Pediatr Gastroenterol Hepatol Nutr.  2020 Jan;23(1):115-120. 10.5223/pghn.2020.23.1.115.

Newly Diagnosed Klippel-Trenaunay Syndrome Presenting with Rectal Polyposis in a Male Pediatric Patient: A Case Report

Affiliations
  • 1Department of Pediatrics, Pusan National University Children's Hospital, Pusan National University School of Medicine, Yangsan, Korea. moonmissing@gmail.com

Abstract

Klippel-Trenaunay syndrome (KTS) is a rare disorder characterized by a triad of abnormal bone and soft tissue growth, the presence of a port-wine stain, and venous malformations. Gastrointestinal (GI) manifestations of KTS are relatively common and generally do not cause significant problems. However, persistence can lead to chronic GI blood loss or even massive bleeding in rare cases. The majority of the severe GI manifestations associated with KTS present as vascular malformations around the GI tract and exposed vessels can lead to serious bleeding into the GI tract. Herein, we report a case of a 16-year-old boy with severe iron deficiency anemia who was previously misdiagnosed as hemorrhoid due to small amount of chronic bleeding. The actual cause of chronic GI bleeding was from an uncommon GI manifestation of KTS as rectal polyposis.

Keyword

Gastrointestinal hemorrhage; Polyps; Vascular malformations

MeSH Terms

Adolescent
Anemia, Iron-Deficiency
Gastrointestinal Hemorrhage
Gastrointestinal Tract
Hemorrhage
Hemorrhoids
Humans
Klippel-Trenaunay-Weber Syndrome*
Male*
Polyps
Port-Wine Stain
Vascular Malformations
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