Serpentine-like syndrome associated with encephalocele

Abstract

Congenital short oesophagus and with intrathoracic development of the stomach is a rare developmental anomaly of the gastrointestinal system. Knowledge regarding its aetiology and management is limited. We report a case in which, in addition to short oesophagus and intrathoracic stomach, an encephalocele was identified during the antenatal period. After the postpartum examination, encephalocele, split notochord malformation, midline localized liver and congenital short oesophagus were confirmed. The report of this case adds to the body of knowledge on this rare condition.