cleft lip and palate

By: Dr. Waqar Jeelani

Layout Introduction Incidence Classification Embryological Background Etiology Diagnosis Problems of individuals with clefts Treatment of Cleft Lip and Palate Specialties involved in the treatment of Cleft Lip and

Palate Timings of surgical repair Cheilorraphy Palatorrhaphy Alveolar Cleft Grafts Secondary Surgical Procedures Valopharyngeal Insufficiency

IntroductionWhat is Cleft Lip and Palate?Congenital abnormal space or gap in the

upper lip, alveolus and palate

IncidenceMore common in south far Asians:

1 in 500Less frequent in Africans:

1 in 2000Prevalence in Europeans and Americans:

1 in 750*Prevalence in Pakistan:

1 in 523

*Elahi MM, Jackson IT, Elahi O, Khan AH, Mubarak F, Tariq GB, Mitra A. Epidemiology of cleft lip and cleft palate in Pakistan. Otolaryngol Clin North Am. 2007 Feb; 40(1):27-60

IncidenceBoys are more affected than girls by 3:2Cleft Lip and Palate occur twice as often in

boys as in girlsIsolated Clefts of Palate are more often in

girls75% of Clefts are Unilateral, rest are

BilateralLeft side is more involved than right side

Head and Neck of 4-Week Old Embryo

Trigeminal nerve

Facial nerve

Glossopharyngealnerve

Vagus nerve

Embryological Background

Embryological BackgroundDevelopment of the

Lip:Unpaired Frontonasal

ProminenceMedial and Lateral

Nasal prominences 2 maxillary

prominences2 mandibular

prominences

Embryological BackgroundFusion defects can occur anywhere

between these prominencesThe defect in the fusion between the

frontonasal and maxillary will lead to cleft lip

Embryological BackgroundDevelopment of Palate: We have two parts of two different embryonic origins: 1 ) primary palate : the triangular part of hard palate anterior to

incisor foramen which originate from the premaxilla ( frontonasal prominences).

develop between 4develop between 4thth and 8 and 8thth week of gestation week of gestation 2 ) secondary palate : remaining part of the hard palate and all soft

palate posterior to incisor foramen which comes from palatine shelves of the maxillary prominences

develop between 8develop between 8thth and 12 and 12thth week of gestation week of gestation

Embryological Background Various theories have been given for its

development. 1. Alteration in intrinsic palatal shelf

force 2. Failure of tongue to drop down 3. Non fusion of shelves4. Rupture of cyst formed at the site of

fusion

ClassificationWe classify as the follows: its combined (cl+cp) or isolated cleft(cl

or cp)?

is it unilateral or bilateral?

is it complete (if it cross the nasal philtrum) or incomplete ( if it doesn’t cross the nasal philtrum.

Classification Systems

Karnahan’s Classification Millard’s Modification of Karnahan’s Classifcation

Lip

Alveolus

Primary PalateHard Palate (Maxillary)Hard Palate (Palatine)Soft Palate

Kriens “LAHSHAL”Kriens “LAHSHAL”L = Lip (right)A = Alveolus (right)H = Hard Palate (right)S = Soft Palate (median)H = Hard Palate (left)A = Alveolus (left)L = Lip (left)

Capital letter = complete cleftLowercase letter = incomplete cleft“.” or “-” = normal

ExamplesLA….l = complete right cleft lip and alveolus, incomplete left cleft lipLAHS = complete right unilateral cleft lip, alveolus, hard, and soft palate

Other Types of CleftsMicroform Cleft:

May look like a little dent in the red part of the lip a scar from the lip up to the nostril.

Muscle tissue underneath the cleft can be affected and may require surgery

Submucous Cleft Palate:Midline deficiency or lack of

muscular tissueOften a submucous cleft palate is

associated with a bifid or cleft uvula

Posterior nasal spine is almost always missing

Speech Problems are common

Prenatal DiagnosisCleft lip can be easily

diagnosed by performing ultrasonography in the second trimester

Diagnosing a cleft palate with ultrasonography is very difficult

Three-dimensional imaging has been introduced to prenatal ultrasonography diagnostics of cleft anomalies

Diagnosis Advantages of Prenatal

Diagnosis:1. Time for parental education2. Time for parental

psychological preparation3. Opportunity to investigate

other associated anomalies4. Gives parents the choice of

continuing the pregnancy5. Opportunity for fetal

surgery

Etiology“Actually no one knows exactly what causes

clefts”Multiple factors may be involved, like:

Genetics (inherited characteristic) from one or both parents .

Environmental factorsDrugs: corticosteroids (anti-inflammatory),

phenytoin (anticonvulsant), retinoid.Infections: like rubella during pregnancy. Alcohol consumption, smoking, hypoxia during

pregnancy, some of dietary and vitamins deficiencies (like folic acid and vitamin A deficiency)

Maternal Age

GENETICSCL/P Normal parents, one child with CL/P 4% risk CL/P in next child Normal parents, two kids with CL/P 9% risk CL/P in next child One parent CL/P, no affected kids 4% risk CL/P in next child One parent CL/P, one child CL/P 17% risk CL/P in next

child Risk of CL/P in siblings increases with severity of deformity - child with unilateral CL risk CL/P next child 2.5% - child with bilateral CL/P risk CL/P next child 5.7%CP Normal parents, one child with CP 2% risk of CP in next child Normal parents, 2 children with CP 7% risk of CP in next child Parent with CP, no affected children 6% risk for next child Parent with CP, one child with CP 15% risk for next child

• All infants with clefs must be evaluated for presence of other anomalies• Associated nomalies are more common in infants with isolated CP

Nonsyndromic CleftNonsyndromic Cleft

Pierre Robin Sequence is the most common associated nonsyndromic anomaly

is a relative term describing the small size of the lower jaw ) and Glossoptosis (is a medical condition and abnormality which refers to the downward displacement or retraction of the tongue)

Syndromic CleftSyndromic Cleft

Common Syndromes Stickler (25% of syndromic CP) – autosomal dominant type 2 collagen gene Pierre Robin, ocular/hearing/joint

malformations

Velocardiofacial (Shprintzen’s) – 15% of syndromic CP

Autosomal dominant, variable expressivity, CATCH 22 – Deletions at 22q11,

Facial, cardiovascular, immunologic, developmental anomalies

Van der Woude’s (19% of syndromic CL/P and CP)

Autosomal dominant CL/P or CP with bilateral lower lip pits

Problems Associated With Cleft Lip and Palate

FeedingDental problemsNasal Deformity and Esthetic ProblemsEar ProblemsSpeech DifficultiesAssociated Anomalies

Feeding DifficultiesCleft lip= makes it more difficult for an

infant to suck on a nippleCleft Palate= may cause formula or breast

milk to be accidently taken up into the nasal cavity

Inability to create negative pressure inside oral cavity

Frequent regurgitationsUpper respiratory tract infections

Dental ProblemsLocal Dental Problems:

Congenitally Missing teeth, Hypodontia, Hyperdontia, Oligodontia

Presence of natal and neonatal teethAnamalies of tooth morphology like microdontia,

macrodontia etcFused teethEnamel HypoplasiaPoor periodontal support, early loss of teethGemination, Dilacerations

Orthodontics Problems:Class III tendencyAnterior and Posterior Cross biteSpacing and crowding

Nasal Deformity and Esthetic ProblemsFacial DisfigurementsPoor nasal shapeScar marks of surgeriesPoor lip function during

speechPoor dental alignment and

smile

Ear ProblemsMiddle ear disease - 22% to 88% Conductive hearing loss and chronic

suppurative otitis media may result Repeated tympanostomy tube placement

Why do cleft kids have eustachian tube dysfunction? Abnormal curvature of the eustachian tube

lumenCephalometric data - width and angulation of

the skull base with respect to the eustachian tube are different

Abnormal insertions of the tensor and levator veli palatini muscles into the cartilages and skull base

Palatal muscle dysfunction

Speech Problems:Hearing loss hampers proper development of

speechVelopharyngeal Insufficiency (VPI)Abnormal airPoor pronunciation of Bilabial, Labiodental,

Linguoalveolar sounds

Schedule of TreatmentBirth:

Initial Assessment Pre-surgical assessment

3 Month: Primary Lip repair

9-18 month: Palate Repair

2 Year: Speech assessment

3-5 Year: Lip Revision Surgery

8-9 Year: Initial interventional

Orthodontics Preparation for alveolar

bone grafting

10 Year: Alveolar Bone Grafts

12-14 Year: Definite Orthodontics

16 Year: Nasal Revision Surgery

17-20 Year: Orthognathic Surgery

Multidisciplinary Cleft Lip And Palate TeamGenetic ScientistPediatricianPedodontistOrthodontistOral and Maxillofacial SurgeonProsthodontistENT SurgeonPlastic SurgeonPsychiatristSpeech TherapistSocial Worker

FeedingCleft lip= makes it more difficult for an infant to suck on

a nippleuse special nipples to allow the baby to latch

properly (either pump or use formula)

Cleft Palate= may cause formula or breast milk to be accidentally taken up into the nasal cavity don’t feed baby without palatal obturator

(prosthetic palate) feed in an upright position to keep milk from

coming out of the nose

Mead Johnson/Enfamil Cleft Feeder Special Needs Feeder / Haberman Feeder

Pigeon Feeder Dr. Brown’s Natural Flow to relieve gas

Presurgical Orthopeadics:1. Reduces the size of cleft; Aids in Surgery2. Partial obturation aids in feeding3. Parental Reassurance at a crucial time

Maxillary StrappingNasoalveolar Moulding Appliances (NAM)

Require orthopedic repositioning of the nasal cartilages, columella, nasal tip, and lateral wall of the vestibule

Presurgical infant nasal remodelling nasal molding

Nasal molding by post surgical nasal stenting

Facial taping 2 to 3 months

BILATERAL CLEFT LIPThe most challenging

condition1. The premaxilla is

extremely protrusive

2. The premaxilla and prolabium can be of variable size

3. The columella is deficient/almost nonexistent

4. The palatal shelves are collapsed

Protrusive maxilla imperative to be

repositioned

Premaxillary orthopedics with inraoral aplliance

Denture adhesive

Elastic strap

Latham Appliance

Rule of Ten Primary repair- repaired at approximately 10

weeks The surgeon usually uses the “Rule of Ten”The child weighs 10 pounds The child has a hemoglobin of at least 10

grams The child has a white count of no higher than

10,000 The child is at least 10 weeks of age

Surgical TechniquesCleft Lip Repair

unilateral rotation-

advancement flap developed by Millard

complicationsdehiscence

infection excess tension

Surgical TechniquesCleft Lip Repair

bilateral bilateral rotation

advancement with attachment to premaxilla mucosa

Cleft Palate Repair - TimingDorf and Curtin

10% occurrence of articulation errors when palatoplasty was completed by 1 year

86% incidence of articulation errors when repair was complete after 1 year

Haapanen and Rantala – Significantly fewer children in the groups

repaired before 18 months had hypernasal speech, articulation errors, or required secondary surgery to correct speech

Cleft Palate RepairSchweckendick’s Primary VeloplastyV-Y PushbackVon Langenbeck Palatal RepairFurlow Palatoplasty

Cleft Palate RepairSchweckendick’s Primary Veloplasty

Incisions made in soft palateMuscle bundles released from the posterior

hard palate and rotatedReconstruction of levator sling Closure of mucosal layers separately

Cleft Palate RepairV-Y Pushback (WARDILL OPERATION)

Two uni-pedicled flaps (greater palatine artery) and one or two anteriorly based pedicled flaps

Posterior flaps rotated in a V-Y advancement technique - increasing the length of the palate

Nasal mucosa not closedImproved speech results compared with bipedicled

techniques Indicated for incomplete clefts

Von Langenbeck Operation:

Cleft Palate RepairFurlow Palatoplasty

Lengthens the soft palateReconstructs the muscle sling. Also commonly used to correct velopharyngeal

insufficiency in patients with submucous cleft palateSpeech outcomes are improved compared with other

palatoplasty techniques.

How do you manage ear disease? 96% of children with cleft

palate required tympanostomy tube placement

50% of these children required repeat tympanostomy tube placement.

Frequency of otitis media decreases as the child with CP ages

Audiology and tympanometry as well as exams / clinical history

Orthodontic Treatment of Transitional Dentition The purpose the dentition adjacent to

cleft has to be orthodontically repositioned to prepare the cleft side for the secondary alveolar bone graft

Preparing the maxillary arch for a bone graft (6-12 months) :

1. Bonded edgewise appliance2. Supported with a maxillary expander quad

helix expander

Alveolar Bone GraftingPrimary Bone Grafting

Bone graft done at the time of primary cheiloplasty

Bone graft done during the first 2 years of lifeBone graft done prior to the eruption of the

primary canine

Secondary Bone GraftingDone before eruption of the permanent

canineUsually when the root of the canine is 1/3 to

2/3 formedUsually between ages 8-10In CLP dental age is usually behind

chronological age

Quadhelix to expand prior to ABG

angle brackets to keep roots away from cleft

Alveolar Bone Grafting1. Provide bone for the eruption and/or

orthodontic repositioning of teeth2. Closure of oro-nasal fistulas3. Support and elevation of the alar base4. Stabilization of the pre-maxilla in bilateral

cases5. Provide continuity of the alveolar ridge

Types of bone grafts Autogenous

Cancellous- iliac crest Cortical- calvarium, mandible Cortico-cancellous- iliac, rib, tibia, mandible

*Allogeneic Graft resorbs, remodels, may contribute to osteoinduction and

osteoconduction **Alloplast

Bone grows into, around alloplast No active osteoinduction but some osteoconduction Teeth do not erupt through alloplast

*Nique T, Fonseca RJ, et al: Particulate allogeneic bone grafts into maxillary alveolar clefts in humans- A preliminary report. J Oral Maxillofac Surg 45: 386-392, 1987

**Horswell BB, El Deeb M: Nonporous HA in the repair of alveolar cleft defect in a primate model. J Oral Maxiilofac Surg 47:946-952, 1989

Alveolar Bone Grafting

Alveolar Bone Grafting

Preoperative Cleft Defect Postoperative Bone Graft

•Correction of anterior crossbite•Arch expansion

Quad HelixExpansion screws

Orthodontic Treatment For Permanent Dentition

Orthognathic SurgeryMidfacial

AdvancementLeForte osteotomies

leave vascular pedicle attached in back of maxilla - prevents necrosis

RhinoplastyRhinoplasty

standard techniques tip projection alar rotation columellar length

Age =17-20

Surgical Management of Velopharyngeal InsufficiencyMajor Goals of Surgery• Close the gap or hole between the roof of

the mouth and the nose. • Reconnect the muscles that normally make

the palate work. • Make the repaired palate long enough so

that when the muscles are working, the palate can perform its function properly.

Velopharyngeal Insufficiency VPI

Wardill Operation: WY push back

Dorrance and Brown’s – U shaped push back palatoplasty

Prosthetic Management of VPI

Thank You!