Preservation of native sigmoid colon for secondary continent cystostomy after multivisceral transplantation for chronic intestinal pseudo-obstruction.

Abstract

Chronic intestinal pseudo-obstruction (CIPO) is characterized by severe digestive +/- urinary dysmotility. If the conservative management fails, multivisceral transplantation (MVT) may be needed. However, urinary dysmotility remains after MVT and requires to continue urinary catheterizations and/or drainage. We report on a boy with severe CIPO complicated by (1) chronic intestinal obstruction requiring total parenteral nutrition, decompression gastrostomy, and ileostomy; (2) recurrent line infections; (3) hepatic fibrosis; and (4) distension of the bladder and upper urinary tract, and recurrent urinary infections, leading to non-continent cystostomy for urinary drainage. He underwent MVT at the age of 5 years. The transplant included the liver, stomach, duodenum and pancreas, small bowel, and right colon. The distal native sigmoid colon was preserved. Fifteen months later, he underwent a pull through of the transplanted right colon (Duhamel's procedure), together with a tube continent cystostomy (Monti's procedure) using the native sigmoid. Postoperative course was uneventful, and the remaining ileostomy was closed 3 months later. Five years post-transplant, he is alive and well. He is fed by mouth with complementary gastrostomy feeding at night. He has 3-6 stools per day, with occasional soiling. The cystostomy is used for intermittent urinary catheterization 4 times/day and continuous drainage at night. He is dry, with rare afebrile urinary infections, normal renal function, and un-dilated upper urinary tract. Conclusion: in severe CIPO with urinary involvement, preservation of the distal native sigmoid colon during MVT allows secondary creation of a continent tube cystostomy, which is useful to manage persistent urinary disease.

• Authors: Julien Grosman, Yves Aigrain, Olivier Goulet, Florence Lacaille, Carmen Capito, Christophe Chardot
• Journal: Pediatric transplantation (Pediatr Transplant) Vol. 26 Issue 2 Pg. e14180 (Mar 2022) ISSN: 1399-3046 [Electronic] Denmark
• PMID: 34747091 (Publication Type: Case Reports)
• Copyright: © 2021 Wiley Periodicals LLC.

Topics

• Catheter-Related Infections (therapy)
• Child, Preschool
• Colon, Sigmoid
• Cystostomy (methods)
• Gastrostomy
• Humans
• Ileostomy
• Intestinal Obstruction (surgery)
• Intestinal Pseudo-Obstruction (surgery)
• Liver Cirrhosis (surgery)
• Male
• Parenteral Nutrition
• Urinary Tract Infections (therapy)
• Viscera (transplantation)