cleft palate
TRANSCRIPT
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The Cleft Palate
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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
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Case Study
Cleft palateCleft palate
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Incidence
Cleft lipCleft lip 1/1000 live births1/1000 live births
Cleft palateCleft palate 1/2000 live births1/2000 live births
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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
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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
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Case Study
MicrognathiaMicrognathia
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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
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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
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The NICU team consults ENT…
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What might your flexible fiberoptic laryngoscopy show?
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Doctor, what is in your differential diagnosis?EnvironmentalEnvironmental AlcoholAlcohol Tobacco smokeTobacco smoke PhenytoinPhenytoin Retinoic acidRetinoic acid Folate DeficiencyFolate Deficiency
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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
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Syndromes
Robin Sequence Robin Sequence MicrognathiaMicrognathia GlossoptosisGlossoptosis Cleft palateCleft palate Associated Associated
abnormalities - 52% to abnormalities - 52% to 83%83%
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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.
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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
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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
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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.
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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
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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
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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
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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
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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
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Latham Appliance
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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.
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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
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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
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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
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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)