Diagnosis and management of congenital rectourethral fistula in a child with long tubular duplication of the colon and Klippel-Feil syndrome

J Pediatr Surg. 2011 Nov;46(11):2184-6. doi: 10.1016/j.jpedsurg.2011.08.022.

Abstract

We describe a unique association of congenital rectourethral fistula with long tubular duplication of the colon in a boy with Klippel-Feil syndrome and Sprengel deformity. He presented with a rectourethral fistula after surgical repair of a tubular duplication of the terminal ileum, colon, and proximal rectum. Preoperative identification of the fistula was challenging and was only achieved after cystoscopy with injection of methylene blue under pressure through Foley catheters placed into the anus and distal stoma of a colostomy. Surgical repair was performed through the posterior sagittal approach. The patient is doing well after 4 years of follow-up.

MeSH terms

  • Colon / abnormalities*
  • Colostomy
  • Constipation / etiology
  • Cystoscopy
  • Humans
  • Infant
  • Klippel-Feil Syndrome / complications*
  • Male
  • Methylene Blue
  • Rectal Fistula / congenital*
  • Rectal Fistula / diagnosis
  • Rectal Fistula / surgery
  • Urethral Diseases / congenital*
  • Urethral Diseases / diagnosis
  • Urethral Diseases / surgery
  • Urinary Fistula / congenital*
  • Urinary Fistula / diagnosis
  • Urinary Fistula / surgery

Substances

  • Methylene Blue