Shelly J. King

APPENDICOVESICOSTOMY AND NEWER ALTERNATIVES FOR THE MITROFANOFF PROCEDURE: RESULTS IN THE LAST 100 PATIENTS AT RILEY CHILDREN′S HOSPITAL

PURPOSE We present our experience using the various Mitrofanoff techniques to create a continent catheterizable stoma as an adjunct to continent urinary tract reconstruction in children and young adults. MATERIALS AND METHODS Between 1990 and 1998 a Mitrofanoff procedure was performed at our institution in 55 male and 45 female patients with a mean age of 10.5 years. The etiology of incontinence was diverse but more than 90% of the patients had neurogenic bladder, the epispadias-exstrophy complex or a cloacal anomaly. Surgery included appendicovesicostomy in 57 cases, a Yang-Monti ileovesicostomy in 21, continent vesicostomy in 21 and formation of a tapered ileal segment as a catheterizable channel in 1. Simultaneously bladder augmentation was performed in 52 patients, bladder neck reconstruction was done in 48 and a Malone antegrade colonic enema stoma was constructed for fecal incontinence in 17. RESULTS The abdominal stoma is continent in 98 of our 100 patients. Mean followup is 2 years (range 2 months to 8 years) with the longer followup in the appendicovesicostomy group. One patient with stomal incontinence who underwent revision is now dry. Postoperative complications requiring an additional procedure developed in 20 patients, including stomal stenosis in 12. Continent vesicostomy was most prone to stomal problems (6 of 21 patients, 29%). CONCLUSIONS The Mitrofanoff procedure is a reliable technique for creating a continent catheterizable urinary stoma. Appendicovesicostomy continues to be our first option for this procedure, although we have also had good results with the Yang-Monti ileovesicostomy and continent vesicostomy. These newer options have allowed preservation of the appendix for the Malone antegrade colonic enema stoma procedure in patients with urinary and fecal incontinence.

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.

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).

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

The three papers in this section cover a wide range of subjects. Authors from Indianapolis present a three‐decade experience in upper and lower urinary tract outcomes after the surgical repair of cloacal malformations. An objective assessment of their results of hypospadias surgery is described by authors from Manchester. Finally, the question is asked by authors from Athens; is bladder muscle decompensation in boys with a history of PUV caused by secondary bladder neck obstruction?

CLOACAL EXSTROPHY: THE NEUROLOGICAL IMPLICATIONS

Congenital anomalies commonly accompany the abdominal wall defects of cloacal exstrophy with an incidence as high as 100% in some series. The urological literature cites an incidence of vertebral and/or spinal cord anomalies in 75% of cases. We reviewed the records of the 17 patients with cloacal exstrophy treated at our institution since 1978. Abnormalities of the vertebral column or spinal cord were identified in 16 patients, including 11 of 12 (92%) fully evaluated by myelography, computerized tomography or magnetic resonance imaging of the lumbosacral spinal cord. Nine patients had a lipomeningocele and 4 had a lipomyelocystocele. Spinal exploration, repair and cord untethering were done in 11 patients of whom 2 subsequently underwent reoperation for re-tethering the cord. A total of 15 patients is ambulatory although 5 require lower extremity braces. The incidence of spinal cord or vertebral anomalies in our patients with cloacal exstrophy approaches 100%, which is higher than commonly cited in the urological literature. We recommend magnetic resonance imaging of the spinal cord as part of the initial evaluation of newborns with cloacal exstrophy. Lifelong attention to the risk of spinal cord tethering can optimize neurological outcome in affected individuals.

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.

TAP WATER AND THE MALONE ANTEGRADE CONTINENCE ENEMA: A SAFE COMBINATION?

PURPOSE The Malone antegrade continence enema provides independence and improved quality of life in patients with fecal incontinence or intractable constipation. However, isolated reports of fatal hypernatremia after irrigation with normal saline have raised safety concerns about frequent colonic irrigation in children. Significant electrolyte abnormalities have also been reported with hypertonic phosphate and high colonic tap water enemas. Because our patients routinely use tap water for Malone antegrade continence enema irrigations, we examined the safety profile of this practice MATERIALS AND METHODS In the last 3.5 years 71 patients at our institution have used antegrade tap water enemas for managing fecal incontinence or intractable constipation. Standard serum electrolytes were measured RESULTS We obtained 101 sets of serum electrolyte measurements in 71 patients at a mean of 8.4 months postoperatively (range 1 to 33). A girl who presented with severe hyponatremia and hypochloremia had not used the Malone antegrade continence enema for several days. The most interesting finding was significantly elevated sodium and chloride in 1 case 6 weeks after surgery that was associated with tap water treated with a home softening system. Electrolytes reverted to normal 1 week after using untreated tap water CONCLUSIONS We did not detect significant hyponatremia or hypochloremia in any patient using tap water for Malone antegrade continence enema irrigation. Although dangerous electrolyte abnormalities are rare, potential morbidity in those cases warrants periodic evaluation. Due to the elevated sodium content in softened tap water families should be alerted to use untreated tap water for preparing enemas.

Lower Urinary Tract Reconstruction Using Stomach and the Artificial Sphincter

We implanted the artificial urinary sphincter at gastrocystoplasty in 13 male and 5 female patients. The diagnoses were classical bladder exstrophy (8 patients), myelodysplasia (8), cloacal exstrophy (1) and bilateral ectopic ureters (1). Mean patient age was 14.3 years (range 7 to 32.5). Six patients (5 with bladder exstrophy and 1 with clocal exstrophy) had failed bladder neck continence procedures, 3 myelodysplastic patients had failed artificial urinary sphincter placement, and 1 exstrophy patient had failed a Young-Dees bladder neck repair and artificial urinary sphincter placement. The sphincter cuff was placed around the reconstructed bladder neck in these patients, while in the remainder the artificial urinary sphincter was placed around the intact bladder neck. Mean followup was 20.3 months (range 1 to 5). Of the patients 16 (88%) are continent day and night, while 2 are wet. A total of 11 patients (61%) use Valsalvas maneuver alone for voiding and the remainder use Valsalvas maneuver and/or clean intermittent catheterization for bladder evacuation. Complications related to the artificial urinary sphincter were recurrent pump erosion requiring conversion to a Mitrofanoff continent stoma in 1 patient, and mechanical dysfunction requiring pump cuff and reservoir replacement in 3. There were no complications due to infection. Our report demonstrates that the combination of augmentation gastrocystoplasty and an artificial urinary sphincter leads to urinary continence and can allow for spontaneous urination. The rate of infectious complications is not increased when the 2 procedures are combined simultaneously.

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 B12 Therapy Effective for Vitamin B12 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.