A Rare Case of Atypical Haemolytic Syndrome Following Right Retrograde Intrarenal Surgery (RIRS)

TL;DR

A rare case of atypical hemolytic uraemic syndrome following kidney surgery is reported, highlighting the importance of multidisciplinary care.

Contribution

This is the first reported case of aHUS as a postoperative complication following endourology procedures.

Findings

A 46-year-old woman developed aHUS after right retrograde intrarenal surgery.

Treatment with eculizumab and plasma exchanges led to normalization of hemolytic parameters.

The patient successfully underwent a second surgery with no complications.

Abstract

Renal failure, non-immune hemolytic anemia, and thrombocytopenia are the hallmarks of the uncommon but potentially fatal complement system-related illness known as atypical hemolytic uraemic syndrome (aHUS). We describe the case of a 46-year-old woman who underwent right retrograde intrarenal surgery (RIRS) for a 1.5 cm urinary stone in the renal pelvis and experienced septic shock on postoperative day 1 caused by aHUS. The patient received continuous venovenous hemodiafiltration (CVVHDF) for six days, eculizumab, a monoclonal antibody that blocks terminal complement activation, and three plasma exchanges with fresh frozen plasma, resulting in a progressive normalization of hemolytic parameters. Two months after the RIRS, the patient underwent percutaneous nephrolithotripsy to remove residual kidney stones. Three days before the surgery, she received a dose of eculizumab. The procedure went smoothly, and there were no postoperative complications. To the best of our knowledge, this is the report that mentions aHUS as a postoperative complication following endourology procedures. This report focuses on multidisciplinary diagnosis, treatment, and follow-up strategies for this unique complication in urology.

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