Giant Omphalocele with OEIS Complex - A Case Report

Abstract

An omphalocele represents an embryological defect of the umbilical ring and medial segments of the two lateral abdominal folds during fetal growth and can be divided into two groups depending on the size of the hernial defect. A major or giant omphalocoele is classified as a 5 cm or larger defect. The incidence is nearly 2.5 cases per 10,000 live births. The hernial sac may contain small and large bowel, stomach, liver, spleen, urinary bladder, gonads. A combination of defects including omphalocele, exostrophy of cloaca, imperforate anus, spinal defects is known as OEIS complex, affecting 1 in 2,00,000 to 4,00,000 pregnancies. During routine dissection in the Department of Anatomy, RIMS, Imphal, a 26 weeks gestational aged foetus of giant omphalocele with OEIS complex in was observed, with short umbilical cord, adherent to membrane was found with right sided scoliosis, imperforated anus with no external genitalias. On removal of the membrane all the abdominal viscera were malpositioned, malformed, malrotated and were found outside the abdominal cavity including the heart and both the lungs. Serial ultrasonography can be used to accurately diagnose abdominal wall defect in utero for monitoring fetal growth.