International Urology and Nephrology 36: 239–243, 2004. © 2004 Kluwer Academic Publishers. Printed in the Netherlands. 239 Spontaneous nephrocutaneous fistula-2 unusual case reports with review of literature M. S. Ansari, Iqbal Singh & P. N. Dogra Department of Urology, All India Institute of Medical Sciences, New Delhi – 29, India Abstract. We report two unusual cases of spontaneous nephrocutaneous fistulas. They were discovered to be the cases of neglected calculous disease in a poorly functioning kidney and tuberculous pyelonephritis respectively. A timely nephrectomy cured their debilitating condition and prevented further morbidity. We have discussed the etio-pathogenesis, clinical and diagnostic approach towards the cases of nephrocutaneous fistula. The literature regarding nephrocutaneous fistula has been reviewed and discussed. A stepwise algorithm has been proposed to manage cases of nephrocutaneous fistulas. Key words: Nephrocutaneous fistula, Nephrocutaneous sinus, Renocutaneous fistula, Spontaneous renal fistula Introduction Spontaneous nephrocutaneous fistula (NCF) is rare condition. Twelve published cases of nephrocu- taneous fistula have been reported till date [1–12]. Spontaneous NCF from asymptomatic kidney stones without prior surgery are also very rare [7, 12]. Where as initial reports [3, 7] have suggested genitourinary tuberculosis as the commonest cause, all recent reports [1, 4–8] suggest a calculus non functioning kidney as the usual etiology. We have reviewed the literature regarding a spontaneous nephrocutaneous fistula and have suggested a clinical and diagnostic-therapeutic algorithm to approach such cases. Case report Case 1 A 45-year-old man presented with complaints of insi- dious onset of purulent discharge from the left flank fistula for the last three months with past history of low-grade fever and left flank pain for the past three months. An x-ray KUB revealed right renal pelvis staghorn calculus, the intravenous pyelogram revealed a nonfunctioning left kidney (Figure 1). The retrograde pyelogram ruled out any evidence of reflux and ureteric obstruction, the communication with the kidney and the fistula was evident on the Figure 1. The intravenous pyelogram showing the non-functioning left kidney.