr Surg 84:219, 1989.

88. Zuckerkandl E: Fossae praenasales. Normale undpathologische. Anat Nasenhohle 1(2):48, 1882.

89. Noyes FB: Case report. Angle Orthod 9:160, 1939.90. Von Binder KH: Dysostosis maxillo-nasalis, ein

arhinencephaler Missbildungskomplex. DeutscheZahnaerztl Z 17:438, 1962.

91. Munro IR, Sinclair WJ, Rudd NL: Maxillonasal dysplasia(Binder’s syndrome). Plast Reconstr Surg 63:657, 1979.

92. Posnick JC, Tompson B: Binder syndrome: staging ofreconstruction and skeletal stability and relapse patternsafter Le Fort I osteotomy using miniplate fixation. PlastReconstr Surg 99:961, 1997.

93. Holmstrom H: Clinical and pathologic features ofmaxillonasal dysplasia (Binder’s syndrome): significance ofthe prenasal fossa on etiology. Plast Reconstr Surg 78:559,1986.

94. Rival JM, Gherga-Negrea A, Mainard R, Delaire J: Dysostosemaxillo-nasale de Binder. J Genet Hum 22:263, 1974.

95. Olow-Nordenram M, Valentin J: An etiologic study ofmaxillonasal dysplasia: Binder’s syndrome. Scand J DentRes 96:69, 1988.

96. Holmstrom H: Surgical correction of the nose and midfacein maxillonasal dysplasia (Binder’s syndrome). Plast ReconstrSurg 78:568, 1986.

97. Jackson IT, Moos KF, Sharpe DT: Total surgical manage-ment of Binder’s syndrome. Ann Plast Surg 7:25, 1981.

98. Banks P, Tanner B: The mask rhinoplasty: a technique forthe treatment of Binder’s syndrome and related disorders.Plast Reconstr Surg 92:1038, 1993.

99. Converse JM: Restoration of facial contour by bone graftsintroduced through the oral cavity. Plast Reconstr Surg6:295, 1950.

100. Converse JM, Horowitz SL, Valauri AJ, et al: The treat-ment of nasomaxillary hypoplasia: a new pyramidal naso-orbital maxillary osteotomy. Plast Reconstr Surg 45:527,1970.

101. Ragnell A: Nasomaxillary retroposition in children. Suc-cessive reconstruction of the nose: a preliminary report.Nord Med 77:847, 1967.

102. Henderson D, Jackson IT: Naso-maxillary hypoplasia: theLe Fort II osteotomy. Br J Oral Surg 11:77, 1973.

103. Steinhauser EW: Variations of Le Fort II osteotomies forcorrection of midfacial deformities. J Maxillofac Surg8:258, 1980.

104. Tessier P, Tulasne JF, Delaire J, et al: Therapeutic aspectsof maxillonasal dysostosis (Binder syndrome). Head NeckSurg 3:207, 1981.

105. Holmstrom H, Kahnberg KE: Surgical approach in severecases of maxillonasal dysplasia (Binder’s syndrome). SwedDent J 12:3, 1988.

106. Rune B, Aberg M: Bone grafts to the nose in Binder’ssyndrome (maxillonasal dysplasia): a follow-up of elevenpatients with the use of profile roentgenograms. PlastReconstr Surg 101:297, 1998.

107. Wolfe SA: Lengthening the nose: a lesson from craniofa-cial surgery applied to posttraumatic and congenital defor-mities. Plast Reconstr Surg 94:78, 1994.

108. McCollum AGH, Wolford LM: Binder syndrome: litera-ture review and long-term follow-up on two cases. Int JAdult Orthod Orthognath Surg 13:45, 1998.

109. Robin P: Backward fall of the root of the tongue as causeof respiratory disturbances. Bull Acad Med (Paris) 89:37,1923.

110. Fletcher MM, Blum SL, Blanchard CL: Pierre Robinsyndrome: pathophysiology of obstructive episodes. Laryn-goscope 79:547, 1969.

111. Kiskadden WS, Dietrich SR: Review of the treatment ofmicrognathia. Plast Reconstr Surg 12:365, 1953.

112. Douglas B: The treatment of macrognathia associated withobstruction by a plastic procedure. Plast Reconstr Surg1:300, 1946.

113. Randall P: The Robin sequence: Micrognathia andglossoptosis with airway obstruction. In: McCarthy JG (ed),Plastic Surgery. Philadelphia, WB Saunders, 1990. Vol 4,Ch 63, pp 3123-3134.

114. Pashayan HM, Lewis MB: Clinical experience with theRobin sequence. Cleft Palate J 21:270, 1984.

115. Routledge RT: The Pierre Robin syndrome: A surgicalemergency in the neonatal period. Br J Plast Surg 13:204,1960.

116. Denny AD, Talisman R, Hanson PR, Recinos RF: Mandibu-lar distraction osteogenesis in very young patients to cor-rect airway obstruction. Plast Reconstr Surg 108:302,2001.

117. Jackson IT, Tanner NSB, Hide TAH: Frontonasal encepha-locele—“long nose hypertelorism”. Ann Plast Surg 11:490,1983.

118. Simpson DA, David DJ, White J: Cephaloceles: treatment,outcome, and antenatal diagnosis. Neurosurgery 15:14,1984.

119. Suwanwela C, Suwanwela N: A morphological classifica-tion of scincipital encephalomeningoceles. J Neurosurg36:201, 1972.

120. Mazzola RF: Congenital malformations in the frontonasalarea: Their pathogenesis and classification. Clin Plast Surg3(4):573, 1976.

121. David DJ et al: Fronto-ethmoidal meningoencephaloceles:morphology and treatment. Br J Plast Surg 37:271, 1984.

122. Jacob OJ, Rosenfeld JV, Watters DAK. The repair of frontalencephaloceles in Papua New Guinea. Aust N Z J Surg64:856, 1994.

123. Cohen MM: Craniosynostosis and syndromes with cranio-synostosis: incidence, genetics, penetrance, variability,and new syndrome updating. Birth Defects 15 (5B):13,1979.

124. Humphreys RP: Encephalocele and dermal sinuses. In:Cheek WR, Marlin AE, McLone DG, et al (eds), PediatricNeurosurgery: Surgery of the Developing NervousSystem, 3rd ed. Philadelphia, WB Saunders, 1994, p 96.

125. Charoonsmith T, Suwanwela C: Frontoethmoidalencephalomeningocele with special reference to plasticreconstruction. Clin Plast Surg 1:27, 1974.

126. Pollack IF: Management of encephaloceles and craniofa-cial problems in the neonatal period. Neurosurg ClinNorth Am 9:121, 1998.

127. David DJ: Cephaloceles: Classification, pathology, andmanagement. A review. J Craniofac Surg 4:192, 1993.


54

128. Forcada M, Montandon D, Rilliet B: Frontoethmoidalcephaloceles: Transcranial and transfacial surgical treat-ment. J Craniofac Surg 4:203, 1993.

129. Smit CSF et al: Frontoethmoidal meningoencephaloceles:A review of 14 consecutive patients. J Craniofac Surg4:210, 1993.

130. Holmes AD, Meara JG, Kolker AR, et al: Frontoethmoidalencephaloceles: reconstruction and refinements. JCraniofac Surg 12:6, 2001.

131. Persing JA, Jane JA, Shaffrey M: Virchow and the pathogen-esis of craniosynostosis: A translation of his original work.Plast Reconstr Surg 83:738, 1989.

132. Alden TD, Lin KY, Jane JA: Mechanisms of prematureclosure of cranial sutures. Child Nerv Syst 15:670, 1999.

133. Robin NH: Molecular genetic advances in understandingcraniosynostosis. Plast Reconstr Surg 103:1060, 1999.

134. Shillito J, Matson DD: Craniosynostosis: A review of 519surgical patients. Pediatrics 41:829, 1968.

135. Shuper A et al: The incidence of isolated craniosynostosisin the newborn infant. Am J Dis Child 139:85, 1985.

136. Marchac D, Renier D: Craniofacial Surgery for Cranio-synostosis. Boston, Little Brown, 1982.

137. Oakes WJ: Craniosynostosis. In: Serafin D, Georgiade NG(eds), Pediatric Plastic Surgery. St Louis, CV Mosby,1984. Vol 1, Ch 26, pp 404-439.

138. Cohen MM Jr, MacLean RE: Anatomic, Genetic, Noso-logic, Diagnostic, and Psychosocial Considerations. In:Cohen MM Jr, MacLean RE (eds), Craniosynostosis. Diag-nosis, Evaluation, and Management, 2nd Ed. New York,Oxford Univ Press, 2000, Ch 11, p 119.

139. Huang MHS, Gruss JS, Clarren SK, et al: The differentialdiagnosis of posterior plagiocephaly: true lambdoid synos-tosis versus positional molding. Plast Reconstr Surg 98:765,1996.

140. American Academy of Pediatrics Task Force on InfantPositioning and SIDS: Positioning and SIDS. Pediatrics89:1120, 1989.

141. Losee JE, Mason AC: Deformational plagiocephaly: diag-nosis, prevention, and treatment. Clin Plast Surg 32:53,2005.

142. Vies JS, Colla C, Weber JW, et al: Helmet versus nonhelmettreatment in non-synostotic positional posterior plagio-cephaly. J Craniofac Surg 11:572, 2000.

143. Latham RA: Maxillary development and growth: Theseptopremaxillary ligament. J Anat 107:471, 1970.

144. Latham RA: Growth and development of the craniofacialskeleton. In: Serafin D and Georgiade NG (eds), PediatricPlastic Surgery. St Louis, CV Mosby, 1984. Vol 1, Ch 14,pp 216-229.

145. Moss ML: The pathogenesis of premature cranial synosto-sis in man. Acta Anat (Basel) 37:351, 1959.

146. Cohen MM Jr: Sutural Pathology. In: Cohen MM Jr,MacLean RE (eds), Craniosynostosis. Diagnosis, Evalua-tion, and Management, 2nd Ed. New York, Oxford UnivPress, 2000, Ch 5.

147. Albright AL, Byrd RP: Suture pathology in craniosynostosis.J Neurosurg 54:384, 1981.

148. Graham JM Jr, deSaxe M, Smith DW: Sagittal craniosteno-sis: fetal head constraint as one possible cause. J Pediatr95(5 Pt 1):747, 1979.

149. Graham JM Jr, Badura RJ, Smith DW: Coronal craniosteno-sis: fetal head constraint as one possible cause. Pediatrics65:995, 1980.

150. Kloss JL: Craniosynostosis secondary to ventriculoatrialshunt. Am J Dis Child 116:315, 1968.

151. Shunts and problems in shunts. Monogr Neural Sci 8:1-228, 1982.

152. Noetzel MJ et al: Hydrocephalus and mental retardation incraniosynostosis. J Pediatr 107:885, 1985.

153. Robinson DC, Hall R, Munro DS: Graves disease. An unusualcomplication: raised intracranial pressure due to prematurefusion of skull sutures. Arch Dis Child 44:252, 1969.

154. Breugem CC, van R Zeeman BJ: Retrospective study ofnonsyndromic craniosynostosis treated over a 10-yearperiod. J Craniofac Surg 10:140, 1999.

155. Cohen SR, Dauser RC, Gorski JL: Insidious onset of familialcraniosynostosis. Cleft Palate Craniofac J 30:401, 1993.

156. Opperman LA, Sweeney TM, Redmon J, et al: Tissueinteractions with underlying dura mater inhibit osseousobliteration of developing cranial sutures. Dev Dyn 198:312,1993.

157. Opperman LA, Passarelli RW, Morgan EP, et al: Cranialsutures require tissue interactions with dura mater to resistosseous obliteration in vitro. J Bone Miner Res 10:1978,1995.

158. Hobar PC et al: The role of the dura in cranial boneregeneration in the immature animal. Plast Reconstr Surg92:405, 1993.

159. Hobar PC, Masson JA, Wilson R, Zerwekh J: The impor-tance of the dura in craniofacial surgery. Plast ReconstrSurg 98:217, 1996.

160. Greenwald JA, Mehrara BJ, Spector JA, et al: Biomolecularmechanisms of calvarial bone induction: immature versusmature dura mater. Plast Reconstr Surg 105:1382, 2000.

161. Greenwald JA, Mehrara BJ, Spector JA, et al: Immatureversus mature dura mater. II: Differential expression ofgenes critical to calvarial reossification. Plast Reconstr Surg106:630, 2000.

162. Levine JP, Bradley JP, Roth DA, et al: Studies in cranialsuture biology: regional dura mater determines overlyingsuture biology. Plast Reconstr Surg 101:1441, 1998.

163. Opperman LA, Persing JA, Sheen R, Ogle RC: In theabsence of periosteum, transplanted fetal and neonatal ratcoronal sutures resist osseous obliteration. J Craniofac Surg5:327, 1994.

164. Delezoide AL, Benoist-Lasselin C, Legeai-Mallet L, et al:Spatio-temporal expression of FGFR 1, 2 and 3 genesduring human embryo-fetal ossification. Mech Dev 77:19,1998.

165. Mangasarian K, Li Y, Mansukhani A, Basilico C: Mutationassociated with Crouzon syndrome causes ligand-inde-pendent dimerization and activation of FGF receptor-2. JCell Physiol 172:117, 1997.

166. Most D, Levine JP, Chang J, et al: Studies in cranial suturebiology: up-regulation of transforming growth factor-beta1and basic fibroblast growth factor mRNA correlates withposterior frontal cranial suture fusion in the rat. PlastReconstr Surg 101:1431, 1998.

167. Mehrara BJ, Mackool RJ, McCarthy JG, et al: Immuno-localization of basic fibroblast growth factor and fibroblastgrowth factor receptor-1 and receptor-2 in rat cranialsutures. Plast Reconstr Surg 102:1805, 1998.

168. Opperman LA, Nolen AA, Ogle RC: TGF-beta 1, TGF-beta2 and TGF-beta 3 exhibit distinct patterns of expressionduring cranial suture formation and obliteration in vivo andin vitro. J Bone Miner Res 12:301, 1997.


55

169. Opperman LA, Chhabra A, Cho RW, Ogle RC: Cranialsuture obliteration is induced by removal of transforminggrowth factor (TGF)-beta 3 activity and prevented byremoval of TGF-beta 2 activity from fetal rat calvaria invitro. J Craniofac Genet Dev Biol 19:164, 1999.

170. Mehrara BJ, Most D, Chang J, et al: Basic fibroblast growthfactor and transforming growth factor beta-1 expression inthe developing dura mater correlates with calvarial boneformation. Plast Reconstr Surg 104:435, 1999.

171. Lin KY, Nolen AA, Gampper TJ, et al: Elevated levels oftransforming growth factors beta 2 and beta 3 in lambdoidsutures from children with persistent plagiocephaly. CleftPalate Craniofac J 34:331, 1997.

172. Morriss-Kay GM, Iseki S, Johnson D: Genetic control of thecell proliferation-differentiation balance in the developingskull vault: roles of fibroblast growth factor receptor signal-ling pathways. Novartis Found Symp 232:102, 2001.

173. Britto JA, Chan JCT, Evans RD, et al: Differential expressionof fibroblast growth factor receptors in human digital devel-opment suggests common pathogenesis in complexacrosyndactyly and craniosynostosis. Plast Reconstr Surg107:1331, 2001.

174. Cohen MM Jr: Discussion of “Differential expression offibroblast growth factor receptors in human digital devel-opment suggests common pathogenesis in complexacrosyndactyly and craniosynostosis”, by Britto JA, ChanJCT, Evans RD, et al. Plast Reconstr Surg 107:1339, 2001.

175. Jabs EW, Muller U, Li X, et al: A mutation in thehomeodomain of the human MSX2 gene in a family af-fected with autosomal dominant craniosynostosis. Cell75:443, 1993.

176. Vainio S, Karavanova I, Jowett A, Thesleff I: Identificationof BMP-4 as a signal mediating secondary induction be-tween epithelial and mesenchymal tissues during earlytooth development. Cell 75:45, 1993.

177. Graham A, Francis-West P, Brickell P, Lumsden A: Thesignalling molecule BMP4 mediates apoptosis in therhomboencephalic neural crest. Nature 372:684, 1994.

178. Amaya E, Musci TJ, Kirschner MW: Expression of adominant negative mutant of the FGF receptor disruptsmesoderm formation in Xenopus embryos. Cell 66:257,1991.

179. Preston A, Post J, Keats B, et al: A gene for Crouzoncraniofacial dysostosis maps to the long arm of chromo-some 10. Nature Genet 7:149, 1994.

180. Reardon W, Winter R, Rutland P, et al: Mutations in thefibroblast growth factor receptor 2 gene cause Crouzonsyndrome. Nature Genet 8:98, 1994.

181. Muenke M, Shell U, Hehr A, et al: A common mutation inthe fibroblast growth factor receptor 1 gene in Pfeiffersyndrome. Nature Genet 8:269, 1994.

182. Rutland P, Pulleyn L, Reardon W, et al: Identical mutationsin the FGFR2 gene cause both Pfeiffer and Crouzonsyndrome phenotypes. Nature Genet 9:173, 1995.

183. Wilkie A, Slaney S, Oldridge M, et al: Apert syndromeresults from localized mutations of FGFR2 and is allelic withCrouzon syndrome. Nature Genet 9:165, 1995.

184. Lajeunie E, Ma H, Bonaventure J, et al: FGFR2 mutationsin Pfeiffer syndrome (letter). Nature Genet 9:108, 1995.

185. Gripp KW, McDonald-McGinn D, Gaudenz K, et al:Identification of a genetic cause for isolated unilateralcoronal synostosis: a unique mutation in the fibroblastgrowth factor receptor 3. J Pediatr 132:714, 1998.

186. Mulliken JB, Steinberger D, Kunze S, Muller U: Moleculardiagnosis of bilateral coronal synostosis. Plast ReconstrSurg 104:1603, 1999.

187. Biesecker LG: Strike three for GLI3. Nat Genet 17:259,1997.

188. Vortkamp A, Gessler M, Grzeschik KH: GLI3 zinc-fingergene interrupted by translocations in Greig syndromefamilies. Nature 352:539, 1991.

189. Wild A, Kalff-Suske M, Vortkamp A, et al: Point mutationsin human GLI3 cause Greig syndrome. Hum Mol Genet6:1979, 1997.

190. Lee MS, Lowe GN, Strong DD, et al: TWIST, a basic helix-loop-helix transcription factor, can regulate the humanosteogenic lineage. J Cell Biochem 75:566, 1999.

191. Ghouzzi V el, Le Merrer M, Perrin-Schmitt F, et al: Muta-tions of the TWIST gene in the Saethre-Chotzen syndrome.Nat Genet 15:42, 1997.

192. Howard TD, Paznekas WA, Green ED, et al: Mutations inTWIST, a basic helix-loop-helix transcription factor, inSaethre-Chotzen syndrome. Nat Genet 15:36, 1997.

193. Paznekas WA, Cunningham ML, Howard TD, et al: Ge-netic heterogeneity of Saethre-Chotzen syndrome, due toTWIST and FGFR mutations. Am J Hum Genet 62:1370,1998.

194. Rice DP, Aberg T, Chan Y, et al: Integration of FGF andTWIST in calvarial bone and suture development. Devel-opment 127:1845, 2000.

195. Ting K, Vastardis H, Mulliken JB, et al: Human NELL-1expressed in unilateral coronal synostosis. J Bone Miner Res14:80, 1999.

196. Ting K: Personal communication, 2000. Cited in WarrenSM, Greenwald JA, Spector JA, et al: New developmentsin cranial suture research. Plast Reconstr Surg 107:523,2001.

197. Warren SM, Greenwald JA, Spector JA, et al: New devel-opments in cranial suture research. Plast Reconstr Surg107:523, 2001.

198. Kaiser G: Sagittal synostosis—Clinical significance andresults of three different methods of craniectomy. Child’sNerv Syst 4:223, 1988.

199. Marchac D, Renier D: Intracranial pressure in cranio-synostosis. J Neurosurg 57:370, 1982.

200. Renier D, Marchac D: Craniofacial surgery for cranio-synostosis: Functional and morphological results. AnnAcad Med Singapore 17:415, 1988.

201. Fok H et al: Relationship between intracranial pressureand intracranial volume in craniosynostosis. Br J Plast Surg45:394, 1992.

202. Gault DT et al: Intracranial pressure and intracranialvolume in children with craniosynostosis. Plast ReconstrSurg 90:377, 1992.

203. Kapp-Simon KA et al: Longitudinal assessment of mentaldevelopment in infants with nonsyndromic craniosynosto-sis with and without cranial release and reconstruction.Plast Reconstr Surg 92:831, 1993.

204. Renier D, Marchac D: Discussion of “Longitudinal assess-ment of mental development in infants with nonsyndromiccraniosynostosis with and without cranial release and re-construction,” by K A Kapp-Simon et al. Plast ReconstrSurg 92:840, 1993.

205. Kapp-Simon KA: Mental development and learning disor-ders in children with single suture craniosynostosis. CleftPalate Craniofac J 35:197, 1998.


56

206. Virtanen R, Korhonen T, Fagerholm J, Viljanto J:Neurocognitive sequelae of scaphocephaly. Pediatrics103(4 Pt 1):791, 1999.

207. Gault DT et al: Intracranial volume in children withcraniosynostosis. J Craniofac Surg 1:1, 1990.

208. Gosain AK: Discussion of Polley JW, Charbel FT, Kim D,MaFee MF: Nonsyndromal craniosynostosis: longitudinaloutcome following cranio-orbital reconstruction in infancy.Plast Reconstr Surg 102:629, 1998.

209. Posnick JC et al: Indirect intracranial volume measure-ments using CT scans: Clinical applications for cranio-synostosis. Plast Reconstr Surg 89:34, 1992.

210. Polley JW, Charbel FT, Kim D, MaFee MF: Nonsyndromalcraniosynostosis: longitudinal outcome following cranio-orbital reconstruction in infancy. Plast Reconstr Surg102:619, 1998.

211. David LR, Wilson JA, Watson NE, Argenta LC: Cerebralperfusion defects secondary to simple craniosynostosis. JCraniofac Surg 7:177, 1996.

212. Taylor WJ, Hayward RD, Lasjaunias P, et al: Enigma ofraised intracranial pressure in patients with complex cran-iosynostosis: the role of abnormal intracranial venousdrainage. J Neurosurg 94:377, 2001.

213. Cohen SR, Persing JA: Intracranial pressure in single-suturecraniosynostosis. Cleft Palate Craniofac J 35:194, 1998.

214. Barritt J, Brooksbank M, Simpson D: Scaphocephaly:Aesthetic and psychosocial considerations. Dev Med ChildNeurol 23:183, 1981.

215. Arndt EM et al: Psychosocial adjustment of 20 patients withTreacher Collins syndrome before and after reconstructivesurgery. Br J Plast Surg 40:605, 1987.

216. Pertschuk MJ, Whitaker LA: Psychosocial outcome ofcraniofacial surgery in children. Plast Reconstr Surg 82:741,1988.

217. Ousterhout DK, Vargervik K: Aesthetic improvementresulting from craniofacial surgery in craniosynostosis syn-dromes. J Craniomaxillofac Surg 15:189, 1987.

218. Barden RC et al: The physical attractiveness of faciallydeformed patients before and after craniofacial surgery.Plast Reconstr Surg 82:229, 1988.

219. Barden RC et al: Emotional and behavioral reactions tofacially deformed patients before and after craniofacialsurgery. Plast Reconstr Surg 82:409, 1988.

220. Collmann H, Sorensen N, Krauss J: Consensus: trigono-cephaly. Childs Nerv Syst 12:664, 1996.

221. Lajeunie E, Le Merrer M, Marchac D, Renier D:Syndromal and nonsyndromal primary trigonocephaly:analysis of a series of 237 patients. Am J Med Genet75:211, 1998.

222. Sadove AM, Kalsbeck JE, Eppley BL, Javed T: Modificationsin the surgical correction of trigonocephaly. Plast ReconstrSurg 85:853, 1990.

223. Thompson DN, Malcolm GP, Jones BM, et al: Intracranialpressure in single-suture craniosynostosis. Pediatr Neurosurg22:235, 1995.

224. Sidoti EJ Jr, Marsh JL, Marty-Grames L, Noetzel MJ: Long-term studies of metopic synostosis: frequency of cognitiveimpairment and behavioral disturbances. Plast ReconstrSurg 97:276, 1996.

225. Bottero L, Lajeunie E, Arnaud E, et al: Functional outcomeafter surgery for trigonocephaly. Plast Reconstr Surg102:952, 1998.

226. Posnick JC, Lin KY, Chen P, Armstrong D: Metopicsynostosis: quantitative assessment of presenting defor-mity and surgical results based on CT scans. Plast ReconstrSurg 93:16, 1997.

227. Havlik RJ, Azurin DJ, Bartlett SP, Whitaker LA: Analysis andtreatment of severe trigonocephaly. Plast Reconstr Surg103:381, 1999.

228. McCarthy JG, Glasberg SB, Cutting CB, et al: Twenty-yearexperience with early surgery for craniosynostosis: I.Isolated craniofacial synostosis—results and unsolved prob-lems. Plast Reconstr Surg 96:272, 1995.

229. McCarthy JG, Karp NS, LaTrenta GS, Thorne GH: Theeffect of early fronto-orbital advancement on frontal sinusdevelopment and forehead aesthetics. Plast Reconstr Surg86:1078, 1990.

230. Anderson H, Paranchos GS: Craniosynostosis: a review ofthe literature and indications for surgery. Acta PediatrScand 57:47, 1968.

231. Burstein FD, Hudgins RJ, Cohen SR, Boydston WR: Surgi-cal correction of severe scaphocephalic deformities. JCraniofac Surg 5(4):228, 1994.

232. Persing JA, Jane JA, Edgerton MT: Surgical treatment ofcraniosynostosis. In: Persing JA, Edgerton MT, Jane JA (eds),Scientific Foundations of Surgical Treatment of Cranio-synostosis. Baltimore, Williams & Wilkins, 1989, p 117.

233. Ingraham FD, Alexander E Jr, Matson DD: Clinical studiesin craniosynostosis: analysis of fifty cases and description ofa method of surgical treatment. Surgery 27:518, 1948.

234. Venes JL, Sayers MP: Sagittal synostectomy. Technicalnote. J Neurosurg 44:390, 1976.

235. Olds MV, Storrs B, Walker ML: Surgical treatment of sagittalsynostosis. Neurosurgery 18:345, 1986.

236. Greene CS Jr, Winston KR: Treatment of scaphocephalywith sagittal craniectomy and biparietal morcellation.Neurosurgery 23:196, 1988.

237. Ocampo RV Jr, Persing JA: Sagittal synostosis. Clin PlastSurg 21(4):563, 1994.

238. Esparza J, Cordobes F, Munoz MJ, et al: Tratamiento de lacraneosinostosis sagital (escaphocefalia), por medio de lacorreccion quirurgica inmediata. An Esp Pediatr 45:143,1996.

239. Friede H, Lauritzen C, Figueroa AA: Roentgen-cephalometric follow-up after early osteotomies in patientswith scaphocephaly. J Craniofac Surg 7:96, 1996.

240. Norwood CW, Alexander E, Davis CH, Kelly DL: Recur-rent and multiple suture closures after craniectomy forcraniosynostosis. J Neurosurg 41:715, 1974.

241. Jane JA, Edgerton MT, Futrell JW, Park TS: Immediatecorrection of sagittal synostosis. J Neurosurg 49:705, 1978.

242. Vollmer DG, Jane JA, Park TS, Persing JA: Variants of sagittalsynostosis: strategies for surgical correction. J Neurosurg61:557, 1984.

243. Chadduck WM, Chadduck JB, Boop FA: The subarachnoidspaces in craniosynostosis. Neurosurgery 30:867, 1992.

244. Epstein N, Epstein F, Newman G: Total vertex craniectomy forthe treatment of scaphocephaly. Childs Brain 9:309, 1982.

245. Marsh JL, Jenny A, Galic M, et al: Surgical management ofsagittal synostosis. A quantitative evaluation of two tech-niques. Neurosurg Clin North Am 3:629, 1991.

246. Christophis P, Junger TH, Howaldt H-P: Surgical correction ofscaphocephaly: experiences with a new procedure andfollow-up investigations. J Craniomaxillofac Surg 29:33, 2001.


57

247. Sutton LN, Barlett SP, Duhaine AC, Markakis D: Totalcranial vault reconstruction for the older child with scapho-cephaly. Pediatr Neurosurg 19:63, 1993.

248. Alvarez-Garijo JA, Cavadas PC, Vila MM, Alvarez-Llanas A:Sagittal synostosis: results of surgical treatment in 210patients. Childs Nerv Syst 17:64, 2001.

249. Marchac D, Renier D, Broumand S: Timing of treatmentfor craniosynostosis and faciocraniosynostosis: a 20-yearexperience. Br J Plast Surg 47:211, 1994.

250. Snively SL, Albin M: Sagittal synostosis in the older patient.Presented at the Annual Meeting of the American CleftPalate Association, San Francisco, April 1989.

251. Mulliken JB, Vander Woude DL, Hansen M, et al: Analysisof posterior plagiocephaly: deformational versus synosto-tic. Plast Reconstr Surg 103:371, 1999.

252. Huang MHS, Mouradian WE, Cohen SR, Gruss JS: Thedifferential diagnosis of abnormal head shapes: separatingcraniosynostosis from positional deformities and normalvariants. Cleft Palate Craniofac J 35:204, 1998.

253. McComb J: Treatment of functional lambdoid synostosis.Neurosurg Clin North Am 2:665, 1991.

254. O’Broin ES, Allcutt D, Earley MJ: Posterior plagiocephaly:proactive conservative management. Br J Plast Surg 52:18,1999.

255. Ellenbogen RG, Gruss JS, Cunningham ML: Update oncraniofacial surgery: the differential diagnosis of lambdoidsynostosis/posterior plagiocephaly. Clin Neurosurg 47:303,2000.

256. David DJ, Menard RM: Occipital plagiocephaly. Br J PlastSurg 53:367, 2000.

257. Persing JA et al: Lambdoid synostosis: Surgical consider-ations. Plast Reconstr Surg 81:852, 1988.

258. Mohr G, Hoffman HJ, Munro IR, et al: Surgical manage-ment of unilateral and bilateral coronal craniosynostosis:21 years of experience. Neurosurgery 2:83, 1978.

259. Hansen M, Mulliken JB: Frontal plagiocephaly. Diagnosisand treatment. Clin Plast Surg 21(4):543, 1994.

260. Marsh JL, Gado MH, Vannier MW, Stevens WG: Osseousanatomy of unilateral coronal synostosis. Cleft Palate J23:87, 1986.

261. Bruneteau RJ, Mulliken JB: Frontal plagiocephaly: synosto-tic, compensational, or deformational. Plast Reconstr Surg89:21, 1992.

262. Bruneteau RJ, Mulliken JB: Frontal plagiocephaly: Synos-totic, compensational, or deformational. Plast ReconstrSurg 89:21, 1992.

263. Lo LJ, Marsh JL, Pilgram TK, Vannier MW: Plagiocephaly:differential diagnosis based on endocranial dysmorphology.Plast Reconstr Surg 97:282, 1996.

264. Lo L-J, Marsh JL, Kane AA, Vannier MW: Orbitaldysmorphology in unilateral coronal synostosis. Cleft Pal-ate Craniofac J 33:190, 1996.

265. Posnick JC: Anterior plagiocephaly: unilateral coronalsynostosis and skull molding. In: Posnick JC (ed), Cranio-facial and Maxillofacial Surgery in Children and YoungAdults. Philadelphia, WB Saunders, 2000. Vol 1, Ch 9.

266. Wagner JD, Cohen SR, Maher H, et al: Critical analysis ofresults of craniofacial surgery for nonsyndromic bicoronalsynostosis. J Craniofac Surg 6(1):32, 1995.

267. Bertelson TI: The premature synostosis of the cranialsutures. Acta Ophthalmol 51:1, 1958.

268. Marchac D, Renier D, Jones BM: Experience with the“floating forehead.” Br J Plast Surg 41:1, 1988.

269. McCarthy JG, Glasberg SB, Cutting CB, et al: Twenty-yearexperience with early surgery for craniosynostosis: II. Thecraniofacial synostosis syndromes and pansynostosis—re-sults and unsolved problems. Plast Reconstr Surg 96:284,1995.

270. Persing J, Babler W, Winn HR, et al: Age as a critical factorin the success of surgical correction of craniosynostosis. JNeurosurg 54:601, 1981.

271. Cohen MM Jr: Phenotypic/molecular correlations. Am JMed Genet 56:334, 1995.

272. Wheaton SW: Two specimens of congenital cranialdeformities in infants in association with fusion of thefingers and toes. In: Smith DW (ed), RecognizablePatterns of Human malformation. Major Problems inClinical Pediatrics Vol 7, Philadelphia, WB Saunders,1982, p 308.

273. Tessier P: Apert’s Syndrome: AcrocephalosyndactylyType I. In: Caronni EP (ed), Craniofacial Surgery. Boston,Little Brown, 1985. Ch 27, pp 280-303.

274. Jackson IT: Craniofacial Dysostosis. In: Serafin D andGeorgiade NG (eds), Pediatric Plastic Surgery. St Louis,CV Mosby, 1984. Vol 1, Ch 27, pp 440-466.

275. Tessier P: Relationship of craniostenoses to craniofacialdysostoses and to faciostenoses. A study with therapeuticimplications. Plast Reconstr Surg 48:224, 1971.

276. Pollard ZF: Bilateral superior oblique muscle palsy associ-ated with Apert’s syndrome. Am J Ophthalmol 106:337,1988.

277. Ousterhout DK, Melsen B: Cranial base deformity inApert’s syndrome. Plast Reconstr Surg 69:254, 1982.

278. Upton J: Apert syndrome. Classification and pathologicanatomy of limb anomalies. Clin Plast Surg 18(2):321,1991.

279. Clauser L, Galie M, Hassanipour A, Calabrese O: Saethre-Chotzen syndrome: review of the literature and report ofa case. J Craniofac Surg 11:480, 2000.

280. Cohen MM Jr: Pfeiffer syndrome update: clinical subtypesand guidelines for differential diagnosis. Am J Med Genet45:300, 1993.

281. Moore MH, Lodge ML, Clark BE: The infant skull inPfeiffer’s syndrome. J Craniofac Surg 6(6):483, 1995.

282. Winter RM, Pfeiffer syndrome. Am J Med Genet 49(3):357,1994.

283. Muenke M, Schell U, Hehr A, et al: A common mutationin the fibroblast growth factor receptor 1 gene in Pfeiffersyndrome. Nature Genet 8:269, 1994.

284. Kerr NC, Wilroy RS Jr, Kaufman RA: Type 3 Pfeiffersyndrome with normal thumbs. Am J Med Genet 66:138,1996.

285. Schell U, Hehr A, Feldman GH, et al: Mutations in FGFR1and FGFR2 cause familial and sporadic Pfeiffer syndromeHum Mol Genet 4:323, 1995.

286. Taravath S, Tonsgard JH: Cerebral malformations in Car-penter syndrome. Pediatr Neurol 9:230, 1993.

287. Poole MD: Surgical caution with Carpenter’s syndrome. JCraniomaxillofac Surg 21:93, 1993.

288. Tessier P: The definitive plastic surgical treatment of thesevere facial deformities of craniofacial dysostosis. Crouzon’sand Apert’s diseases. Plast Reconstr Surg 48:419, 1971.

289. Cohen MM Jr: Craniosynostosis and syndromes withcraniosynostosis: incidence, genetics, penetrance, vari-ability, and new syndrome updating. Birth Defects15(5B):13, 1979.


58

290. Dodge HW, Wood MW, Kennedy RL: Craniofacial dysos-tosis: Crouzon’s disease. J Pediatr 23:98, 1959.

291. Costaras-Volarich M, Pruzansky S: Is the mandible intrin-sically different in Apert and Crouzon syndromes? Am JOrthod 85:475, 1984.

292. Rosen HM, Whitaker LA: Cranial base dynamics in cran-iofacial dysostosis. J Maxillofac Surg 12:56, 1984.

293. Proudman TW, Moore MH, Abbott AH, David DJ:Noncraniofacial manifestations of Crouzon’s disease. JCraniofac Surg 5(4):218, 1994.

294. Kreiborg S: Crouzon syndrome: A clinical androentgencephalometric study. Scand J Plast Reconstr Surg18 (Suppl):38, 1981.

295. Kreiborg S et al: Comparative three-dimensional analysisof CT-scans of the calvaria and cranial base in Apert andCrouzon syndromes. J Craniomaxillofac Surg 21:181,1993.

296. Posnick JC, Liin KY, Khawar BJ, Armstrong D: Crouzonsyndrome: quantitative assessment of presenting defor-mity and surgical results based on CT scans. Plast ReconstrSurg 92:1027, 1993.

297. Cohen MMJ: Craniofrontonasal dysplasia. Birth Defects15:85, 1979.

298. Sedano HE, Gorlin RJ: Frontonasal malformation as a fielddefect and in syndromic associations. Oral Surg 65:704,1988.

299. Orr DJA, Slaney S, Ashworth GJ, Poole MD: Craniofrontonasal dysplasia. Br J Plast Surg 50:153, 1997.

300. Hemmer KM, McAlister WH, Marsh JL: Cervical spineanomalies in the craniosynostosis syndromes. Cleft PalateJ 24:328, 1987.

301. Parry CH: Collections from the Unpublished MedicalWritings of the Late Caleb Hillier Parry. London,Underwoods, 1825, p 478-480.

302. Romberg MH: Klinische Ergebnisse. Berlin, A Forstner,1846, p 75-81.

303. Eulenberg A: Lehrbuch der functionellen Nerven-krankheiten. Berlin, S Karger, 1871.

304. Rogers BO: Progressive facial hemiatrophy (Romberg’sdisease): a review of 772 cases. In: Transactions of theThird International Congress of Plastic Surgery. Wash-ington, Excerpta Medica, 1963, p 681-689.

305. Moebius PJ: Der umschriebene Gesichtsschwund. In:Northanagel CWH (ed), Specielle Pathologie undTherapie, Vol 2, Pt 2. Vienna, A Holder, 1912.

306. Mendel G: Uber Hemiatrophia facialis. Dtsch MedWochenschr 14:321, 1888.

307. Moss ML, Crikelair GJ: Progressive facial hemiatrophyfollowing cervical sympathectomy in the rat. Arch Oral Biol1:254, 1960.

308. Pensler JM, Murphy GF, Mulliken JB: Clinical and ultra-structural studies of Romberg’s hemifacial atrophy. PlastReconstr Surg 85:669, 1990.

309. Moore MH, Wong KS, Proudman TW, David DJ: Pro-gressive hemifacial atrophy (Romberg’s disease): skel-etal involvement and treatment. Br J Plast Surg 46:39,1993.

310. Jurkiewicz MJ, Nahai F: The use of free revascularizedgrafts in the amelioration of hemifacial atrophy. PlastReconstr Surg 76:44, 1985.

311. Inigo F, Rojo P, Ysunza A: Aesthetic treatment of Romberg’sdisease: experience with 35 cases. Br J Plast Surg 46:194,1993.

312. Dunkley MP, Stevenson JH: Experience with the free“inverted” groin flap in facial soft tissue contouring; areport on 6 flaps. Br J Plast Surg 43:154, 1990.

313. Tweed AEJ, Manktelow RT, Zuker RM: Facial contourreconstruction with free flaps. Ann Plast Surg 12:313,1984.

314. Harashina T, Fujino T: Reconstruction in Romberg’sdisease with free groin flap. Ann Plast Surg 7:289, 1981.

315. Williams HB, Crepeau RJ: Free dermal fat flaps to the face.Ann Plast Surg 3:1, 1979.

316. Koshima I, Inagawa K, Urushibara K, et al: Deep inferiorepigastric perforator dermal-fat or adiposal flap for correc-tion of craniofacial contour deformities. Plast ReconstrSurg 106:10, 2000.

317. Mordick TG II, Larossa D, Whitaker L: Soft-tissue recon-struction of the face: a comparison of dermal-fat graftingand vascularized tissue transfer. Ann Plast Surg 29:390,1992.

318. Upton J, Albin RE, Mulliken JB, Murray JE: The use ofscapular and parascapular flaps for cheek reconstruction.Plast Reconstr Surg 90:959, 1992.

319. Longaker MT, Siebert JW: Microvascular free-flap correc-tion of severe hemifacial atrophy. Plast Reconstr Surg96:800, 1995.

320. Rees TD, Ashley FL, Delgado JP: Silicone fluid injectionsfor facial atrophy: a 10-year study. Plast Reconstr Surg52:118, 1973.

321. De la Fuente A, Tavora T: fat injections for the correctionof facial lipodistrophies: a preliminary report. AestheticPlast Surg 12:39, 1988.

322. De la Fuente A, Jimenez A: Latissimus dorsi free flaps forrestoration of facial contour defects. Ann Plast Surg 22:1,1989.

323. Jones GC et al: Juvenile neurofibroma: Behavior andtreatment of extensive and residual tumors. ArchOtolaryngol 112:1191, 1986.

324. Nichols RD et al: Meningioma in the parotid region.Laryngoscope 97:693, 1987.

325. Humphreys DH, Schwartz MR, Jenkins HA: Meningioma:A case of transcranial recurrence managed by base-of-skulltechnique and a review of the tumor. Otolaryngol HeadNeck Surg 93:563, 1985.

326. Jackson IT: Management of Craniofacial Tumors. In:Caronni EP (ed), Craniofacial Surgery. Boston, LittleBrown, 1982. Ch 29, pp 315-328.

327. Shah JP et al: Craniofacial resections for tumors involvingthe base of the skull. Am J Surg 154:352, 1987.

328. Kennerdell JS, Maroon JC, Malton ML: Surgical approachesto orbital tumors. Clin Plast Surg 15:273, 1988.

329. Sundaresan N, Shah JP: Craniofacial resection for anteriorskull base tumors. Head Neck Surg 10:219, 1988.

330. Panje WR et al: The transfacial approach for combinedanterior craniofacial tumor ablation. Arch Otolaryngol115:301, 1989.

331. Jackson IT, Adham MN, Marsh WR: Use of the galealfrontalis myofascial flap in craniofacial surgery. Plast ReconstrSurg 77:905, 1986.

332. Arden RL, Mathog RH, Thomas LM: Temporalis muscle-galea flap in craniofacial reconstruction. Laryngoscope97:1336, 1987.

333. Fisher J, Jackson IT: Microvascular surgery as an adjunct tocraniomaxillofacial reconstruction. Br J Plast Surg 42:146,1989.


59

334. Colen SR: Selective microvascular procedures inoculoplastic surgery. Clin Plast Surg 15:283, 1988.

335. Cohen SR, Bartlett SP, Whitaker LA: Reconstruction of latecraniofacial deformities after irradiation of the head andface during childhood. Plast Reconstr Surg 86:229, 1990.

336. Edgerton MT, Persing JA, Jane JA: The surgical treatmentof fibrous dysplasia. With emphasis on recent contribu-tions from craniomaxillofacial surgery. Ann Surg 202:459,1985.

337. Ricalde P, Horswell BB: Craniofacial fibrous dysplasia ofthe fronto-orbital region: a case series and literaturereview. J Oral Maxillofac Surg 59:157, 2001.

338. Barat M, Rybak LP, Mann JL: Fibrous dysplasia masquerad-ing as chronic maxillary sinusitis. Ear Nose Throat J 68:42,1989.

339. Posnick JC: Fibrous dysplasia of the craniomaxillofacialregion: current clinical perspectives. Br J Oral MaxillofacSurg 36:264, 1998.

340. Liakos GM, Walker CB, Carruth JAS: Ocular complicationsin fibrous dysplasia. Br J Ophthalmol 68:611, 1979.

341. Forbes G, Gorman CA, Brennan MD, et al: Ophthalmalogyof Graves disease: Computerized volume measurementsof the orbital fat in muscles. Am J Neuroradiol 7(4):651,1986.

342. Lello GE amd Sparrow OC: Craniofacial polyostotic fibrousdysplasia. J Maxillofac Surg 13:267, 1985.

343. Papay F, Morales L, Flaharty P, et al: Optic nerve decom-pression in cranial base fibrous dysplasia. J Craniofac Surg6(1):5, 1995.

344. Schwartz DT, Alpert M: The malignant transformation offibrous dysplasia. Am J Med 32:393, 1962.

345. Zachariades N et al: Cherubism. Int J Oral Surg 14:138,1985.

346. Van Horn PE Jr, Dahlin DC, Bickel WH: Fibrous Dysplasia:a clinical study of orthopedic surgical cases. Mayo ClinProc 38:175, 1963.

347. Derome P: Les tumeurs spheno-ethmodiales (possibilitesd’exenese et de reparation chirurgicales). Rapport de laSociete de Neuro- chirurgie de Langue Francaise.

348. Chen Y-R, Breidahl A, Chang C-N: Optic nerve decom-pression in fibrous dysplasia: indications, efficacy, andsafety. Plast Reconstr Surg 99:22, 1997.

349. Weisman JS, Hepler RS, Vinters HV: Reversible visual losscaused by fibrous dysplasia. Am J Ophthalmol 110:244,1990.

350. Lauritzen C, Alberius P, Santanelli F, et al: Repositioning ofcraniofacial tumorous bone after autoclaving. Scand J PlastReconstr Hand Surg 25:161, 1991.

351. Bradley PF: A 2-stage procedure for reimplantation ofautogenous freeze-treated mandibular bone. J OralMaxillofac Surg 40:278, 1982.

352. Bradley PF: Modern trends in cryosurgery of bone in themaxillo-facial region. Int J Oral Surg 7:405, 1978.

353. Chen Y-R, Noordhoff MS: Treatment of craniomaxillofacialfibrous dysplasia: How early and how extensive? PlastReconstr Surg 86:835, 1990.

354. Hansen-Knarhoi M, Poole MD: Preoperative difficulties indifferentiating intraosseous meningiomas and fibrous dys-plasia around the orbital apex. J Craniomaxillofac Surg22(4):226, 1994.

355. Chapurlat RD, Delmas PD, Liens D, Meunier PJ: Long-term effects of intravenous pamidronate in fibrous dyspla-sia of bone. J Bone Miner Res 12:1746, 1997.

356. Clark C, Hobar PC, and Rajagopal U: Characterization offibrous dysplasia in the nude mouse model: Evidence forestrogen dependent growth. Surgical Forum 44:741,1993.

357. Krastinova-Lolov K, Hamza F: the surgical management ofcranio-orbital neurofibromatosis. Ann Plast Surg 36(3):263,1996.

358. Poole MD: Experiences in the surgical treatment ofcranioorbital neurofibromatosis. Br J Plast Surg 42:155,1989.

359. Marchac D: Intracranial enlargement of the orbital cavityand palpebral remodeling for orbitopalpebral neurofibro-matosis. Plast Reconstr Surg 73:534, 1984.

360. Snyder BJ, Hanieh A, Trott JA, David DJ: Transcranialcorrection of orbital neurofibromatosis. Plast ReconstrSurg 102:633, 1998.

361. Jackson IT et al: Orbitotemporal neurofibromatosis: Clas-sification and treatment. Plast Reconstr Surg 92:1, 1993.

362. Lannelongue M: De la craniectomie dans la microcephalie.C R Acad Sci Paris 110:1382, 1890.

363. Lane LC: Pioneer craniectomy for relief of mental imbe-cility due to premature sutural closure and microcephalus.JAMA 18:49, 1892.

364. Waterhouse N: The history of craniofacial surgery. FacPlast Surg 9:143, 1993.

365. Gillies H, Harrison SH: Operative correction by osteotomyof recessed malar maxillary compound in a case of ox-ycephaly. Br J Plast Surg 3:123, 1950-51.

366. Converse JM, Smith B: An Operation for Congenital andTraumatic Hypertelorism. In: Troutman RC, Converse JM,Smith B (eds), Plastic and Reconstructive Surgery of theEye and Adnexa. Washington, Butterworths, 1962.

367. Tessier P: Osteotomies totales de la face. Syndrome deCrouzon. Syndrome d’Apert. Oxycephalies. Scapho-cephalies. Turricephalies. Ann Chir Plast 12:273, 1967.

368. Tessier P: Orbital hypertelorism: I. Successive surgicalattempts. Materials and methods. Causes and mecha-nisms. Scand J Plast Reconstr Surg 6:135, 1972.

369. Tessier P, Guiot G, Derome P: Orbital hypertelorism: II.Definite treatment of orbital hypertelorism (ORH) by cran-iofacial or by extracranial osteotomies. Scand J PlastReconstr Surg 7:39, 1973.

370. Tessier P: Experiences in the treatment of orbitalhypertelorism. Plast Reconstr Surg 53:1, 1974.

371. Le Fort R: Experimental study of fractures of the upper jaw.Parts I and II. Reprinted in Plast Reconstr Surg 50:497,1972. Part III, Plast Reconstr Surg 50:600, 1972.

372. Caronni EP: Preface. In: Caronni EP (ed), CraniofacialSurgery. Boston, Little Brown, 1982.

373. Obwegeser HL: Surgical correction of dish-face defor-mity. Plast Reconstr Surg 43:351, 1969.

374. Munro IR: The Luhr fixation system for the craniofacialskeleton. Clin Plast Surg 16:41, 1989.

375. Marsh JL: The use of the Wurtzberg system to facilitatefixation in facial osteotomies. Clin Plast Surg 16:49, 1989.

376. Munro IR: Rigid fixation and facial asymmetry. Clin PlastSurg 16:187, 1989.

377. Fuente del Campo A: Rigid fixation and osteotomy designin frontal orbital advancement osteotomies. Clin Plast Surg16:205, 1989.

378. Beals SP, Munro IR: The use of miniplates incraniomaxillofacial surgery. Plast Reconstr Surg 79:33,1987.


60

379. Munro IR, Fearon JA, Bruce DA: Complications of rigidfixation in infants and small children. Presented at the FifthInternational Congress of the International Society ofCraniofacial Surgery, Oaxaca, Mexico, October 1993.

380. Yu JY, Goldberg DS, Bartlett SP, Whitaker LA: The longterm positional stability of microfixation on the growingswing craniofacial skeleton. Presented at the annual meet-ing of the American Society of Plastic and ReconstructiveSurgeons, San Diego, CA, September 1994.

381. Goldberg DS, Bartlett SP, Yu JC, et al: Critical review ofmicrofixation in pediatric craniofacial surgery. J CraniofacSurg 6(4):301, 1995.

382. Papay FA, Hardy S, Morales L Jr, et al: “False” migration ofrigid fixation appliances in pediatric craniofacial surgery. JCraniofac Surg 6(4):309, 1995.

383. Berryhill WE, Rimell FL, Ness J, et al: Fate of rigid fixationin pediatric craniofacial surgery. Otolaryngol Head NeckSurg 121:269, 1999.

384. Orringer JS, Barcelona V, Buchman SR: Reasons forremoval of rigid internal fixation devices in craniofacialsurgery. J Craniofac Surg 9:40, 1998.

385. Ahn DK, Sims CD, Randolph MA, et al: Craniofacialskeletal fixation using biodegradable plates and cyanoacry-late glue. Plast Reconstr Surg 99:1508, 1997.

386. Eppley BL, Sadove AM: Effects of resorbable fixation oncraniofacial skeletal growth: alteration in plate size. JCraniofac Surg 5:110, 1994.

387. Eppley BL, Sadove AM: Resorbable coupling fixation incraniosynostosis surgery: experimental and clinical appli-cations. J Craniofac Surg 6(6):477, 1995.

388. Wiltfang J, Merten HA, Schultze-Mosgau S, et al: Biode-gradable miniplates (LactoSorb): long-term results in infantminipigs and clinical results. J Craniofac Surg 11:239,2000.

389. Tharanon W, Sinn DP, Hobar PC, et al: Surgical outcomesusing bioabsorbable plating systems in pediatric craniofa-cial surgery. J Craniofac Surg 9:441, 1998.

390. Kurpad SN, Goldstein JA, Cohen AR: Bioresorbable fixa-tion for congenital pediatric craniofacial surgery: a 2-yearfollow-up. Pediatr Neurosurg 33:306, 2000.

391. Imola MJ, Hamlar DD, Shao W, et al: Resorbable platefixation in pediatric craniofacial surgery: long-term out-come. Arch Facial Plast Surg 3:79, 2001.

392. Ambay RS, Mount DL: Craniofacial Anomalies I: Cepha-lometries and Orthognathic Surgery. Selected Read PlastSurg 10(17) Pt 1, 2007.

393. Shillito J, Matson DD: Craniosynostosis: A review of 519surgical patients. Pediatrics 41:829, 1968.

394. Epstein N, Epstein F, Newman G: Total vertex craniectomyfor the treatment of scaphocephaly. Child’s Brain 9:309,1982.

395. Hoffmann HJ, Mohr G: Lateral canthal advancement of thesupraorbital margins. J Neurosurg 45:376, 1976.

396. Marchac D: Radical forehead remodeling for craniosteno-sis. Plast Reconstr Surg 61:823, 1978.

397. Marchac D, Renier D: Craniofacial surgery for cranio-synostosis. Scand J Plast Reconstr Surg 15:235, 1981.

398. Hendel PM, Nadell JM: Projection geometry and stress–reduction techniques in craniofacial surgery. Plast ReconstrSurg 83:217, 1989.

399. Albin RE et al: Trigonocephaly: Refinements in recon-struction. Experience with 33 patients. Plast Reconstr Surg76:202, 1985.

400. Munro IR, Hoffmann HJ, Hendrick EB: Total cranial vaultreshaping in craniofacial surgery. In: Transactions of theSixth International Congress of Plastic and Recon-structive Surgery, Paris, Masson, 1975, p 158.

401. Jackson IT, Hide TAH, Barker DT: Transposition cranio-plasty to restore forehead contour in craniofacial deformi-ties. Br J Plast Surg 31:127, 1978.

402. Ortiz Monasterio F, Fuente del Campo A, Carrillo A:Advancement of the orbits and the midface in one piece,combined with frontal repositioning for the correction ofCrouzon’s deformities. Plast Reconstr Surg 61:507, 1978.

403. Marchac D, Renier D: Commentary on cranial vaultexpansion. J Craniofac Surg 4:174, 1993.

404. Marchac D, Renier D, Broumand S: Timing of treatmentfor craniosynostosis and faciocraniosynostosis: a 20-yearexperience. Br J Plast Surg 47:211, 1994.

405. McCarthy JG et al: The effect of early fronto-orbitaladvancement on frontal sinus development and foreheadaesthetics. Plast Reconstr Surg 86:1078, 1990.

406. Bartlett SP, Whitaker LA, Marchac D: The operativetreatment of isolated craniofacial dysostosis (plagioceph-aly): A comparison of the unilateral and bilateral tech-niques. Plast Reconstr Surg 85:677, 1990.

407. David DJ, Sheen R: Surgical correction of Crouzon syn-drome. Plast Reconstr Surg 85:344, 1990.

408. Marchac D, Renier D: Treatment of craniosynostosis ininfancy. Clin Plast Surg 14:61, 1987.

409. McCarthy JG et al: Early surgery for craniofacial synostosis—An 8-year experience. Plast Reconstr Surg 73:521, 1984.

410. Dufresne CR et al: Volumetric quantification of intracranialand ventricular volume following cranial vault remodeling:A preliminary report. Plast Reconstr Surg 79:24, 1987.

411. Marsh JL, Vannier MW: Cranial base changes followingsurgical treatment of craniosynostosis. Cleft Palate J 23(Suppl1):9, 1986.

412. Friede H, Lilja J, Laurizen C, et al: Skull morphology afterearly craniotomy in patients with premature synostosis ofthe coronal suture. Cleft Palate J 23 (Suppl 1):1, 1986.

413. Persing JA, Jane JA, Edgerton MT: Surgical Treatment ofCraniosynostosis. In: Persing JA, Edgerton MT, Jane JA(eds), Scientific Foundations and Surgical Treatment ofCraniosynostosis. Baltimore, Williams & Wilkins, 1989,pp 117-238.

414. Heeckt P, Muhlbauer W, Anderl H, et al: Early radicaltreatment of pancraniofacial synostosis. Ann Plast Surg30:312, 1993.

415. Whitaker LA et al: Craniosynostosis: An analysis of thetiming, treatment, and complications in 164 consecutivepatients. Plast Reconstr Surg 80:195, 1987.

416. Roddi R, Vaandrager JM, Gilbert PM, van der Meulen JC:Reshaping of the skull in the early surgical correction ofscaphocephaly. J Craniomaxillofac Surg 21:226, 1993.

417. Wall SA, Goldin JH, Hockley AD, et al: Fronto-orbital re-operation in craniosynostosis. Br J Plast Surg 47:180, 1994.

418. McCarthy JG, Glasberg SB, Cutting CB, et al: Twenty-yearexperience with early surgery for craniosynostosis: I.Isolated craniofacial synostosis—results and unsolved prob-lems. Plast Reconstr Surg 96:272, 1995.

419. McCarthy JG, Glasberg SB, Cutting CB, et al: Twenty-yearexperience with early surgery for craniosynostosis: II. Thecraniofacial synostosis syndromes and pansynostosis—re-sults and unsolved problems. Plast Reconstr Surg 96:284,1995.


61

420. Tessier P: Dysostoses cranio-faciales. Osteotomies totalesde la face. Transactions of the Fourth InternationalCongress of Plastic and Reconstructive Surgery,Amsterdam, Excerpta Medica, 1969.

421. Tessier P: Total osteotomy of the middle third of the facefor faciostenosis or for sequelae of le Fort III fractures. PlastReconstr Surg 48:533, 1971.

422. Van der Meulen JC: The pursuit of symmetry in cranio-facial surgery. Br J Plast Surg 29:85, 1976.

423. Van der Meulen JC: Medial faciotomy. Br J Plast Surg32:339, 1979.

424. Anderl H et al: Frontofacial advancement with bonyseparation in craniofacial dysostosis. Plast Reconstr Surg71:303, 1983.

425. Wolfe SA et al: The monobloc frontofacial advancement:Do the pluses outweigh the minuses? Plast Reconstr Surg91:977, 1993.

426. Fearon JA, Whitaker LA: Complications with facial ad-vancement: A comparison between the Le Fort III andmonobloc advancements. Plast Reconstr Surg 91:990,1993.

427. Munro IR, Das SK: Improving results in orbital hypertelorismcorrection. Ann Plast Surg 2:499, 1979.

428. Edgerton MT, Jane JA: Vertical orbital dystopia—Surgicalcorrection. Plast Reconstr Surg 67:121, 1981.

429. Byrd HS, Hobar PC: Optimizing the management ofsecondary zygomatic fracture deformities. Aesthetic andfunctional considerations. Clin Plast Surg 19:259, 1992.

430. De Ponte FS, Fadda T, Rinna C, et al: Early and late surgicaltreatment of orbital dystopia in craniofacial malformation.J Craniofac Surg 8(1):17, 1997.

431. Currarina G, Silverman FN: Orbital hypotelorism,arhinencephaly and trigonocephaly. Radiology 74:206,1960.

432. Evereklioglu C, Doganay S, Hamdi ER, et al: Interpupillaryindex: a new parameter for hypo-hypertelorism. JCraniomaxillofac Surg 29:191, 2001.

433. Converse JM et al: Ocular hypertelorism andpseudohypertelorism. Advances in surgical treatment.Plast Reconstr Surg 45:1, 1970.

434. McCarthy JG et al: Hypertelorism correction in the youngchild. Plast Reconstr Surg 86:214, 1990.

435. Ortiz Monasterio F, Medina O, Musolas A: Geometricalplanning for the correction of orbital hypertelorism. PlastReconstr Surg 86:650, 1990.

436. Van der Meulen JC, Vaandrager J: Surgery related to thecorrection of hypertelorism. Plast Reconstr Surg 71:6,1983.

437. Kennedy RE: The effect of early enucleation on the orbit.Am J Ophthalmol 73:80, 1965.

438. Tessier P: Expansion chirurgicale de l’orbite. Les orbitestrop petites. Exophthalmies Basedowiennes. Exorbitismedes dysostoses cranio-faciales. Atresies orbitaires des jeunesenuclees. Tumeurs orbitaires. Ann Chir Plast 14:207,1969.

439. Marchac D et al: Orbital expansion for anophthalmia andmicroorbitism. Plast Reconstr Surg 59:486, 1977.

440. Elisevich K, Bite U, Colcleugh R: Microorbitalism: Atechnique for orbital rim expansion. Plast Reconstr Surg88:609, 1991.

441. Lo AKM et al: The role of tissue expanders in an anophthalmicanimal model. Plast Reconstr Surg 86:399, 1990.

442. Rodallec A et al: Anophthalmie congenitale. Controle del’osteogenese par prothese expansive intraorbitaire. J FrOphthalmol 11:661, 1988.

443. Drommer RB: The history of the “Le Fort I osteotomy”. JMaxillofac Surg 14:119, 1986.

444. Munro IR, Beals SP, Griffin GJ: The self-retained Le Fort Iosteotomy. Plast Reconstr Surg 80:843, 1987.

445. Bell WH, Levy BM: Revascularization and bone healingfollowing total maxillary osteotomy. J Dent Res 82:96,1973.

446. Pepersack WJ: Tooth vitality after alveolar segmentalosteotomy. J Maxillofac Surg 1:85, 1973.

447. Henderson D, Jackson IT: Naso-maxillary hypoplasia—The Le Fort II osteotomy. Br J Oral Surg 2:77, 1973.

448. Converse JM et al: The treatment of nasomaxillary hypo-plasia. A new pyramidal naso-orbital maxillary osteotomy.Plast Reconstr Surg 45:527, 1970.

449. Bachmayer DI, Ross RB, Munro IR: Maxillary growthfollowing Le Fort III advancement surgery in Crouzon,Apert, and Pfeiffer syndromes. Am J Orthod DentofacOrthop 90:420-430, 1986.

450. Kaban LB et al: Midface position after Le Fort III advance-ment. Plast Reconstr Surg 73:758, 1984.

451. McCarthy JG et al: Le Fort III advancement osteotomy inthe growing child. Plast Reconstr Surg 74:343, 1984.

452. McCarthy JG et al: The Le Fort III advancement osteotomyin the child under 7 years of age. Plast Reconstr Surg86:633, 1990.

453. Whitaker LA et al: Combined report of problems andcomplications in 793 craniofacial operations. Plast ReconstrSurg 64:198, 1979.

454. Munro IR, Sabatier RE: An analysis of 12 years ofcraniomaxillofacial surgery in Toronto. Plast Reconstr Surg76:29, 1985.

455. David DJ, Cooter RD: Craniofacial infection in 10 years oftranscranial surgery. Plast Reconstr Surg 80:213, 1987.

456. Poole MD: Complications in craniofacial surgery. Br J PlastSurg 41:608, 1988.

457. Codovilla A: On a means of lengthening in the lower limbs,the muscles and tissues that are shortened through defor-mity. Am J Orthop Surg 2:352, 1905.

458. Ilizarov GA: The principles of the Ilizarov method. BullHosp Joint Dis Orthop Inst 48:1, 1988.

459. Ilizarov GA: A new principle of osteosynthesis with the useof crossing pins and rings. In: Collection of scientificworks of the Kurgan Regional Scientific Medical Soci-ety. Kurgan Med Soc 145-160, 1954.

460. Snyder CC, Levine GA, Swanson HM, Browne EZ Jr:Mandibular lengthening by gradual distraction. Prelimi-nary report. Plast Reconstr Surg 51:506, 1973.

461. McCarthy JG, Stelnicki EJ, Grayson BH: Distraction osteo-genesis of the mandible: a ten-year experience. SeminOrthod 5:3, 1999.

462. Habal MB: A future domain distractor for the facialskeleton. J Craniofac Surg 4:414, 1995.

463. Polley JW, Cohen M, Reisberg D, et al: Monobloccraniomaxillofacial distraction osteogenesis in a newbornwith severe craniofacial synostosis: a preliminary report. JCraniofac Surg 6:421, 1995.

464. Chin M, Toth BA: Le Fort III advancement with gradualdistraction using internal devices. Plast Reconstr Surg100:819, 1997.


62

465. Tavakoli K, Walsh WR, Bonar F, et al: The role of latencyin mandibular osteodistraction. J Craniomaxillofac Surg26:209, 1998.

466. Cedars MG, Linck DL II, Chin M, Toth BA: Advancementof the midface using distraction techniques. Plast ReconstrSurg 103:429, 1999.

467. Farhadieh RD, Gianoutsos MP, Dickinson R, Walsh WR:Effect of distraction rate on biomechanical, mineralization,and histologic properties of an ovine mandible model.Plast Reconstr Surg 105:889, 2000.

468. Troulis MJ, Glowacki J, Perrott DH, Kaban LB: Effects oflatency and rate on bone formation in a porcine mandibu-lar distraction model. J Oral Maxillofac Surg 58:507, 2000.

469. Ilizarov GA: Clinical application of the tension-stress effectfor limb lengthening. Clin Orthop Rel Res 250:8, 1990.

470. Aronson J: Experimental and clinical experience with distrac-tion osteogenesis. Cleft Palate Craniofac J 31:473, 1994.

471. Rachmiel A, Laufer D, Jackson IT, et al: Midface membra-nous bone lengthening: a one-year histological and mor-phological follow-up of distraction osteogenesis. CalcifTissue Int 62:370, 1998.

472. Smith SW, Sachdeva RCL, Cope JB: Evaluation of theconsolidation period during osteodistraction using com-puted tomography. Am J Orthod Dentofacial Orthop116:254, 1999.

473. Felemovicius J, Ortiz Monasterio F, Gomez Radillo LS,Serna A: Determining the optimal time for consolidationafter distraction osteogenesis. J Craniofac Surg 11:430,2000.

474. Molina F: Discussion of “Craniofacial distraction osteogen-esis: a review of 3278 cases” by Mofid MM, Manson PN,Robertson BC, et al. Plast Reconstr Surg 108:1115, 2001.

475. Tharanon W, Sinn DP: Mandibular distraction osteogen-esis with multidirectional extraoral distraction device inhemifacial microsomia patients: three-dimensional treat-ment planning, prediction tracings, and case outcomes. JCraniofac Surg 10:202, 1999.

476. Gateno J, Teichgraeber JF, Aguilar E: Computer planningfor distraction osteogenesis. Plast Reconstr Surg 105:873,2000.

477. Gateno J, Allen ME, Teichgraeber JF, Messersmith ML: Anin vitro study of the accuracy of a new protocol for planningdistraction osteogenesis of the mandible. J Oral MaxillofacSurg 58:985, 2000.

478. Pensler JM, Goldberg DP, Lindell B, Carroll NC: Skeletaldistraction of the hypoplastic mandible. Ann Plast Surg34:130, 1995.

479. McCarthy JG, Williams JK, Grayson BH, Crombie JS: Con-trolled multiplanar distraction of the mandible: devicedevelopment and clinical application. J Craniofac Surg9:322, 1998.

480. Douglas LR, Douglass JB, Nakeeb S, et al: Intraoral distrac-tion osteogenesis in the baboon mandible using a tooth andbone-anchored appliance. J Oral Maxillofac Surg 58:49,2000.

481. Guerrero CA, Bell WH, Contasti GI, Rodriguez AM: In-traoral mandibular distraction osteogenesis. Semin Orthod5:35, 1999.

482. Hollier LH, Gosain A, Stelnicki E, et al: Craniofacialdistraction osteogenesis. J Craniofac Surg 10:268, 1999.

483. Rachmiel A, Aizenbud D, Eleftheriou S, et al: Extraoral vs.intraoral distraction osteogenesis in the treatment of hemi-facial microsomia. Ann Plast Surg 45:386, 2000.

484. Polley JW, Figueroa AA, Chardel FT, et al: Monobloccraniomaxillofacial distraction osteogenesis in a newbornwith severe craniofacial synostosis: a preliminary report. JCraniofac Surg 6:421, 1995.

485. Cohen SR: Craniofacial distraction with a modular internaldistraction system: evolution of design and surgical tech-niques. Plast Reconstr Surg 103:1592, 1999.

486. Cohen SR, Holmes RE, Amis P, Fichtner H: Internalcraniofacial distraction with biodegradable devices: earlystabilization and protected bone regeneration. J CraniofacSurg 11:354, 2000.

487. Cohen SR, Holmes RE: Internal Le Fort III distraction withbiodegradable devices. J Craniofac Surg 12:264, 2001.

488. Schmelzeisen R, Neumann G, Fecht R von der: Distractionosteogenesis in the mandible with a motor-driven plate: apreliminary animal study. Br J Oral Maxillofac Surg 34:375,1996.

489. Swennen G, Schliephake H, Dempf R, et al: Craniofacialdistraction osteogenesis: a review of the literature. Part I:clinical studies. Int J Oral Maxillofac Surg 30:89, 2001.

490. Lauritzen C, Munro IR, Ross RB: Classification and treat-ment of hemifacial microsomia. Scand J Plast ReconstrSurg 19:33, 1985.

491. Vento AR, LaBrie RA, Mulliken JB: The O.M.E.N.S. classi-fication of hemifacial microsomia. Cleft Palate CraniofacJ 28:68, 1991.

492. Munro IR: One-stage reconstruction of the temporoman-dibular joint in hemifacial microsomia. Plast Reconstr Surg66:6999, 1980.

493. McCarthy JG: The role of distraction osteogenesis in thereconstruction of the mandible in unilateral craniofacialmicrosomia. Clin Plast Surg 21:625, 1994.

494. McCormick SU, McCarthy JG, Grayson BH, et al: Effectof mandibular distraction on the temporomandibularjoint: Part 1, canine study. J Craniofac Surg 6(5):358,1995.

495. McCormick SU, Grayson BH, McCarthy JG, Staffenberg D:Effect of mandibular distraction on the temporomandibularjoint: Part 2, clinical study. J Craniofac Surg 6(5):364,1995.

496. Arnett GW: A redefinition of bilateral sagittal osteotomy(BSO) advancement relapse. Am J Orthod Dentofac Orthop104:506, 1993.

497. Ellis E III, Hinton RJ: Histologic examination of the tem-poromandibular joint after mandibular advancement withand without rigid fixation. J Oral Maxillofac Surg 49:1316,1991.

498. McNeill C, ed: Temporomandibular Disorders: Guide-lines for Classification, Assessment, and Management.Chicago, Quintessence Publ, 1993, p 27-38.

499. Enlow DH: Handbook of Facial Growth. Philadelphia,WB Saunders, 1975, p 25-57.

500. Moss ML, Salentijin L: The primary role functional matricesin facial growth. Am J Orthod 55:566, 1969.

501. Guyette TW, Polley JW, Figueroa A, et al: Changes inspeech following unilateral mandibular distraction osteo-genesis in patients with hemifacial microsomia. CleftPalate Craniofac J 38:179, 2001.

502. Hoffmeister B, Marcks C, Wolff K: The floating boneconcept in intraoral mandibular distraction. Presented atthe 14th Congress of the European Association for Cranio-Maxillofacial Surgery, Helsinki, Finland, 1998. JCraniomaxillofac Surg 26(Suppl 1):76, 1998.


63

503. Kunz C, Hammer B, Prein J: Manipulation of callus afterlinear distraction: a “lifeboat” or an alternative tomultivectorial distraction osteogenesis of the mandible?Plast Reconstr Surg 105:674, 2000.

504. Denny A, Kalantarian B: Mandibular distraction in neo-nates: a strategy to avoid tracheostomy. Plast ReconstrSurg 109:896, 2002.

505. Ortiz Monasterio F, Molina F, Andrade L, et al: Simulta-neous mandibular and maxillary distraction in hemifacialmicrosomia in adults: avoidal occlusal disasters. PlastReconstr Surg 100:852, 1997.

506. Molina F, Ortiz Monasterio F: Maxillary distraction: Threeyears of clinical experience. Presented at the AnnualMeeting of the American Society of Plastic and Recon-structive Surgeons, Dallas, Texas, November 1996.

507. Ko EW-C, Figueroa AA, Polley JW: Soft tissue profilechanges after maxillary advancement with distraction os-teogenesis by use of a rigid external distraction device: a1-year follow-up. J Oral Maxillofac Surg 58:959, 2000.

508. Ko EW-C, Figueroa AA, Guyette TW, et al: Velopharyngealchanges after maxillary advancement in cleft patients withdistraction osteogenesis using a rigid external distractiondevice: a 1-year cephalometric follow-up. J CraniofacSurg 10:312, 1999.

509. Cohen SR, Rutrick RE, Burstein FD: Distraction osteogenesisof the human craniofacial skeleton: initial experience witha new distraction system. J Craniofac Surg 6(5):368, 1995.

510. Meling TR, Tveten S, Due-Tonnessen BJ, et al: Monoblocand midface distraction osteogenesis in pediatric patientswith severe syndromal craniosynostosis. Pediatr Neurosurg33:89, 2000.

511. Gosain AK, Hanson P: Case reports in midfacial distrac-tion: in search of the optimal technique. In: Chen YR (ed),Craniofacial Surgery VIII. Bologna, Monduzzi Editore,2002.

512. Fearon JA: The Le Fort III osteotomy: to distract or not todistract? Plast Reconstr Surg 107:1091, 2001.

513. Nadal E, Dogliotti PLV, Rodriguez JC, Zuccaro G: Cranio-facial distraction osteogenesis en bloc. J Craniofac Surg11:246, 2000.

514. Cohen SR, Boydston W, Hudgins R, Burstein FD: Monoblocand facial bipartition distraction with internal devices. JCraniofac Surg 10:244, 1999.

515. Hierl T, Hemprich A: Callus distraction of the midface inthe severely atrophied maxilla—a case report. Cleft PalateCraniofac J 36:457, 1999.

516. Komuro Y, Akizuki T, Kurakata M, Ohmori K: Histologicalexamination of regenerated bone through craniofacialbone distraction in clinical studies. J Craniofac Surg 10:308,1999.

517. Glat PM, Staffenberg DA, Karp NS, et al: Multidimensionaldistraction osteogenesis: the canine zygoma. Plast ReconstrSurg 94:753, 1994.

518. Liou EJ, Chen PK, Huang CS, Chen YR: Interdentaldistraction osteogenesis and rapid orthodontic tooth move-ment: a novel approach to approximate a wide alveolarcleft or bony defect. Plast Reconstr Surg 105:1262, 2000.

519. Kobayashi S, Honda T, Saitch A, Kashiwa K: Unilateralcoronal synostosis treated by internal forehead distraction.J Craniofac Surg 10:467, 1999.

520. Matsumoto K, Nakanishi H, Seike T, et al: Application ofthe distraction technique to scaphocephaly. J CraniofacSurg 11:172, 2000.

521. Mofid MM, Manson PN, Robertson BC, et al: Craniofacialdistraction osteogenesis: a review of 3278 cases. PlastReconstr Surg 108:1103, 2001.

522. Stein SC, Schut L: Management of scaphocephaly. SurgNeurol 7:153, 1977.

523. Wolfe SA: Autogenous bone grafting of orbitocranialdefects versus the use of alloplastic materials. In: CaronniEP (ed), Craniofacial Surgery. Boston, Little Brown,1982. Ch 41, pp 476- 489.

524. Lipschitz AH, Kenkel JM: Implantation: Bone, Cartilage,and Alloplasts. Selected Read Plast Surg 10(2), 2004.

525. Cutting CB, McCarthy JG, Berenstein A: Blood supply ofthe upper craniofacial skeleton: The search for compos-ite calvarial bone flaps.. Plast Reconstr Surg 74:603,1984.

526. Spear SL, Wiegering CE: Temporal fossa bone grafts: Anew technique in craniofacial surgery. Plast Reconstr Surg79:531, 1987.

527. Bite U et al: Vascularized skull bone grafts in craniofacialsurgery. Ann Plast Surg 19:3, 1987.

528. Antonyshin O, Colcleugh RG, Anderson C: Growth poten-tial in suture bone inlay grafts: A comparison of vascular-ized and free calvarial bone grafts. Plast Reconstr Surg79:1, 1987.

529. Byrd HS, Hobar PC, Shewmake K: Augmentation of thecraniofacial skeleton with porous hydroxyapatite granules.Plast Reconstr Surg 91:15, 1993.

530. Burstein FD, Cohen SR, Hudgins R, et al: The use ofhydroxyapatite cement in secondary craniofacial recon-struction. Plast Reconstr Surg 104:1270, 1999.

531. Jackson IT, Yavuzer R: Hydroxyapatite cement: an alter-native for craniofacial skeletal contour refinements. Br JPlast Surg 53:24, 2000.

532. Jackson IT, Carls F, Bush K, et al: Assessment andtreatment of facial deformity resulting from radiation tothe orbital area in childhood. Plast Reconstr Surg 98:1169,1996.

533. Nwoku AL, Koch H: Effect of radiation injury on thegrowing face. J Maxillofac Surg 3:28, 1975.

534. Larson EP: Long-term effects of radiation therapy in thehead and neck. Clin Plast Surg 20:485, 1993.

535. Marx RE, Johnson RP: Studies in the radiobiology ofosteoradionecrosis and their clinical significance. OralSurg 64:379, 1987.

536. Guyuron B: The hourglass facial deformity. J Cranio-maxillofac Surg 18:187, 1990.

537. Guyuron B, Dagys AP, Munro IR: Long-term effects oforbital irradiation. Head neck Surg 10:85, 1987.

538. Kawamoto HK Jr: Elective osteotomies and bone graftingof irradiated midfacial bones. J Craniomaxillofac Surg15:199, 1987.

10-17-02CraniofacialAnomaliesPart2

Documents

Transcript of 10-17-02CraniofacialAnomaliesPart2