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Urogenital involvement in the Klippel-Trenaunay-Weber syndrome. Treatment options and results.
| Content Provider | Semantic Scholar |
|---|---|
| Author | Vicentini, Fábio Carvalho Dénes, Francisco Tibor Gomes, Cristiano Mendes Danilovic, Alexandre Srougi, Miguel |
| Copyright Year | 2006 |
| 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 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. |
| File Format | PDF HTM / HTML |
| Alternate Webpage(s) | http://www.brazjurol.com.br/november_december_2006/Vicentini_ing_697_704.pdf |
| PubMed reference number | 17201948v1 |
| Volume Number | 32 |
| Issue Number | 6 |
| Journal | International braz j urol : official journal of the Brazilian Society of Urology |
| Language | English |
| Access Restriction | Open |
| Subject Keyword | Bladder Neoplasm Bladder Tissue Cholestasis Congenital Abnormality Congenital phimosis Cryptorchidism Cutaneous Candidiasis Female Genital Diseases Genitalia Genitourinary system Hematuria Hyperostosis Hypertrophy Klippel-Trenaunay-Weber Syndrome Lymphatic Diseases Male Circumcision Obstruction Patients Pelvic Inflammatory Disease Renal Tissue Ureter Urethra Urethral Diseases Urinary Bladder Urinary Diversion Urologic Neoplasms Vascular anomaly Vulva soft tissue |
| Content Type | Text |
| Resource Type | Article |
