Klippel-Trenaunay syndrome: incidence and treatment of genitourinary sequelae

PURPOSE: Prior reviews regarding genitourinary manifestations of Klippel-Trenaunay syndrome used data acquired from diverse specialty specific articles to define the incidence and sequelae of its genitourinary manifestations. We believe that this resulted in erroneous conclusions regarding the urological complications of Klippel-Trenaunay syndrome. MATERIALS AND METHODS: Data on genitourinary manifestations in patients with Klippel-Trenaunay syndrome treated at 1 institution from 1970 through 2005 were acquired. RESULTS: Of 218 patients with Klippel-Trenaunay syndrome 30% (66 of 218) had genitourinary involvement, including 7% (15) with cutaneous genital abnormalities, 7% (15) with visceral genitourinary involvement and 16% (36) with each type. Intermittent bleeding from cutaneous genital abnormalities developed in 65% of patients (33 of 51). Conservative treatment with compression and/or cauterization was attempted in all 33 patients and it was successful in 64% (21 of 33). Intractable hemorrhage resulted in excision of the cutaneous bleeding site in 36% of cases (12 of 33). A total of 39 hospitalizations for gross hematuria occurred in 9% of the patients (19 of 218). Hematuria developed from the bladder in 11 cases, the urethra in 4 and the kidney in 4. Conservative therapy resolved gross hematuria in 21% of the patients (4 of 19). Refractory hematuria was successfully treated with cauterization in 37% of the patients (7 of 19) and by angiographic embolization in 10% (2 of 19). Intractable gross hematuria resulted in open surgical excision of the bleeding site in 32% of the patients (6 of 19). CONCLUSIONS: The incidence of genitourinary manifestations of Klippel-Trenaunay syndrome is 30%, which is triple the previously reported incidence of 9%. Unlike prior reports stating that the genitourinary abnormalities rarely caused problems, 52% of the patients (34 of 66) with Klippel-Trenaunay syndrome who had urological manifestations eventually required interventional therapy for genitourinary complications.