Nephropathy Following Portosystemic Shunt Surgery in Children With Extrahepatic Portal Venous Obstruction: An Unusual...

Portosystemic shunt (PSS) surgery is a well-established procedure for managing portal hypertension and associated complications of extrahepatic portal venous obstruction (EHPVO) in children. However, the long-term renal effects have not been systematically studied. We aimed to evaluate the prevalence, clinical spectrum, histopathology, treatment response, and outcomes of nephropathy developing after PSS in children with EHPVO.

We conducted an ambispective observational cohort study of 315 EHPVO children undergoing non-selective PSS (August 1996-December 2024). Patients were followed with monitoring and Doppler ultrasonography to assess shunt patency. If the clinician suspected urinary protein loss, then serial measurements of serum creatinine and blood urea nitrogen, and spot/24-h urine protein quantification was done. Renal biopsy was performed if proteinuria was persistent. The clinical, biochemical, and histologic findings, treatment, and outcomes were systematically recorded.

Twelve of 315 children (4%) developed post-shunt nephropathy in a span of 24 months (interquartile range [IQR]: 18-36) from PSS. Renal biopsies were suggestive of membranoproliferative glomerulonephritis (n = 6), immunoglobulin A nephropathy (n = 4), focal segmental glomerulosclerosis (n = 1), and diffuse global glomerulosclerosis (n = 1). Median total cholesterol at presentation was 312 mg/dL (IQR: 268-386) and median triglycerides 196 mg/dL (IQR: 148-268). Treatment included angiotensin receptor blockers (n = 11), immunosuppressive therapy (n = 7), and renal replacement therapy (n = 2). Partial remission occurred in 7 cases; four children progressed to end-stage renal disease, and two died. One patient experienced recurrence of nephropathy in the renal graft.

Post-shunt nephropathy is an under-recognized but serious complication following PSS in EHPVO. Renal surveillance, early biopsy, and timely therapy are critical.