A rare case of accessory maxilla and bilateral Tessier no. 7 clefts, a 10-year follow-up

Abstract

A Tessier no. 7 cleft is a lateral facial cleft which originates from the oral cavity and extends towards the tragus, involving both soft-tissue and skeletal components. A male patient presenting with both maxillary jaw duplication and bilateral Tessier no. 7 clefts, which has been reported only twice in the literature, is described. Bilateral facial clefts, macrostomia and chondro-cutaneous remnants were noted, which were repaired and resected. With further growth, facial asymmetry and asymmetric facial nerve dysfunction became apparent. Radiographic examination showed an accessory maxillary jaw and a flattened and hypoplastic right coronoid process. A maxillary alveolar cleft was also present between the left second bicuspid and the second permanent molar. This case may represent an under-recognized phenotype with an unusual combination of maxillary jaw duplication, macrostomia, Tessier no. 7 clefts, and chondro-cutaneous remnants. A long-term follow-up of these patients is recommended as they often develop craniofacial deformities later in life.