CLEFT LIP AND PALATE

Literature Reading Anne Indrawati

Introduction
The most congenital malformation of the head and neck Evaluation and management require a longterm 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

Introduction
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)

Embriology
Normal embriologic : 2 Phase o I 4 5 weeks gestation upper lip,nose and primary palate or premaxilla (anterior incisive foramen)

II 8-9 weeks gestation Secondary palate(posterior foramen)

Embriology
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)

Embriology

Embriology

Embriology

Understanding cleft embriologies allows an understanding of cleft clasiffication

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Classification

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ANATOMY

02/04/2012

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02/04/2012

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ANATOMY
Greater Palatine foramen: -a greater palatine artery -a greater palatine nervus

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Anatomical Deformity & Facial Growth

1 Cleft Lip Deformity A.Defect in Unilateral Cleft Lip Depens on degree of the cleft, the orbicularis oris muscle, blood supply and innervation INCOMPLETE muscle fiber are intact (hypoplastic across the width of the cleft)

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Anatomical Deformity & Facial Growth

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)
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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
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Anatomical 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
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Anatomical 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

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Anatomical Deformity & Facial Growth

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 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

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Anatomical 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

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Protruding premaxilla; collapse alveolar arches

Previously repair cleft lip and palate; reduce midface growth

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Management
General Phylosophy 1. The care of cleft is complex and should be coordinated cleft team 2. Counseling of parents 3. 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
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Management Early Presurgical Treatment

Feeding plate Preoperative orthopaedic

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

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

Timing & Technique for repair

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Timing for CLEFT SURGERY

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Management
Characteristic Natural Lip

SURGICAL REPAIR

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Management
Cleft Lip Repair
following criteria:

SURGICAL REPAIR

Ideally, the operation should be design to meet 1. Accurate approximation of skin, muscle, and mucosa 2. An inconspicuous scar. 3. Symmetric lip length. 4. Creation of a symmetric Cupids 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 LIP ADHESION

SURGICAL REPAIR

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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Management
Unilateral Lip adhesion

Surgical technique

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Management

Bilateral Lip adhesion

SURGICAL REPAIR

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Management
Cleft Lip Repair
Timing: 1.Traditional-10 weeks (rule of ten) 2.early repair 4-6weeks

SURGICAL REPAIR

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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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Management
Cleft Lip Repair

SURGICAL REPAIR

The Tennisan Randall triangular flap utilizes a lateral, inferior based triangular flap and zplasty 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)
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Management

SURGICAL REPAIR

Unilateral Cleft Lip Repair ( Complete)

Rotation Advanced Method- Millard

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Management

Unilateral Cleft Lip Repair

SURGICAL REPAIR

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Millard rotation- Advancement Repair

Advantage Flexible Minimal tissue discarded Good nasal access Camouflaged suture line Disadvantage Requires experience surgeon Possible excessive tension Extensive underlining required Vertikal scar contraktur Tendency to small nostril

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Management

Unilateral Cleft Lip Repair

SURGICAL REPAIR

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UNILATERAL CLEFT LIP REPAIR TENNISON-RANDALL'S DESIGN

02/04/2012

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Bilateral Cleft Lip Repair

Management

SURGICAL 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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Management

SURGICAL REPAIR

Bilateral Cleft Lip Repair

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
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Management

SURGICAL REPAIR

Bilateral Cleft Lip Repair

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

SURGICAL REPAIR

Bilateral Cleft Lip Repair

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Management

SURGICAL REPAIR

Bilateral Cleft Lip Repair

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Management
Cleft Palate Repair

SURGICAL REPAIR

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Management

SURGICAL REPAIR

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

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Management
Cleft Palate Repair
Many methods for PALATOPLASTY Selected case

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Management

SURGICAL REPAIR

Palatoplasty technique

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.

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Management

SURGICAL REPAIR

Palatoplasty technique

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

SURGICAL REPAIR

Palatoplasty technique

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
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Management

SURGICAL REPAIR

Palatoplasty technique

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

Management

SURGICAL REPAIR

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
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Management

Unilateral Cleft Palate

SURGICAL REPAIR

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Unilateral Cleft Palate Repair

Management

SURGICAL REPAIR

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Unilateral Cleft Palate Repair

Management

SURGICAL REPAIR

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Management

Bilateral Cleft Palate Repair

SURGICAL REPAIR

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Management

SURGICAL REPAIR

Cleft Palate Repair

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Management

Cleft Palate Repair

SURGICAL REPAIR

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Further 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

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

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Further Management
Pharyngeal flap
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
-Local mucopriosteal flaps

(+/- bone graft)

-Delayed until afterchilds permanent incisors have fully errupted

Oronasal fistula
- Closed surgical (two flaps) - Obturator

- soft dental wax

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Further Management 2. OTOLOGIC DISSEASE

1. Virtually all patients with cleft palate have middle ear disease 2. The incidence of middle ear disease decreases with age 3. 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
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HIGHLIGHT 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)
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HIGHLIGHT

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).

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HIGHLIGHT 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.

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HIGHLIGHT

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.

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HIGHLIGHT 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 74

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Celah bibir dan palatum

Labioskisis

Labiopalatoskis 18 bln

Palatoskisis Dewas a > 16 thn

Palatoskisis

- BB 10 pon - Umur 10 mgg - Hb 10

Timpanometri

OME (-) Timpanometri

OME (+)

ProtudingMaksila

OME (-)

OME (+)

-Nasofari ngoskopi -Nasalens -Analisis suara

(-)

(+)

Grome t

Palatoplasti 3-4 thn

Pasang Gromet

Orthodonti Speech Therapy Labio plasti Prosthodonti VP I Nasofaringoskopi Nasalens 02/04/2012 OME: Otitis Media Efusi Faringo plasti Baik VP I (-) Palatoplasti Faringoplasti Rinoplasti (Cleft Lipnose) 76