Kohli H. et al., 2022: Primary hyperoxaluria type 1: urologic and therapeutic management.
Kohli H, Kurtz MP.
Boston Children's Hospital, Department of Urology, Boston, MA, USA.
While the surgical approaches available in primary hyperoxaluria (PH) are common to all patients requiring intervention for urolithiasis, the indications for treatment and their corresponding toxicities are unique. Being a rare disease, we are guided by case series. This review summarizes the available literature highlighting the important disease-specific considerations. Shockwave lithotripsy (SWL) is of particular interest. It is generally the first-line treatment for stones in children, but here the stones produced will be relatively resistant to fragmentation. In addition, there are concerning reports in children of sudden unilateral decline in function in the treated kidney as measured by nuclear renography. Percutaneous nephrostolithotomy might intuitively seem favorable given the shortest drain duration and the ability to treat larger stones efficiently but, similar to SWL, rapid chronic kidney disease (CKD) progression has been seen postoperatively. Ureteroscopy is therefore generally the safest option, but considerations regarding stent encrustation, the growth of residual fragments and the large volume of stone often faced may limit this approach. The surgeon must balance the above with consideration of the patient's CKD status when considering a plan of monitoring and treating stones in PH.
Clin Kidney J. 2022 May 17;15(Suppl 1):i14-i16. doi: 10.1093/ckj/sfab187. eCollection 2022 May. PMID: 35592623. FREE ARTICLE
Treating patients (children) with PH (primary hyperoxaluria) is difficult. Stone-free rates after SWL are generally low because of the dominance of COM (calcium oxalate monohydrate) in the stones. Moreover, there are reports of cases of paediatric patients with reduced renal function after repeated SWL.
Improved stone-free rates are recorded with URS (RIRS) and, as second alternative, PCNL.
Difficulties to determine the stone status both before and after treatment is associated with the simultaneous nephrocalcinosis.