The Cleft Palate

Case Study

Full term infant, Full term infant, NSVDNSVD

Respiratory Distress Respiratory Distress at birthat birth

Numerous O2 Numerous O2 desaturationsdesaturations

Unable to feedUnable to feed

Case Study

Cleft palateCleft palate

Incidence

Cleft lipCleft lip 1/1000 live births1/1000 live births

Cleft palateCleft palate 1/2000 live births1/2000 live births

Classification - Veau Clefts of soft palate onlyClefts of soft palate only Clefts of soft and hard palate Clefts of soft and hard palate

reaching anteriorly to reaching anteriorly to incisive foramenincisive foramen

Complete unilateral alveolar Complete unilateral alveolar clefts - generally involve the clefts - generally involve the lip as welllip as well

Bilateral alveolar clefts - Bilateral alveolar clefts - associated with bilateral associated with bilateral clefts of the lipclefts of the lip

Classification A -A - Incomplete cleft of the Incomplete cleft of the

secondary palatesecondary palate B -B - Complete cleft of the Complete cleft of the

secondary palate secondary palate C-C- Incomplete cleft of the Incomplete cleft of the

primary and secondary palates primary and secondary palates D -D - Unilateral complete cleft Unilateral complete cleft

of the primary and secondary of the primary and secondary palatespalates

E -E - Bilateral complete cleft of Bilateral complete cleft of the primary and secondary the primary and secondary palatespalates

Case Study

MicrognathiaMicrognathia

Case Study

GlossoptosisGlossoptosis Increase in frequency of O2 desaturations Increase in frequency of O2 desaturations

that improve when neonate is placed in that improve when neonate is placed in prone positionprone position

Timeline of Care

Neonatal to one month of ageNeonatal to one month of age Refer to cleft palate teamRefer to cleft palate team Medical diagnosisMedical diagnosis Genetic counselingGenetic counseling Address psychosocial issuesAddress psychosocial issues Airway managementAirway management Provide feeding instructionsProvide feeding instructions Monitor growthMonitor growth Hearing screeningHearing screening

The NICU team consults ENT…

What might your flexible fiberoptic laryngoscopy show?

Doctor, what is in your differential diagnosis?EnvironmentalEnvironmental AlcoholAlcohol Tobacco smokeTobacco smoke PhenytoinPhenytoin Retinoic acidRetinoic acid Folate DeficiencyFolate Deficiency

Doctor, what is in your differential diagnosis?Syndromes

van der Woude syndrome - Autosomal dominant van der Woude syndrome - Autosomal dominant disorder is on chromosomedisorder is on chromosome

Syndromes

Robin Sequence Robin Sequence MicrognathiaMicrognathia GlossoptosisGlossoptosis Cleft palateCleft palate Associated Associated

abnormalities - 52% to abnormalities - 52% to 83%83%

Syndromes

Velocardiofacial syndromeVelocardiofacial syndrome

Overlaps with DiGeorge syndrome and Overlaps with DiGeorge syndrome and conotruncal anomaly face syndromeconotruncal anomaly face syndrome

CATCH 22: cardiac anomalies (usually CATCH 22: cardiac anomalies (usually conotruncal), conotruncal), aabnormal facies, bnormal facies, tthymic disorders, hymic disorders, ccleft palate, and left palate, and hhypocalcemia caused a deletion of ypocalcemia caused a deletion of chromosome chromosome 2222 at the q11.2 region. at the q11.2 region.

Syndromes

Stickler syndromeStickler syndrome Most common syndrome found in children with Most common syndrome found in children with

Robin Sequence - 14% to 34% of casesRobin Sequence - 14% to 34% of cases Sensorineural hearing lossSensorineural hearing loss

Myopia - Risk of retinal detachment and early Myopia - Risk of retinal detachment and early cataractscataracts

Hypermobilityof jointsHypermobilityof joints

Stiffness and premature osteoarthritisStiffness and premature osteoarthritis

Airway management

Prone positioning, which allows the tongue to fall Prone positioning, which allows the tongue to fall away from the posterior pharyngeal wall.away from the posterior pharyngeal wall.

Gavage feeding may be needed in such casesGavage feeding may be needed in such cases Glossopexy or lip-tongue adhesion in infants Glossopexy or lip-tongue adhesion in infants TracheotomyTracheotomy Mandibular distraction osteogenesisMandibular distraction osteogenesis

Seibert’s Technique Lip Adhesion

Medially and laterally based rectangular flaps from the margin of the Medially and laterally based rectangular flaps from the margin of the cleftcleft

Tension-bearing suture through the membranous septum.Tension-bearing suture through the membranous septum. Unilateral lip adhesion: Unilateral lip adhesion: A,A, landmarks and incisions; landmarks and incisions; B,B, flaps elevated flaps elevated

and undermined; and undermined; C,C, placement of 3-0 nylon retention suture; placement of 3-0 nylon retention suture; D,D, final final suturing.suturing.

Airway Management

One hundred seven patients with Pierre Robin SequenceOne hundred seven patients with Pierre Robin Sequence 74 (69.2%) - positioning alone 74 (69.2%) - positioning alone 29 - tongue lip adhesion29 - tongue lip adhesion

Airway obstruction relieved in 20 (83.3%)Airway obstruction relieved in 20 (83.3%) 10 - Tracheostomy10 - Tracheostomy

Including six (20.7%) who initially underwent a Including six (20.7%) who initially underwent a TLATLA

TimelineOne to Four months

Monitor feeding and growthMonitor feeding and growth Monitor ears and hearing Monitor ears and hearing Repair cleft lip with possible PE tube Repair cleft lip with possible PE tube

placementplacement

Incidence of Eustachian Tube Dysfunction Middle ear disease - 22% to 88% Middle ear disease - 22% to 88% Conductive hearing loss and chronic Conductive hearing loss and chronic

suppurative otitis media may result suppurative otitis media may result Repeated tympanostomy tube placementRepeated tympanostomy tube placement

Why do cleft kids have eustachian tube dysfunction? Paratubal cartilage abnormalities Paratubal cartilage abnormalities Hypoplasia of the lateral cartilage relative to the Hypoplasia of the lateral cartilage relative to the

medial cartilage medial cartilage Abnormal curvature of the eustachian tube lumenAbnormal curvature of the eustachian tube lumen Cephalometric data - width and angulation of the Cephalometric data - width and angulation of the

skull base with respect to the eustachian tube are skull base with respect to the eustachian tube are different different

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

Palatal muscle dysplasia Palatal muscle dysplasia

How do you manage ear disease?

96% of children with cleft palate required 96% of children with cleft palate required tympanostomy tube placementtympanostomy tube placement

50% of these children required repeat 50% of these children required repeat tympanostomy tube placement. tympanostomy tube placement.

Frequency of otitis media decreases as the child Frequency of otitis media decreases as the child with CP ageswith CP ages

Audiology and tympanometry as well as exams / Audiology and tympanometry as well as exams / clinical historyclinical history

First set are frequently placed during cleft lip or First set are frequently placed during cleft lip or cleft palate repaircleft palate repair

Latham Appliance

Cleft Lip RepairRule of 10’s

Surgery > 10 weeks oldSurgery > 10 weeks old Weight > 10 poundsWeight > 10 pounds Hemoglobin level > 10 g/dL Hemoglobin level > 10 g/dL

Cleft Lip RepairGoals

Bridge the cleftBridge the cleft Create a complete muscular sling around the entire Create a complete muscular sling around the entire

circumference of the oral cavitycircumference of the oral cavity Approximate cleft edges Approximate cleft edges Maintain Cupid's bow and philtral dimpleMaintain Cupid's bow and philtral dimple Align vermillion borderAlign vermillion border Create an intact nasal floor and sillCreate an intact nasal floor and sill Produce symmetry of the alar base and columellaProduce symmetry of the alar base and columella Reconstituting the circumferential integrity of the Reconstituting the circumferential integrity of the

orbicularis oris muscleorbicularis oris muscle

Cleft Lip Repair - Types

Millard Repair - Rotation advancement Millard Repair - Rotation advancement techniquetechnique

Randall - Graham - Triangular flap Randall - Graham - Triangular flap interpositioninterposition

Rose - Thompson - Straight line repair, Risk Rose - Thompson - Straight line repair, Risk of vertical contractureof vertical contracture

Millard Repair

Downward and lateral rotation of the medial segment of the cleft lip Downward and lateral rotation of the medial segment of the cleft lip combined with the medial advancement of the lateral cleft segment into the combined with the medial advancement of the lateral cleft segment into the defectdefect

Bridges the gap Bridges the gap Maintains the rotation flap in positionMaintains the rotation flap in position Maintains the amount of vertical height gained by the rotation flap and back Maintains the amount of vertical height gained by the rotation flap and back

cutcut

TimelineFive to Fifteen Months

Monitor feeding, growth, and developmentMonitor feeding, growth, and development Repair cleft palateRepair cleft palate Monitor ears and hearingMonitor ears and hearing Instructions in oral hygieneInstructions in oral hygiene

Cleft Palate Repair - Timing

Dorf and CurtinDorf and Curtin 10% occurrence of articulation errors when 10% occurrence of articulation errors when

palatoplasty was completed by 1 yearpalatoplasty was completed by 1 year 86% incidence of articulation errors when 86% incidence of articulation errors when

repair was complete after 1 yearrepair was complete after 1 year Haapanen and Rantala - Significantly fewer Haapanen and Rantala - Significantly fewer

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

Cleft Palate Repair

V-Y PushbackV-Y Pushback Two Flap PalatoplastyTwo Flap Palatoplasty Four Flap PalatoplastyFour Flap Palatoplasty Schweckendick’s Primary VeloplastySchweckendick’s Primary Veloplasty Furlow PalatoplastyFurlow Palatoplasty

Cleft Palate RepairV-Y Pushback

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

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

Nasal mucosa not closedNasal mucosa not closed Improved speech results compared with bipedicled techniques Improved speech results compared with bipedicled techniques Indicated for incomplete cleftsIndicated for incomplete clefts

Cleft Palate RepairSchweckendick’s Primary Veloplasty

Incisions made in soft palateIncisions made in soft palate Muscle bundles released from the posterior hard Muscle bundles released from the posterior hard

palate and rotatedpalate and rotated Reconstruction of levator sling Reconstruction of levator sling Closure of mucosal layers separatelyClosure of mucosal layers separately

Cleft Palate RepairFurlow Palatoplasty

Lengthens the soft palateLengthens the soft palate Reconstructs the muscle Reconstructs the muscle

sling. sling. Also commonly used to Also commonly used to

correct velopharyngeal correct velopharyngeal insufficiency in patients insufficiency in patients with submucous cleft palatewith submucous cleft palate

Speech outcomes are Speech outcomes are improved compared with improved compared with other palatoplasty other palatoplasty techniques.techniques.

Cleft Palate RepairComplications

Oronasal fistula - 8.7% to 23%Oronasal fistula - 8.7% to 23%

Sites of fistulization are typically the Sites of fistulization are typically the anterior hard palate and the junction of anterior hard palate and the junction of the hard and soft palate.the hard and soft palate.

Velopharyngeal insufficiencyVelopharyngeal insufficiency

TimelineTwo to Five years old

Monitor speech and language developmentMonitor speech and language development Manage velopharyngeal insufficiencyManage velopharyngeal insufficiency Monitor ears and hearing; ear tubes if Monitor ears and hearing; ear tubes if

indicatedindicated Assess development and psychosocial Assess development and psychosocial

needsneeds Consider lip/nose revision before schoolConsider lip/nose revision before school

TimelineSix to Eleven years old

Monitor speech and language; manage Monitor speech and language; manage velopharyngeal insufficiencyvelopharyngeal insufficiency

Orthodontic evaluation and treatmentOrthodontic evaluation and treatment Alveolar bone graftAlveolar bone graft Monitor school and psychosocial needsMonitor school and psychosocial needs

TimelineTwelve to Twenty One years old

Monitor school and psychosocial needsMonitor school and psychosocial needs Orthodontics and restorative dentistryOrthodontics and restorative dentistry Genetic counselingGenetic counseling Rhinoplasty (if needed)Rhinoplasty (if needed) Orthognathic surgery (if needed)Orthognathic surgery (if needed)