4.3 Cleft Lip and Cleft Palate

Cleft lip with or without cleft palate, and cleft palate alone, are referred to collectively as orofacial clefts. Descriptions for each of these conditions follow. To aid understanding of the individual conditions, the structure of a normal palate is shown in Fig. 4.7.

 

  Fig. 4.7. Normal palate
Normal Palate illustration
Normal palate

Cleft palate (Q35, Q35.1, Q35.3, Q35.5, Q35.59, Q35.9, Q87.0)

Cleft palate is characterized by an incomplete fusion of the secondary palate and can affect the soft and the hard palate (see Fig. 4.8), or only the soft palate. Laterality of cleft palate is difficult to ascertain and some believe it does not exist.

  Fig. 4.8. Cleft hard palate with cleft soft palate
Cleft Palate illustration
Cleft hard palate with cleft soft palate (Q35.59)[1]
Cleft Palate photograph
Cleft hard palate with cleft soft palate (Q35.59)[1]

 

Relevant ICD-10 codes and Royal College of Paediatrics and Child Health (RCPCH) uses
Relevant ICD-10 codes and Royal College of Paediatrics and Child Health (RCPCH) uses:
Q35 Cleft palate (avoid using this general code if more specific information is available) Fissure of palate; Palatoschisis
Q35.1 Cleft hard palate
Q35.3 Cleft soft palate
Q35.5 Cleft hard palate with cleft soft palate
Q35.59 Complete cleft palate, Cleft hard palate with cleft soft palate
Q35.9 Cleft palate, unspecified
Q87.0 Robin (sequence or defect; includes micrognathia, posterior displacement of the tongue, cleft palate)a
Note: most specialists do not agree on the existence of cleft palate laterality, as this defect is the result of a failure of the palatal shelves to fuse in the midline. For this reason, the preferred codes are: Q35.1 Cleft hard palate; Q35.3 Cleft soft palate; Q35.59 Cleft hard palate with cleft soft palate; Q35.9 Cleft palate and Q87.0 Robin (sequence or defect; includes micrognathia, posterior displacement of the tongue, cleft palate).
Exclusions
Exclusions
Q35.7 Cleft uvula, Bifid uvula
Q35.9 Submucous cleft
Q37-Q37.9  Cleft palate with cleft lipb
Q38.5 Absence of uvula
Exclusions
aICD-10 lists Robin sequence as an exclusion from the Q35 series. However, because it is such a common condition, the 87.0 code is suggested.
bCleft palate with cleft lip is included in a separate group (see following).

Cleft lip (Q36, Q36.0, Q36.9, Q36.90, Q36.99)

Cleft lip (see Fig. 4.9) is characterized by a partial or complete fissure of the upper lip. It can be unilateral or bilateral, and can be associated with a cleft of the gum. Cases of cleft lip with a cleft of the primary palate (anterior to the incisive foramen) is coded as cleft lip alone, because clefts of the primary palate involve only the alveolus, and are embryologically related to cleft lip and different from clefts of the secondary palate.

  Fig. 4.9. Cleft lip
Cleft lip unilateral illustration
a. unilateral (Q36.9)
Cleft lip unilateral photograph
b. unilateral (Q36.9)[2]
Cleft lip bilateral photograph
c. bilateral (Q36.0)[3]

 

Relevant ICD-10 codes and RCPCH uses
Relevant ICD-10 codes and RCPCH uses:
Q36 Cleft lip (avoid using this general code if more specific information is available)
Cheiloschisis; Congenital fissure of lip; Harelip; Labium leporinum
Q36.0 Cleft lip, bilateral
Q36.9 or Q36.90  Cleft lip, specified as unilateral
Q36.99 Cleft lip, unspecified
Exclusions
Exclusions
Q36.1 Cleft lip, median
Q37–Q37.9  Submucous cleft
Q37–Q37.9  Cleft palate with cleft lipa
Pseudocleft or microform cleft lip (no associated ICD-10 codes)
Oblique facial clefts (Tessier type facial clefts) (no associated ICD-10 codes)
aCleft palate with cleft lip is included in a separate group (see following).

Cleft palate with cleft lip (Q37, Q37.0, Q37.1, Q37.2, Q37.3, Q37.4, Q37.5, Q37.8, Q37.9, Q37.99)

Cleft palate with cleft lip is characterized as a cleft of the upper lip extending through the primary and secondary palates, with or without extension through the soft palate (see Fig. 4.10).

  Fig. 4.10. Cleft palate with bilateral cleft lip
Cleft palate with unilateral cleft lip (Q37.10)
Fig. 4.20 Cleft palate with unilateral cleft lip
Cleft palate with bilateral cleft lip (Q37.0)[4]

 

Relevant ICD-10 codes and RCPCH uses
Relevant ICD-10 codes and RCPCH uses:
Q37 Cleft palate with cleft lip (avoid using this general code if more specific information is available)
Q37.0 Cleft hard palate with bilateral cleft lip
Q37.10  Cleft hard palate with cleft lip, specified as unilateral
Q37.19  Cleft hard palate with cleft lip, unspecified
Q37.2 Cleft soft palate with bilateral cleft lip
Q37.3 Cleft soft palate with unilateral cleft lip
Q37.4 Cleft hard palate and soft palate with bilateral cleft lip
Q37.5 Cleft hard palate and soft palate with unilateral cleft lip
Q37.59  Cleft hard palate and soft palate with cleft lip, unspecified
Q37.8 Unspecified cleft palate with bilateral cleft lip
Q37.9 Unspecified cleft palate with unilateral cleft lip
Q37.99  Cleft palate with cleft lip, unspecified
Exclusions
Exclusions
Oblique facial clefts (Tessier type facial clefts)
Q04.2 Cases with known or probable holoprosencephaly
Q79.80  Cases with known or probable amniotic band/constriction band presence a
aICBDSR recommends using Q79.80 to identify the presence of an amniotic band. The anomaly is coded as the code for the specific anomaly, as well as the Q79.80 amniotic band code.
  1. Photograph source: courtesy of CDC-Beijing Medical University collaborative project.
  2. Photograph source: courtesy of Jaime Frías MD, USA.
  3. Photograph source: courtesy of Pedro Santiago DMD and Miguel Yanez MD, FACS, USA.
  4. Photograph source: courtesy of Estudio Colaborativo Latino Americano de Malformaciones Congénitas (ECLAMC).