Neonatology Book

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Cleft LipAka: Cleft Palate

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  1. See Also
    1. Lactation for Infant with Cleft Lip or Palate
  2. Epidemiology
    1. Incidence
      1. Cleft Lip: 1 in 750 white births
      2. Isolated Cleft Palate: 1 in 2500 white births
  3. 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
  4. 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
  5. 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
  6. 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
  7. 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
  8. 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
  9. 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"
  10. References
    1. Behrman (2000) Nelson Pediatrics, p. 1111-12
    2. Kirschner (2000) Otolaryngol Clin North Am 33:1191
    3. Weintraub (2000) Otolaryngol Clin North Am 33:1171

Cleft Lip (C0008924)

Definition (MSH)Congenital defect in the upper lip where the maxillary prominence fails to merge with the merged medial nasal prominences. It is thought to be caused by faulty migration of the mesoderm in the head region.
Definition (CSP)congenital defect in the upper lip where the maxillary prominence fails to merge with the merged medial nasal prominences; thought to be caused by faulty migration of the mesoderm in the head region.
ConceptsCongenital Abnormality (T019)
ICD9749.1, 749.1, 749.10
MSHD002971
EnglishCheiloschisis, CL - Cleft lip, Cleft Lip, Cleft Lips, Congenital fissure of lip, hare lip, HARELIP, Harelips, Labium leporinum, LIP CLEFT
Spanishfisura congenita de labio, labio leporino, labio leporinum, queilosquisis
Parent Conceptscongenital oral/facial/cranial defect (C0596372), Congenital Abnormality (C0000768), Oral cavity (C0226896), Cleft Lip (C0008924), Cleft palate with cleft lip (C0158646), Lip Diseases (C0023760), Mouth Abnormalities (C0026633), Congenital anomaly of lip (C0431561), Duplicate concept (C1274013)
SourcesAOD, COSTAR, CSP, CST, DXP, ICD9CM, MSH, MTHICD9, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)


Cleft Palate (C0008925)

Definition (MSH)Congenital fissure of the soft and/or hard palate, due to faulty fusion.
Definition (CSP)congenital fissure of the soft and/or hard palate, due to faulty fusion.
ConceptsCongenital Abnormality (T019) , Disease or Syndrome (T047)
ICD9749.0, 749.00
MSHD002972
EnglishCleft hard and soft palate, Cleft of secondary palate, CLEFT PALATE, Cleft Palates, CP, CP - Cleft palate, CPI, PALATE CLEFT, Palatoschisis, Uranoschisis, Uranostaphyloschisis
Spanishfisura palatina, paladar hendido, palatosquisis, uranosquisis, uranostafilosquisis
Parent ConceptsCongenital deformity of face (C0266617), congenital oral/facial/cranial defect (C0596372), Congenital Abnormality (C0000768), Oral cavity (C0226896), Cleft Palate (C0008925), Cleft palate with cleft lip (C0158646), Jaw Abnormalities (C0022360), Mouth Abnormalities (C0026633), Nasopharyngeal Diseases (C0027438), Respiratory System Abnormalities (C0035238), Congenital anomaly of upper respiratory system (C0265734), Congenital digestive system anomalies (C0266015), Disorder of oral soft tissues (C0266998), Soft tissue lesion (C0410013), Congenital anomaly of head (C0520555), Oropharyngeal disorders (C0553694), Congenital abnormality of nose and nasopharynx (C0585356), Congenital anomaly of palate (C1290576), Congenital anomaly of digestive organ (C1290614), Disorder of palate (C1290785), Ambiguous concept (C1274012), Duplicate concept (C1274013)
SourcesAOD, COSTAR, CSP, CST, DXP, ICD9CM, LCH, MSH, MTH, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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