Taiwanese Journal of Obstetrics and Gynecology
Volume 46, Issue 2 , Pages 103-110, June 2007

Syndromes and Disorders Associated with Omphalocele (II): OEIS Complex and Pentalogy Of Cantrell

  • Chih-Ping Chen

      Affiliations

    • Corresponding Author InformationCorrespondence to: Dr Chih-Ping Chen, Department of Obstetrics and Gynecology, Mackay Memorial Hospital, 92, Section 2, Chung-Shan North Road, Taipei, Taiwan

Departments of Obstetrics and Gynecology, and Medical Research, Mackay Memorial Hospital, Taipei, Department of Biotechnology and Bioinformatics, Asia University, and College of Chinese Medicine, China Medical University, Taichung, Taiwan

Accepted 30 March 2007.

Article Outline

Summary 

Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex is characterized by a combination of omphalocele, exstrophy of the bladder, an imperforate anus, and spinal defects. Pentalogy of Cantrell is characterized by a combination of a midline supraumbilical abdominal wall defect, a defect of the lower sternum, a defect of the diaphragmatic pericardium, a deficiency of the anterior diaphragm, and congenital cardiac anomalies. This article provides a comprehensive review of OEIS complex and pentalogy of Cantrell, including the pathogenesis, prenatal diagnosis, differential diagnosis, and associated malformations. Omphalocele is an important sonographic marker for OEIS complex and pentalogy of Cantrell. Prenatal detection of an abdominal wall defect associated with multiple midline defects should alert one to the possibility of OEIS complex and pentalogy of Cantrell and prompt the genetic investigation and counseling of the disorders.

Key Words:  OEIS complex , omphalocele , pentalogy of Cantrell

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PII: S1028-4559(07)60003-5

doi:10.1016/S1028-4559(07)60003-5

Taiwanese Journal of Obstetrics and Gynecology
Volume 46, Issue 2 , Pages 103-110, June 2007

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