Orthognathic Surgery in the Mobius Syndrome Patient

Mobius syndrome has been well described in the literature since von Graffe’s seminal report in 1880. It is defined by the congenital, non-progressive palsy of the sixth and seventh cranial nerves, with occasional involvement of other cranial nerves. Facial and abducens nerve involvement can be unilateral or bilateral, and paralysis can be partial or complete. The incidence of Mobius syndrome has not been determined, although it is estimated to occur in 1:50,000 live births, with an equal gender distribution. The pathophysiology of Mobius syndrome is not completely understood. It is attributed to brainstem dysfunction, caused by agenesis of the involved cranial nerve nuclei, prompted by either environmental or genetic causes. Despite its rarity, the dental and maxillofacial management of the Mobius patient warrants attention. The management of soft tissue manifestations in these patients, particularly treatment of facial muscle paralysis, is well described. The treatment of other common maxillofacial abnormalities, however, has not been studied as extensively. A recent search of the literature on the hard tissue management of Mobius patients afforded only three case reports on a total of four patients. Three patients who underwent dentofacial deformity correction with orthognathic surgery are presented. This is the largest case series on orthognathic surgery in the Mobius syndrome patient, performed at one institution by one surgeon. Common dental and maxillofacial features of the Mobius patient and pertinent surgical treatment planning recommendations are also discussed.