Spontaneous Nephrocutaneous Fistula: A Case Report, Update of the Literature and Management Algorithm

Aims: Spontaneous nephrocutaneous fistula (NCF) is a rare and severe condition. The aim of this study was to update the data of the literature about this disease and, reporting and considering also our experience in 1 case of NCF, to make a new clinical algorithm. Methods: This study was done with a review of all the literature and a comparison with our experience. Results: The characteristic sign of spontaneous NCF is a flank sinus discharging material. Physical examination and computed tomography usually lead to the diagnosis. The main predisposing factors are renal stones/staghorn calculi, xanthogranulomatous pyelonephritis and renal tuberculosis. In the vast majority of the cases kidneys are poorly functioning or nonfunctioning. Surgical approach is the common management and usually consists of open nephrectomy. We create a clinical management algorithm obtained by a review of the literature and our experience with spontaneous NCF. Conclusions: Spontaneous NCF represents a very serious renal disease leading to loss of the kidney in the vast majority of cases. We propose a revised and user-friendly clinical diagnostic-therapeutic algorithm for spontaneous NCF based on the worldwide literature. (C) 2014 S. Karger AG, Basel