[Bowel obstruction by hamartomatous polyp in a pediatric patient with tuberous sclerosis].

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[Bowel obstruction by hamartomatous polyp in a pediatric patient with tuberous sclerosis].

Arch Argent Pediatr. 2017 06 01;115(3):e157-e161

Authors: Scrigni AV, Bottero A, Bosaleh A, Aisenberg N, Paglia M, Manfrin L, Luong TC

Abstract
Tuberous sclerosis is an autosomal dominant disorder with a wide clinical spectrum of disease. It is characterized by development of benign tumors in multiple organs due to a disturbance in cellular growth and differentiation. It usually affects skin, brain, heart and kidney. Gastrointestinal involvement is rare and mainly restricted to adults and sigmoid colon and rectum. In children there are only two cases; our patient would be the third. We present a patient with tuberous sclerosis who began at the first month of life with repeated intestinal subocclusion. The videocolonoscopy showed a mass protruding into the lumen of the ascending colon. Right hemicolectomy was performed. The anatomopathological report corresponded to a hamartomatous polyp. The clinical and nutritional evolution in the postoperative period was very favorable. Although uncommon, the hamartomatous polyp should be considered in the differential diagnosis of intestinal occlusion in pediatrics.

PMID: 28504501 [PubMed - indexed for MEDLINE]

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