Lateral cleft lip and macrostomia: Case report and review of the literature.

Lateral clefts are rare in occurrence. The lateral cleft is cause by failure of fusion of the maxillary and mandibular dermatomes. It is also associated with preaurical tags. We present a case of a lateral cleft of the lip with multiple bilateral preauricular tags that was repaired using triangular flaps.

INTRODUCTION

Lateral cleft of the lip or macrostomia is also known as Tessier cleft type 7, and is one of the rarest facial anomalies.[1] Although the reported incidence of cleft lip is 1 in 3000-5000, the incidence of lateral cleft lip is 1 in 100,000-300,000 and constitutes about 0.3-1% of all facial clefts.[2]

This lateral cleft is due to failure of fusion of the maxillary and mandibular processes of the first and second branchial arch or due to disruption in the processes after fusion. It is commonly associated with macrostomia and defects of the first branchial arch. The patients commonly also have deformities like preauricular tags, sinuses and Goldenhar syndrome (bilateral lateral clefts). This cleft can present as slight widening of the mouth to a cleft extending up to the ear. It can be bilateral, but most of the reported cases are unilateral and do not extend beyond the anterior border of the masseter.[3] Our case is a unilateral transverse cleft with multiple bilateral preauricular tags.

CASE REPORT

A 10-month-old male child presented with lateral cleft lip of the left side with multiple bilateral preauricular tags [Figure 1]. The main complaints of the parents were abnormal wide mouth, salivary drooling and facial deformity. The obstetrical and family history was insignificant. The child was evaluated for ophthalmic, auditory and facial nerve abnormalities. The renal ultrasound was normal. X-ray of the skull was performed to rule out structural anomalies of the skull. The new commissar was marked. The upper and the lower lip flaps were raised in a triangular fashion. The orbicularis muscle was separated from the mucosa and the skin. The repair was performed in three layers, i.e. the mucosa, muscles and skin [Figure 2]. The preauricular tags were also excised and the skin was repaired.

Figure 1

Clinical photo showing unilateral cleft and bilateral preauricular skin tags

Figure 2

Repaired mucosa and orbicularis muscle at the new lateral commissure

DISCUSSION

Transverse clefts or the lateral cleft lip is more common in the male child and, although most of the reported cases are unilateral, there are quite a few bilateral occurrences as well.[4] Our case is also a unilateral cleft. The additional anomalies reported with the lateral clefts are hemifacial microsomia, preauricular skin tags, microtia, absence of the Eustachian tube, temporomandibular joint, zygomatic arch, polydactyly and cardiac and renal anomalies.[4567] Correction of sialorrhea, speech problems and compromised chewing ability are important factors to intervene early. Reconstruction of the orbicularis oris is critical for good appearance.[89] The aim of surgery is to create symmetrical commissure with minimal scarring. The reconstruction of the commissure needs proper attention. There are various techniques described for the rotational flaps, like Z plasty by Longacre et al.,[10] modified Estlander-like flap by Nagai and Weinstein,[7] W plasty, simple linear flap and triangular flap. We have used the triangular flap technique in our patient. The well-repaired orbicularis oris counters the lateral contractile force of the skin scar.[10]

CONCLUSION

Lateral cleft lip, although rare in occurrence, requires precise correction. The well-mobilized and reconstructed orbicularis is key in the repair. The triangular flaps of the skin give satisfactory results.