Title

Covered cloacal exstrophy - A poorly recognized condition: Hints for a correct diagnosis

Document Type

Conference Proceeding

Publication Date

12-1-2013

Journal

Journal of Pediatric Surgery

Volume

48

Issue

12

DOI

10.1016/j.jpedsurg.2013.08.011

Keywords

Cloacal exstrophy; Cloacal exstrophy variant; Covered cloacal exstrophy

Abstract

Introduction: Covered cloacal exstrophy requires a high index of suspicion for its diagnosis. Low implantation of the umbilical cord, separated pubic bones, and anorectal malformation are the most common signs. Methods: Thirty-one patients with this defect were retrospectively analyzed. Results: Besides the anorectal malformation, the patients had important unique anatomic findings, including a colon shorter than 20 cm (17 patients) and absent bladderneck (27 patients). Twenty-four patients underwent a colonic pullthrough; of those, only 5 of them have voluntary bowel movements. Twelve patients underwent a urinary reconstruction. Eleven of them are dry with catheterization, and one leaks in between catheterization. Two patients are urinary continent. Conclusions: Covered exstrophy is a serious condition. Externally, the patients may look like having a rather simple malformation. However, the intra-abdominal findings are similar to those seen in cloacal exstrophy. An early correct diagnosis is important to plan a reconstructive strategy and to adjust the parent's expectations concerning bowel and urinary function. In addition to the traditional prognostic factors for bowel and urinary control (sacral ratio, tethered cord, and level of the rectum) these patients have other anatomic defects (absent bladderneck and short colon) that negatively affect the functional prognosis. © 2013 Elsevier Inc. All rights reserved.

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