Klippel-Trénaunay syndrome: 2 case reports and a review of genitourinary manifestations.
OBJECTIVE
We summarize the literature and present our experience with genitourinary manifestations of the Klippel-Trénaunay syndrome, which can lead to challenging management problems.


METHODS
We report on 2 patients with genitourinary manifestations of the Klippel-Trénaunay syndrome and performed a MEDLINE review of the literature using the key words "Klippel-Trénaunay," "vascular malformation" and "genitourinary."


RESULTS
Genitourinary manifestations were cited in 18 articles, including 1,174 cases of the Klippel-Trénaunay syndrome, detailing the presentation and management of bladder, external genitalia and retroperitoneal involvement in the Klippel-Trénaunay syndrome. The overall genitourinary symptoms in patients with the Klippel-Trénaunay 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-Trénaunay 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-Trénaunay syndrome and provide insight for the clinician to formulate individual therapies for these patients.
Furness
P D
PD
Department of Pediatric Urology, Childrens Hospital and University of Colorado Health Science Center, Denver, Colorado, USA.
Barqawi
A Z
AZ
Bisignani
G
G
Decter
R M
RM
D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007715:Klippel-Trenaunay-Weber Syndrome / Q000150:complications / Q000175:diagnosis*; D008297:Male; D052801:Male Urogenital Diseases / Q000209:etiology*