A Rare Case of Omphalocele, Exstrophy of Bladder, Imperforate Anus and Spinal Defect Complex with Genital Anomalies in a Term Neonate

Abstract

A 22-year-old pregnant female presented at 39.1 weeks of gestation in third stage of labour with hand prolapse to our emergency department. She immediately underwent emergency lower segment caesarean section (LSCS). There was no significant antenatal history of exposure to teratogenic drugs or radiation, infection, diabetes mellitus or hypertension. There was no history of treatment for infertility. No antenatal registration or antenatal scan was done. She delivered a live baby weighing 2.8 kg with head circumference of 34.5cm, who had multiple congenital abnormalities. There was no history of consanguinity or similar malformations in previous deliveries. There was presence of omphalocele, exstrophy of bladder, imperforate anus, and genital anomalies (Figure 1 and 2) along with bilateral congenital talipes equinovarus (CTEV) and lumbosacral meningocele. X-ray thoracolumbar spine lateral view showed lumbosacral meningocele. Diagnosis of OEIS complex was made. Ultrasound (USG) of the abdomen and pelvis confirmed OEIS but the gender could not be evaluated due to obscure genital organs. Chromosomal analysis could not be done as the parents financial status did not permit for any future work up and intervention. The neonate died on day 5 of life. OEIS complex is a severe form of exstrophy-epispadias complex which results from improper closure of ventral abdominal wall due to failure of cephalocaudal and lateral folding with associated defects of cloaca and urorectal septum.1 The term OEIS complex was proposed to describe findings by Carey et al based on a retrospective search of medical records of 175 ARTICLE HISTORY Received 3 November 2020 Accepted 31 January 2021