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BMJ Case Reports 2018; doi:10.1136/bcr-2017-224162
  • Rare disease
  • CASE REPORT

An unusual presentation of gall bladder papillomatosis in association with metachromatic leukodystrophy

  1. Tarun John K Jacob2
  1. 1Department of Paediatric Surgery, Christian Medical College, Vellore, Vellore, Tamil Nadu, India
  2. 2Department of Paediatric Surgery, Christian Medical College and Hospital Vellore, Vellore, Tamil Nadu, India
  1. Correspondence to Dr Jujju Jacob Kurian, jujjujacobkurian{at}gmail.com
  • Accepted 5 October 2018
  • Published 1 November 2018

Summary

A 5-year-old boy with metachromatic leukodystrophy, debilitated by spastic quadriparesis presented to us with massive ascites and respiratory distress. A subtotal cholecystectomy was performed on him from another centre for a gall bladder mass a year before he came to us. Imaging revealed a polypoidal frond-like mass arising from the gall bladder fossa which was supplied by a hypertrophied branch of the right hepatic artery. A decision was made to offer surgical resection preceded by embolisation of the feeding vessel. At surgery, a polypoidal frond-like mass in communication with the peritoneal cavity was seen arising from the remnant gall bladder bed with over 4 L of mucoid ascites. The mass along with the remnant gall bladder was removed. Biopsy revealed villous papilloma of the gall bladder. The child is well and asymptomatic at 5-month follow-up.

Footnotes

  • Contributors JJK was involved in the conception and design, acquisition of data and drafting the case report. TJKJ was involved with revising it critically for important intellectual content.

  • 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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