馬蹄腎合併腎盂及輸尿管泌尿道上皮癌- 病例報告

任駿逸、康智雄

高雄長庚紀念醫院泌尿科

Urothelial carcinoma of ureter and kidney in horseshoe kidney: A case report

Chun-Yi Jen, Chih-Hsiung Kang

Department of Urology, Kaohsiung Chang Gung Memorial Hospital and Chang Gung

 University College of Medicine

Background: Horseshoe kidney is the most common congenital renal fusion anomaly, with incidence reported about 1/400. There are highly variations of fusion part and location, pathway of upper urinary tract, and renal vasculature. Horseshoe kidney is assumed to double to quadruple the risk for urothelial carcinoma (UC), and nephroureterectomy with bladder cuff excision is the gold standard for most patients with UC of the renal pelvis or ureter. Thus, the importance of thorough preoperative evaluation and surgical planning could not to be underestimated.

Case presentation: A 64-year-old man with end-stage renal disease (ESRD) under hemodialysis, and recurrent bladder UC treated with transurethral resection of bladder tumor (TURBT) several times. Regular monitoring of renal sonography showed right hydronephrosis, and computed tomography demonstrated right upper ureter tumor consistent with a UC. Right hand-assisted retroperitoneoscopic nephroureterectomy (HARNU) with bladder cuff excision was performed. The renal pedicle and the isthmus of horseshoe kidney were both controlled with Endo-GIA staplers. No additional selective ligation of the exposed arteries was required.

Conclusions: We report a case of horseshoe kidney with ESRD and UC both in the ureter and the horseshoe kidney treated by HARNU with bladder cuff excision. Standard two-dimensional computed tomography was arranged to illustrate the individual renal vasculature for surgical planning, and hand-assisted maneuver provided further information and identification of vessels during the surgery of horseshoe kidney.   Endo-GIA staplers can be used to control the renal pedicle and the isthmus of horseshoe kidney without additional selective ligation of the exposed arteries.

Keywords: Horseshoe kidney; Renal fusion; Urothelial carcinoma; End-stage renal disease