Search

Kidney dysfunction in spina bifida is usually detected by low estimated glomerular filtration rate or ultrasound based hydronephrosis. We assessed the diagnostic test characteristics of hydronephrosis for detecting low estimated glomerular filtration rate, hypothesizing that hydronephrosis has low sensitivity compared to cystatin C based estimated glomerular filtration rate.We conducted a single center, retrospective cohort study, including patients with spina bifida from 2012-2017 with 2 kidneys and complete data needed to calculate estimated glomerular filtration rate via multiple pediatric (age 1-17.9 years) or adult (age ≥18 years) estimating equations. We evaluated the association of hydronephrosis status (high grade, low grade or none) with estimated glomerular filtration rate, adjusting for small kidney size and scarring, and calculated diagnostic test characteristics of hydronephrosis for low estimated glomerular filtration rate.We analyzed 247 patients (176 children and 71 adults). Mean±SD age was 13.7±6.6 years, and 81% of patients had myelomeningocele. Hydronephrosis (77% low grade) was found in 35/176 children and 18/71 adults. Hydronephrosis was associated with low estimated glomerular filtration rate in stepwise fashion, independent of kidney size and scarring. However, across cystatin C based pediatric equations, any hydronephrosis (compared to none) had 23%-48% sensitivity, and high grade hydronephrosis (compared to none or low grade) had 4%-15% sensitivity for estimated glomerular filtration rate90 ml/min/1.73 mHydronephrosis was associated with low estimated glomerular filtration rate but had poor sensitivity for cystatin C based estimated glomerular filtration rate90 ml/min/1.73 m

AIMS Current estimated glomerular filtration rate (eGFR) equations may be inaccurate in patients with spina bifida (SB) because of reduced muscle mass and stature. Cross-sectional and longitudinal variability of eGFR were analyzed in these patients across multiple equations, hypothesizing greater variability in creatinine-based than cystatin-C (Cys-C)-based equations. METHODS This retrospective cohort study included children (age, 1-17.9 years) and adults (≥18 years) with SB from 2002-2017 at a large SB clinic. Those without all data needed to calculate eGFR were excluded. Four pediatric and three adult eGFR equations were compared for cross-sectional outcomes of eGFR and elevated office blood pressures using chronic kidney disease (CKD) stage classification, and for longitudinal outcome of eGFR slope over time using covariance pattern models accounting for repeated measures. RESULTS One hundred and eighty two children and 75 adults had greater than or equal to 1 set of data measurements; 118 and 52, respectively, had greater than or equal to 2 sets. The pediatric bedside Schwartz equation had the highest median eGFR and coefficient of variation. CKD stage classification by eGFR showed large differences across equations in children, with rates of eGFR

Background Clubfoot occurs in 30% to 50% of patients with spina bifida. The Ponseti casting method has changed treatment of idiopathic clubfoot to a primarily nonoperative regimen. The Ponseti method is now widely applied to clubfoot in spina bifida, however, few studies report treatment outcomes. Most available studies include heterogeneous diagnoses or short-term results. The purpose of this study is to report midterm outcomes in patients with spina bifida and clubfoot treated with the Ponseti method. Methods IRB-approved retrospective chart review of 17 consecutive patients (26 feet) below 1 year of age with spina bifida and clubfoot treated with Ponseti method. Charts reviewed for age at treatment initiation, number of casts, surgeries performed, recurrence of deformity, and further treatments. Primary outcome was recurrence of deformity requiring further treatment. Data were analyzed using t tests for means and χ tests for categorical data. Results Initial correction was achieved in 26 of 26 feet patients. A total of 23 of 26 feet patients underwent a surgical procedure for the tendo-Achilles at an average age of 105 days, 12 percutaneous tenotomies (percT) and 11 open tendonectomy (openT). At average follow-up of 5 (1.8 to 7.5) years, 11 feet (42.3%) in 8 patients were successfully treated with Ponseti method. Of the 15 feet (57.7%) with recurrence, 10 required posterior releases, 4 posterior-medial-lateral releases and 1 tendon transfers. Average age at further treatment was 1.5 years (0.9 to 3.1 y). Those with recurrence required more casts before tendon surgery (7.6 vs. 6.1, P=0.02). A total of 100% patients (12/12) with percT had recurrence of deformity, compared with 18% (2/11) of patients with openT (P Conclusions Midterm evaluation of Ponseti method for clubfoot in spina bifida shows a successful outcome in 42.3%. Recurrence with openT was significantly lower than percT and also substantially lower than previously published recurrence rates in spina bifida (33.3% to 68%). In spina bifida, Ponseti method leads to reliable initial correction and is useful to decrease extensive soft tissue release. An open excision of the Achilles should be performed. Families should be counseled about high risk of recurrence and potential need for further treatment. Level of evidence Level III-retrospective comparative study.

INTRODUCTION AND OBJECTIVES:Emerging adults with spina bifida (SB) may face inaccurate kidney function assessment as they bridge the age gap between pediatric and adult equations for estimated glom...