Cleft Lip

Aka: Cleft Lip, Cleft Palate

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II. Epidemiology

  1. Incidence
    1. Cleft Lip: 1 in 750 white births
    2. Isolated Cleft Palate: 1 in 2500 white births

III. Risk Factors

  1. Family History (e.g. van der Woude Syndrome)
    1. Recurrence rate: 2-6% depending on history
  2. Ethnicity
    1. Asian patients have highest risk
    2. Black patients have lowest risk
  3. Epilepsy and maternal anticonvulsant use

IV. Pathophysiology

  1. Embryonic Timing of lip and Palate defects
    1. Occurs prior to 37 days post-conception
  2. Cleft Lip
    1. Failed medial nasal and Maxillary closure
    2. Extension to Cleft Palate in 42% of cases
  3. Isolated Cleft Palate
    1. Failed fusion of palatal shelves

V. Signs

  1. Cleft Lip (with or without Cleft Palate)
    1. Variable involvement
      1. May involve only vermilion border
      2. Some cases extend into Palate and floor of nose
    2. May be associated with tooth abnormalities
    3. Unilateral or bilateral cleft
    4. Often associated with Columella abnormality
  2. Isolated Cleft Palate
    1. Midline defect starts at uvula
    2. May involve soft and Hard Palate and incisive foramen

VI. Associated Conditions

  1. Pierre Robin Syndrome (Pierre Robin Sequence)
    1. Micrognathia
    2. U-Shaped Cleft Palate
    3. Glossoptosis
  2. EEC Syndrome
    1. Ectrodactyly (Split hand and foot)
    2. Ectodermal dysplasia
    3. Cleft Lip and Palate
  3. Trisomy 13 Syndrome
    1. Holoprosencephaly
    2. Amnion Rupture Sequence
  4. Hypothalamus and Pituitary abnormalities
    1. Isolated hypothalamic Hormone deficit
    2. Panhypopituitarism
    3. Septo-optic dysplasia
    4. Kallmann's Syndrome

VII. Management: Feeding

  1. Breast Feeding infant
    1. See Lactation for Infant with Cleft Lip or Palate
  2. Bottle Feeding infant
    1. Consider plastic obturator to cover Palate
    2. Soft artificial nipple with large openings
    3. Squeezable bottle

VIII. Management: Surgery

  1. Perioperative Concerns
    1. Pre-operative criteria
      1. No signs of infection
      2. Child well hydrated
    2. Post-operative care
      1. Gentle nasal aspiration
      2. Fluid intake only for first 3 weeks after surgery
      3. Child must be kept from touching surgical site
  2. Cleft Lip Repair
    1. Z-Plasty repair with staggered Sutures minimizes scar
    2. Repaired at age 3 months
    3. Revision may be needed at age 4-5 years
  3. Corrective Nasal Surgery
    1. Repaired at lip repair or at adolescence
  4. Cleft Palate Repair
    1. Timing of repair variable based on type of defect
    2. Usually repaired before age 1 for normal speech
    3. Contoured speech bulb if surgery delayed after age 3

IX. Complications

  1. Recurrent Otitis Media
  2. Hearing Loss
  3. Tooth malposition requiring orthodontics
  4. Speech deficits (may persist after repair)
    1. Difficulty with explosive sounds: "p, b, d, t, h, y"
    2. Difficulty with hissing sounds: "s, sh, ch"

X. References

  1. Behrman (2000) Nelson Pediatrics, p. 1111-12
  2. Kirschner (2000) Otolaryngol Clin North Am 33:1191-215 [PubMed]
  3. Weintraub (2000) Otolaryngol Clin North Am 33:1171-89 [PubMed]

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