P D Furness1, A Z Barqawi, G Bisignani, R M Decter. 1. Department of Pediatric Urology, Childrens Hospital and University of Colorado Health Science Center, Denver, Colorado, USA.
Abstract
PURPOSE: We summarize the literature and present our experience with genitourinary manifestations of the Klippel-Trénaunay syndrome, which can lead to challenging management problems. MATERIALS AND 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.
PURPOSE: We summarize the literature and present our experience with genitourinary manifestations of the Klippel-Trénaunay syndrome, which can lead to challenging management problems. MATERIALS AND 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.
Authors: Peter Rubenwolf; Alexander Roosen; Elmar W Gerharz; Antje Kirchhoff-Moradpour; Kassa Darge; Hubertus Riedmiller Journal: Int Urol Nephrol Date: 2006 Impact factor: 2.370
Authors: Gaspare Cucinella; Giuseppe Di Buono; Girolamo Geraci; Federica Ricupati; Giuseppe Gullo; Elisa Maienza; Giorgio Romano; Giulia Bonventre; Giuseppe Amato; Giorgio Romano; Salvatore Buscemi; Antonino Agrusa Journal: Front Surg Date: 2022-05-12
Authors: Carlo Bettocchi; Marco Spilotros; Giuseppe Lucarelli; Emanuele Utano; Francesco Sebastiani; Lukas Bittner; Pasquale Ditonno; Michele Battaglia Journal: Medicine (Baltimore) Date: 2019-08 Impact factor: 1.817
Authors: Salih Samo; Muhammed Sherid; Husein Husein; Samian Sulaiman; Margaret Yungbluth; John A Vainder Journal: Case Rep Gastrointest Med Date: 2013-06-03