The Cleft Palate Case Study Full term infant, NSVD

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The Cleft Palate
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Case Study

• Full term infant, NSVD
• Respiratory Distress at birth
• Numerous O2 desaturations
• Unable to feed

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Case Study

• Cleft palate

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Incidence

• Cleft lip
• 1/1000 live births
• Cleft palate
• 1/2000 live births

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Classification - Veau

• Clefts of soft palate only
• Clefts of soft and hard palate reaching
  anteriorly to incisive foramen
• Complete unilateral alveolar clefts - generally
  involve the lip as well
• Bilateral alveolar clefts - associated with
  bilateral clefts of the lip

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Classification

• A - Incomplete cleft of the secondary palate
• B - Complete cleft of the secondary palate
• C- Incomplete cleft of the primary and secondary
  palates
• D - Unilateral complete cleft of the primary and
  secondary palates
• E - Bilateral complete cleft of the primary and
  secondary palates

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Case Study

• Micrognathia

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Case Study

• Glossoptosis
• Increase in frequency of O2 desaturations that
  improve when neonate is placed in prone position

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Timeline of Care

• Neonatal to one month of age
• Refer to cleft palate team
• Medical diagnosis
• Genetic counseling
• Address psychosocial issues
• Airway management
• Provide feeding instructions
• Monitor growth
• Hearing 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?

• Environmental
• Alcohol
• Tobacco smoke
• Phenytoin
• Retinoic acid
• Folate Deficiency

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Doctor, what is in your differential
diagnosis?Syndromes

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

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Syndromes

• Robin Sequence
• Micrognathia
• Glossoptosis
• Cleft palate
• Associated abnormalities - 52 to 83

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Syndromes

• Velocardiofacial syndrome
• Overlaps with DiGeorge syndrome and conotruncal
  anomaly face syndrome
• CATCH 22 cardiac anomalies (usually
  conotruncal), abnormal facies, thymic disorders,
  cleft palate, and hypocalcemia caused a deletion
  of chromosome 22 at the q11.2 region.

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Syndromes

• Stickler syndrome
• Most common syndrome found in children with Robin
  Sequence - 14 to 34 of cases
• Sensorineural hearing loss
• Myopia - Risk of retinal detachment and early
  cataracts
• Hypermobilityof joints
• Stiffness and premature osteoarthritis

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Airway management

• Prone positioning, which allows the tongue to
  fall away from the posterior pharyngeal wall.
• Gavage feeding may be needed in such cases
• Glossopexy or lip-tongue adhesion in infants
• Tracheotomy
• Mandibular distraction osteogenesis

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Seiberts Technique Lip Adhesion

• Medially and laterally based rectangular flaps
  from the margin of the cleft
• Tension-bearing suture through the membranous
  septum.
• Unilateral lip adhesion A, landmarks and
  incisions B, flaps elevated and undermined C,
  placement of 3-0 nylon retention suture D, final
  suturing.

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Airway Management

• One hundred seven patients with Pierre Robin
  Sequence
• 74 (69.2) - positioning alone
• 29 - tongue lip adhesion
• Airway obstruction relieved in 20 (83.3)
• 10 - Tracheostomy
• Including six (20.7) who initially underwent a
  TLA

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TimelineOne to Four months

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

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Incidence of Eustachian Tube Dysfunction

• Middle ear disease - 22 to 88
• Conductive hearing loss and chronic suppurative
  otitis media may result
• Repeated tympanostomy tube placement

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Why do cleft kids have eustachian tube
dysfunction?

• Paratubal cartilage abnormalities
• Hypoplasia of the lateral cartilage relative to
  the medial cartilage
• Abnormal curvature of the eustachian tube lumen
• Cephalometric 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 dysplasia

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

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Latham Appliance
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Cleft Lip RepairRule of 10s

• Surgery gt 10 weeks old
• Weight gt 10 pounds
• Hemoglobin level gt 10 g/dL

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Cleft Lip RepairGoals

• Bridge the cleft
• Create a complete muscular sling around the
  entire circumference of the oral cavity
• Approximate cleft edges
• Maintain Cupid's bow and philtral dimple
• Align vermillion border
• Create an intact nasal floor and sill
• Produce symmetry of the alar base and columella
• Reconstituting the circumferential integrity of
  the orbicularis oris muscle

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Cleft Lip Repair - Types

• Millard Repair - Rotation advancement technique
• Randall - Graham - Triangular flap interposition
• Rose - Thompson - Straight line repair, Risk of
  vertical contracture

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Millard Repair

• Downward and lateral rotation of the medial
  segment of the cleft lip combined with the medial
  advancement of the lateral cleft segment into the
  defect
• Bridges the gap
• Maintains the rotation flap in position
• Maintains the amount of vertical height gained by
  the rotation flap and back cut

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TimelineFive to Fifteen Months

• Monitor feeding, growth, and development
• Repair cleft palate
• Monitor ears and hearing
• Instructions in oral hygiene

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Cleft Palate Repair - Timing

• Dorf 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

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Cleft Palate Repair

• V-Y Pushback
• Two Flap Palatoplasty
• Four Flap Palatoplasty
• Schweckendicks Primary Veloplasty
• Furlow Palatoplasty

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Cleft Palate RepairV-Y Pushback

• 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 closed
• Improved speech results compared with bipedicled
  techniques
• Indicated for incomplete clefts

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Cleft Palate RepairSchweckendicks Primary
Veloplasty

• Incisions made in soft palate
• Muscle bundles released from the posterior hard
  palate and rotated
• Reconstruction of levator sling
• Closure of mucosal layers separately

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Cleft Palate RepairFurlow Palatoplasty

• Lengthens the soft palate
• Reconstructs the muscle sling.
• Also commonly used to correct velopharyngeal
  insufficiency in patients with submucous cleft
  palate
• Speech outcomes are improved compared with other
  palatoplasty techniques.

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Cleft Palate RepairComplications

• Oronasal fistula - 8.7 to 23
• Sites of fistulization are typically the
  anterior hard palate and the junction of the hard
  and soft palate.
• Velopharyngeal insufficiency

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TimelineTwo to Five years old

• Monitor speech and language development
• Manage velopharyngeal insufficiency
• Monitor ears and hearing ear tubes if indicated
• Assess development and psychosocial needs
• Consider lip/nose revision before school

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TimelineSix to Eleven years old

• Monitor speech and language manage
  velopharyngeal insufficiency
• Orthodontic evaluation and treatment
• Alveolar bone graft
• Monitor school and psychosocial needs

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TimelineTwelve to Twenty One years old

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