CLEFT LIP AND PALATE

CLEFT LIP AND PALATE

Literature ReadingAnne Indrawati

Literature ReadingAnne Indrawati

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IntroductionIntroductionIntroductionIntroduction

• The most congenital malformation of the head and neck

• Evaluation and management require a long-term comprehensive and multidisiplinary program

• Have numerous associated problems• Cleft lip with/without C P 1:1000 –

1 :2000 -In native americans 3,6/1000 birth & - in Asians, whites and black 0,4/1000 birth

• The most congenital malformation of the head and neck

• Evaluation and management require a long-term comprehensive and multidisiplinary program

• Have numerous associated problems• Cleft lip with/without C P 1:1000 –

1 :2000 -In native americans 3,6/1000 birth & - in Asians, whites and black 0,4/1000 birth

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In Cleft palate is constant among ethnic group

• Sex ratio: Male :female Cleft Lip with or without 2 : 1

Cleft Palate (isolated) 1 : 2• Prevalence of cleft in cleft population is as

follow: 45% cleft lip, alveolus and palate 25% cleft lip only or lip and

alveolus 30% cleft of secondary palate

In Cleft palate is constant among ethnic group

• Sex ratio: Male :female Cleft Lip with or without 2 : 1

Cleft Palate (isolated) 1 : 2• Prevalence of cleft in cleft population is as

follow: 45% cleft lip, alveolus and palate 25% cleft lip only or lip and

alveolus 30% cleft of secondary palate

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IntroductionIntroductionIntroductionIntroduction

• Can be subcatategorized as syndromic and non syndromic

• The etiology of syndromic clefts may be single gene transmission (mendelian inheritance) , chromosomal aberrations (trisomi,deletions or tranlocation), teratogenic (talidomide,etanol), and enviromental (maternal diabetic melitus)

• Can be subcatategorized as syndromic and non syndromic

• The etiology of syndromic clefts may be single gene transmission (mendelian inheritance) , chromosomal aberrations (trisomi,deletions or tranlocation), teratogenic (talidomide,etanol), and enviromental (maternal diabetic melitus)

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Normal embriologic : 2 Phase

o I 4 – 5 week’s gestation

upper lip,nose and primary palate or premaxilla (anterior incisive foramen)

o II 8-9 week’s gestation Secondary palate(posterior foramen)

Normal embriologic : 2 Phase

o I 4 – 5 week’s gestation

upper lip,nose and primary palate or premaxilla (anterior incisive foramen)

o II 8-9 week’s gestation Secondary palate(posterior foramen)

Embriology

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Malformation cleft on anterior (lip and alveolus) or cleft on posterior (secondary palate only), or both of them

The classic submucous cleft of the soft palate (bifid uvula, midline diastasis of levator m.,loss of posterior nasal spine or notching)

Malformation cleft on anterior (lip and alveolus) or cleft on posterior (secondary palate only), or both of them

The classic submucous cleft of the soft palate (bifid uvula, midline diastasis of levator m.,loss of posterior nasal spine or notching)

EmbriologyEmbriology

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EmbriologyEmbriology

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EmbriologyEmbriology

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EmbriologyEmbriology

Understanding cleft embriologies allows an understanding of cleft clasiffication

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Classification

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ANATOMY

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ANATOMYANATOMYGreater Palatine foramen:

-a greater palatine artery

-a greater palatine nervus

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Anatomical Deformity & Facial GrowthAnatomical Deformity & Facial Growth1 Cleft Lip DeformityA.Defect in Unilateral Cleft Lip Depens on degree of the cleft, the orbicularis

oris muscle, blood supply and innervation INCOMPLETE muscle fiber are intact

(hypo-plastic across the width of the cleft)

1 Cleft Lip DeformityA.Defect in Unilateral Cleft Lip Depens on degree of the cleft, the orbicularis

oris muscle, blood supply and innervation INCOMPLETE muscle fiber are intact

(hypo-plastic across the width of the cleft)

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DEFECT: (for COMPLETE)1. The orbicularis muscle is oriented

upward,parallel to cleft margins, and the orbicularis sfingter is disrupted

2. The maxilla is hipoplastic on the cleft side

3. The nasal ala on cleft side is inferioly,posteriorly and laterally displaced

4. The collumella is displaced to the cleft side

5. The medial crus is shorter and the lateral crus is longer on cleft lower lateral cartilage (LLC)

DEFECT: (for COMPLETE)1. The orbicularis muscle is oriented

upward,parallel to cleft margins, and the orbicularis sfingter is disrupted

2. The maxilla is hipoplastic on the cleft side

3. The nasal ala on cleft side is inferioly,posteriorly and laterally displaced

4. The collumella is displaced to the cleft side

5. The medial crus is shorter and the lateral crus is longer on cleft lower lateral cartilage (LLC)

Anatomical Deformity & Facial Growth

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Anatomical Deformity & Facial Growth

Anatomical Deformity & Facial Growth

6. The dome on the cleft side is lower, resulting in alar flattening and horizontal nostril shape

7. The alveolar defect passes through the developing dentition

8. The nasal floor is absent

9. The caudal septum is deviated to the non cleft side and there is an obstructing septal spur on the cleft side

6. The dome on the cleft side is lower, resulting in alar flattening and horizontal nostril shape

7. The alveolar defect passes through the developing dentition

8. The nasal floor is absent

9. The caudal septum is deviated to the non cleft side and there is an obstructing septal spur on the cleft side

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Anatomical Deformity & Facial GrowthAnatomical Deformity & Facial Growth

B.Defect in Bilateral Cleft Lip:

1. Muscle fibers are absent in prolabial segment

2. The levator palatini muscles is primarily responsible for elevating the palate

3. The vermilion is absen in prolabial segment

4. The prolabial segment has

disminished blood supply

5. The prolabium is under

develop vertically and over

develop horizontally 18

Anatomical Deformity & Facial GrowthAnatomical Deformity & Facial Growth

6.The columella is short

7.The nasal floor absent bilaterally

8.The central portion of alveolar arch is displaced

anterioly and superiorly

9.The premaxilla is mobile

10.The nasal tip is widened

6.The columella is short

7.The nasal floor absent bilaterally

8.The central portion of alveolar arch is displaced

anterioly and superiorly

9.The premaxilla is mobile

10.The nasal tip is widened

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Anatomical Deformity & Facial GrowthAnatomical Deformity & Facial Growth2. Defect in Cleft Palate

1. The velopharyngeal sling is disrupted;the muscle insert into the medial margin of the cleft and posterior hard palate

2. The cleft may involve only the soft palate, the hard palate (secondary palate),or the complete primary and secondary palate.

3. The nasal and oral cavity comunicated freely, resulting in velopharyngeal insufficiency.

4. A submucous cleft palate may be difficult diagnose

2. Defect in Cleft Palate

1. The velopharyngeal sling is disrupted;the muscle insert into the medial margin of the cleft and posterior hard palate

2. The cleft may involve only the soft palate, the hard palate (secondary palate),or the complete primary and secondary palate.

3. The nasal and oral cavity comunicated freely, resulting in velopharyngeal insufficiency.

4. A submucous cleft palate may be difficult diagnose

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Anatomical Deformity & Facial GrowthAnatomical Deformity & Facial Growth

The classic physical finding are :

Zona pellucida (hyperlucent gray area in midline soft palate),Bifid uvula,Notch in posterior hard palate

Nasopharyngoscopy during speech is the most sensitive diagnostic tool

The classic physical finding are :

Zona pellucida (hyperlucent gray area in midline soft palate),Bifid uvula,Notch in posterior hard palate

Nasopharyngoscopy during speech is the most sensitive diagnostic tool

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Anatomical Deformity & Facial GrowthAnatomical Deformity & Facial Growth

3. Facial Growth The facial skeleton is frequently deformed

in patients with cleft lip and palate Collapse the alveolar arches, midface retrussion and resultant malocclusion There is controversy regarding the relationship between surgical procedures and maxillary growth in term of the sequencing of surgical effect on maxillofacial growth, the various surgical tehniques and experience

3. Facial Growth The facial skeleton is frequently deformed

in patients with cleft lip and palate Collapse the alveolar arches, midface retrussion and resultant malocclusion There is controversy regarding the relationship between surgical procedures and maxillary growth in term of the sequencing of surgical effect on maxillofacial growth, the various surgical tehniques and experience

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Protruding premaxilla; collapse alveolar arches

Previously repair cleft lip and palate; reduce midface growth

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ManagementManagement

General Phylosophy1. The care of cleft is complex and should be

coordinated cleft team 2. Counseling of parents3. Feeding difficulties

4. Airway issues may required early management

5. Speech problems are found in 25 % 6. Cleft palate is associated with COME ( 95%)7. Patient with CL+/-P will required surgical

procedures throughout their childhood and into adolescense

General Phylosophy1. The care of cleft is complex and should be

coordinated cleft team 2. Counseling of parents3. Feeding difficulties

4. Airway issues may required early management

5. Speech problems are found in 25 % 6. Cleft palate is associated with COME ( 95%)7. Patient with CL+/-P will required surgical

procedures throughout their childhood and into adolescense 25

The feeding plate functions simultaneously as an orthodontic treatment device: realigment of the maxillary segments and molding of alveolar arch

Management Early Presurgical Treatment

Preoperative orthopaedic

Feeding plate

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Management Preoperative diagnosticManagement Preoperative diagnostic• General Examination candidate for

surgery, checking for evidence of an infection, deficience vitamin

Anatomical factors (Unilateral/bilateral, complete/incomplete, etc)

• Standart photographic views (face from the front, intra oral views of maxillary arch)

• Radiographic examination (upper jaw in patients with bilateral and bilateral total cleft)

• Jaws model

• General Examination candidate for surgery, checking for evidence of an infection, deficience vitamin

Anatomical factors (Unilateral/bilateral, complete/incomplete, etc)

• Standart photographic views (face from the front, intra oral views of maxillary arch)

• Radiographic examination (upper jaw in patients with bilateral and bilateral total cleft)

• Jaws model Timing & Technique for repair 28

Timing for CLEFT SURGERY

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Management SURGICAL REPAIRCharacteristic Natural Lip

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Management SURGICAL REPAIR

Management SURGICAL REPAIR

Cleft Lip RepairIdeally, the operation should be design to meet

following criteria:

1. Accurate approximation of skin, muscle, and mucosa

2. An inconspicuous scar.

3. Symmetric lip length.

4. Creation of a symmetric Cupid’s bow

5. Creation of a philtrum dimple,and a labial sulcus

6. Symmetric nostril and collumella

7. Easy adaptability of the procedure to various cleft

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Management SURGICAL REPAIRManagement SURGICAL REPAIR• LIP ADHESION A Lip adhession convert a complete cleft into

an incomplete cleft lip, allowing the definitive lip repair.(2-4 weeks of age)

Indication : 1. Wide unilateral complete cleft of

lip,alveolus, and palate with initial closure by convensional might produce undue tension on suture lip

2. Symmetric wide bilateral complete cleft with an extremely protruding premaxilla

3. To introduce symmetry to ansymmetryc bilateral cleft lip.

• LIP ADHESION A Lip adhession convert a complete cleft into

an incomplete cleft lip, allowing the definitive lip repair.(2-4 weeks of age)

Indication : 1. Wide unilateral complete cleft of

lip,alveolus, and palate with initial closure by convensional might produce undue tension on suture lip

2. Symmetric wide bilateral complete cleft with an extremely protruding premaxilla

3. To introduce symmetry to ansymmetryc bilateral cleft lip.

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Advantages :

- Convert complete to incomplete cleft

- Improves alveolar arch alignment

- Posible prolabial growth

- Assist with feeding

- Psychologic benefit to parents

Disadvantage:

- Increased scar tissue

- Additional operation

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Unilateral Lip adhesion

Management Surgical technique

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Bilateral Lip adhesionManagement SURGICAL REPAIR

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Management SURGICAL REPAIR

Cleft Lip Repair

Timing:

1.Traditional-10 weeks

(rule of ten)

2.early repair –4-6weeks

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Cleft Lip Repair Management SURGICAL REPAIR

If no medical contraindication, and lip adhesion has not been performed previously, definitive repair 10-14 weeks of age (In USA : Rule of Ten)

The Millard Rotation advancedment rotates the medial lip segment downward and advanced the lateral lip segment (Most commmon repair in USA)

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The Tennisan Randall triangular flap utilizes a lateral, inferior based triangular flap and z-plasty transposition

Advantages include utility with wide cleft and minimal discarding of tissue

Disadvantages include Z shaped scar and lack of flexibility with need for precise measurements

Initial treatment of nasal deformity should occur at time of primary cleft repair (primary rhinoplasty)

Management SURGICAL REPAIR

Cleft Lip Repair

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Rotation Advanced Method- Millard

Management SURGICAL REPAIR

Unilateral Cleft Lip Repair ( Complete)

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Unilateral Cleft Lip RepairManagement SURGICAL REPAIR

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Millard rotation- Advancement Repair

Millard rotation- Advancement Repair

Advantage Disadvantage

Flexible Requires experience surgeon

Minimal tissue discarded Possible excessive tension

Good nasal access Extensive underlining required

Camouflaged suture line Vertikal scar contraktur

Tendency to small nostril

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Unilateral Cleft Lip Repair

Management SURGICAL REPAIR

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UNILATERAL CLEFT LIP REPAIRTENNISON-RANDALL'S DESIGN

Management SURGICAL REPAIRBilateral Cleft Lip Repair

1. The goals surgical repair identical to those in unilateral cleft

2. The bilateral cleft can be closed in a single procedure, which offers the following advantages : increased lip and nasal symmetry, mucosa lined labial sulcus, good orbicularis oris muscle function

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3.The bilateral defect can be repair in stages : widest cleft repaired first, second cleft repaired several months later, Staged repair result in poor orbicularis oris muscle function, Lip can eventually be to long,Trifurcation scar beneath collumella is difficult to camouflage

3.The bilateral defect can be repair in stages : widest cleft repaired first, second cleft repaired several months later, Staged repair result in poor orbicularis oris muscle function, Lip can eventually be to long,Trifurcation scar beneath collumella is difficult to camouflage

Management SURGICAL REPAIR

Management SURGICAL REPAIR

Bilateral Cleft Lip Repair

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Management SURGICAL REPAIRBilateral Cleft Lip Repair

Required two stages if there is asymmetric bilateral cleft lip (rotated premaxilla). Sometimes need presurgical orthopedic before definitive repair 46

Management SURGICAL REPAIRBilateral Cleft Lip Repair

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Management SURGICAL REPAIRBilateral Cleft Lip Repair

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Management SURGICAL REPAIR

Cleft Palate

Repair…

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• Timing Operation : Consider the effect for : speech,maxillofacial

growth, occlusion, and anatomical factor. The Anatomical factors are: Cleft type, width,

degree of protrussion of premaxilla-prolabial, collaps alveolar,etc

• Timing Operation : Consider the effect for : speech,maxillofacial

growth, occlusion, and anatomical factor. The Anatomical factors are: Cleft type, width,

degree of protrussion of premaxilla-prolabial, collaps alveolar,etc

Management SURGICAL REPAIR

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Many methods for PALATOPLASTY

Selected case

Management Cleft Palate Repair…

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•The Schweekendiek two stage repair closes the sof palate cleft and leaves the hard palate cleft for obturation with a prothesis until delayed closure at 4 to 5 years. Minimal disturbance of facial grwoth. Requires frequent chages of prothesis. Result in significant speech disorder if not properly obturated. Not frequently used.

Management SURGICAL REPAIR

Palatoplasty technique

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Von Langenbaceck’’s palatoplasty advances bipedicle mucoperiosteal flaps : Easy to perform,Decreased denuded palatal bone, does not provide increased palatal length

Management SURGICAL REPAIR

Palatoplasty technique

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V-Y Push Back Palatoplasty retrodisplaces two posteriorly based mucoperiosteal flaps by a V to Y closure techq: Lengthens the palate, Leaves a large, raw palatal surface

Management SURGICAL REPAIR

Palatoplasty technique

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•Two Flap Palatoplasty (Bardach) utilizes two posterioly placed mucoperiosteal flap that extend to the alveolar cleft. Good for complete cleft of palatal/alveolus

Management SURGICAL REPAIR

Palatoplasty technique

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The Furlow Palatoplasty utilizes a double reversing Z plasty of musculomucosa and mucosa only flaps to repair the palatal cleft. Usually used for submucosal or soft palate cleft. Good speech results with proper muscle aligment. Dificult for wide cleft

Management SURGICAL REPAIRPalatoplasty technique

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Management SURGICAL REPAIRUnilateral Cleft Palate

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Management SURGICAL REPAIRUnilateral Cleft Palate Repair

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Management SURGICAL REPAIRUnilateral Cleft Palate Repair

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Management SURGICAL REPAIRBilateral Cleft Palate Repair

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Management SURGICAL REPAIRCleft Palate Repair

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Management SURGICAL REPAIRCleft Palate Repair

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Further ManagementFurther Management

Associated problems :1. SPEECH : VPI, Fistula - Generally avoided for aproximately 6-

12 months after repair 80% good speech production

Diagnosis of Velopharyngeal

Insufficiency - VPI result in hypernasal speech and

nasal escape in CP even after repair

Associated problems :1. SPEECH : VPI, Fistula - Generally avoided for aproximately 6-

12 months after repair 80% good speech production

Diagnosis of Velopharyngeal

Insufficiency - VPI result in hypernasal speech and

nasal escape in CP even after repair

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• Treatment VPI: -Initial speech trainning -Failure of speech th/ (6-12 months)

a dental obturator or surgical procedure

-Pharyngeal implants and rolls can create an artificial passavant’s ridge( Inj of Teflon paste)

-Pharyngeal flap utilizes a posterior pharyngeal mucosa/muscle flap to create two lateral ports (ideal in patients with good lateral wall motion and poor AP motion)

• Treatment VPI: -Initial speech trainning -Failure of speech th/ (6-12 months)

a dental obturator or surgical procedure

-Pharyngeal implants and rolls can create an artificial passavant’s ridge( Inj of Teflon paste)

-Pharyngeal flap utilizes a posterior pharyngeal mucosa/muscle flap to create two lateral ports (ideal in patients with good lateral wall motion and poor AP motion) 64

Treatment VPI:Treatment VPI:

Timing : 6 or 7 years of age (after an adequate period of intensive speech theraphy and full evaluation)

Need Tracheostomy

Timing : 6 or 7 years of age (after an adequate period of intensive speech theraphy and full evaluation)

Need Tracheostomy

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Pharyngeal flap

Further Management

Post op : liquid diet for 3 weeks.

Operative risk :

Bleeding from donor site

Stenosis of lateral airway portals

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Further Management

Pharyngeal flap

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Fistula

Nasolabial fistula

Oronasal fistula

-Local mucopriosteal flaps

(+/- bone graft)

-Delayed until afterchild’s permanent incisors have fully errupted

- Closed surgical (two flaps)

- Obturator

- soft dental wax68

2. OTOLOGIC DISSEASE 1. Virtually all patients with cleft palate

have middle ear disease2. The incidence of middle ear disease

decreases with age3. Factor contributing to eusthachian tube

dysfunction in CP include : ineffective tubal, dilatatation of tensor

veli palatini secondary to muscular hypoplasia and malposition and Nasoharyngeal reflux and contamination

4. Ventilation tube are placed

2. OTOLOGIC DISSEASE 1. Virtually all patients with cleft palate

have middle ear disease2. The incidence of middle ear disease

decreases with age3. Factor contributing to eusthachian tube

dysfunction in CP include : ineffective tubal, dilatatation of tensor

veli palatini secondary to muscular hypoplasia and malposition and Nasoharyngeal reflux and contamination

4. Ventilation tube are placed

Further Management

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• Cleft lip and palate are the most common congenital malformations involving the head and neck, and a cleft palate team approach best provides long-term multidisciplinary management.

• Cleft lip and palate occurs in 1 of 1,000 births; cleft palate alone occurs in 1 of 2,000 births. Clefts occur in children with recognizable syndromes or as an isolated deformity (nonsyndromic)

• Cleft lip and palate are the most common congenital malformations involving the head and neck, and a cleft palate team approach best provides long-term multidisciplinary management.

• Cleft lip and palate occurs in 1 of 1,000 births; cleft palate alone occurs in 1 of 2,000 births. Clefts occur in children with recognizable syndromes or as an isolated deformity (nonsyndromic)

HIGHLIGHTHIGHLIGHT

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• Complex genetic and environmental interactions are present in most nonsyndromic clefts.

• Lip and palate embryologic development occurs in two phases: the first beginning at 4 to 5 weeks (lip, nose, premaxilla) and the second beginning at 8 to 9 weeks (secondary palate).

• Complex genetic and environmental interactions are present in most nonsyndromic clefts.

• Lip and palate embryologic development occurs in two phases: the first beginning at 4 to 5 weeks (lip, nose, premaxilla) and the second beginning at 8 to 9 weeks (secondary palate).

HIGHLIGHTHIGHLIGHT

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• The relative prevalence of cleft types include complete cleft lip, alveolus, and palate, 45%; cleft lip with or without cleft alveolus, 25%; and clefts of the secondary palate only, 30%.

• Critical psychosocial and nutritional issues should be addressed in the neonatal period or even prenatally.

• The relative prevalence of cleft types include complete cleft lip, alveolus, and palate, 45%; cleft lip with or without cleft alveolus, 25%; and clefts of the secondary palate only, 30%.

• Critical psychosocial and nutritional issues should be addressed in the neonatal period or even prenatally.

HIGHLIGHTHIGHLIGHT

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• The rule of tens is used to determine suitable age for lip repair: the infant is at least 10 weeks old, weighs about 10 pounds, and has a hemoglobin of 10 g.

• Cleft palate repair is usually performed at 8 to 12 months of age as long as the child is gaining weight and growing in a normal fashion.

• The rule of tens is used to determine suitable age for lip repair: the infant is at least 10 weeks old, weighs about 10 pounds, and has a hemoglobin of 10 g.

• Cleft palate repair is usually performed at 8 to 12 months of age as long as the child is gaining weight and growing in a normal fashion.

HIGHLIGHTHIGHLIGHT

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• In many cases, ongoing evaluation and management are needed and determined by the cleft palate team members. This can include surgical correction of secondary lip and nasal deformities, dental and orthodontic care, speech therapy (for both treatment and assessment for articulation errors, compensatory errors, and velopharyngeal incompetence), routine otologic and audiologic care, and orthognathic surgery

• In many cases, ongoing evaluation and management are needed and determined by the cleft palate team members. This can include surgical correction of secondary lip and nasal deformities, dental and orthodontic care, speech therapy (for both treatment and assessment for articulation errors, compensatory errors, and velopharyngeal incompetence), routine otologic and audiologic care, and orthognathic surgery

HIGHLIGHTHIGHLIGHT

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Palatoskisis

- BB 10 pon- Umur 10 mgg- Hb 10

ProtudingMaksila

(-)

Labio plasti

Celah bibir dan palatum

Labioskisis Labiopalatoskis Palatoskisis

Timpanometri

(+)

OME (-)

Orthodonti

Prosthodonti

OME (+)

Gromet

18 bln

PalatoplastiFaringoplasti

Rinoplasti(Cleft Lipnose)

Timpanometri

OME (-) OME (+)

Dewasa

> 16 thn

-Nasofari ngoskopi-Nasalens-Analisis suara

Palatoplasti Pasang Gromet

Speech Therapy

3-4 thn

VP I VP I (-)

Faringo plasti

Baik

NasofaringoskopiNasalens

OME: Otitis Media Efusi