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Indian Journal of Surgery

, Volume 80, Issue 3, pp 272–274 | Cite as

Surgery for Blue Rubber Bleb Nevus Syndrome—a Case Report

  • U. Aravindan
  • R. Ganesan
  • M. Thamarai Kannan
Case Report
  • 105 Downloads

Abstract

Blue rubber bleb nevus syndrome (BRBNS) is a rare disease characterised by multiple venous malformations and haemangioma in the skin and visceral organs. The lesion often involves the cutaneous and gastrointestinal tract. Most common visceral organ affected is the GIT. Most predominant region involved in the GIT is small bowel. However, vascular lesions can occur anywhere from oral mucosa to anal canal. GIT bleed is relatively slow, resulting in minor, chronic and occult blood loss. The syndrome in the GIT may also present with severe complications such as rupture, volvulus, intussusceptions and even death. Cutaneous malformations are usually asymptomatic and do not require treatment. The treatment of GIT lesions is determined by the extent of intestinal involvement and severity of the disease. Most patients respond to supportive therapy such as iron supplementation and blood transfusion. Surgical resection, endoscopic sclerosis and laser photocoagulation have been proposed for more significant haemorrhage and severe complications. Here, we present a case of BRBNS in a 13-year-old girl involving the GIT especially the large bowel, presenting with the complaints of bleeding per rectum and iron deficiency anaemia. Initially, endoscopic sclerotherapy was performed, but to no response. Hence, proceeded with surgical resection.

Keywords

Blue rubber bleb nevus syndrome Haemangioma Vascular malformations Gastrointestinal bleeding 

Notes

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

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Copyright information

© Association of Surgeons of India 2017

Authors and Affiliations

  • U. Aravindan
    • 1
  • R. Ganesan
    • 2
  • M. Thamarai Kannan
    • 3
  1. 1.Department of SGE, TMCHThanjavurIndia
  2. 2.Department of MGE, TMCHThanjavurIndia
  3. 3.Department of General Surgery ,TMCHThanjavurIndia

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