A rare combination of Tessier 2 cleft with Goldenhar syndrome

Abstract

The incidence of rare craniofacial clefts ranges from 1.43 to 4.85/100,000 live births. Most craniofacial centers report an incidence of 9.4-34 cases of rare clefts for every 1000 clefts. Among these Tessier clefts, the number 2 cleft is the rarest. We report one such case of complete number 2 nasal cleft in a 3-year-old boy, with features of Goldenhar syndrome, such as preauricular tags, epibulbar dermoid, and coloboma of middle one-third of the upper eyelid. This combination is extremely rare and has only been reported once before in literature. Reconstruction of the nasal cleft was carried out using a modification of Denonvilliers Z-plasty along with a composite graft from the right helix and rotation of dorsal nasal skin. Rare facial cleft combinations reinforce the embryological basis that they are likely to result from the disruption in migration of neural crest cells from rhombomeres to branchial arches by genetic manipulation by teratogens, rather than other simplistic theories.

Keywords

• facial clefts
• goldenhar syndrome
• nasal clefts
• syndromic clefts
• tessier 2 cleft