Complete Fusion of the Maxillamandibular: Report of a Rare Case and Review of the Literature

Abstract

The maxillomandibular fusion is a very rare condition, with no more than forty cases described in the literature. Adhesions of bone and/or soft tissue between the mandible and maxilla manifest themselves in the inability to open the mouth added to impacts on mandibular growth, nutrition and speech. This condition can be isolated or, when congenital, associated with other anomalies such as cleft lip and palate, aglossia, or Van der Woude syndrome. In the present paper, we report a case of maxillomandibular fusion treated in our department at University Hospital, between February 2011 and June 2014. The case is a congenital maxillomandibular fusion in a two-year-old infant, associated with a syndrome of Van der Woude. We discuss the diagnostic and treatment difficulties on the anaesthetic and surgical levels and the action to be taken to avoid recurrence. To date, some classifications have been suggested in the literature, but there is no standard treatment protocol. Early treatment is necessary to allow freedom of the upper airway, and ensure proper nutrition and good growth of facial bones. The success of surgery is conditioned by an adequate physiotherapy follow-up likely to guarantee the non-recurrence of the lesion.