People with spina bifida use their MACE on long-term follow-up: A single institutional retrospective cohort study.

OBJECTIVE: While the Malone antegrade continence enema (MACE) facilitates bowel movements in patients with spina bifida (SB) and neuropathic bowel, little is known about its long-term use. We aimed to assess long-term MACE use and potential risk factors for disuse. METHODS: All patients with SB who underwent MACE procedures at our institution were retrospectively reviewed. Main outcome was MACE disuse (no longer catheterizing the MACE for antegrade enemas) based on self-report on a clinic questionnaire, or medical record for patients last seen before introducing the questionnaire 5 years ago. Survival analysis used two timeframes: time after surgery (Analysis 1) and chronological age: accounting for older children reaching adulthood earlier (Analysis 2). RESULTS: Overall, 411 patients (54% female, 78% shunted, 65% augmented) underwent a MACE procedure at median 7.9 years old (median follow-up: 8.4 years). Thirty-three (8%) patients no longer used their MACE. Most common reasons for doing so were channel/stomal stenosis (61%) and excision at colostomy or other abdominal surgery (12%). Bowel management afterwards included oral agents ± enemas (55%), Chait tube (30%), colostomy (12%). After correcting for differential follow-up, 90% of participants used their MACE at 10 years and 87% at 15 years after surgery. Based on chronological age, 97% used their MACE at 15 years old, 92% at 20 and 81% at 30 (Summary Figure). On multivariate analysis, umbilical MACEs were 2.4 times more likely to be disused than right lower quadrant MACEs (p = 0.04). Without correcting for chronological age (Analysis 1), patients undergoing MACE surgery at older ages were more likely to stop MACE use (p = 0.03). However, after accounting for chronological age (Analysis 2), patients undergoing a MACE procedure at older ages were no more likely to stop its use (p = 0.47, Figure). Gender, SB type, shunt status, mobility status, bladder augmentation or a urinary catheterizable channel were not associated with stopping MACE use (p ≥ 0.10). COMMENT: Participants were regularly followed in multi-disciplinary SB clinics. We did not assess continence, satisfaction or long-term urinary channel use, making it premature to recommend optimal stomal locations. CONCLUSIONS: Most patients with SB followed by a multi-disciplinary team continue using their MACE; 1% stopped MACE use annually, particularly after adolescence. This strongly suggests it is an effective bowel management method and transitioning to self-care plays a role in maintaining long-term MACE use. Umbilical MACEs may be at high risk of disuse, but all people with a MACE can benefit from support as they transition to adult care.

Age-based risk of end-stage kidney disease in patients with myelomeningocele.

OBJECTIVE: We aimed to quantify end-stage kidney disease (ESKD) risk after infancy in individuals with myelomeningocele (MMC) followed by urology in the modern medical era and to assess if ESKD risk was higher after surgery related to a hostile bladder. METHODS: We retrospectively reviewed patients with MMC followed by urology at our institution born ≥ 1972 (when clean intermittent catheterization was introduced) past 1 year of age (when mortality is highest, sometimes before establishing urology care). ESKD was defined as requiring permanent peritoneal/hemodialysis or renal transplantation. Early surgery related to hostile bladder included incontinent vesicostomy, bladder augmentation, detrusor Botulinum A toxin injection, ureteral reimplantation, or nephrectomy for recurrent urinary tract infections. Survival analysis and proportional hazards regression were used. Sensitivity analyses included: risk factor analysis with only vesicostomy, timing of surgery, including the entire population without minimal follow-up (n = 1054) and only patients with ≥ 5 years of follow-up (n = 925). RESULTS: Overall, 1029 patients with MMC were followed for a median of 17.0 years (49% female, 76% shunted). Seven patients (0.7%) developed ESKD at a median 24.3 years old (5 hemodialysis, 1 peritoneal dialysis, 1 transplantation). On survival analysis, the ESKD risk was 0.3% at 20 years old and 2.1% at 30 years old (Figure). This was ∼100 times higher than the general population (0.003% by 21 years old, p < 0.001). Patients who underwent early surgery for hostile bladder had higher ESKD risk (HR 8.3, p = 0.001, 6% vs. 1.5% at 30 years). On exploratory analyses, gender, birth year, shunt status and wheelchair use were not associated with ESKD risk (p ≥ 0.16). Thirty-year ESKD risk was 10% after early vesicostomy vs. 1.4% among children without one (p = 0.001). Children undergoing bladder surgery between 1.5 and 5 years old had a higher risk of ESKD. No other statistically/clinically significant differences were noted. COMMENT: Patients with MMC remain at risk of progressive renal damage throughout life. We relied on the final binary ESKD outcome to quantify this risk, rather than imprecise glomerular filtration rate formulas. Analysis was limited by few people developing ESKD, inconsistent documentation of early urodynamic findings and indications for bladder-related surgery. CONCLUSIONS: While ESKD is relatively uncommon in the MMC population receiving routine urological care, affecting 2.1% of individuals in the first 3 decades, it is significantly higher than the general population. Children with poor bladder function are likely at high risk, underlining the need for routine urological care, particularly in adulthood.

Ileal enterocystoplasty and B12 deficiency in pediatric patients.

PURPOSE: Vitamin B12 deficiency is a feared complication of enterocystoplasty but it has never been demonstrated in pediatric patients who have undergone ileal enterocystoplasty. We reviewed our series of more than 500 bladder augmentations in an attempt to define the timing and risk of vitamin B12 deficiency in pediatric patients after bladder augmentation. MATERIALS AND METHODS: From October 2004 to present we obtained serum B12 values in patients who had undergone bladder augmentation at our institution. We looked at patients who had undergone ileal enterocystoplasty and who were 18 years or younger at the time of augmentation. Any B12 value that was obtained while on any form of B12 supplementation was excluded. These criteria resulted in 79 patients with 105 B12 values. B12 values of 200 pg/ml or less were considered "low," and values between 201 and 300 pg/ml were considered "low-normal." RESULTS: There was a statistically significant correlation between followup time and serum B12 (p = 0.0001). The probability of low B12 increased as followup time increased (p = 0.007), as did the probability of low-normal B12 (p = 0.005). Starting at 7 years postoperatively 6 of 29 patients (21%) had low B12 values, while 12 of 29 (41%) had low-normal values. CONCLUSIONS: Pediatric patients who have undergone ileal enterocystoplasty are at risk for development of vitamin B12 deficiency. These patients are at the highest risk beginning at 7 years postoperatively, and the risk increases with time. We recommend an annual serum B12 value in children beginning at 5 years following bladder augmentation.

Upper and lower urinary tract outcome after surgical repair of cloacal malformations: a three-decade experience.

OBJECTIVE: To report the urological outcome of the surgical correction of persistent cloaca, which is technically demanding and may require many procedures in an effort to preserve renal function and provide urinary continence. PATIENTS AND METHODS: A retrospective chart review from 1971 to 2003 identified 23 patients with cloacal malformations (two posterior, 21 classical) that were reconstructed. The confluence of the urethra, vagina and rectum was noted to be high in 16, low in five and unknown in two; one patient was a conjoined twin. RESULTS: The mean (range) follow-up was 9.3 (0.4-31.6) years. Urinary anomalies included 14 patients with renal anomalies (six solitary kidneys, four renal dysplasia, two pelvi-ureteric junction, one each duplex and crossed fused) and two duplicated bladders. Vesico-ureteric reflux was present in 13 patients (57%), hydronephrosis at birth in 13, a bony vertebral abnormality in 14 and the VACTERL association in four. Total urogenital mobilization (TUM) was used in six patients and spinal cord untethering in four; a nephrectomy was required in three and partial nephrectomy in one. Upper tract dilation was still present in six patients. Age-adjusted creatinine levels were abnormal in four (18%) patients and borderline in another six (26%). In the nine patients with a solitary kidney (six solitary, three after nephrectomy), the age-adjusted creatinine level was abnormal in two and borderline in four. A vesicostomy was initially performed in 11 patients. The method of bladder emptying is known in 22; 10 void, 11 require clean intermittent catheterization (five abdominal stoma, six urethral) and one was diverted with a conduit. Of the 18 patients aged > 47 months 15 were continent (14 complete > 4 h, one partial 2-4 h), and three are wet (one conduit). Reconstruction of the lower urinary tract included four bladder augmentations (one ureteric, one ileal, two colon), five bladder neck procedures (two artificial sphincter, one each bladder neck repair, sling, bladder neck division) and six catheterizable channels (one now with a colon conduit). The ureters were re-implanted in 12 patients. CONCLUSION: Although the surgical correction of this rare malformation is complex, the upper urinary and lower urinary tract outcome can be favourable, albeit after several reconstructive procedures. TUM has emerged as the primary method for vaginal reconstruction, but the long-term lower tract outcome after this procedure is awaited.