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"

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Ontology: Cleft Lip (C0008924)

Definition (NCI) A congenital abnormality consisting of one or more clefts (splits) in the upper lip, which may be accompanied by a cleft palate; it is the result of the failure of the embryonic parts of the lip to fuse.
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.
Concepts Congenital Abnormality (T019)
MSH D002971
ICD9 749.10, 749.1
ICD10 Q36 , Q36.9
SnomedCT 204609007, 204606000, 156941008, 80281008
LNC LA14411-5
English Cleft Lip, Cleft Lips, Harelip, Harelips, Lip, Cleft, Lips, Cleft, CLEFT LIP, HARELIP, Cleft lip, unspecified, hare lip, cleft lip (physical finding), cleft lip, cleft lip (diagnosis), Lip cleft, Hare lip, Cleft lip NOS, congenital fissure of lip, Cleft Lip [Disease/Finding], Cleft lip NOS (disorder), Cleft lip, unspecified (disorder), Cleft lip, Cheiloschisis, Congenital fissure of lip, Labium leporinum, CL - Cleft lip, Cleft lip (disorder), harelip, cheiloschisis, cleft; lip, labium leporinum, lip; cleft, lip; fissure, congenital, Cleft lip, NOS
French FENTE LABIALE, Chéiloschisis, Bec-de-lièvre, non précisé, BEC-DE-LIEVRE, Bec-de-lièvre, Fente labiale
German LIPPENSPALTE, Hasenlippe, Lippenspalte, unspezifisch, Lippe, gespalten, HASENSCHARTE, Cheiloschisis, Hasenscharte, Lippenspalte, Labium leporinum
Portuguese LABIO LEPORINO, Queilosquize, Lábio leporino NE, Fenda labial, Fissura Labial, Lábio leporino, Fenda Labial, Lábio Leporino
Spanish LABIO LEPORINO, Labio leporino, Fisura de labio, Labio hendido de localización indeterninada, Queiloquisis, labio leporino, SAI, labio leporino, no especificado, labio leporino, no especificado (trastorno), labio leporino, SAI (trastorno), Labio Hendido, fisura congénita de labio, labio leporino (trastorno), labio leporino, labio leporinum, queilosquisis, Labio hendido, Labio Leporino
Dutch gespleten lip, niet-gespecificeerd, cheiloschisis, hazenlip, gespleten; lip, lip; fissuur, congenitaal, lip; gespleten, gespleten lip, Gespleten lip, Hazenlip, Lipspleet, Spleet, lip-
Italian Labioschisi, non specificata, Cheiloschisi, Labbro leporino, Labioschisi
Japanese 口唇裂、詳細不明, コウシンレツ, トシン, コウシンレツショウサイフメイ, 口唇披裂, 口唇裂, みつくち, 兎唇, 唇披裂, 唇裂
Swedish Läppspalt
Czech rozštěp rtu, Rozštěp rtu, blíže neurčený, Rozštěp rtu, Zaječí ret, Cheiloschíza, cheiloschisis
Finnish Huulihalkio
Russian GUBY RASSHCHELINA, NEZARASHCHENIE GUBY, ZAIACH'IA GUBA, KHEILOSKHIZIS, ГУБЫ РАСЩЕЛИНА, ЗАЯЧЬЯ ГУБА, НЕЗАРАЩЕНИЕ ГУБЫ, ХЕЙЛОСХИЗИС
Korean 입술갈림증
Croatian RASCJEP USNE
Polish Rozszczep wargi, Warga zajęcza
Hungarian Nyúlajak, Ajak sulcus, Nyúlajakl, Ajakhasadék, Ajakhasadék, nem meghatározott, Cheiloschisis
Norwegian Hareskår, Haremunn, Labium leporinum, Medfødt kløft i overleppe, Leppespalte

Ontology: Cleft Palate (C0008925)

Definition (NCI) A congenital abnormality consisting of a fissure in the midline of the hard and/or soft palate; it is the result of the failure of the two sides of the palate to fuse during embryonic development.
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.
Concepts Congenital Abnormality (T019) , Disease or Syndrome (T047)
MSH D002972
ICD9 749.00, 749.0
ICD10 Q35 , Q35.9
SnomedCT 204593009, 156940009, 268196005, 204605001, 87979003, 253986002
LNC LA14412-3
English Cleft Palates, Palate, Cleft, Palates, Cleft, CLEFT PALATE, Cleft palate, unspecified, Cleft Palate, cleft palate (diagnosis), cleft palate, Palate cleft, Cleft palate NOS, Cleft Palate [Disease/Finding], secondary cleft palate, cleft palates, Cleft palate, unspecified (disorder), (Palatoschisis) or (unspecified cleft palate) (disorder), Cleft palate NOS (disorder), (Palatoschisis) or (unspecified cleft palate), Cleft-palate, Cleft palate, Palatoschisis, Uranoschisis, CP - Cleft palate, Cleft palate (disorder), Palatoschisis (disorder), cleft; palate, hard and soft, cleft; palate, soft with hard, cleft; palate, deformity; palate, cleft (congenital), palate; cleft, palatoschisis, Cleft palate, NOS
German GAUMENSPALTE, Gaumenspalte, unspezifisch, Gaumen, gespalten, Gaumenspalte, Palatoschisis
Dutch gespleten verhemelte, niet-gespecificeerd, verhemeltespleet, palatoschisis, gehemelte; gespleten, gespleten; gehemelte, hard met zacht, gespleten; gehemelte, zacht met hard, gespleten; gehemelte, misvorming; gehemelte, gespleten (congenitaal), Gespleten gehemelte, niet gespecificeerd, gespleten verhemelte, Gehemeltespleet, Gespleten gehemelte, Gespleten verhemelte, Palazoschizis, Schizis, palazo-, Verhemeltespleet
French Fente palatine, non précisée, Division du voile du palais, Fissure du palais, Palatoschisis, Fissure palatine, FISSURE PALATINE, Fente palatine, Division palatine
Italian Palatoschisi, non specificata, Palatoschisi
Portuguese Fenda palatina NE, FENDA PALATINA, Palatosquise, Fenda Palatina, Fenda palatina, Fissura Palatina
Spanish Paladar hendido de localización indeterminada, Hendidura palatina, FISURA PALATINA, Fisura del Paladar, Palatosquisis, uranosquisis, SAI, uranosquisis, SAI (trastorno), hendidura palatina, no especificada (trastorno), hendidura palatina, no especificada, Paladar Hendido, fisura palatina (trastorno), fisura palatina, paladar hendido, palatosquisis (trastorno), palatosquisis, uranosquisis, Paladar hendido
Japanese 口蓋裂、詳細不明, 口蓋裂, コウガイレツ, コウガイレツショウサイフメイ
Swedish Gomspalt
Czech rozštěp patra, Rozštěp patra, Rozštěp patra, blíže neurčený, Palatoschiza, rozštěp měkkého patra, uranoschisis
Finnish Suulakihalkio
Russian PALATOSKHIZIS, NEBA RASSHCHELINA, НЕБА РАСЩЕЛИНА, ПАЛАТОСХИЗИС
Korean 입천장갈림증, 상세불명의 입천장갈림증
Croatian RASCJEP NEPCA
Polish Rozszczep podniebienia
Hungarian Szájpad sulcus, Sulcus szájpad, nem meghatározott, Palatoschisis, Szájpadhasadék
Norwegian Ganespalte