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.

Sacral nerve stimulator success after filum section for refractory dysfunctional voiding.

INTRODUCTION: Filum section (FS) has been used to treat tethered cord syndrome (on MRI or occult) in pediatric patients with refractory dysfunctional voiding (DV). While controversial, some groups have previously reported significant improvement in patients' symptoms after FS, even in the presence of a normal preoperative spinal MRI. Until recently, it was our practice to refer patients with DV to pediatric neurosurgery for evaluation, MRI, and possible FS. OBJECTIVES: We report our experience with sacral nerve stimulator (SNS) placement for pediatric patients with refractory DV after failed FS with the primary outcome being complications and explantations. The secondary outcome being change in quality of life. STUDY DESIGN: We retrospectively reviewed all consecutive patients <18 years old who underwent second stage SNS placement after FS at our institution between November 2012 and December 2019. We abstracted rate of 2nd stage implantation, complication, and explantation. We also collected age-appropriate preoperative and postoperative 15-question quality of life questionnaires (PedsQL™). The PedsQL scores ranged 0-100, higher numbers correlating with higher quality of life. A paired t-test was used for statistical analysis. RESULTS: Overall, 23 children proceeded to the second stage SNS due to persistent symptoms following FS. Median age at SNS implantation was 10.3 years (IQR 7.5-11.8 years), and 13 were female (56.5%). There were no intraoperative complications. Median follow-up was 2.8 years (IQR 1.1-3.7 years). One patient had the SNS removed due to a need for MRI, one for resolution of symptoms, and 4 patients underwent lead revision for lead fracture and return of symptoms (17.4%). All 15 patients who completed both preoperative and postoperative PedsQL reported significantly improved overall scores after SNS (Figure). Median scores improved from 61.7 to 86.7 (p < 0.0001). DISCUSSION: Symptom relief in the child with refractory DV using SNS is not new. However, we sought to review our results in those with previous FS for tethered cord syndrome. While our study is limited by its small size, we assessed both surgical and patient-reported outcomes. Our patients' marked improvement in quality of life is similar to that of other reported pediatric SNS cohorts. CONCLUSION: SNS placement after FS is feasible, safe, and can result in significant improvement in overall quality of life. Given these findings, we have changed our practice to offer SNS in patients with refractory DV with a normal lumbosacral spinal MRI. For those with MRI abnormalities, we seek neurosurgical evaluation for FS (if deemed necessary) prior to considering SNS.

Troubleshooting problems with antegrade continent enema flushes: The Indiana university algorithm.

INTRODUCTION: Antegrade continence enemas have transformed treatment and improved the quality of life in children with neuropathic bowel, refractory constipation and fecal incontinence. However, it can often be difficult to manage problems that arise with ACE flushes. OBJECTIVE: We report the use of an online tool designed for nurses to help troubleshoot calls for problems associated with antegrade continence enema (ACE) flushes as well as update our algorithm for managing refractory constipation/fecal incontinence in a large single institution experience. STUDY DESIGN: We developed an online tool based on our management protocol for managing refractory constipation/fecal incontinence (Summary Figure). Patient frequency and bother was assessed prior to the intervention and at one month after the intervention using 5- and 4-point Likert scales respectively. Patient demographics, MACE/Chait information, type of difficulty, volume of flush, and use of additives were recorded. Nurses were also interviewed prior to using the tool and 14 months after its development with regards to taking these phone calls and the helpfulness of the tool. RESULTS: Over 14 months, the nurses received 22 patients calls via the nursing triage line regarding ACE flush problems and prospectively collected data. Half reported multiple episodes of fecal incontinence. Other complaints included no response to flush (8, 36.4%), occasional episodes of liquid fecal incontinence (2, 9.1%) and time of flush exceeding 60 min (1, 4.5%). While patients did not report decreased frequency of problems as a result of nurse troubleshooting using the ACE algorithm (2.5 vs. 2, p = 0.55), patients did report a significant improvement in their bother scores (4 vs. 2, p = 0.02). All but one patient reported that the recommendation was "some" or "a lot" helpful on follow up interview. The nurses all indicated that the tool helped "some" or "a lot." DISCUSSION: The antegrade continence enema is valuable in managing neurogenic bowel, refractory constipation, and fecal incontinence, however, some patients experience problems with flushes that can often be difficult to manage. CONCLUSION: Patients reported less bother with their bowel issues after using our algorithm for managing refractory constipation/fecal incontinence and nurses reported that the tool was helpful.

Radiographic abnormalities, bladder interventions, and bladder surgery in the first decade of life in children with spina bifida.

BACKGROUND: Spina bifida (SB) patients are at increased risk for hydronephrosis, bladder storage and emptying problems, and renal failure that may require multiple bladder surgeries. METHODS: We retrospectively reviewed patients born with SB 2005-2009, presenting to our institution within 1 year of birth. Outcomes at 8-11 years old included final renal/bladder ultrasound (RBUS) results, clean intermittent catheterization (CIC) use, anticholinergic use, surgical interventions, and final renal function. We excluded those without follow-up past age 8 and/or no RBUS or fluoroscopic urodynamic images (FUI) within the first year of life. Imaging was independently reviewed by four pediatric urologists blinded to radiologists' interpretation and initial findings compared with final outcomes. RESULTS: Of 98 children, 62 met inclusion criteria (48% male, 76% shunted). Median age at last follow-up was 9.6 years. Upon initial imaging, 74% had hydronephrosis (≥ SFU grade 1), decreasing to 5% at 10 years (p < 0.0001). Initially, 9% had ≥ SFU grade 3 hydronephrosis, decreasing to 2% (p = 0.13). CIC and anticholinergic use increased from 61% and 37% to 87% and 86%, respectively (p = 0.001 and p < 0.0001, respectively). With follow-up, 55% had surgical intervention and 23% had an augmentation. Of children with a serum creatinine/cystatin-C at 8-11 years old, one had confirmed chronic kidney disease (stage 2). CONCLUSIONS: Despite initial high incidence of hydronephrosis, this was low grade and resolved in the first decade of life. Additionally, the 8-11-year incidence of kidney disease and upper tract changes was low due to aggressive medical management.

Comparing inpatient versus outpatient bowel preparation in children and adolescents undergoing appendicovesicostomy.

PURPOSE: The need for mechanical inpatient bowel preparation (IBP) in reconstructive pediatric urology has come under scrutiny, secondary to literature demonstrating little benefit regarding outcomes. Starting in 2013, a majority of patients undergoing reconstructive procedures at our institution no longer underwent IBP. We hypothesized that outpatient bowel preparation (OBP) would reduce length of stay (LOS) without increasing postoperative complications after appendicovesicostomy surgery. MATERIALS AND METHODS: An institutional database of patients undergoing lower urinary tract reconstruction between May 2010 and December 2014 was reviewed. Starting in 2013, a departmental decision was made to replace IBP with OBP. Patients undergoing an augmentation cystoplasty or continent ileovesicostomy were excluded because of insufficient numbers undergoing OBP. Patients undergoing IBP were admitted 1 day prior to surgery and received polyethylene glycol/electrolyte solution. A personalized preoperative OBP was introduced in 2013. Cost data were obtained from the Pediatric Health Information System. RESULTS: Sixty-seven patients met the inclusion criteria, with 30 (44.8%) undergoing IBP. There were no differences with respect to gender, age, presence of ventriculoperitoneal shunt, body mass index, glomerular filtration rate, preoperative diagnosis, operative time, and prior or simultaneous associated surgeries (p ≥ 0.07). Patients undergoing an IBP had a longer median LOS (7 vs. 5 days, p = 0.0002) and a higher median cost (US$4,288, p = 0.01). Postoperative complications in both groups were uncommon and were classified as Clavien-Dindo grade 1-2, with no statistical difference (IBP 20.0% vs. OBP 5.4%, p = 0.13). No serious postoperative complication occurred, such as a dehiscence, bowel obstruction, or shunt infection. DISCUSSION: This is the first analysis of hospitalization costs and IBP, showing a higher median cost of US$4288 compared with OBP. The LOS was shorter with an OBP (figure), similar to a previous report. Similar complication rates between the groups add to the growing body of literature that avoidance of IBP is safe in pediatric lower urinary tract reconstruction. Being a retrospective review of a practice change, differences in care that influenced cost and LOS may be missing. Also, as the surgeons do not know if a usable appendix is initially present, our data may not extrapolate to all patients. Despite these potential limitations, our data support the safety of utilizing OBP in patients with a high likelihood of a usable appendix, including those undergoing a synchronous Malone antegrade continence enema via a split-appendix technique. CONCLUSION: In patients undergoing an appendicovesicostomy, preoperative IBP led to longer LOS and higher costs of hospitalization. OBP was not associated with increased risks of postoperative complications.

Validation of QUALAS-T, a health-related quality of life instrument for teenagers with spina bifida.

INTRODUCTION: We aimed to develop and validate a self-reported QUAlity of Life Assessment in Spina bifida for Teenagers (QUALAS-T). MATERIAL AND METHODS: We drafted a 46-question pilot instrument using a patient-centered comprehensive item generation/refinement process. A group of 13-17 years olds with spina bifida (SB) was recruited online via social media and in person at SB clinics (2013-2015). Healthy controls were recruited during routine pediatrician visits. Final questions were identified based on clinical relevance, factor analysis and domain psychometrics. Teenagers with SB completed the validated generic Kidscreen-27 instrument. RESULTS: Median age of 159 participants was 15.2 years (42.0% male, 77.4% Caucasian), similar to 58 controls (p ≥ 0.06). There were 102 online and 57 clinic participants (82.8% of eligible). Patients, parents and an expert panel established face and content validity of the 2-domain, 10-question QUALAS-T. Internal consistency and test-retest reliability were high for the Family and Independence and Bladder and Bowel domains (Cronbach's alpha: 0.76-0.78, ICC: 0.72-0.75). The Bladder and Bowel domain is the same for QUALAS-T , QUALAS-A for adults and QUALAS-C for children. Correlations between QUALAS-T domains were low (r = 0.34), indicating QUALAS-T can differentiate between distinct HRQOL components. Correlations between QUALAS-T and Kidscreen-27 were also low (r ≤0.41). QUALAS-T scores were lower in teenagers with SB than without (p <0.0001). CONCLUSIONS: QUALAS-T is a short, valid HRQOL tool for adolescents with SB, applicable in clinical and research settings. Since the Bladder & Bowel domains for all QUALAS versions are the same, Bladder and Bowel HRQOL can be measured on the same scale from age 8 through adulthood.

Design and Methodological Considerations of the Centers for Disease Control and Prevention Urologic and Renal Protocol for the Newborn and Young Child with Spina Bifida.

PURPOSE: Care of children with spina bifida has significantly advanced in the last half century, resulting in gains in longevity and quality of life for affected children and caregivers. Bladder dysfunction is the norm in patients with spina bifida and may result in infection, renal scarring and chronic kidney disease. However, the optimal urological management for spina bifida related bladder dysfunction is unknown. MATERIALS AND METHODS: In 2012 the Centers for Disease Control and Prevention convened a working group composed of pediatric urologists, nephrologists, epidemiologists, methodologists, community advocates and Centers for Disease Control and Prevention personnel to develop a protocol to optimize urological care of children with spina bifida from the newborn period through age 5 years. RESULTS: An iterative quality improvement protocol was selected. In this model participating institutions agree to prospectively treat all newborns with spina bifida using a single consensus based protocol. During the 5-year study period outcomes will be routinely assessed and the protocol adjusted as needed to optimize patient and process outcomes. Primary study outcomes include urinary tract infections, renal scarring, renal function and bladder characteristics. The protocol specifies the timing and use of testing (eg ultrasonography, urodynamics) and interventions (eg intermittent catheterization, prophylactic antibiotics, antimuscarinic medications). Starting in 2014 the Centers for Disease Control and Prevention began funding 9 study sites to implement and evaluate the protocol. CONCLUSIONS: The Centers for Disease Control and Prevention Urologic and Renal Protocol for the Newborn and Young Child with Spina Bifida began accruing patients in 2015. Assessment in the first 5 years will focus on urinary tract infections, renal function, renal scarring and clinical process improvements.

Quality of Life Assessment in Spina Bifida for Children (QUALAS-C): Development and Validation of a Novel Health-related Quality of Life Instrument.

OBJECTIVE: To develop and validate a self-reported health-related QUAlity of Life Assessment in Spina bifida for Children (QUALAS-C). METHODS: We drafted a 27-question pilot instrument using a patient-centered comprehensive item generation and refinement process. It was administered to a sample of children 8-12 years old with spina bifida (SB) recruited online via social media and in person at an outpatient SB clinic (January 2013-September 2014). Healthy controls were recruited at routine pediatrician visits. Validation and final questions were determined based on clinical relevance, high loadings on factor analysis, and domain psychometrics. Children with SB also completed the validated generic Kidscreen-27 instrument. RESULTS: Median age of 150 participants was 9.6 years (60.7% male, 72.7% Caucasian), similar to 46 controls (P ≥ .10). There were 97 online and 53 clinic participants (89.0% and 84.2% of eligible, respectively). Face and content validities of the 2-domain, 10-question QUALAS-C were established by patients, parents, and experts. Internal consistency and test-retest reliability was high for the Esteem & Independence and Bladder & Bowel domains (Cronbach's alpha: 0.72-0.76, ICC: 0.74-0.77). Correlations between QUALAS-C domains were low (r = 0.51), indicating that QUALAS-C can differentiate between two distinct health-related quality of life components. Correlations between QUALAS-C and Kidscreen-27 were also low (r ≤ 0.44). QUALAS-C scores were significantly lower in children with SB than without (P < .0001). CONCLUSION: QUALAS-C is a short, valid health-related quality of life tool for children with SB. It will be useful in clinical and research settings.

Vitamin B12 deficiency in patients after enterocystoplasty.

INTRODUCTION: Serum B12 deficiency is a known sequlae of enterocystoplasty. The complications of B12 deficiency include megaloblastic anemia, neuropsychiatric disease, and demyelinating diseases such as peripheral neuropathy. Some studies have suggested that underlying disease states may be more important than enteric absorptive capacity in predicting acquired B12 deficiency. A 38% incidence of low or low-normal serum B12 in patients who have undergone enterocystoplasty has previously been reported, and oral B12 supplementation has been demonstrated to be an effective short-term therapy; however, the long-term results remain unclear. AIMS: This study hypothesized that oral vitamin B12 supplementation in patients with B12 deficiency following enterocystoplasty is an effective long-term treatment. Additionally, it sought to determine if underlying disease state predicts B12 deficiency following enterocystoplasty. DESIGN: Children who underwent enterocystoplasty at the present institution prior to August 2007 were reviewed. Patients with non-ileal augment, insufficient follow-up or hematologic disorders were excluded. Patients with low or low-normal B12 levels were included. Treatment consisted of daily oral therapy of 250 mcg or monthly parenteral therapy of 1000 mcg IM. Separately, the institutional database of 898 patients who underwent enterocystoplasty was searched and patients with at least one post-operative B12 level were highlighted. The indication for enterocystoplasty was classified as neuropathic or non-neuropathic. RESULTS: Twenty-three patients met inclusion criteria. The mean follow-up was 49 months (range 5-85) following initial abnormal B12 level. On the last follow-up, 4/23 (17%) patients had normal serum B12 levels. No patients reported sequelae of long-term B12 deficiency. In the secondary investigation, 113 patients met inclusion criteria. A total of 101 had neuropathic indications for enterocystoplasty, and 12 had non-neuropathic indications. At any time during follow-up, 48/101 (47.5%) neuropathic patients had low or low-normal B12 levels, and 4/12 (33.3%) non-neuropathic patients had low or low-normal B12 levels during follow-up (P = 0.54) (Figure). DISCUSSION: The initial success of oral B12 deficiency treatment following enterocystoplasty does not persist over time. This contradicts previous results with short duration follow-up. Underlying disease as the indication for enterocystoplasty did not predict B12 deficiency risk. The study was limited by the small number of patients with B12 deficiency who were started on treatment, as well as by the small number of patients with non-neuropathic indications for enterocystoplasty. CONCLUSION: The aims of the study were met. Further investigation is required to assess predictors of B12 deficiency following enterocystoplasty.

QUAlity of Life Assessment in Spina bifida for Adults (QUALAS-A): development and international validation of a novel health-related quality of life instrument.

PURPOSE: Health-related quality of life (HRQOL) is important in spina bifida (SB) management. No clinically useful, comprehensive instrument incorporating bladder/bowel domains exists. We aimed to develop and validate a self-reported QUAlity of Life Assessment in Spina bifida for Adults (QUALAS-A). METHODS: We drafted the 53-question pilot instrument using a comprehensive item generation/refinement process. It was administered to an international convenience sample of adults with SB and controls recruited online via social media and in person at outpatient SB clinics (January 2013-September 2014). Final questions were determined by: clinical relevance, high factor loadings and domain psychometrics in an Internal Validation Sample randomly selected from United States participants (n = 250). External validity was evaluated in United States and International External Validation Samples (n = 165 and n = 117, respectively). Adults with SB completed the validated general WHOQOL-BREF and International Consultation on Incontinence Questionnaire (ICIQ). RESULTS: Mean age of 532 participants was 32 years (32.7 % males, 85.0 % Caucasian), similar to 116 controls (p ≥ 0.08). There were 474 online and 58 clinical participants (61.1 % eligible). Face validity and content validity of the 3-domain, 15-question QUALAS-A were established by patients, families and experts. Internal consistency and test-retest reliability were high for all domains (Cronbach's alpha ≥ 0.70, ICC ≥ 0.77). Correlations between QUALAS-A and WHOQOL-BREF were low (r ≤ 0.60), except for high correlations with Health and Relationships domain (0.63 ≤ r ≤ 0.71). Bladder and Bowel domain had a high correlation with ICIQ (r = -0.70). QUALAS-A scores were lower among adults with SB than without (p < 0.0001). QUALAS-A had good statistical properties in both External Validation Samples (Cronbach's alpha 0.68-0.77). CONCLUSIONS: QUALAS-A is a short, valid HRQOL tool for adults with SB.

Mortality after bladder augmentation in children with spina bifida.

PURPOSE: Renal failure has been a leading cause of death for children with spina bifida. Although improvements in management have increased survival, current data on mortality are sparse. Bladder augmentation, a modern intervention to preserve renal function, carries risks of morbidity and mortality. We determined long-term mortality and causes of death in patients with spina bifida treated with bladder augmentation. MATERIALS AND METHODS: We retrospectively reviewed the records of patients with spina bifida who underwent bladder augmentation between 1979 and 2013. Those born before 1972 or older than 21 years at augmentation were excluded. Demographic and surgical data were collected. Outcomes were obtained from medical records, death records and the Social Security Death Index. Fisher exact and Wilcoxon rank-sum tests and Kaplan-Meier plots were used for analysis. RESULTS: Of 888 patients in our bladder reconstruction database 369 with spina bifida met inclusion criteria. Median followup was 10.8 years. A total of 28 deaths (7.6%) occurred. The leading causes of mortality were nonurological infections (ventriculoperitoneal shunt related, decubitus ulcer fasciitis, etc) and pulmonary disease. Two patients (0.5%) died of renal failure. No patient died of malignancy or bladder perforation. Patients with a ventriculoperitoneal shunt had a higher mortality rate than those without a shunt (8.9% vs 1.5%, p = 0.04). CONCLUSIONS: Previously reported mortality rates of 50% to 60% in patients with spina bifida do not appear to apply in children who have undergone bladder augmentation. On long-term followup leading causes of death in patients with spina bifida after bladder augmentation were nonurological infections rather than complications associated with augmentation or renal failure.

Is oral vitamin B(12) therapy effective for vitamin B(12) deficiency in patients with prior ileocystoplasty?

PURPOSE: We previously identified vitamin B(12) deficiency as a potential long-term consequence in pediatric patients with prior ileocystoplasty despite adequate preservation of terminal ileum. Vitamin B(12) deficiency can result in hematological and neurological deficits, of which some are irreversible. Deficiency discovered after ileocystoplasty is purportedly due to B(12) malabsorption since the principal absorption site is ileum. B(12) deficiency due to malabsorption is typically treated with intramuscular injection to ensure adequate treatment. We determined whether oral vitamin B(12) supplementation could increase serum vitamin B(12) in patients with deficiency who underwent ileocystoplasty. MATERIALS AND METHODS: During followup after ileocystoplasty we identified patients with low (200 pg/dl or less) or low normal (200 to 300 pg/dl) vitamin B(12). Oral vitamin B(12) was begun at 250 µg. Serum B(12) was assessed at 1, 2 and 3-month intervals after beginning therapy. RESULTS: A total of 128 patients with a mean followup of 83 months after ileocystoplasty had vitamin B(12) levels available for review. Of these patients 36 (28%) had a level of 300 pg/dl or less with a level of 200 pg/dl or less in 16 (13%). After oral vitamin B(12) treatment serum levels increased from a mean 235 to 506 pg/dl (114%) upon initial measurement (p <0.001). Subsequent measurements continued to increase from the first posttreatment level (p <0.05). No adverse effects were noted during a mean 4-month followup. CONCLUSIONS: To our knowledge this is the first study to show that oral vitamin B(12) effectively increases serum levels in pediatric patients with prior ileocystoplasty.

Tap water irrigation and additives to optimize success with the Malone antegrade continence enema: the Indiana University algorithm.

PURPOSE: We report the long-term effectiveness of standard tap water for Malone antegrade continence enema irrigation as well as our algorithm for managing refractory constipation/fecal incontinence in a large single institution experience. MATERIALS AND METHODS: We retrospectively reviewed the charts of 256 Malone antegrade continence enema procedures performed for chronic constipation and/or incontinence due to neuropathic bowel. Continence, type of fluid used to irrigate the colon, volume of flushes and the need for additives were recorded and a database was created. All patients were initially treated with tap water irrigation. Those in whom tap water irrigation failed underwent complete bowel cleanout with enemas and GoLYTELY via the Malone antegrade continence enema, followed by a gradual increase in irrigation volume. If this was unsuccessful, additives of mineral oil, MiraLAX or glycerin were added to the irrigant daily. RESULTS: A total of 236 patients with at least 6 months of followup were included in this study. Mean age at surgery was 10.2 years (range 2 to 36) and mean followup in the entire cohort was 50 months (range 6 to 115). Mean volume of colonic flushes was 642 ml (range 100 to 1,000). Of the patients 196 (83.1%) achieved total fecal continence with tap water flushes alone. Using additives increased the overall continence rate to 93.6% (p <0.0001). CONCLUSIONS: The Malone antegrade continence enema procedure has proved invaluable for treating children with refractory constipation. When additives are used in conjunction with water flushes, they can significantly improve the overall fecal continence rate in partially continent children.

The Malone antegrade continence enema: single institutional review.

PURPOSE: We report the long-term outcome of the Malone antegrade continence enema procedure in the treatment of chronic constipation and fecal incontinence in children with neuropathic bowel. MATERIALS AND METHODS: We performed a retrospective chart review of 256 Malone antegrade continence enema procedures. Patient age at surgery, bowel segment used, location of stoma, number and type of revisions required, and fecal continence were documented and a database was created. RESULTS: A total of 236 patients with at least 6 months of followup are included in this report. There were 172 in situ appendicocecostomies, 23 split appendix channels, 9 appendicocecostomies with cecal extension, 22 Yang-Monti ileocecostomies and 10 colon flap channels performed. A total of 112 males (47%) and 124 females (53%) were included in the study. Median patient age at surgery was 9 years (range 2 to 36) and median followup for the entire cohort was 50 months (6 to 115). A total of 56 surgical revisions were performed in 39 patients (17%). Median time to first revision was 9.5 months (range 1 to 105). Stomal stenosis occurred in 32 patients (14%). Overall, surgical revisions were required in 14% of in situ appendix channels (24 of 172), 22% of split appendix channels (5 of 23), 33% of appendix channels with cecal extension (3 of 9), 18% of Yang-Monti Malone antegrade continence enema channels (4 of 22) and 30% of colon flap channels (3 of 10). Independent of channel technique, surgical revisions were required in 15% of umbilical stomas and 18% of lower quadrant stomas (p = 0.516). Two patients had minimal stomal leakage, and 94% (221 of 236) achieved fecal continence with irrigations. CONCLUSIONS: The long-term results of the Malone antegrade continence enema channel in a large cohort of patients with neuropathic bowel and chronic constipation are encouraging. Of the patients 17% will require revision surgery, and patients/parents should be counseled accordingly. In our series in situ appendicocecostomy had the lowest revision rate on long-term followup, although the difference was not statistically significant (p = 0.226).

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.

The Malone antegrade continence enema procedure: quality of life and family perspective.

PURPOSE: Since introducing the Malone antegrade continence enema (MACE) procedure into our practice, it has been our bias that social confidence and independence are significantly improved and satisfaction is overwhelmingly high. We objectively determine outcomes after the MACE to refine patient selection, and maximize the quality of perioperative counseling and teaching. MATERIALS AND METHODS: An anonymous questionnaire was mailed to all patients who had undergone the MACE procedure within the last 4 years. Patient/parent satisfaction, impact on quality of life and clinical outcome were assessed with Likert scales. Demographic information, MACE specifics, preoperative expectations, and unanticipated benefits and problems were also recorded. RESULTS: A total of 65 questionnaires were returned from our first 92 patients (71%). Myelodysplasia was the primary diagnosis in 88% of patients. Complete or near complete fecal continence was achieved in 77% of patients and all others had improved incontinence. The highest level of satisfaction was reported by 89% of patients. Social confidence and hygiene were significantly improved. Daily time commitment, pain/cramping, intermittent constipation and time for fine-tuning the regimen were cited as unanticipated issues. CONCLUSIONS: The MACE procedure has received high praise from patients and families after years of battling constipation and fecal incontinence. Significant improvement rather than perfection is the realistic expectation. Objective feedback from patients and families will continue to improve patient selection and education.