Klippel-Trenaunay syndrome: 2 case reports and a review of genitourinary manifestations

Purpose: We summarize the literature and present our experience with genitourinary manifestations of the Klippel-Trenaunay syndrome, which can lead to challenging management problems. Materials and Methods: We report on 2 patients with genitourinary manifestations of the Klippel-Trenaunay syndrome and performed a MEDLINE review of the literature using the key words "Klippel-Trenaunay," "vascular malformation" and "genitourinary." Results: Genitourinary manifestations were cited in 18 articles, including 1,174 cases of the Klippel-Trenaunay syndrome, detailing the presentation and management of bladder, external genitalia and retroperitoneal involvement in the Klippel-Trenaunay syndrome. The overall genitourinary symptoms in patients with the Klippel-Trenaunay syndrome seem to occur in the more severe cases and usually involve cutaneous vascular malformations of the trunk, pelvis and genitalia. Conclusions: Intra-abdominal and intrapelvic extension of the vascular malformations of the Klippel-Trenaunay syndrome frequently occurs concurrently with the lower abdominal, pelvic cutaneous involvement of the external genitalia, as in our 2 cases and in our review of the literature. These data provide a better understanding of the spectrum of genitourinary manifestations in the Klippel-Trenaunay syndrome and provide insight for the clinician to formulate individual therapies for these patients.