Coupon Accepted Successfully!


Cleft Lip & Palate

  1. Epidemiology
    1. a. Cleft lip with palate is most common (45%)
      b. Cleft palate 40%
      c. Cleft lip 15% 
    2. Cleft lip and palate represents the second most frequently occurring congenital deformity (after clubfoot deformity).
    3. 1/700 overall incidence for facial clefting. 
    4. Clefts can be unilateral or bilateral; Left side more common for unilateral
    5. Syndromic clefting accounts for 50-60% pts Cleft lip, cleft palate or both affects approximately 1 in 750.
  2. Embryology
    1. Failure of fusion of medial nasal & lateral maxillary process causes cleft lip.
    2. Failure of fusion of two lateral palatine process causes cleft palate.
    3. Weeks 5 & 6: Maxillary processes grow medially & fuse with frontonasal process
    4. Failure here > cleft lip +/- primary (anterior) palate
    5. Weeks 7 & 8: Tongue descent, migration & fusion of palatal shelves
    6. Failure here > cleft secondary (posterior) palate (Pierre-Robin, & other)
  3. Anatomy
    1. The palatine processes of the maxilla and horizontal lamina of the palatine bones form the hard palate.
    2. Its blood supply is mainly from the greater palatine artery.
    3. The nerve supply is via the anterior palatine and nasopalatine nerves.
    4. The soft palate is a fibromuscular shelf made up of several muscles attached like a sling to the posterior portion of the hard palate.
    5. It closes off the nasopharynx by tensing and elevating, thereby contacting Passavants ridge posteriorly.
    6. The soft palate consists of the tensor veli palatini, the levator veli palatini, the musculus uvulae, the palatoglossus, and palatopharyngeus muscles.
    7. CN V supplies the tensor veli palatini, while CN IX and CN X innervate the others.
    8. The levator veli palatini is the primary elevator of the palate.
Examples of Unilateral and Bilateral Cleft Lip
Normal roof of mouth One-sided cleft lip Two-sided cleft lip
Examples of cleft palate
Cleft of back of soft palate Complete cleft of soft palate Cleft of soft and hard palates Complete cleft of lip and palate
  1. Etiologies
    1. Idiopathic
    2. Teratogens: ethanol (FAS), anti-convulsants, steroids, chemo, excess Vita A
    3. Maternal / intra-uterine conditions: infant of diabetic mom, amniotic bands
    4. Chromosomal abnormalities, monogenic causes (AR, AD, XL)
    5. Pierre – Robinson Syndrome:
      1. Microganathia
      2. Cleft palate
      3. Glosoptosis
  2. Genetics
    1. Nonsyndromic inheritance of facial clefting is multifactorial.
    2. Familial inheritance of both cleft lip and palate occurs with varying frequency, depending on whether a parent or sibling is affected.
    3. Common syndromes with cleft palates include Apert's, Stickler's and Treacher Collins. Van der Woude's and Waardenberg's syndromes are associated with cleft lip with or without cleft palate.
  3. Diagnosis -- Newborn Physical Exam
    1. Inspect lip & oropharynx
    2. Palpate palate with gloved finger (submucous cleft is not visible, but can be felt in bony palate underlying mucous membranE.. This diagnosis is often missed until later childhood when speech problems arise.
  4. Newborn Feeding
    1. Isolated cleft lip rarely causes feeding problems
    2. Cleft lip & palate or palate alone may require special nipples, occluders.
  5. Surgical Treatment
    1. Age of repair:
      Cleft lip – 6 months
      Cleft palate – soft – 6 months
      Cleft palate – hard – 12 - 18 months
      Cleft lip with palate – stage 1 - lip and soft palate repair at 6 months
      Stage 2 – hard palate – 12 to 18th months
    2. Many surgical technique available – lip – milard rotational advancement Flap Technique.
      Palate – V-Y platoplasty
    3. Cleft lip: "Rule of Tens" -- ten weeks, ten pounds, hemoglobin of 10
    4. Cleft palate: around 1 year, before speech develops
    5. Subsequent surgical revisions required for cleft lip after one year of primary repair as child grows
    6. Palatal repair- repaired at approximately 9-12 months
    7. Secondary repair- if needed- repaired at approximately 4-6 years
    8. Alveolar cleft- repaired at 8-10 years
    9. Rhinoplasty if needed required at 16 – 18 years.
  6. Associated Problems in Childhood
    1. ENT problems: often requires ear tubes
    2. Hearing loss (cleft palatE. conductive as well as sensory neural deafness: Cleft palate is very often associated with eustachian tube dysfunction due to an abnormal insertion of the levator and tensor veli palatini muscles into the posterior margin of the hard palate. In addition to middle ear effusion, the patients also appear to have an increased incidence of cholesteatoma (7%).
    3. Indications for myringotomy and tube insertion include a significant conductive hearing loss or persistent
    4. middle ear effusion, recurrent otitis media, or tympanic membrane retraction.
    5. Speech problems, often speech therapy (cleft palatE.: It is estimated that 75% of patients have
    6. velopharyngeal competence following primary cleft palate surgery, and this can be increased to 90-95% with directed secondary procedures.
    7. Dental problems, usually orthodontics – Hypo or hyper dentation.
    8. Multi-disciplinary cranio-facial teams address child's multiple needs
  7. Airway problems:
    1. May arise in children with cleft palates, especially those with concomitant structural or functional anomalies. eg, Pierre-Robin sequence is the combination of micrognathia, cleft palate, and glossoptosis. 
  8. Van der Woude Syndrome (VWS)
    About three percent of people with a cleft have VWS. VWS is inherited in an autosomal dominant pattern. Features of VWS include:
    1. Mounds or depressions (pits) on the lower lip
    2. Cleft lip, with or without cleft palate
    3. Cleft palate alone
    4. Missing teeth

Test Your Skills Now!
Take a Quiz now
Reviewer Name