OEIS complex: A case report and literature review of a rare polymalformative syndrome

Introduction: The OEIS complex or syndrome is the most severe form of the exstrophy-epispadias complex and is characterized by the existence of an omphalocele, bladder exstrophy, anal imperforation and spina bifida. We report an atypical case of OEIS complex associated with further birth defects admitted to our department. The diagnostic and therapeutic approach to the complex congenital malformations involved are highlighted, as well as difficulties encountered in a limited resource setting.

Case Report: This was a neonate referred for the management of a congenital malformation on the first day of life. The baby was born vaginally at 35 weeks of gestation with a birth weight of 2000 g and significant major risk factors for neonatal sepsis. Two antenatal ultrasounds had revealed fetal uropathy. On admission, the clinical exam revealed: a type 1 omphalocele, an anorectal malformation with a recto-urinary fistula and a covered lumbosacral dysraphism. Paraclinical examinations revealed an associated cardiac anomaly. The karyotype analysis was not done due to lack of financial means. Supportive care was given and the neonate underwent surgical reconstruction of birth defects on day 20.

Conclusion: The OEIS complex is a rare morbid congenital polymalformative syndrome in neonates. Patients require keen diagnostic enquiry and immediate postnatal multidisciplinary management, with long-term follow-up.