You have accessJournal of UrologyStone Disease: Evaluation & Medical Management (I)1 Apr 20132106 SURGICAL INTERVENTION FOR PRIMARY HYPEROXALURIA: A SINGLE INSTITUTION SERIES Alonso Carrasco, Candace Granberg, and Amy Krambeck Alonso CarrascoAlonso Carrasco Rochester, MN More articles by this author , Candace GranbergCandace Granberg Rochester, MN More articles by this author , and Amy KrambeckAmy Krambeck Rochester, MN More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2013.02.2015AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES Primary hyperoxaluria (PH) is an inborn error of glyoxylate metabolism resulting in markedly elevated systemic oxalate levels with subsequent urolithiasis, nephrocalcinosis, and end stage renal disease (ESRD). We present our experience with surgical urolithiasis management in patients with PH. METHODS A retrospective chart review from 1994-2012 was perform to identify patients with diagnosis of PH. RESULTS A total of 149 patients with PH were identified with median follow-up of 11.6 years (range: 0-51). Sixty-five percent were diagnosed before the age 18 years, and 19% presented with ESRD. The rate of PH subtype was 77% type 1, 11% type 2, 10% type 3, and 2% non-1,2,3. Median age at initial symptom and subsequent diagnosis were 5.6 years (range: 0.1-53) and 10.9 years (range: 0-74), respectively. A median delay of 2.7 years (range: 0-62) from first symptom to diagnosis was observed. ESRD developed in 34 (32%) patients at a median time from diagnosis of 15.4 years (range: 0.1-51). A total of 368 urologic surgical interventions for urolithiasis were documented in 68 (46%) patients with an average of 5.84 procedures (range: 1-29) per patient. Procedures included shock wave lithotripsy 152 (47%), ureteroscopy 95 (29%), percutaneous nephrolithotomy 61 (19%), cystoscopy 39 (12%), and open neprolithotomy 16 (5%). Acute stone events occurred at a rate of one every 2.83 years (range: 0-21.5). Patients required an average of 1.36 procedures (range: 1-4) for management of the stone(s), which subsequently afforded a mean of 3.62 years (range: 0.25-21.5) without the need of additional interventions. A total of 9 complications (Clavien grade IIIa-IVa) were observed in 8 patients, which included intervention for retained symptomatic stone in 7 (77%) and development of ESRD in 2 (22%) in the immediate postoperative period secondary to systemic oxalosis crisis. CONCLUSIONS Primary hyperoxaluria is a heterogeneous disease which can pose a significant diagnostic and treatment challenge. Despite optimal medical and surgical management, patients experience recurrent acute stone events requiring multiple urologic interventions. Rendering these patients stone free is not always attainable due to severe parenchymal calcification and it must be recognized that significant complications such as ESRD can occur secondary to surgical intervention. © 2013 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 189Issue 4SApril 2013Page: e863 Advertisement Copyright & Permissions© 2013 by American Urological Association Education and Research, Inc.MetricsAuthor Information Alonso Carrasco Rochester, MN More articles by this author Candace Granberg Rochester, MN More articles by this author Amy Krambeck Rochester, MN More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...