Aicha Birem, Matthieu Peycelon, Sébastien Faraj, Mathilde Glenisson, Garance Martin, Florent Guérin, Thomas Blanc, Luca Pio
In this contemporary multicentre cohort, paediatric XGP affected very young, predominantly male children with left-sided disease and was diagnosed earlier than in historical series. Nephrectomy remains the cornerstone for diffuse, non-functional disease, but minimally invasive approaches were feasible and a parenchyma-sparing or conservative strategy appeared feasible and safe in highly-selected children with focal disease, preserved differential function, or a clearly correctable underlying uropathy. We propose a pragmatic decision framework integrating differential function, focality and the presence of a treatable causal lesion. This series challenges the historical paradigm of systematic nephrectomy in paediatric XGP and supports a more individualized approach guided by renal function and disease focality.
BACKGROUND: Xanthogranulomatous pyelonephritis (XGP) is a rare chronic destructive granulomatous pyelonephritis. Paediatric experience is limited and mostly historical. The largest published series reported nephrectomy in 95.4 %. Whether this radical-only paradigm still reflects best practice in the cross-sectional imaging and minimally invasive surgery era is unclear.
MATERIAL AND METHODS: We retrospectively reviewed all paediatric patients (≤16 years) treated for XGP at four tertiary paediatric-surgery centres of Assistance Publique - Hôpitaux de Paris (AP-HP), the university hospital trust of the greater Paris region (Bicêtre, Robert-Debré, Necker-Enfants Malades and Trousseau), between January 2005 and December 2024. Diagnosis required a compatible cross-sectional imaging pattern and/or histological confirmation. Demographics, prior uropathy, clinical and microbiological findings, imaging, treatment strategy (radical, parenchyma-sparing, or purely conservative), perioperative course and follow-up were analysed.
RESULTS: Twelve children were included (9 male, 3 female; M:F 3:1; median age 47 months, range 10 months-6 years). The left kidney was involved in 11/12 (91.7 %). Pre-existing uropathy was documented in 7/12 (58.3 %). All patients had fever; nephrolithiasis was identified in 8/12 (66.7 %), with Proteus mirabilis being the predominant isolate (4/12, 33.3 %). All received broad-spectrum intravenous antibiotics. Nine children (75 %) underwent nephrectomy - by open approach (n = 4), retroperitoneoscopy (n = 4) or robot-assisted laparoscopy (n = 1) - while three (25 %) were managed without nephrectomy (percutaneous drainage; ureteric stent and delayed ureteroscopy; Cohen reimplantation for severe vesicoureteric reflux). Intra-operative complications occurred in 3/9 surgical patients (33.3 %; 25 % of the whole cohort). At median follow-up of 47 months, compensatory contralateral hypertrophy was universal, no recurrent infection occurred in the parenchyma-sparing group, and one child (8.3 %) progressed to stable CKD stage 3.
CONCLUSIONS: In this contemporary multicentre cohort, paediatric XGP affected very young, predominantly male children with left-sided disease and was diagnosed earlier than in historical series. Nephrectomy remains the cornerstone for diffuse, non-functional disease, but minimally invasive approaches were feasible and a parenchyma-sparing or conservative strategy appeared feasible and safe in highly-selected children with focal disease, preserved differential function, or a clearly correctable underlying uropathy. We propose a pragmatic decision framework integrating differential function, focality and the presence of a treatable causal lesion. This series challenges the historical paradigm of systematic nephrectomy in paediatric XGP and supports a more individualized approach guided by renal function and disease focality.