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We describe a young woman with the Klippel-Trenaunay-Weber syndrome in whom gross unilateral hematuria developed. A renal arteriogram showed a left renal artery aneurysm. Artery reconstruction was impossible and left nephrectomy was performed. Pathological examination showed multiple renal pelvic hemangiomas as the cause of the gross hematuria.
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Campistol et al. (1988) studied this question.
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