Renal Hemangioma and Renal Artery Aneurysm in the Klippel-Trenaunay Syndrome

Campistol, J. M.; Agustı́, Carles; Torrás, A; Campo, Elı́as; Abad, Cipriano; Revert, L

doi:10.1016/s0022-5347(17)41507-2

Cited by 31 publications

(19 citation statements)

References 19 publications

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“…Hematuria is a common symptom in patients with genitourinary and pelvic vascular malformation necessitating therapeutic intervention. Hematuria as a presenting symptom has been reported in children [1][2][3], but massive hematuria in adults associated with KTS is rare [7][8][9]. We report 2 cases of hematuria in adults with KTS.…”

Section: Introductionmentioning

confidence: 84%

“…There have been 3 case reports of significant hematuria in adult patients with KTS [7][8][9]. In 2 patients, the upper tract was the source of bleeding.…”

Section: Discussionmentioning

confidence: 99%

“…In 2 patients, the upper tract was the source of bleeding. Renal artery aneurysm and hemangioma were diagnosed respectively [7,8]. Both patients required nephrectomy for control of hemorrhage.…”

Section: Discussionmentioning

confidence: 99%

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Massive Hematuria in Adults with Klippel-Trenaunay Syndrome Associated with Vascular Malformation of the Bladder

Shekarriz¹,

Upadhyay²,

Smith³

et al. 2000

Urol Int

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Although hematuria has been reported in children with Klippel-Trenaunay syndrome, it is a rare presentation in the adult population. Two cases of massive hematuria in adults with Klippel-Trenaunay syndrome are reported here. A unique feature was venous malformations of the bladder which were responsible for massive recurrent bleeding in 1 patient. The clinical presentation and management are discussed. Conservative endoscopic and arteriographic control seems to be appropriate as initial management in these patients.

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Section: Introductionmentioning

confidence: 84%

“…There have been 3 case reports of significant hematuria in adult patients with KTS [7][8][9]. In 2 patients, the upper tract was the source of bleeding.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Massive Hematuria in Adults with Klippel-Trenaunay Syndrome Associated with Vascular Malformation of the Bladder

Shekarriz¹,

Upadhyay²,

Smith³

et al. 2000

Urol Int

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“…Multifocal lesions in the same kidney have been reported in 12% of cases. Hemangioma may be part of a congenital disorder such as the Klippel-Trenaunay syndrome, Sturge-Weber syndrome and systemic angiomatosis [1,5,6]. Hemangioma occurs usually in the mucosa or subepithelial tissue of the pelvis (48.7%), in the pyramid (42.1%) and in the cortex of the kidney (9.2%) [1,7].…”

Section: Discussionmentioning

confidence: 99%

Cavernous Hemangioma of the Renal Hilum Presenting as an Avascularized Solid Mass

Virgili

Stasi²,

Bove³

et al. 2003

Urol Int

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“…Bladder involvement is estimated to occur in 2.3% to 6% of the patients, penoescrotal vascular malformations in 8.5% and vaginal or vulvar in 9.5% of the patients (1). Cases of kidney, renal artery and ureteral involvement have rarely been reported (1,(6)(7)(8). Our series is in accor- dance with these numbers, with a prevalence of bladder and genital involvement and one patient with ureteral obstruction associated with a massive pelvic vascular malformation.…”

Section: Commentsmentioning

confidence: 99%

Urogenital involvement in the Klippel-Trenaunay-Weber syndrome: treatment options and results

et al. 2006

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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.

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Renal Hemangioma and Renal Artery Aneurysm in the Klippel-Trenaunay Syndrome

Cited by 31 publications

References 19 publications

Massive Hematuria in Adults with Klippel-Trenaunay Syndrome Associated with Vascular Malformation of the Bladder

Massive Hematuria in Adults with Klippel-Trenaunay Syndrome Associated with Vascular Malformation of the Bladder

Cavernous Hemangioma of the Renal Hilum Presenting as an Avascularized Solid Mass

Urogenital involvement in the Klippel-Trenaunay-Weber syndrome: treatment options and results

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