A case of prenatally diagnosed prune belly syndrome variant and congenital pouch colon in the United States: A case report

Authors

  • Mignote Yilma University of California, San Francisco
  • Karen Trang University of California, San Francisco
  • Marisa Schwab University of California, San Francisco
  • Max Bowman University of California, San Francisco
  • Mark Sugi University of California, San Francisco
  • Jesse Courtier University of California, San Francisco
  • Laurence Baskin University of California San Francisco
  • Doruk Ozgediz University of California, San Francisco

DOI:

https://doi.org/10.47338/jns.v10.1012

Keywords:

Prune belly syndrome, Congenital pouch colon, Congenital colovesicular fistula, Anorectal malformation

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.

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Author Biographies

Mignote Yilma, University of California, San Francisco

Fellow | National Clinician Scholars Program at UCSF

Research Resident | Department of Surgery 

 

 

 

Karen Trang, University of California, San Francisco

General Surgery Resident 

Marisa Schwab, University of California, San Francisco

General Surgery Resident

Max Bowman, University of California, San Francisco

Department of Urology

Mark Sugi, University of California, San Francisco

Asst Professor Clinical Radiology

Jesse Courtier, University of California, San Francisco

Professor Clinical Radiology

Laurence Baskin, University of California San Francisco

Department of Urology

Professor in Urology

Doruk Ozgediz, University of California, San Francisco

Associated Professor of Surgery

Division of Pediatric Surgery

Director, Center for Global Surgery and Health Equity

 

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Published

2021-09-25

How to Cite

1.
Yilma M, Trang K, Schwab M, Bowman M, Sugi M, Courtier J, Baskin L, Ozgediz D. A case of prenatally diagnosed prune belly syndrome variant and congenital pouch colon in the United States: A case report. J Neonatal Surg [Internet]. 2021Sep.25 [cited 2021Oct.19];10:45. Available from: https://www.jneonatalsurg.com/ojs/index.php/jns/article/view/1012