ESPU Congress 2017 - Abstract book

19–22 APRIL, 2017, BARCELONA, SPAIN

09:35–09:38

S6-2 (PP)

XANTHOGRANULOMATOUS PYELONEPHRITIS

IN A PAEDIATRIC COHORT (1963–2016): OUTCOMES FROM

A LARGE SINGLE-CENTER SERIES

Ionica STOICA

, Fardod O’KELLY

, Michael MCDERMOTT

and Feargal QUINN

1) Our Lady’s Childrens Hospital, Paediatric Urology, Dublin, IRELAND - 2) Our Lady’s Childrens Hospital, Urological

Surgery, Dublin, IRELAND - 3) Our Lady’s Childrens Hospital, Pathology, Dublin, IRELAND

PURPOSE

Xanthogranulomatous pyelonephritis (XGP) is an uncommon chronic destructive granulomatous

inflammation of the kidney first described in 1916 and affecting 6/1000 cases of pyelonephritis. Its

manifestations are varied, and with a limited number of cases in the literature, the optimal diagnosis

and management of XGP in the paediatric cohort is still unknown.

MATERIAL AND METHODS

The medical records of children who were treated for XGP in our unit from 1963 to 2016, inclu-

sive, were retrospectively reviewed. Information recorded for each patient included age, sex, past

medical history, clinical and biochemical characteristics, diagnostic procedures, treatment methods,

histopathologic findings and outcome.

RESULTS

64 children with a median age of 6.04 (1.1-14.81), with a M:F ratio 1.29:1 underwent nephrectomy

for XGP and had median follow-up 9.96yrs (1.19-14.9). The most common presentations were

being constitutionally unwell (60.6%), pain (57.6%), urinary tract infections (51.5%) and an ab-

dominal mass (36.4%). Pyrexia was present in 53.1%. Biochemical abnormalities included anaemia

(84.8%), thrombocytosis (81.8%) and hypomagnesemia (66.7%). There was an 82.6% concordance

between intraoperative cultures and positive mid-stream urines. Index kidneys were significantly

larger than the contralateral side (mean 1.4cm; p=0.002). XGP staging demonstrated extension

beyond the kidney in 78.8% kidneys. CT was performed in 10 cases.DMSA showed 0-10% function

in 87.5% cases. Surgical procedures included nephrectomy (n= 61), partial nephrectomy (n=3).

Perioperative complications colonic resections (n= 5) and abscess formation in 18%.

CONCLUSIONS

This is the largest series to date of XGP in a paediatric cohort. XGP should be included in the

differential diagnosis of all children presenting with perirenal or psoas abscess, renal mass and/or

non-functioning kidney associated with/or without urolithiasis. Clinical awareness and a high index

of suspicion is required to achieve the correct preoperative diagnosis and appropriate management.