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Case Reports in Surgery
Volume 2014, Article ID 746323, 4 pages
Case Report

Prune Belly Syndrome with Overlapping Presentation of Partial Urorectal Septum Malformation Sequence in a Female Newborn with Absent Perineal Openings

1College of Medicine, Alfaisal University, Riyadh 11533, Saudi Arabia
2College of Medicine, King Saud University, P.O. Box 2454, Riyadh 11451, Saudi Arabia
3Department of Paediatric Surgery, King Faisal Specialist Hospital and Research Center, Riyadh 12713, Saudi Arabia

Received 21 September 2014; Accepted 24 November 2014; Published 9 December 2014

Academic Editor: Gregorio Santori

Copyright © 2014 Azhar Farooqui et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Prune belly syndrome (PBS) is a rare congenital anomaly characterized in males by a triad of anomalous genitourinary tract, deficient development of abdominal wall muscles, and bilateral cryptorchidism. Although similar anomalies have been reported in females, by definition they do not full fill the classical triad. Urorectal septum malformation sequence (URSM) is a lethal condition characterized by presence of ambiguous genitalia, absent perineal openings (urogenital and anal), and lumbosacral abnormalities. In this original case report, the authors discuss the presentation and management of what would be analogous to a Woodhouse category 1 PBS in a female newborn associated with an overlapping presentation of URSM.