FAIRCLOTH ET AL

J Oral Maxillofac Surg 49535-542. 1991

Cherubism Involving a mother and Daughter:

Case Reports and Review of the Literature

LT COL W. JACKSON FAIRCLOTH JR, DDS, USAF, DC,* LT COL RICHARD C. EDWARDS, DDS, USAF, DC,t AND

COL VINCENT W. FARHOOD, DDS, USAF, DC+

535

Cherubism is an uncommon, benign fibro-osse- ous lesion of the jaws that causes a progressive, painless, symmetrical expansion of the maxilla and mandible with a predilection for the mandible. The World Health Organization classifies cherubism as a nonneoplastic bone lesion.’ It was first reported in 1933 by Dr W.A. Jones of Kingston, Ontario, in his description of a family with three of five children affected by bilateral cystic jaw lesions associated with cheek fullness, cervical lymphadenopathy without an apparent direct relationship to the jaw lesion, and an upward tilt of the eyes with exposure of a rim of lower sclera.* The “grotesque cherubic appearance” of these children caused him to sug- gest the term familial multilocular disease of the jaws for this condition. He later coined the term cherubism after the cherubs of Renaissance art with their “eyes-raised-to-heaven” look imparted by lower face enlargement and retraction of the lower lids. Since then, authors have employed other de- scriptive terms such as familial fibrous dysplasia of the jaws, hereditary fibrous dysplasia of the jaws, disseminated juvenile fibrous dysplasia, bilateral gi- ant cell tumors of the jaws, fibro-osseous dysplasia

Received from the Department of Oral and Maxillofacial Sur- gery, David Grant USAF Medical Center, Travis Air Force Base, CA.

* Formerly, Chief Resident; presently, Chief, Department of Oral and Maxillofacial Surgery, 1 Medical Group (TAC), Lang- ley AFB, VA.

t Attending Staff. $ Formerly Assistant Chairman and Residency Training Direc-

tor. The views expressed in this article are those of the authors and

do not reflect the official policy or position of the Department of Defense or the US government.

Address correspondence and reprint requests to Dr Faircloth: Department of Oral and Maxillofacial Surgery, 1 Medical Group (TAC), Langley AFB, VA 236f55000.

This is a US government work. There are no restrictions on its use.

0278-2391/91/4905-0018$0.00/O

of the jaws, familial intraosseous fibrous swelling of the jaws, familial bilateral giant cell tumors of the jaws, familial multilocular cystic disease of the jaws, and familial fibrous swellings of the jaws.384

The familial nature of cherubism is well estab- lished. Anderson, McClendon, and Cornelius re- ported that cherubism is hereditary, with an auto- somal dominant pattern of inheritance and a pene- trance of 80% (100% in males, 50% to 70% in females) with variable expressivity.’ Peters com- piled a family tree of greater than 100 members that demonstrated the hereditary nature of cherubism. He noted that the condition was represented in each generation, indicating either autosomal dominance or sex-linked dominance or recessiveness. Exam- ples of male to male transmission eliminated the sex-linked possibility and confirmed the autosomal dominant pattern. For purposes of genetic counsel- ing, he estimated a 40% possibility of an affected family member producing an affected offspring.6 Because cherubism is transmitted from one parent to a percentage of the children, descendents of un- affected children have been found clinically free of the condition. In the family first reported by Jones, however, two additional children had only radio- graphic evidence of disease without any cosmetic deformities. For this reason, when a family is being investigated, one must be careful to evaluate radio- graphically the jaws of all family members and adult relatives to prevent affected persons being classed as normal.’ The following case reports illustrate this point.

Report of Cases

Case 1

A 23-year-old white woman initially presented to the David Grant USAF Medical Center Department of Oral and Maxillofacial Surgery in August 1988 requesting sur- gical recontouring of an asymmetrical mandibular de-

536 CHERUBISM INVOLVING A MOTHER AND DAUGHTER

formity. Her medical history was noncontributory except for a biopsy documented diagnosis of cherubism at age 16. A review of her family history uncovered no known cases of cherubism involving her parents, four siblings, or relatives. Her mother died at age 44 from uterine cancer. She last saw her biologic father at age 3. A review of old photographs of her parents revealed no obvious cosmetic facial deformities suggestive of cherubism.

Physical examination showed a well-developed, well- nourished woman with firm, asymmetrical fullness of the lower third of the face. Greater deformity was evident on the left than on the right (Fig 1). The upper and middle thirds of the face were symmetrical and well propor- tioned, with no evidence of disease. The oral cavity was of normal size and held a full complement of 28 perma- nent teeth. The four third molars were congenitally ab- sent. She had significant maxillary and mandibular ante- rior crowding. At the time of her initial evaluation, she had severe chronic gingivitis secondary to poor oral hy- giene and malpositioned teeth. Excessive display of the maxillary incisors and labial gingiva suggested mild ver- tical maxillary excess. Other remarkable physical find- ings were a nontender, firm, movable, right submandib- ular lymph node and a functional II/VI systolic ejection murmur.

Laboratory studies, including a complete blood count and differential count, as well as measurement of electro- lytes, serum calcium, phosphorus, alkaline phosphatase, SGOT/AST, lactate dehydrogenase, and a urinalysis were all within normal limits. A panoramic radiograph clearly showed multilocular radiolucencies throughout the man- dible (Fig 2). The disease involved the anterior half of the right ramus and the anterior two thirds of the left ramus, including both coronoid processes. The posterior rami and both condyles were free of involvement. The most extensive involvement was seen throughout the mandib-

FIGURE 1. Frontal view of patient at initial visit.

FIGURE 2. Panoramic radiograph showing multilocular radio- lucencies and inferior border expansion.

ular body. Expansion of the inferior borders produced a considerable increase in the height of the mandible. More expansion was noted on the left, particularly beneath the molars and premolars. Except for the mandibular first molars, all teeth had short, tapered roots. Both inferior alveolar canals were displaced to the inferior border. No maxillary involvement was noted.

The lateral cephalometric radiograph clearly demon- strated expansion of the inferior border of the anterior body and symphyseal regions, as well as perforation of the labial cortex.

A CT scan of the mandible was performed from which a computer-generated anatomical model of the patient’s mandible was fabricated (Cemax Corporation, Santa Clara, CA) (Fig 3). The scan revealed a bubbly, lytic ap- pearance throughout the mandible, with sparing of the angle and the condyles (Fig 4). Anteriorly, the cortical bone of the mandible was extremely thin and showed disruption of the labial cortex. Overall, the appearance was one of expansion with well-defined margins. An in- cidental finding of multiple, small, rounded soft-tissue densities in the maxillary sinuses was consistent with small mucous retention cysts. The radiologist interpreted the CT findings as consistent with cherubism.

At surgery, the entire mandible between the right and left angles was degloved and explored. Much of the lat- eral cortex in the left body and symphysis was thin or nonexistent. The cystic spaces were full of soft, friable, reddish-brown, fibrous tissue that bled freely. A thorough debridement of this tissue was performed using assorted curettes and rongeur forceps. No perforations of the me- dial cortex or involvement of the left inferior alveolar

FIGURE 3. Computer-generated anatomic model of the man- dible showing both cavitation and bony expansion.

FAIRCLOTH ET AL 537

FIGURE 4. Axial CT view demonstrating generalized involve- ment of the mandible with disruption of the labial cortex. The condyles are spared.

neurovascular bundle were found. Similar findings were seen on the right side except for involvement of the right inferior alveolar nerve by the soft-tissue mass. All lateral and inferior border irregularities were contoured with a combination of reciprocating and oscillating saws and bone files. Multiple biopsy specimens were sent for ex- amination .

The microscopic diagnosis of the biopsy specimens was giant cell granulomalike lesion consistent with cherubism (Fig 5). The moderately collagenous, hypercellular con- nective tissue contained rather closely packed histiocytes and fibroblastic cells as well as numerous interspersed, moderately sized, multinucleated giant cells and foci of extravasated red blood cells (Fig 6). Brown, granular, hemosiderin pigment was abundantly present throughout the fibrotic areas. The fibrohistiocytic cells displayed a focal storiform pattern.

The patient’s case was followed clinically and radio- graphically over the next year. Her recovery was com-

FIGURE 5. Multinucleated giant cells (hematoxylin-eosin stain, original magnification X500).

FIGURE 6. Moderately collaginous, hypercellular, connective tissue containing histiocytes and fibroblastic cells and moder- ately sized multinucleated giant cells (hematoxylin-eosin stain, original magnification X 125).

plicated only by a right body of the mandible infection during the fifth postoperative month. This infection re- solved after incision and drainage and a IO-day course of oral penicillin VK 500 mg four times daily. In December 1989, she underwent a reduction genioplasty to contour residual irregularities in the chin region. Soft tissue sub- mitted for microscopic examination was histologically identical to the original specimens. Her recovery was un- eventful and she was pleased with the cosmetic result. Follow-up radiographs demonstrated increased calcifica- tion throughout the mandible, but especially in the right mandibular ramus and body. The inferior borders regen- erated bilaterally and good bony symmetry was evident.

Case 2

An especially interesting observation in case 1 was the involvement of the daughter (Fig 7). The mother was un- aware of her daughter’s condition until a staff oral sur- geon noted the child’s enlarged cheeks. A panoramic ra- diograph demonstrated symmetrical, multilocular cystic lesions involving the entire body and ramus except the condyles. The lesions were well-defined and separated by bony septa. She was followed over 18 months with serial radiographs and she began to show bilateral maxillary involvement in addition to increasing expansion of the mandibular body and symphysis regions (Fig 8). There was evidence of a thin residual cortex of bone and areas of perforation at the lower border of the mandible. Nei- ther inferior alveolar canal could be identified. Severe derangement of the mandibular deciduous and developing permanent dentitions was evident, along with aplasia of the maxillary permanent first and second molars. The roots of the maxillary deciduous posterior teeth were pre- maturely resorbed. The mandibular deciduous incisors were lost prematurely, and severe root resorption was apparent on the remaining lower deciduous teeth. The radiographs also revealed agenesis of the mandibular per- manent incisors and the remainder of the mandibular per- manent teeth appeared to be floating within the cystic spaces. Lateral and anteroposterior cephalometric radio- graphs further demonstrated the extensive two-jaw in- volvement, with the maxillary antra obliterated by the lesions. The extensive soft-tissue deformity was also ev- ident radiographically (Fig 9).

538 CHERUBISM INVOLVING A MOTHER AND DAUGHTER

FIGURE 7. Mother 3 months after second surgical procedure and daughter at age 5 years.

On physical examination, the daughter was an alert, well-developed, and well-nourished S-year-old with no apparent systemic disease. Her cheeks were full, with symmetrical bilateral swellings present along the inferior border of the mandible. The protuberant jaw masses were firm and nontender. No cervical adenopathy was noted. Intraorally, thickened maxillary and mandibular alveolar ridges were present. Severe malocclusion was evident due to derangement of the mandibular deciduous denti- tion.

A complete blood count and differential count, as well as measurement of electrolytes and serum calcium, and a urinalysis were all within normal limits. The serum phos- phorus was 4.7 mg/dL (2.0 to 4.0), and the alkaline phos- phatase was 280 U/L (38 to 126). She was subsequently diagnosed as having cherubism based on her clinical and radiographic appearance and positive family history.

Review of the mother’s childhood portraits revealed onset of the condition around 3 years of age. However, while most cases of cherubism exacerbate early and re- gress by puberty, our patient showed only mild symmet- rical lower face enlargement at the mandibular angles un-

FIGURE 9. Lateral cephalometric radiograph showing exten- sive two-jaw involvement.

til age 12 when the facial deformity increased (Fig 10). By the age of 16 the lower facial deformity was significant and she had become asymmetrical, with the left side larger than the right (Fig 11). At this age, esthetic con- cerns caused her to seek professional advice and a biopsy documented her condition as cherubism.

Her condition stabilized over the next 6 years and at the age of 23 she sought surgical assistance to improve her facial appearance. After two surgical procedures in- volving osseous recontouring and curettage of the cystic lesions, she obtained an appearance she considered ac-

FIGURE 8. Panoramic radiograph demonstrates symmetrical, multilocular cystic involvement of the maxilla and mandible. De- rangement of the deciduous and permanent dentition is evident. FIGURE 10. Mother at 12 years of age.

FAIRCLOTH ET AL 539

FIGURE 11. Mother at 16 years of age.

ceptable (Fig 12). Follow-up radiographs demonstrated bone regeneration and improved mandibular symmetry, which supports the observation of other authors that sur- gical intervention stimulates osseous healing of cherubic lesions (Fig 13). *’ Soft-tissue redundancy remains, par- ticularly in the submental region, but should improve as the soft tissues gradually adapt to the new bony contours.

The daughter presented as an unusually extensive case

FIGURE 13. Panoramic radiograph 16 months following first surgery (4 months after second surgery) shows bone regenera- tion and improved mandibular symmetry.

of cherubism, corresponding to a grade 3 presentation in the Fordyce classification system. She is currently in the rapidly expanding phase of the disease and is already ex- periencing functional difficulties with mastication and speech. Her intellectual and social behavior is normal, but continued abnormal facial growth is likely to cause emotional and social disturbances as she approaches pu- berry. Friedman and Eisenbud reported patients becom- ing uncommunicative and morose during adolescence.24

To date, no treatment has been advocated for the daughter in the hope that little progression of the disease will occur after she reaches 6 years of age. The child will remain under close observation in case a speech or air- way problem necessitates surgical intervention. Worsen- ing cosmetic deformity may eventually justify surgical re- contouring for social and emotional reasons because these children may be the subject of ridicule as they get older.6 Benefits of surgical intervention must be weighed against risks. Radical treatment could damage the devel- oping dentition and important structures such as the in- ferior alveolar neurovascular bundles. Also, there have been reported cases in which surgery can aggravate the lesions leading to recurrence with a more aggressive course.3*4.12*20 Many factors must be considered in man- aging these young patients and no perfect treatment op- tions are available.

Discussion

The pathogenesis of cherubism remains contro- versial. No cause and effect relationship with trauma, infection, or hemorrhage has ever been ver- ified. In keeping with the hereditary nature of the condition, Anderson et al suggested that a geneti- cally induced biochemical abnormality stimulates the giant cell lesions characteristic of cherubism.* Jones’ opinion that the lesions were of odontogenic origin, perhaps related to dentigerous cysts, was based on the fact that they were localized in or near tooth-bearing areas.2 Zachariades et al speculated cherubism was a manifestation of fibrous dysplasia and that it represented a perivascular fibrosis that resulted in reduced oxygenation of bone and an al- teration of the mesenchyme occurring during the development of bone. 9 Other theories presented

FIGURE 12. Mother 4 months after final surgery. over the years include latent hyperparathyroidism,

540 CHERUBISM INVOLVING A MOTHER AND DAUGHTER

benign neoplasia with hormonal dependence, and aberration of ossification in membranous bone- producing fibrous dysplastic lesions.3,‘0

Atfected children appear normal at birth. Bilat- eral swelling tends to occur between 2 and 4 years of age, but a case has been reported that arose at 14 months.“’ Males tend to be affected twice as often as females. A rapid increase in jaw size is noted, with maximum enlargement occurring within 2 years of onset in most cases.l* By age 7, the lesions become static or progress relatively slowly until puberty.’ At puberty, facial deformity remains static, and tends to improve, although radiographi- tally the appearance remains abnormal. During the late teens, the disease may undergo spontaneous involution, with the maxilla regressing earlier than the mandible.‘y3 It is not uncommon for one side to improve while the other remains static.’ Facial ap- pearance may return to almost normal by the fourth or fifth decade, although most patients seek surgical recontouring of their residual deformity during their 20s.’

Several theories have been reported to explain the cherubic appearance. Khasla and Korobkin thought the appearance was due to a combination of expanded orbital floor and poorly supported lower eyelids.13 Caffey and Williams attributed it to ten- sion on the infraorbital tissue caused by the en- larged maxilla.i4 Burland suggested the cause was bulging of the orbital floor and anterolateral wall of the maxillary sinus, with consequent lack of the in- ferior orbital rim.15

Maxillary lesions frequently give rise to a gro- tesque appearance, with associated problems in- volving speech, respiration, deglutition, and mas- tication.’ The classic cherubic appearance is not the important diagnostic sign once thought, because many affected children do not demonstrate the up- ward gaze phenomenon. In most cases, the disease is not fully developed and the mandible is the only bone involved, resulting in a clinical picture that lacks the lateral malar and infraorbital expansion and the cherubic look.16

Painless, bilateral, facial swelling, with character- istic fullness of the cheeks, is the most important clinical sign in the majority of cherubism patients. The mandible is the first bone affected. The rami and body areas are most commonly involved, pro- ducing a broadening at the angles.6 Grunebaum re- ported two cases of nonfamilial cherubism in which mandibular involvement was restricted to the sym- physis region. l6

Submandibular and tonsillar lymphadenopathy of unknown significance is a constant finding in cherubism.i7 The submandibular nodes are large,

ranging from 2 to 4 cm in diameter, nontender, and mobile, and appear 1 to 2 years after the onset of jaw changes, usually regressing within 5 years, and becoming impalpable after age 12.” Biopsy shows reticuloendothelial hyperplasia, prominent germinal centers, interseptal and capsular fibrosis, edema, and chronic lymphadenitis.’

Maxillary involvement causes more disfigure- ment than seen with isolated mandibular lesions. The maxillary tuberosity is the most common site. The infraorbital area, anterior part of the maxilla, and the orbital floor also may be affected.’ The pal- ate may be diffusely enlarged, especially anteriorly. Enlarged alveolar ridges produce a narrow V- shaped palate or obliteration of the palatal vault.13 Extreme tumerous swellings ultimately cause a gro- tesque appearance with rare functional problems in- volving speech, respiration, deglutition, limited range of jaw motion, and mastication.’ Rietkohl et al reported a case of visual disturbance thought to be a consequence of extraocular muscle imbalance secondary to unequal globe displacement. This case involved eventual orbital floor curettage with min- imal improvement in vision.3 Caffey and Williams also documented a case where maxillary lesions completely obliterated the maxillary sinuses, which later became normally pneumatized with regression of the jaw lesions. l4

In subtle cases, detection may be based on dental problems such as premature loss of teeth and non- eruption of permanent molars. Reported dental ab- normalities include dental agenesis (primarily of permanent second and third molars), pressure of deformed or resorbed roots, displacement of teeth and follicles, premature exfoliation of deciduous teeth, sloughing and/or noneruption of permanent teeth, and malocclusion. Both deciduous and per- manent teeth are widely displaced in areas of expansion.’ The fibrous replacement of bone is implicated in displacement of the deciduous den- tition. ’

Although extremely rare, cases of extrafacial skeletal involvement have been reported by several investigators. Previously reported skeletal lesions include the upper humerus, the triquetral bone bi- laterally, anterior ribs, and the upper femoral necks.5T’0*‘8 McClendon et al conjectured that skel- etal lesions might be a more common finding if skel- etal radiographic surveys were used in the diagnosis of cherubism.5

Laboratory values are nondiagnostic. Serum cal- cium, phosphorus, and alkaline phosphatase levels tend to be within normal limits. While an occasional child with cherubism will have elevated alkaline phosphatase levels, the child population at large fre-

FAIRCLOTH ET AL 541

quently has levels two to three times the adult range. ’

Radiographic Findings

Cherubism is usually recognized radiographically between 18 months and 2 years of age. The bilateral progressive bony changes are classic for the condi- tion and occasionally provide the only evidence of the disease. Irregular, multilocular, well-defined, translucent, cystic spaces causing expansion of bone and sparing only a thin layer of cortex are usually seen. Areas of cortex are commonly perfo- rated and thin bony septae separate the cystic spaces. Teeth may be displaced, unerupted, or ap- pear to be floating in the cystlike spaces. In the mandible, the inferior alveolar canal is frequently displaced as the lesion occupies the alveolar pro- cess, angle, and ramus.’ Radiolucent expansion is commonly seen in the coronoid processes, but it never involves the condyles. Periosteal new bone formation is never present. I6

Radiographically, the maxillary lesions in the tu- berosities and adjacent alveolar bone are similar to those in the mandible. The maxillary antra may be completely obliterated only to become normally pneumatized as the lesions regress.14 By age 8 to 12 years, tine trabeculae of bone begin to appear in the radiolucent areas, first in the alveolar processes and later in other areas of the jaws.7 During the third to fourth decades, radiolucent areas fill with bone of a dense granular pattern and the jaw assumes a more normal contour.‘3

Microscopic Findings

The lesions of cherubism are not distinctive his- tologically and are difficult to differentiate from other giant cells containing fibro-osseous lesions, making diagnosis independent of the clinical find- ings impossible. The histologic picture shows a loose, highly vascular, fibrous stroma with a mod- erate number of unevenly distributed multinucleat- ed giant cells of the osteoclastic type. Gorlin de- scribed the stroma as water-logged and granular, and considered this a feature unique to cherubism. l9 Giant cells tend to be arranged near hemorrhagic foci and degenerating hemosiderin. Blood vessels within the lesion tend to be well-formed, with large endothelial cells.’ Numerous investigators have ob- served the presence of eosinophilic material around small capillaries. Hamner histochemically demon- strated this perivascular cufftng to be collagen pro- tein and felt its presence was of value in the diag- nosis of cherubism.17 Mature lesions exhibit prom-

inent collaginous and fibrous tissue as the number of multinucleated giant cells diminish.’

Treatment

Treatment of cherubism has not been standard- ized, making it difficult to recommend manage- ment. The possibility of spontaneous involution is recognized, but the frequency is not known because most patients have been treated surgically prior to adulthood.3 Because few functional disturbances arise, treatment is usually based on the rate of pro- gression of the lesion, the extent of involvement, and the emotional state of the patient.” Many in- vestigators, therefore, recommend deferring treat- ment until after puberty unless psychological prob- lems warrant earlier treatment. Prompt recurrence is likely when surgery is performed at an early age. Riefkohl et al reported a case in which repeated conservative curettage (a total of 10 surgeries over a 12-year period) were followed by prompt recur- rence until the disease stabilized during the pa- tient’s early 20s. They speculated that more radical treatment initially may have reduced the number of surgical procedures, but may also have increased morbidity through damage to the permanent denti- tion and permanent facial disfigurement.3 Others re- port excellent results with surgical contouring of expanded lesions in late stage disease or after the disease has become dormant.*’ Contouring after the age of 20 years should be curative.“*‘* Dukart et al proposed that surgical stimulation accelerates os- seous deposition.*’

Radiation therapy is ineffective and contraindi- cated in view of the risks of osteoradionecrosis, interference with dentofacial growth and develop- ment, and the affect on future surgical proce- dures.3*5 Peters reported on 10 patients who had been subjected to radiation therapy, five of whom suffered some form of growth disturbance. One had a slight hypoplasia of the zygomatic region; two had mandibular hypoplasia; another had both maxillary and mandibular underdevelopment and a short in- competent soft palate; and a fifth expired at the age of 18 years from a fibrosarcoma of the maxilla.6

Differential Diagnosis

Cherubism is diagnosed primarily on the basis of a positive family history, characteristic facies, early extensive bilateral mandibular swelling, absence of other bone pathology, biopsy specimens containing giant cells within fibrous tissue, submandibular lymphadenopathy, and impacted or congenitally ab- sent permanent molars.‘* Not all features need to be

542 CHERUBISM INVOLVING A MOTHER AND DAUGHTER

present to establish a positive diagnosis. Cherubism may also be confused with other fibrous and/or cys- tic lesions of the jaws. Polyostotic fibrous dyspla- sia, for example, can be ruled out because of its later onset (second to third decade), asymmetrical distribution, predilection for the maxilla and zygo- matic bones, and the fact that the lesions stabilize rather than involute in adulthood.** Histologically, cherubism and central giant cell granuloma may be indistinguishable. However, giant cell granuloma can be excluded on clinical grounds because it is not a bilateral condition, is not inherited, does not re- gress in adulthood, and has a predilection for the anterior mandible.5,23 Multiple dentigerous or fol- licular cysts on occasion may be familial, but do not cause generalized jaw swelling or involve the coro- noid process, and have not been described in chil- dren younger than 5 years of age.3 Ameloblastomas are rarely multiple and tend to occur in older chil- dren and young adults. Reticuloendotheliosis of the Hand-Schuller-Christian type rarely causes facial swelling or bone expansion, and there are signs of the disease elsewhere. Bone changes in hyperpara- thyroidism rarely cause isolated jaw lesions, but do cause abnormal serum calcium and phosphorus levels. I4

In 1976, Fordyce suggested a grading system based on the location and severity of the jaw le- sions. Grade 1 lesions are limited primarily to the mandibular rami. Grade 2 cases involve the ramus and body of the mandible, resulting in the congen- ital absence of the third and occasionally the second mandibular molar teeth. The tuberosity of the max- illa is also affected. Grade 3 lesions affect the man- dible and maxilla in their entirety, resulting in con- siderable facial disfigurement. ’

Acknowledgment

The authors would like to thank Dr Orson P. Cardon, Chief, Department of Oral and Maxillofacial Surgery, USAF Hospital Homestead AFB, Florida, for his contributions to the mother’s initial surgical procedure. Our thanks, also, go to Dr Lee J. Slater, Chairman, Department of Oral Pathology, David Grant USAF Medical Center, Travis AFB, California, for his histologic interpretation of the biopsy specimens.

References

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2. Jones WA: Familial multilocular cystic disease of the jaw, Am J Cancer 17:946, 1933

3. Rietkohl R, Georgiade GS, Georgiade NG: Cherubism. Ann Plast Surg 1485, 1985

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