Incidence of nephrolithiasis after bladder augmentation in people with spina bifida.

INTRODUCTION: Risks of nephrolithiasis after bladder augmentation in people with spina bifida (SB) remain unclear. Annual incidence of nephrolithiasis in the general population is 0.01% for 10-14 years old, 0.07% for 15-19 years old and 0.2% for 20-24 years old. Our aim was to assess the incidence and risk factors of nephrolithiasis in SB patients after augmentation. METHODS: Patients with SB and augmentation followed at our institution were retrospectively reviewed (born ≥1972, surgery 1979-2019). Patients were screened annually with renal bladder ultrasound and abdominal radiograph. Main outcome was nephrolithiasis treatment. Kaplan-Meier survival and Cox proportional hazards analysis were used. Possible predictors were assessed using stepwise forward selection (variables with p < 0.1 on univariate analysis included in multivariate analysis). RESULTS: 427 patients with SB and augmentation were included (51.8% female, 74.9% shunted). Median age at augmentation was 8.5 years (median follow-up: 12.4 years, ileum segment: 81.0%, bladder neck procedure: 60.7%, urinary channel: 74.2%) and 28.8% developed bladder stones. Overall, 47 (11.0%) patients were treated for nephrolithiasis. After correction for differential follow-up, nephrolithiasis was treated in 7.3% at 10 years, 13.2% at 15 years, and 18.0% at 20 years (Figure). Patients presented with either a urinary tract infection (46.8%), on screening (44.7%), or pain (8.5%). Stones were treated percutaneously, endoscopically or by ESWL (63.8%/34.0%/10.7%, respectively). Most were calcium stones (58.3%). On multivariate analysis, compared to younger patients, patients augmented at ≥10 years of age had 1.84 times the risk of nephrolithiasis (p = 0.01). Nephrolithiasis was more common in those who developed bladder stones (HR = 3.00, p < 0.0001). Among those with both renal and bladder stones, bladder stones typically preceded nephrolithiasis (55.2%), were treated concurrently (31.0%) and 13.8% occurred after nephrolithiasis. Gender, wheelchair use, bowel segment used, MACE and skeletal fractures were not associated with higher nephrolithiasis risk (p ≥ 0.11). DISCUSSION: This study of a large cohort of SB patients with long-term follow-up highlights that the risk of nephrolithiasis is cumulative and related to bladder stone formation, age at augmentation and time since augmentation. An association with bladder stones suggests potential shared metabolic causes. The study's retrospective design likely led to underestimating the risk of nephrolithiasis by not capturing spontaneously passed stones. CONCLUSION: Approximately 1% of patients with SB develop nephrolithiasis annually after augmentation. Close long-term surveillance after augmentation is strongly indicated, as nephrolithiasis incidence in augmented patient with SB is at least 10 times higher than general population. Patients with bladder stones are especially at risk.

Use of retrograde pyelogram to plan for miniature open incision in pediatric pyeloplasty.

INTRODUCTION: As robotic-assisted surgery becomes increasingly utilized for pediatric ureteropelvic junction (UPJ) obstruction, open surgeons have countered by using muscle-splitting, miniature (≤2 cm) incisions. To prepare for this type of incision during pyeloplasty, it is necessary to define the exact location of the UPJ. The use of retrograde pyelogram (RPG) at the time of pyeloplasty helps the surgeon to identify the exact location of UPJ, and thus be able to use a muscle-splitting, miniature incision for open pyeloplasty. OBJECTIVE: We hypothesize that when performing a muscle-splitting, miniature incision open approach; preoperative RPG frequently changes the traditional pyeloplasty flank incision at the tip of the 11th or 12th rib. MATERIALS & METHODS: A retrospective review of open pyeloplasties performed by a single surgeon at our institution from 7/1/2010 to 12/31/2018 was performed to determine rate of use of RPG, open pyeloplasty incision location and to determine what factors are predictive of incisional site. RESULTS: 114 of 122 (93.4%) patients with 115 renal units had pyeloplasties with preoperative RPG performed. Of the 8 procedures without RPG, two had a pelvic kidney diagnosed prior to surgery, two had narrow ureteric orifices that were difficult to cannulate, and four had associated reflux. In 31/115 (27%) pyeloplasties the incision was changed from a standard incision position at the 11th or 12th rib to an alternative incision (i.e. extended muscle-transecting incision at the tip of the 11th or 12th rib, or to an alternate incision site including Gibson, McBurney's incision, or low anterior abdominal incision). 84/115 (73.0%) had a miniature (<2 cm) incision at the tip of the 11th or 12th rib. Grade IV hydronephrosis was a significant predictor for changing the traditional incision site (p = 0.02). Preoperative nephrostomy tube insertion was also associated with an increased likelihood of having an alternate incision (p = 0.04). Incision site was not significantly affected by age of the patient at surgery, patient sex, size of the affected kidney, T1/2 times of <30 min, split function of <30%, kidney length differential, or laterality. CONCLUSION: The consistent use of RPG prior to pyeloplasty helps surgeons to plan for a small muscle-splitting, miniature open incisions. In our experience, 27% of pyeloplasties required alternative incision sites based on the results of pre-operative RPG.

Estimating and tracking renal function in children and adults with spina bifida.

PURPOSE: Estimated glomerular filtration rate (eGFR) in the general population is stable in children after 2 years of age until adulthood. In the first three decades after age 18, eGFR decreases by 0.3-0.8 ml/min/1.73 m2/year. Little data exists regarding eGFR changes in the spina bifida (SB) population given variability in muscle mass. In the absence of a validated SB-specific eGFR formula, the performance of different eGFR formulas may vary. We performed a cross-sectional study (1) to determine trends in eGFR with increasing age in children and adults with SB and (2) to compare eGFRs calculated using different formulas. METHODS: We retrospectively reviewed records of patients 2-50 years old with SB followed at our institution (2014-2019). We determined eGFR using four pediatric formulas (2-17 years: CKiDSCr, CKiDCys, CKiDSCr-Cys, ZappitelliSCr-Cys) and four adult formulas (18 + years: MDRDSCr, CKD-EPISCr, CKD-EPICys, CKD-EPISCr-Cys). One eGFR per patient was included (most recent eGFR for those with serial measurements). Patients were categorized as chronic kidney disease (CKD) stage 2 (eGFR 60-89) and Stage 3+ (<60). Non-parametric tests, linear regression, and Spearman's correlation were used for analysis. RESULTS: Among 209 children with SB (median age 10.3 years), depending on the formula used, eGFR decreased by -0.7 to -1.8 ml/min/1.73 m2/year (CKiDCys, CKiDScr, p ≤ 0.001), remained stable (CKiDSCr-Cys, p = 0.41), or increased by +2.7/year (ZappitelliSCr-Cys, p < 0.001) (Figure). The proportion of children with CKD 2 or higher varied between formulas (11.5-58.9%, p < 0.001). Correlations between pediatric formulas were negligible to moderate. Comparing any two formulas, 12.0-65.6% of children were assigned a different CKD stage. Among 164 adults (median age 26.3), eGFR decreased for each formula (range: -1.3 to -2.2/year, p ≤ 0.01) (Figure). The proportion of adults with CKD 2 or higher varied between formulas (9.2-30.5%, p < 0.001). Correlations between adult formulas were moderate to very high. Comparing any two formulas, 8.5-26.8% of adults were assigned a different CKD stage. COMMENT: We cannot reliably determine whether eGFR changed during childhood. Among adults, eGFR decreased with age for every formula evaluated at greater than twice the general population rate. Without a validated SB-specific eGFR formula, we are left with formulas providing different results. CONCLUSIONS: Estimated GFR among adults with SB appears to deteriorate at a higher rate than in the general population. Due to lack of precision of accepted eGFR formulas in the SB population, this may be a real phenomenon, an artifact of inaccurate eGFR formulas, or both. A validated SB-specific eGFR formula is needed.

Adherence with bladder irrigation following augmentation.

BACKGROUND: Patients with bladder augmentation (BA) are routinely counseled to irrigate their bladders daily. However, reports of adherence with this regimen are lacking. OBJECTIVE: To evaluate adherence to a bladder irrigation protocol and identify risk factors associated with adherence among adults with spina bifida (SB) and BA. STUDY DESIGN: Adults with SB after BA followed in a multidisciplinary clinic were identified (2017-2019). All patients or caregivers were taught the importance of and the technique for the bladder irrigation protocol prior to and after BA. Patient demographics (age, gender, ambulatory status, and presence of a caregiver in clinics) and surgical details (type of BA, age at surgery, length of follow-up, presence of a catheterizable channel, position of stoma, bladder neck surgery, presence of Malone antegrade colonic enema or ventriculo-peritoneal shunt, and number of stone surgeries) were obtained from the medical record. Patients reported other variables in a standardized clinic questionnaire. Answers were confirmed by health care providers. The variables included who performs clean intermittent catheterization (CIC), size of catheter, frequency of CIC, use of overnight catheterization, difficulties with CIC, number of UTIs, and continence per urethra and per catheterizable channel. Adherence to bladder irrigation was also assessed in the questionnaire. ?Strict adherence' was defined as bladder irrigation performed ≥6 times/week with ≥120 mL of saline. For statistical analysis, a more lenient definition of ?higher adherence' was used: bladder irrigation ≥2 times/week with at least 60 mL. ?Lower adherence' was defined as ≤1 time/week or with less than 60 mL. Factors associated with ?higher adherence' were assessed with non-parametric tests (Bonferroni-corrected p-value: 0.002). RESULTS: Adherence was assessed in 87 eligible patients (60.9% females; mean age of 28.8 ± 8.2 years). No patient (0.0%) reported ?strict adherence', and 62 and 25 patients (71.3% and 28.7%) reported ?higher' and ?lower' adherence' to bladder irrigation, respectively. Nine patients (10.3%) in the ?lower adherence' group did not irrigate at all. No variables were statistically significant on univariate analysis, including previous bladder stone surgery or having a channel (p ≥ 0.01). On exploratory analysis, higher adherence was only associated with self-catheterizations versus those performed by caregivers (76.7% vs 33.3%, p = 0.01). CONCLUSIONS: Adherence to a bladder irrigation protocol in adults with SB and BA is poor. A history of bladder stones requiring surgery and the presence of a catheterizable channel do not appear to affect adherence. It remains unclear why some patients are more likely than others to irrigate their bladders. Future work will focus on methods to improve adherence.

Beyond changing diapers: stress and decision-making among parents of girls with congenital adrenal hyperplasia seeking consultation about feminizing genital restoration surgery.

INTRODUCTION/BACKGROUND: The impact of having a child with atypical genitalia due to a life-threating chronic medical condition like congenital adrenal hyperplasia (CAH) is poorly understood. OBJECTIVE: The aim of the study was to determine parental stress and impact of CAH on parental decisions, including decisions regarding female genital restoration surgery (FGRS). STUDY DESIGN: The authors surveyed consecutive parents of girls with CAH ≤3 years presenting at a tertiary referral center for FGRS consultation (2016-2019). The survey was developed by three families of daughters with CAH and six clinicians. Nine potentially stressful past experiences were rated on a 6-point Likert scale ('not at all' to 'extremely' stressful). Overall parental stress and strain (broader negative consequences) were reported using validated instruments (Perceived Stress Scale and Caregiver Strain Questionnaire Short Form, respectively). Impact of CAH on past decisions about childcare, social interactions, and who changes diapers were also assessed. Non-parametric tests were used for analysis. RESULTS: Twenty-nine parents (median age: 32years) of 22 consecutive children participated (Prader 3/4/5: 59.1%/36.4%/4.5%). After the study, 20 girls (90.9%) underwent FGRS at a median age of 8 months. The most stressful experiences were having an adrenal crisis ('very much' stressful), waiting for the CAH diagnosis, and making sense of the diagnosis (both 'quite a bit') (Figure 1). Remaining issues were 'somewhat' stressful. Deciding whether to proceed with FGRS was ranked as the least stressful issue. Overall parental stress was similar to overall stress previously reported by spousal caregivers of stroke or heart failure survivors (P ≥ 0.15). Overall parental strain was similar to parents of adolescents receiving mental health counseling (P = 0.77). Congenital adrenal hyperplasia impacted decisions about babysitting, daycare, who changed diapers, and choosing a pediatrician (P ≤ 0.02), but did not impact parental social interactions (P ≥ 0.11). Diapers were typically changed by parents (100.0%) and grandmothers (50.0%). Parents anticipated that some individuals currently not allowed to change diapers would be allowed after FGRS: grandfathers (+18.2%), aunts/uncles (+27.3-32.8%), cousins (+18.2%), and family friends (+45.5%). DISCUSSION: The authors present the first assessment of parental stress with respect to different aspects of care of a daughter with CAH. Larger studies are required to determine if the parental stress associated with these experiences varies over time and how these stressors rank relative to each other through the child's development. CONCLUSION: Parents experience multiple stressors after having a daughter with CAH. Parental stress surrounding a decision about FGRS appears less severe than events pertaining to the diagnosis and medical management of CAH. Congenital adrenal hyperplasia impacts multiple parental decisions.

Superior Mesenteric Artery Syndrome Following Augmentation Cystoplasty.

Superior mesenteric artery syndrome (SMAS) is a rare condition of external duodenal compression in the angle between the superior mesenteric artery and aorta. We report a case of SMAS following augmentation cystoplasty in a young patient.