Clinical Assessment of a Delayed Congenital Bilateral Maxillomandibular Syngnathia –A Comprehensive Management and Literature Review

Abstract

Syngnathia refers to maxillomandibular fusion and may present with other defects, the airway and feeding being the primary considerations. Congenital syngnathia is an extremely rare condition only described predominantly in Asia and with some cases in Africa. To date, only 21 cases of true bony bilateral syngnathia have been reported. Surgical interventions were usually instituted rather early, most cases received surgery within the first 15 days of birth. The oldest age reported to receive a reconstructive surgery was 4 years old to date. Here we describe the surgical management of a bilateral complete extraoral maxillomandibular fusion as well as alveolar fusion intra-orally with temporomandibular joint (TMJ) involvement, who had managed to survive to the age of 9 years without any surgical intervention. The family managed to feed the patient through a Ryles nasogastric prior to the surgical intervention. At presentation, the patient was severely underweight due to malnourishment.  This is also the first report of use of bilateral temporalis flap as an interposition flap to prevent TMJ re-ankylosis. Peri-operatively, a mouth opening of 40mm was achieved. General improvements were confirmed by a stable mouth-opening of 20mm as well as an increased weight of 2 kgs at the 2years post-operative review. & at 4 year review continued to be stable at 20mm with additional weight of 2 kg. The minimal post-operative follow up and care including physiotherapy for jaw movements and speech, the quality of feeding and phonation improved significantly. Additional rehabilitation measures including the distraction of TMJ replacement are being carefully planned to commence when the patient turns 16 year old if the bone quality has improved such that this procedure can be attempted.