Abstract
Congenital maxillomandibular synechia is a rare malformation that is characterized
by a fusion of the maxilla and mandible. The fusion is fibrous or bony and prevents
mouth opening, which causes difficulties in feeding and occasionally in breathing.
Although extremely rare, neonatologists must understand the disease because it can
be fatal and require emergency treatment after birth. We report the case of a very-low-birth-weight
(VLBW) infant with congenital maxillomandibular synechia and other malformations,
including cleft palate, syndactyly, and cryptorchidism. The patient presented with
extremely limited mouth opening, and endotracheal intubation seemed impossible; fortunately,
the patient did not have respiratory distress syndrome. The patient underwent surgical
release of the fibrous bands on days 10 and 17, and good mouth opening was achieved.
The patient was able to consume breast milk orally and was discharged home at a corrected
gestational age of 1 month without recurrence of difficulty in mouth opening or any
sequelae. This is the first reported case of a VLBW infant with congenital maxillomandibular
synechia who required more complicated management of feeding, surgical intervention,
and anesthesia.
Keywords
congenital maxillomandibular synechia - fibrous or bony fusion - difficulty in feeding
and breathing - popliteal pterygium syndrome
