Bilateral transverse facial clefts and accessory maxillae--variant or separate entity?

We report an extremely rare case of bilateral transverse facial clefts and accessory maxillae with severe mandibular hypoplasia in a 7-year-old Chinese boy. The transverse facial clefts had been repaired in infancy. The accessory maxillae were excised via a combined temporal and oral approach. Functional orthodontic appliance therapy was not feasible due to restricted protrusive movements of the mandible. Bimaxillary osteotomies were performed one year later due to failure mandibular catch-up growth to occur. The pathogenesis of the accessory maxillae in the presence of lateral facial clefting is postulated to be compensatory mesenchymal growth from the temporal region, and a case is made for this condition to be considered as a possible syndrome rather than a variant of the transverse facial cleft.