Prevention of chronic kidney disease in spina bifida.

OBJECTIVE: The prevalence of progressive chronic kidney disease (CKD) in children and adults with spina bifida is considerable, rising, and entirely preventable.

REMOVING THE CAUSE: PREVENTION OF SPINA BIFIDA: The best prevention of CKD in spina bifida is prevention of spina bifida itself through strategies that include folate supplementation, ideally before pregnancy.

THE CAUSE OF CKD: Dysfunctional bladder outlet causes febrile Urinary Tract Infections (UTI), even with clean intermittent catheterization (CIC), and subsequent renal scarring. The development of secondary vesicoureteric reflux (VUR) increases the risk of renal scarring and CKD.

FINDING THE IDEAL MARKER FOR MEASUREMENT OF RENAL FUNCTION IN SPINA BIFIDA: Creatinine-based methods are insensitive because of low muscle mass and underdeveloped musculature in the legs. Only Cystatin C-based eGFR can reliably assess global renal function in these patients. However, unilateral renal damage requires nuclear medicine scans, such as (99m)Tc DMSA. (VIDEO)URODYNAMICS STUDIES (UDS): Early treatment is recommended based on UDS with anticholinergics, CIC, and antibiotic prophylaxis when indicated. Overnight catheter drainage, Botox, and eventually augmentation cystoplasty are required for poorly compliant bladders. A continent child or one rendered continent following surgery is at a higher risk of renal damage.

CONCLUSION: A multidisciplinary approach is required to reduce the burden of CKD in patients with spina bifida. The right tools have to be utilized to monitor these patients, particularly if recurrent UTIs occur. Cystatin C eGFR is preferred for monitoring renal damage in these patients, and (99m)Tc DMSA scans have to be used to detect unilateral renal scarring.