Congenital Maxillomandibular Syngnathia: Review of Literature and Proposed New Classification System.

Abstract

Maxillomandibular fusion (syngnathia) is a rare craniofacial anomaly. It occurs as either fibrous (synechiae) or bony (synostosis) fusion of jaw or fusion of mandible to zygoma, tuberosity, hard palate and temporal bone. There are several documentations delineating this condition but owing to its varying presentation, association with both intraoral and extraoral anomalies and syndromes, reviewing it has been as uphill task. The non-unanimous description of cases in the literature and the use of different nomenclatures make it difficult to classify this condition. Extensive search of the literature was done from the year 1936 to 2018 which included a total of 118 cases with 62 cases of bony fusion, 48 cases of fibrous fusion and 8 cases of combined fusion, i.e., fibrous on one site and bony on another side. This paper proposes a novel, simple and explicit classification system on the basis of nature of fusing tissues, location and extent, association with syndrome after reviewing the existing literature. Furthermore, this review reports 4 new cases of syngnathia along with their clinical, radiographic features and their management protocol. This classification can be generalized to all the cases of syngnathia and can further facilitate in its appropriate diagnosis and treatment plan.