1162 | Horseshoe Kidney with Complete Unilateral Duplication of Ureter and Pelvicalyceal System- A Case Report Vipin Tyagi, Tanveer Iqbal Dar, Abdul Rouf Khawaja, Sudhir Chadha Keywords: abnormalities; kidney; humans; urogenital abnormalities; ureter. INTRODUCTION Horseshoe kidney is the most common fusion abnormality in the kidney and oc-curs in 1 per 400 people with the male female ratio of 2:1.(1) Duplication of renal collecting system is the most common upper tract anomaly with an incidence of 0.5-0.8%.(2) Unilateral duplication is six times more frequent than bilateral duplication, with equal incidence on right and left sides.(3) However duplicated system in a horseshoe kidney is very rare. Most patients with horseshoe kidney are asymptomatic, up to 80% have hy- dronephrosis, about 20% develop renal calculi and 1/3-1/2 of cases have another associated anomaly.(2) The aim of this report is to highlight its rarity and technical difficulties in its management. CASE REPORT A 30 years male patient presented to our department with history of left flank pain since last few months. Base line investigations revealed serum creatinine of 1.05 mg/dL, hemoglobin 13.5 g/dL and urine routine-microscopy showed pus cells 15-20/hpf. On evaluation he was found to have horseshoe kidney with bilateral pelvic calculi. Ultrasonography showed left small kidney (7 × 5 × 3 cm) with large pelvic calculus, gross hydronephrosis and thinned out cortex. Intravenous urography showed no contrast uptake on left side and normally function- ing right kidney with right complete duplex system and bilateral renal calculi (Figure). DTPA scan showed left nonfunctioning kidney and normally functioning right kidney. Cystoscopy Corresponding author: Tanveer Iqbal, MD Department of urology and Kidney transplant surgery, Sir Ganga Ram Hospital, New Delhi, 110060, India. Tel: +11 4225 1235 E mail; drtanveerdar@gmail.com Received October 2011 Accepted February 2012 Department of urology, Sir Ganga Ram Hospital, New Delhi, 110060, India, CASE REPORT Case Report 1163Vol. 10 | No. 4 | Autumn 2013 |U R O LO G Y J O U R N A L Horseshoe Kidney with Complete Collecting System Duplication | Tyagi et al showed two ureteric orifices on right side and only one on left side. He was subjected to right PCNL, complete stone clearance was achieved. After one month left heminephrec- tomy was performed through a left flank sub costal incision. Findings of single ureter with three arteries and two veins were noted on left side during open retroperitoneal nephrec- tomy. Left kidney was hydronephrotic with very thin cortex and its ureter was crossing in front of the isthmus. Isthmus was divided at a line where thinned out cortex ended and normal cortex was evident. He recovered well and was dis- charged on 5th post-operative day. Histopathological study of the kidney showed features of chronic pyelonephritis. He is following our outpatient department since then (two months) and is doing well. DISCUSSION Horseshoe kidneys may be a result of teratogenic factors, which may also be responsible for the known increase in the incidence of related congenital anomalies and nephroblasto- ma.(4) Christoffersen and colleagues stated that combination of horseshoe kidneys with bilateral ureteral duplication is a very rare entity. Only two cases have been reported till now. (2,5) He described a case of partial hydronephrosis, bilateral duplication of the pelvis and ureter with horseshoe kidney. (5) Similarly Kuzel and colleagues described a horseshoe kidney with bilateral double pelvis system and double ure- ters.(6) Kevin and colleagues reported a horseshoe kidney with bilateral partial duplex pelvicalyceal system and ureter in a cadaver during its anatomical dissection. He concluded that intravenous urography (IVU) is the main radiological investigation method to diagnose this anomaly.(7) We report a case of unilateral complete duplex system in a horse shoe kidney. We reviewed literature by searching in Google and Pub med database, using “horseshoe kidney with unilateral completely duplicated system” as key words and found no such case reported till date. Khong and colleagues reported a 9-month-old boy who had a horseshoe kidney, associated with bilateral single system ectopic ureters. The right ureteric orifice was located near the midline of a deformed trigone while the grossly dilated left ureter inserted into the posterior urethra.(8) Pode and colleagues reported a case of unilateral triplica- tion of the collecting system in a horseshoe kidney.(9) Clini- cians should be conscious of complete duplex systems in horseshoe kidneys which is very rare and may pose a diag- nostic and interventional challenge. CONFLICT OF INTEREST None declared. Figure. X ray of KUB region showing right side horseshoe kidney with complete duplication of ureter and pelvis. Left renal radio opaque shadow (stone) is seen with no contrast uptake. REFERENCES 1. Glodny B, Petersen J, Hofmann KJ, et al. kidney fusion anom- alies revisited: clinical and radiological analysis of 209 cases of crossed fused ectopia and horseshoe kidney. BJU Int. 2009;103:224-35. 2. Keskin S, Erdogan N, Kurt A, Tan S, Ipek A. Bilateral partial ureteral duplication with double collecting system in horse- shoe kidney. Adv Med Sci. 2009;54:302-304. 1164 | 3. Schlussel RN, Retik AB. Ectopic ureter, ureterocele, and other anomalies of the ureter. Chapter 58. In: Walsh PC (ed). Campbell’s Urology, 8th Edn. Philadelphia: Saunders; 2002. p. 2007-52. 4. Hohenfellner M, Schultz-Lampel D, Lampel A, Steinbach F, Cramer BM, Thuroff JW. Tumor in the horseshoe kid- ney: clinical implications and review of embryogenesis. J Urol.1992;147:1098-102. 5. Christoffersen J, Iversen HG. Partial hydronephrosis in a pa- tient with horseshoe kidney and bilateral duplication of the pelvis and ureter. Scand J Urol Nephrol. 1976;10:91-93. 6. Kuzel M, Makarewicz J, Musial S, Sarzynska M. Case of horse- shoe kidney with bilateral double pelvis system and double ureters (in Polish). Pediatr Pol. 1979;54:407-9. 7. Kevin W. ONGETI, Julius OGENG’O, Hassan SAIDI. A horse- shoe kidney with partial duplex systems. IJAV. 2011;4:55-56. 8. Khong PL, Peh WC, Mya GH, Chan KL, Saing H. Horseshoe kidney with bilateral single system ectopic ureters. Aust N Z J Surg. 1996;66:773-6. 9. Pode D, Shapiro A, Lebensart P. Unilateral triplication of the collecting system in a horseshoe kidney. J Urol. 1983;130:533-4. Case Report