Abstract

Introduction: OEIS syndrome, the most severe form of exstrophy-epispadias complex, is characterized by the existence of an omphalocele, a bladder exstrophy, an anal imperforation and a spina bifida. Case Presentation: Two clinical cases admitted at the first day of life in neonatology department are reported; one was born by caesarean section, weighing 2500 g and the other one vaginally with a weight at 1890 g. The first child’s mother was a primigravid, primiparous and the second mother was multiparous. Prenatal ultrasound was performed in one case. The clinical examination found two exstrophied hemi-bladders with two productive ureteral meatus, separated by a double intestinal orifice, a manifestation of anal imperforation, omphalocele, spina bifida and lumbar vertebral malformations; which corresponds to a typical form of OEIS syndrome. One of the babies had an associated clubfoot. The karyotype could not be performed. Both were dead; the first in the early postoperative period, the second one at 16 days of life without surgery. Conclusion: OEIS syndrome is a rare condition and represents a therapeutic challenge in developing countries. Promoting prenatal diagnosis is essential.

Highlights

  • OEIS syndrome, the most severe form of exstrophy-epispadias complex, is characterized by the existence of an omphalocele, a bladder exstrophy, an anal imperforation and a spina bifida

  • The clinical examination found two exstrophied hemi-bladders with two productive ureteral meatus, separated by a double intestinal orifice, a manifestation of anal imperforation, omphalocele, spina bifida and lumbar vertebral malformations; which corresponds to a typical form of OEIS syndrome

  • It was statistically demonstrated that OEIS complex is a clinically recognized non-random association that, in addition to the four classic malformations is variably associated with spina bifida, genital abnormalities, renal malformations, symphysis pubis diastasis, and limb abnormalities

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Summary

Introduction

The term OEIS complex was first used by Carey et al in 1978 [2]. It was statistically demonstrated that OEIS complex is a clinically recognized non-random association that, in addition to the four classic malformations (omphalocele, exstrophy of the cloaca, imperforate anus, and spine abnormalities) is variably associated with spina bifida, genital abnormalities, renal malformations, symphysis pubis diastasis, and limb abnormalities. The etiology of OEIS complex is thought to be multifactorial, based on the sporadic nature of most of the reported cases. We report 2 cases admitted, in the four months period of the year 2017, in the neonatology department of the University Hospital of Mother and Child Lagune of Cotonou

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