A mild and rare form of Klippel-Trenaunay syndrome presenting with urethral bleeding due to penile hemangioma.

摘要

Klippel-Trenaunay syndrome (KTS) is characterized by a triad of cutaneous port-wine capillary malformations, hemihypertrophy, and varicose veins. Intermittent gross painless hematuria is usually the first clinical sign. An 8-year-old boy with multiple hemangiomas, including glans penis, and associated with KTS presented with urethral bleeding. Radiologic and endoscopic evaluation revealed neither intra-abdominal nor intravesical hemangioma. Urethral bleeding was thought to be related to glanular hemangioma extending to the anterior penile urethra. Although we were able to manage the case conservatively, many patients require endoscopic or surgical interventions. Radiologic and endoscopic evaluations and careful follow-up is essential for diagnosis and prompt treatment.