Klippel-Trenaunay Syndrome Associated With Urinary Tract Hemangiomas: A Case Report

Abstract

The association between Klippel-Trenauney syndrome (KTS) and bladder hemangiomas is rare. The most common clinical manifestation is hematuria. The diagnosis is made from the characteristic cystoscopic appearance of the tumor. We report the case of a patient presenting recurrent macroscopic hematuria in the context of KTS. A cystoscopic evaluation revealed bladder hemangiomas. A conservative approach consisting of bladder irrigation and close follow-up was chosen as therapy. Conservative treatment of bladder irrigation and close follow-up is the recommended initial treatment of moderate and infrequent episodes of hematuria in this context. The more invasive therapeutic options have to be considered especially for frequent or life-threatening episodes of hematuria. This case suggests that conservative treatment may be effective in treating moderate and infrequent episodes of hematuria due to bladder hemangioma in the context of KTS. Further studies are required to adequately establish the effectiveness, limitations, and complications of each approach.

Vesical Haemangioma in Klippel-Trenaunay-Weber Syndrome: A Clinical Case Report

“…The presented case is emblematic of the recurrent and painless gross haematuria that frequently serves as the initial clinical presentation of bladder involvement in KTW syndrome. The etiological underpinnings of these lesions remain speculative, with theories suggesting venous overload as a possible mechanism [3,6,7].…”

“…The syndrome is characterized by diverse clinical features, including cutaneous capillary malformations, varicose veins, soft tissue, and bone hypertrophy [3][4][5]. While KTW typically presents with prominent cutaneous manifestations, its involvement in the urinary tract, particularly through the development of bladder haemangiomas, represents a seldom-encountered clinical condition [5,6].…”

“…In KTW syndrome with urinary tract involvement, patients often present with gross haematuria, and the diagnostic utility of cystoscopy in identifying bladder haemangioma involvement is well established [6]. However, managing such cases can be challenging.…”

“…However, managing such cases can be challenging. Historically, partial cystectomy has been the standard of care for large, life-threatening haemangiomas, although less invasive approaches have been proposed [6,7].…”