RESUMEN
INTRODUCTION: Omphalocele, bladder extrophy, imperforate anus and spinal defect (known as OEIS) is a very rare congenital anomaly with an unknown etiology. In this report we describe a case of an OEIS variant associated with a wide pubic diastasis, bladder extrophy with a recto-bladder neck fistula and a high ano-rectal malformation. This work has been reported in line with the SCARE 2020 criteria. PRESENTATION OF THE CASE: A 30-year-old mother delivered a male baby at 39 weeks through a normal vaginal delivery. Examination revealed multiple congenital anomalies in the form of an Omphalocele, extrophied bladder, imperforate anus, ambiguous genitalia and a large pelvic diastasis. Fecal matter was noted at the most inferior point of the extrophied bladder, raising the suspicion of a recto- vesical fistula. An exploratory laparotomy showed a fistula between the rectum and the neck of the extrophied bladder. A sigmoid colostomy was carried out in addition to a mucous fistula. The fascial defect of the Omphalocele was approximated to the upper border of the extrophied bladder. At the age of 2 years, the baby underwent a bladder extrophy repair, a posterior sagittal anorectoplasty and bilateral osteotomies. DISCUSSION: OEIS complex has been reported to occur with a wide variety of associated anomalies, and this necessitates a thorough investigation in order to formulate an appropriate treatment plan. A prenatal diagnosis of OEIS complex can be made by ultrasound stressing the importance of antenatal follow up and a multidisciplinary approach in management. CONCLUSION: We described a rare variant of an OEIS complex and management of such anomalies requires a multidisciplinary input.