Omphalocele, Exstrophy of the Bladder, Imperforate Anus and Spinal Defect Complex on an Aterm Baby

Omphalocele, exstrophy of bladder/cloaca, imperforate anus, and spinal defects (OEIS complex) is an extremely rare congenital anomaly with varied clinical presentation. The exact aetiology is unknown but genetic associations have also been hypothesized. It results from defective blastogenesis leading to improrer closure of anterior abdominal wall and defective development of cloacae and urogenital septum. We reported a zero-day old baby presented with omphalocele, exstropy of bladder, imperforata anus, ambiguous genitalia and spinal defect. Laboratory examination showed normal result. Radiological imaging showed an exstrophy of bladder, gastroschizis and hemivertebrae CV Th 10, 11, 12. The patient underwent laparotomy, adhesiolisis, bladder closure, caecum tubularization and colostomy. The patology anatomy examination result from intrabladder specimen revealed unidentified spesific organ. He showed improvement and discharged after 26th days of hospitalisation. The patient was planned to undergo sex chromosome examination to determine the sex of the patient. The OEIS complex can be diagnosed based on the clinical manifestation of omphalocele, exstrophy bladder or cloacal, imperforate anus and spinal anomalies. Fetal ultrasound and fetal MRI on 2nd trimester of pregnancy are critical diagnostic. Fetal tomographic ultrasound imaging (TUI) also can be used. The prognosis of OEIS complex depends on the severity of the anomalies, the prevalence of infection and bowel anomalies.