Document Detail

Urogenital involvement in the Klippel-Trenaunay-Weber syndrome. Treatment options and results.
MedLine Citation:
PMID:  17201948     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: Klippel-Trenaunay-Weber syndrome (KTWS) is a congenital condition characterized by vascular malformations of the capillary, venous and lymphatic systems associated to soft tissue and bone hypertrophy in the affected areas. This syndrome may involve bladder, kidney, urethra, ureter and genitals. We report the treatment of 7 KTWS patients with urogenital involvement. MATERIALS AND METHODS: From 1995 to 2005, 7 patients with KTWS were evaluated and the charts of these patients were reviewed. RESULTS: Patients' median age was 19-years (range 4 to 46-years) and only 1 was female. The clinical presentation included genital deformities in 3 cases, hematuria in 2 and urethrorragia in 2, one of which associated with cryptorchidism and phimosis. Three patients had an association of pelvic and genital malformations, including 2 patients with hematuria due to vesical lesions and 1 patient with left ureterohydronephrosis due to a pelvic mass. Two patients had urethral lesions. Treatment included endoscopic laser coagulation for 1 patient with recurrent hematuria and 1 patient with urethrorrhagia, pelvic radiotherapy for 1 patient with hematuria and circumcision in 2 patients with genital deformities. One patient required placement of a double-J catheter to relieve obstruction. Hematuria and urethrorragia were safely and effectively controlled with laser applications. Circumcision was also effective. The patient treated with radiotherapy developed a contracted bladder and required a continent urinary diversion. CONCLUSIONS: Urogenital involvement in patients with KTWS is not rare and must be suspected in the presence of hematuria or significant cutaneous deformity of the external genitalia. Surgical treatment may be warranted in selected cases.
Fabio C Vicentini; Francisco T Denes; Cristiano M Gomes; Alexandre Danilovic; Frederico A Silva; Miguel Srougi
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  International braz j urol : official journal of the Brazilian Society of Urology     Volume:  32     ISSN:  1677-5538     ISO Abbreviation:  Int Braz J Urol     Publication Date:    2006 Nov-Dec
Date Detail:
Created Date:  2007-01-04     Completed Date:  2007-08-21     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101158091     Medline TA:  Int Braz J Urol     Country:  Brazil    
Other Details:
Languages:  eng     Pagination:  697-703; discussion 703-4     Citation Subset:  IM    
Division of Urology, University of Sao Paulo School of Medicine (USP), Sao Paulo, Brazil.
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MeSH Terms
Child, Preschool
Genital Diseases, Female / therapy*
Hematuria / complications
Klippel-Trenaunay-Weber Syndrome / pathology,  therapy*
Male Urogenital Diseases / therapy*
Middle Aged
Pelvis / radiography
Retrospective Studies
Urogenital System / pathology
Comment In:
Int Braz J Urol. 2007 Mar-Apr;33(2):264   [PMID:  17488549 ]

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