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A case of prenatally diagnosed prune belly syndrome variant and congenital pouch colon in the United States: A case report
Author(s)
Mignote Yilma Department of Surgery, University of California San Francisco, CA, USA
Karen Trang Department of Surgery, University of California San Francisco, CA, USA
Marisa Schwab Department of Surgery, University of California San Francisco, CA, USA
Max Bowman Department of Urology, University of California San Francisco, CA, USA
Mark Sugi Department of Radiology and Biomedical Imaging, University of California San Francisco, CA, USA
Jesse Courtier Department of Radiology and Biomedical Imaging, University of California San Francisco, CA, USA
Laurence S. Baskin Department of Urology, University of California San Francisco, CA, USA
Doruk Ozgediz Department of Surgery, University of California San Francisco, CA, USA
Abstract
Background: Prune belly syndrome (PBS) and congenital pouch colon (CPC) are rare congenital syndromes with a low incidence in the United States (U.S.) with most CPC cases being from India. In this case report, we describe, to the best of our knowledge, the first PBS variant and CPC patient in the U.S. Case Presentation: A 30-year-old G2P0010 woman was referred to a tertiary center after an 18-week ultrasound showed a fetal abdominal mass. A prenatal MRI showed a dilated loop of bowel containing a mixture of urine and meconium, oligohydramnios, and a protuberant abdominal wall. Born at 37 weeks, the child’s physical exam was notable for a distended abdomen with thin abdominal musculature, non-palpable bilateral testes, no anal opening, and flat buttocks. Intra-operatively, a dilated cecum/ascending colon was noted with an abrupt change in caliber at the transverse colon, bilateral enlarged ureters, a left testis at the internal ring and no visualized right testis. A colostomy and mucous fistula were created 5 cm from the sigmoid pouch. Conclusion: While most reported cases of CPC undergo single stage repair (one operation) at 1 day of life, our patient underwent the first procedure of a staged repair at 16 hours of life given his clinical instability at the time as well as his unknown urological anatomy in the setting of urinary obstruction. This case demonstrates the importance of fetal imaging, multidisciplinary approach at a tertiary care center, and reinforces a staged repair when necessary
Publication Details
Page(s) 1-45
DOI 10.47338/jns.v10.1012
Published Journal: Journal of Neonatal Surgery, Volume: 10, Issue: 1, Year: 2021
Keywords
Congenital pouch colon Anorectal Malformation Prune belly syndrome Congenital colovesicular fistula
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