BMJ Case Reports 2018; doi:10.1136/bcr-2018-225072
  • Rare disease

Congenital colonic stenosis with absent caecum and appendix: a rare association

  1. Potala Srinivas Rao2
  1. 1Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India
  2. 2Max Smart Hospital, Saket, New Delhi, India
  1. Correspondence to Dr Ruchira Nandan, ruchira.nandan{at}
  • Accepted 3 October 2018
  • Published 1 November 2018


Congenital colonic stenosis is a rare condition with less than 20 cases reported in the literature since 1966. We report an interesting case of a 7-month-old baby girl who presented with features suggestive of acute intestinal obstruction. On exploration, it was a case of ascending colon stenosis with absence of caecum and appendix. A double barrel ileocolostomy was performed. The histopathology confirmed the diagnosis of colonic stenosis and ruled out the presence of Hirschsprung’s disease in the distal colon. The child underwent second stage surgery (stoma closure) after 9 months. In complex cases of congenital colonic stenosis, an early decompressive surgery followed by a delayed second stage closure is recommended in patients with poor general condition.


  • Contributors The case was admitted under the care of DKY at Safdarjang Hospital, New Delhi. The case was successfully managed and operated by DKY and KK. The manuscript has been drafted by RN and KK. PG and PSR helped in revision of the manuscript. The revision of manuscript and the final draft has been seen and approved by all the authors.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Parental/guardian consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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