Bannayan-Riley-Ruvalcaba Syndrome, Rare Etiology of Intestinal Hamartomatouspolyposis: A case report

  • Zannatul Ferdous Sonia Department of Pediatric Gastroenterology & Nutrition, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
  • Md Rukunuzzaman Associate Professor, Department of Pediatric Gastroenterology & Nutrition, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
  • ASM Bazlul Karim Chairman, Department of Pediatric Gastroenterology & Nutrition, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
  • Afsana Yasmin Resident (Phase B), Department of Pediatric Gastroenterology & Nutrition, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
  • Shashi Bhushan Thakur Resident (Phase B), Department of Pediatric Gastroenterology & Nutrition, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
Keywords: Bannayan-Riley-Ruvalcaba, per rectal bleeding, polyposis

Abstract

Bannayan-Riley-Ruvalcaba syndrome (BRRS) is a rare autosomal dominant congenital disorder, characterized bymacrocephaly, lipomas, hamartomas, and pigmented macule in genitalia.Several dozen cases have been reported in the medical literature, but no case has been reported in Bangladesh. We report a case of BRRS in a 11-year-old male child with recurrent per rectal bleeding with hamartomatouscolonic polyposis & multiple subcutaneous lipomas on the anterior abdominal wall.In addition, patient had macrocephaly, intellectual impairment.Bleeding polyps were removed by colonoscopic polypectomy.

Bangladesh J Child Health 2019; VOL 43 (1) :62-65

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Abstract
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PDF
301
Published
2019-04-28
How to Cite
Sonia, Z., Rukunuzzaman, M., Karim, A., Yasmin, A., & Thakur, S. (2019). Bannayan-Riley-Ruvalcaba Syndrome, Rare Etiology of Intestinal Hamartomatouspolyposis: A case report. Bangladesh Journal of Child Health, 43(1), 62-65. https://doi.org/10.3329/bjch.v43i1.41220
Section
Case Reports