PERINATAL FEATURES OF OMPHALOCELE-EXSTROPHY-IMPERFORATE-ANUS-SPINAL DEFECTS (OEIS COMPLEX) ASSOCIATED WITH LARGE MENINGOMYELOCELES AND SEVERE LIMB DEFECTS

Omphalocele-Exstrophy-Imperforate anus-Spinal defects (OEIS complex), a combination of omphalocele, exstrophy of the bladder, an imperforate anus and spinal defects, arises from a single localized defect in the early development of the mesoderm that will later contribute to infra umbilical mesenchyme, cloacal septum, and caudal vertebrae. In this re port, we document the perinatal features of two cases of OEIS complex associated with meningomyeloceles and severe lower limb defects, and d iscuss the prenatal diagnosis, inheritance, and differential diagnosis of this association of malformations. Although long-term survival can be achieved by successful corrective surgery, the associated structur al defects such as large meningomyelocele and severe limb aplasia or h ypoplasia, as seen in our patient, can influence the patient's quality of life. We would like to emphasize that an accurate prenatal diagnos is of OEIS complex and associated malformations is important for the d etailed counseling of the family as well as appropriate perinatal mana gement by the obstetricians, pediatric surgeons, urologists, neurosurg eons, and neonatologists.