Cleft Lip and Palate

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Anatomy of the palate:

  • Levator veli palatini: passes posterior to hamulus and creates muscular sling
    • Runs through palate to elevate palate
    • Innervated by the pharyngeal plexus
  • Palatoglossus runs through anterior tonsillar pillar to depress the palate
  • Tensor veli palatini: innervated by medial pterygoid nerve from the trigeminal nerve. Descends from base of skull adjacent to eustachian tube, courses around hamulus of pterygoid, forms broad aponeurosis with contralateral muscle within anterior soft palate
    • Contributes to eustachian tube pressure modulation
  • Palatopharyngeus: responsible for controlling velopharyngeal sphincter by controlling size, shape, and position
  • Superior pharyngeal constrictor: pharyngeal closure during swallowing and speech.

Incidence of cleft lip and palate:

  • One affected sibling with isolated cleft lip: 2.5% risk of second sibling to have cleft lip
  • One affected sibling with unilateral cleft lip and palate (ULCLP): 4.2% of having a second child with cleft lip and palate.
  • One sibling with BCLP: 6.7% risk of having a second child with cleft lip and palate.
  • Two affected siblings: 10% risk of having a third child with cleft lip and palate.
  • Parent only: 3-4.7% risk of child having isolated cleft lip and palate.
  • Parent and sibling cleft: 17% risk of second child having cleft lip and palate.
  • Cleft lip and palate (CLP) has a higher incidence than cleft palate (CP) alone. CLP have a higher male predominance, higher asian incidence, less syndromic association than cleft palate alone (15 vs 50%).

Congenital Anomalies of cleft lip and palate:

  • Van der Woude Syndrome: common syndrome associated with cleft lip with or without cleft palate
    • Autosomal dominant (50% risk in offspring)
    • Can present as lip pits to bilateral complete cleft lip and palate
    • Due to gene IRF6
  • 22q11.2 or velocardiofacial syndrome: overt or submucous clefting of the palate, hypotonia, cardiac abnormalities, hypocalcemia
    • High suspicion in children with cardiac abnormalities and cleft palate abnormalities.
    • Distinct facial appearance: elongated face with a wide nose, small ears, lower facial muscle tone
    • Detected by FISH for deletion of 22q11.2.
    • Has higher incidence overall of VPI (velopharyngeal insufficiency) due to decreased oropharyngeal tone and learning differences.
  • Pierre Robin presents as a triad: glossoptosis, retrogenia, and respiratory distress
    • Can have high arched or a U shaped cleft.
    • Initial management includes placing neonate in prone position to relieve respiratory distress.
  • Waardenburg syndrome: associated with white forelock and sensorineural hearing loss

Timing of treatment of cleft lip and palate:

  • Nasoalveolar molding (applied shortly after birth)
  • Cleft lip repaired at 3-6 months
  • Cleft palate repair are performed at 9-12 months
  • Alveolar bone grafting 7-12 years
  • LeFort/Rhinoplasty should be performed in late adolescence after skeletal maturity

Nasoalveolar molding: goal is to reposition and approximate the alveolar segments and reshape the nasal cartilage

  • Corrects columella height 
    • In bilateral cleft lip the columella is short

Cleft Lip: developmentally forms at 5-6 weeks gestation.

  • Unilateral cleft lip results from failed fusion of the medial nasal prominence and maxillary prominence
    • Lateral nasal processes form nasal alae
    • Medial nasal prominence form nasal tip, columella, philtrum and premaxilla
    • Frontonasal process: forehead, bridge of nose, root of nose
  • Oblique facial cleft results from failure of fusion of the lateral nasal prominence and maxillary prominence
  • Lateral oral commissure cleft is produced by failed fusion of the mandibular and maxillary prominences
  • Median cleft lip results from failed fusion of the medial nasal prominences
  • Cleft lip techniques:
    • Rotation advancement: can result in short upper lip
      • The non cleft side is rotated, cleft side advanced
      • C flap: columellar flap made from non-cleft side to rotate into columella and lengthen it
      • D flap: or alar base flap 
      • M flap: medially based flap for rotation
      • L flap: from the surface of the lateral lip element used to line the lateral nasal vault between internal mucosa and hair bearing skin
    • Bilateral cleft lip repair may result in a wide philtrum
    • Scar correction aimed at 12 months or later
  • Nasal deformity of cleft lip: inferior border of bony septum is deviated to the cleft side, while anterior nasal spine is deviated to the noncleft side; decreased sagittal projection of pyriform sinus and dentoalveolar arch
    • Ala is lateral inferior and posterior 
    • Premaxilla is rotated and projected outward, laterally maxillary element is collapsed and retro positioned
    • Columella will be short
    • Lip deformities: philtrum termination of the orbicularis oris muscle in the lateral lip is shortened at the margin of the cleft and the muscle inserts on alar wing.
  • Abbe flap: or lip switch flap used to create a functional philtrum in patients who have tightness of the upper lip following cleft lip repair
    • Pedicled on the submucosal labial artery of the lower lip
    • Pedicle divided at 2-4 weeks
    • Normal philtrum is 10-15mm wide

Cleft Palate:

  • Produced by failure of fusion of the medial and lateral palantine processes in weeks 7-8 gestation
  • Veau classification: 
    • Veau I: incomplete cleft of soft palate
    • Veau II: cleft involves soft and hard palate
    • Veau III: complete unilateral cleft lip and palate
    • Veau IV: bilateral cleft lip and palate
  • Isolated cleft palate alone carries 50% risk of other congenital anomalies 
  • In cleft palate the levator veli palatini is clefted and courses sagittally in an anteroposterior direction and is attached abnormally to posterior edge of hard palate. This is repaired in a cleft palate repair.
  • 90% of those with cleft palate have chronic infection and effusion of the middle ear which can result in hearing loss
    • Remember that repair of levator during cleft palate repair can help improve eustachian tube function.
  • Techniques: Veau, Wardill, Kilner; Furlow palatoplasty; Bardach palatoplasty
    • Attempt to reestablish normal anatomy: repair uvulae, levator veli palatini, repair nasal lining, releases abnormal attachments of tensor veli palatini.
  • Submucous cleft palate: bifid uvula, notching of the hard palate, and midline thinning of the posterior palate (zona pellucida which is formed by two parallel bulges of levator muscle cleft)
    • Transillumination due to an absent levator muscle sling. This can present as velopharyngeal insufficiency after tonsillectomy
    • 10-15% of patients will demonstrate velopharyngeal insufficiency with the rest being asymptomatic. It is important to evaluate speech in these patients.
      • Treatment includes restoration of normal anatomy with palatoplasty (typically furlow)
      • Levator will still be clefted and should be repaired.

Bilateral cleft lip and palate:

  • Asymmetric bilateral cleft lip should be repaired simultaneously for best outcomes

Dental Arch:

  • High prevalence of dental anomalies with cleft lip and palate
    • Agenesis is the most common anomaly found in 50% of patients.
    • Most common affected tooth is the permanent lateral incisor on the cleft side.
    • Supranumary teeth are second most frequent anomaly
  • Unrepaired alveolar cleft will reveal a posterior crossbite of maxillary dentition (due to collapse of maxillary arch).
  • Alveolar bone grafting stabilizes dental arch
    • Performed to support tooth health for permanent canine before the canine has fully erupted
      • Too early grafting bad for tooth growth
      • Should be performed in transitional dentition
      • Begins with palatal expansion then grafting
    • Allows for bone support for subsequent placement of endosseous titanium implant; bone stock required for implant is 10-15mm
    • Typically used by iliac bone crest cancellous bone rather than demineralized bone matrix
      • Disadvantages include donor site; failure rates are equivalent.
  • Patients will frequently have absence of teeth in the alveolar cleft or teeth that may be abnormal and require removal
    • Lateral incisors most commonly affected

VPI: velopharyngeal insufficiency is the inability to completely close the velopharyngeal sphincter and is diagnosed on nasoendoscopy. Signs include hypernasal speech.

  • Velopharyngeal port is bordered by the velum anteriorly, lateral pharyngeal walls, and posterior pharyngeal wall
  • Poor lateral wall motion or coronal closure pattern
    • Treatment includes sphincter pharyngoplasty which bring lateral walls more centrally
    • Sphincter pharyngoplasty rotates the posterior tonsillar pillars as superiorly based flaps to line the posterior pharynx and narrowing velopharyngeal sphincter
      • includes harvest of palatopharyngeaus muscle
      • supplied by pharyngeal plexus through the vagus nerve
    • Less nasal obstruction than pharyngeal flap
  • Poor central wall motion or sagittal closure pattern
    • Treatment includes pharyngeal flap: 
      • Harvested as superiorly based flap typically, layers include mucosa, superior constrictor muscle, and buccopharyngeal flap (pretracheal fascia), anterior prevertebral fascia is left (surrounds the cervical column and musculature).
      • Width of the flap based on lateral wall motion
  • Shortened palatal length with good palatal elevation (indicative of anterior placement of levator veli palatini). This can be treated to conversion to furlow palatoplasty
    • Particularly good for patients who have concurrent sleep apnea as pharyngoplasty may worsen sleep apnea.
    • Can be used with associated submucous cleft palates or following conventional pushback palatoplasty.
    • Preoperative velopharyngeal gap (determined by pre-op nasoendoscopy) is the most important determinant of velar competence after a furlow palatoplasty.
  • Very small central gap after adenoidectomy
    • Palatal soft tissue augmentation
  • Poor wall movement: should treat with palatal elevator which functions to elevate the soft palate. 
  • Palatal obturator used to block oronasal fistulas
  • Hyponasal speech: describes the sound production when not enough air gets through the velopharyngeal sphincter resulting in muffled nasal voice
    • May result from pharyngoplasty
    • Should evaluate for speech abnormalities and obstructive sleep apnea
  • Sleep Apnea: severe midface hypoplasia + pharyngoplasty can lead to obstructive sleep apnea as confirmed on polysomnogram
    • Initial treatment is with trial of CPAP (continuous positive airway pressure)
    • LeFort I may be performed in adolescence, but in children results in permanent injury to unerupted teeth
    • Tracheostomy will bypass his midface level obstruction and used if all other options fail

Maxillary Hypoplasia: appreciated by an acute SNA angle (normal 80-82), less signifies hypoplasia. Common in patients with cleft lip and palate.

  • Frequently leads to Class III occlusion
    • Class occlusion based on relationship from mesiobuccal cusp of maxillary first molar to the buccal groove of the mandibular first molar
    • Class I: mesiobuccal cusp of maxillary first molar lies within the buccal groove of mandibular first molar
    • Class II: mesiobuccal cusp of maxillary first molar lies anterior to the buccal groove of mandibular first molar
    • Class III: mesiobuccal cusp of maxillary first molar lies distal or posterior to buccal groove of mandibular first molar
  • Skeletal maturity can be assessed via cephalometric Xray
  • LeFort I: can advance 10mm
    • Can result in changes including midfacial projection and fullness, increased upper lip fullness, decreased upper lip height, decreased depth of nasolabial folds, increased tooth show, nasal tooth rates cephalad, increases nasolabial fold angle
  • Maxillary distraction, for advancing >10mm (use for SNA angle less than normal)
    • Also allows maintenance of current velopharyngeal anatomy
  • Mandibular setback (or sagittal split osteotomy) indicated for when SNB > 77 indicating class III


  • Blood supply to tongue comes from the ventral third (paired lingual arteries).

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