Urogenital involvement in the Klippel-Trenaunay-Weber syndrome: treatment options and results
Int. braz. j. urol
;
32(6): 697-704, Nov.-Dec. 2006. ilus
Article
in English
| LILACS
| ID: lil-441370
ABSTRACT
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 ANDMETHODS:
From 1995 to 2005, 7 patients with KTWS were evaluated and the charts of these patients were reviewed.RESULTS:
PatientsÆ 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.
Full text:
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Index:
LILACS (Americas)
Main subject:
Klippel-Trenaunay-Weber Syndrome
/
Male Urogenital Diseases
/
Genital Diseases, Female
Type of study:
Observational study
/
Risk factors
Limits:
Adolescent
/
Adult
/
Child, preschool
/
Female
/
Humans
/
Male
Language:
English
Journal:
Int. braz. j. urol
Journal subject:
Urology
Year:
2006
Type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
University of Sao Paulo/BR
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