網膜色素変性と難聴を伴つた巨大水腎症の1例

A CASE OF GIANT HYDRONEPHROSIS WITH RETINITIS PIGMENTOSA AND LOSS OF HEARING

緒言

　水腎症はしばしば遭遇する疾患であるが，内容液量が1lを越えるいわゆる巨大水腎症は比較的まれである。最近，著者らは，内容液量が10.3lに及んだ右巨大水腎症の1例を経験したので報告する。本症例ではさらに網膜色素変性と難聴を伴つており，これら三症候を伴う症例の報告は現在のところなく，極めてまれな症例と思われる。

The patient was 49-year-old man, who entered our clinic with the chief complaint of hematuria. An intravenous pyelography and a renoscintigram showed a large left kidney with non-visualization of the right kidney. Displacement of the abdominal aorta to the left and non visualizing right kidney were also demonstrated by the renal angiography. Hydronephrosis of the right kidney was diagnosed. Preoperatively 10, 300 ml of pelvic fluid was aspirated. Right nephrectomy was performed. The size of the specimen was 36×21×12cm. Vascular blockade of the pelvi-ureteral junction and stenosis or torsion of the ureter were not seen.