Literature DB >> 28892921

A Rare Case of Genital Malformation with Omphalocele, Exstrophy of Bladder, Imperforate Anus and Spinal Defect Complex-Autopsy Findings.

K Mamatha1, B R Yelikar2, Varsha R Deshpande3, B S Disha3.   

Abstract

Omphalocele, Exstrophy of cloaca, Imperforate anus and Spinal defects (OEIS) is a severe manifestation of exstrophy-epispadias sequence with a combination of defects including OEIS. It results from improper closure of anterior abdominal wall and defective development of cloaca and urogenital septum due to defect in blastogenesis during the 4th week of gestation. Identification of this complex is important through foetal autopsy as this condition can recur in siblings. Prenatal diagnosis also helps to prevent foetal death with appropriate management in the less severe cases. In severe cases, termination of pregnancy is considered. A primigravida with 28 weeks of gestation had delivered a live baby with multiple congenital anomalies; baby died after 10 minutes. These anomalies were grouped under OEIS complex.

Entities:  

Keywords:  Extrophy of cloaca; Gestation; Placenta

Year:  2017        PMID: 28892921      PMCID: PMC5583874          DOI: 10.7860/JCDR/2017/28277.10307

Source DB:  PubMed          Journal:  J Clin Diagn Res        ISSN: 0973-709X


  4 in total

1.  The OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects): recurrence in sibs.

Authors:  N M Smith; H M Chambers; M E Furness; E A Haan
Journal:  J Med Genet       Date:  1992-10       Impact factor: 6.318

2.  OEIS complex: prenatal ultrasound and autopsy findings.

Authors:  Z Ben-Neriah; S Withers; M Thomas; A Toi; K Chong; A Pai; L Velscher; S Vero; S Keating; G Taylor; D Chitayat
Journal:  Ultrasound Obstet Gynecol       Date:  2007-02       Impact factor: 7.299

Review 3.  Prenatal ascertainment of OEIS complex/cloacal exstrophy - 15 new cases and literature review.

Authors:  Kim Keppler-Noreuil; Sue Gorton; Florence Foo; Jerome Yankowitz; Catherine Keegan
Journal:  Am J Med Genet A       Date:  2007-09-15       Impact factor: 2.802

4.  OEIS complex associated with chromosome 1p36 deletion: a case report and review.

Authors:  Ayman W El-Hattab; Josh C Skorupski; Michael H Hsieh; Amy M Breman; Ankita Patel; Sau Wai Cheung; William J Craigen
Journal:  Am J Med Genet A       Date:  2010-02       Impact factor: 2.802

  4 in total

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