Cleft Lip Only

Cleft lip is characterized by a partial or complete fissure of the upper lip. It can be unilateral (Fig. 24, panels a and b) or bilateral (Fig. 24, panel c). The cleft lip can extend through the gum, but not beyond the incisive foramen. If the cleft extends further backwards into the secondary palate it becomes a different entity – a cleft lip with cleft palate.

Fig. 24. Cleft lip

Fig. 24. Cleft lip

Photograph source: b: Dr Jaime Frías (EE. UU.); c: Dr Pedro Santiago and Dr Miguel Yanez (EE. UU.).

Fig. 25. Anatomy of the lip and palate

Fig. 26. Median cleft lip

Fig. 25. Anatomy of the lip and palate
Fig. 26. Median cleft lip

Photograph source: Prof Tahmina Banu, Chittagong Research Institute for Children Surgery, Bangladesh.

Diagnosis

Prenatal. Cleft lip can be suspected prenatally but can easily be missed or misdiagnosed. Cases identified or suspected prenatally should be confirmed postnatally before inclusion in a surveillance programme.

Postnatal. Cleft lip is easily recognized on physical examination after delivery. Check the palate carefully to rule out cleft lip with cleft palate.

Clinical and epidemiologic notes

Rarer conditions that can be confused with typical cleft lip are median cleft lip and atypical or Tessier type clefts.
  • Median cleft lip can be distinguished from bilateral cleft lip by examination of the philtrum. In median cleft lip, there is no remnant of tissue (philtrum) in the area below the nasal septum. In bilateral cleft lip a midline remnant of tissue is always present (Fig. 24, panel c; also, see below for more information on median cleft lip).
  • Atypical or Tessier type craniofacial clefts are a group of clefting defects that involve the cranial and/or facial skeleton. Unlike cleft lip, where the cleft extends up towards the nose, the 14 various Tessier’s clefts extend through radiating axes of facial and cranial bones, including towards the eye or nasolacrimal canal, or even more laterally towards the ear.
  • Cleft lip is occasionally associated with other birth defects or syndromes.
Additional clinical tips:
  • Always check the palate to exclude cleft palate with cleft lip; this is important both for surveillance and for clinical care.
  • Check for lip pits in the lower lip (Fig. 23), in the child and in the parents – it is a sign of a genetic condition (van der Woude syndrome) with high recurrence risk (a parent may have the pits but not the cleft).

Checklist for high-quality reporting

Cleft Lip – Documentation Checklist
Describe in detail, including:
  • Laterality – right, left, or bilateral.
  • Lower lip (Fig. 23) – pits present or absent (when present, van der Woude syndrome should be suspected).
  • Extension of the cleft lip – minimum, partial, or total involvement of the gum extending at most through the alveolus to the incisive foramen (not beyond).

Describe procedures to assess further additional malformations, and if present describe.

Take and report photographs: Very useful; can be crucial for review.

Report whether specialty consultation(s) were done, and if so, report the results.

Key visuals:

Anatomy of the lip and palate. Note that in bilateral cleft lip, a median remnant of the philtrum is still present. Note also that the clefting can extend to the gum or alveolus but does not extend beyond the incisive foramen (thus involving only the primary palate) (see Fig. 25).

Median cleft lip. In median cleft lip, there is no remnant of tissue (philtrum) in the area below the nasal septum (see Fig. 26). Median cleft lip is part of a condition called absent premaxilla and is often an external finding in holoprosencephaly, a severe brain malformation. Absent premaxilla and holoprosencephaly are associated with genetic syndromes, including trisomy 13.