Congenital fusion of the jaws

Abstract

Congenital fusion of the jaws is quite rare. It may be unilateral or bilateral and involves only the soft tissues or both the hard and soft tissues. This anomaly may be seen separately or in association with syndromes. Maxillomandibular fusion restricts mouth opening causing problems in feeding, swallowing and respiration. This condition can be easily treated. However, in long-standing cases, growth anomalies from TMJ ankylosis may occur.

Syngnathism in an infant born to consanguineous parents

“…The present parental consanguinity may be of interest, and may point to an autosomal recessive pattern of inheritance, as has been suggested previously in a few more reports [Ugurlu et al, 1999;Yazdi and Fakhraee, 2000;Mir et al, 2007;Mortazavi and Motamedi, 2007]. Further reports of patients with this infrequent malformation are needed in order to better understand genetic and teratogenic influences.…”

“…Dawson described a case with unilateral jaw fusion associated with mosaic trisomy 8 [Dawson et al, 1997]. From our search, in four cases there was reported consanguinity and/or familial occurrence [Ugurlu et al, 1999;Yazdi and Fakhraee, 2000;Mir et al, 2007;Mortazavi and Motamedi, 2007]. We found only one patient from Turkey, born to consanguineous parents, who had a sibling with a similar malformation [Ugurlu et al, 1999].…”

Congenital zygomatico-maxillo-mandibular fusion: a brief case report and review of literature

“…Congenital maxillomandibular fusion (syngnathia) is a rare deformity seen in infants that often appears as part of a syndrome and can vary in severity from single mucosal bands (synechiae) to complete bony fusion (synostosis), which is less common than synechiae. It may affect soft and/or hard tissue and can restrict maximum mouth opening causing problems with feeding and respiration 4,8,11 .…”

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

“…In all cases, early treatment is essential. A long-term immobilisation would lead to the risk of ankylosis of Temporo Mandibular Joints, making a surgical treatment more complicated [9]. Early surgical release of adhesions is recommended to ensure a normal diet, prevent obstruction of the upper airway, allow normal mandibular function and ensure proper growth [9].…”