Anthony J. Casale

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.

BLADDER CALCULI IN THE PEDIATRIC AUGMENTED BLADDER

PURPOSE Bladder augmentation is now a commonly accepted treatment in children with neuropathic bladder and other bladder anomalies. Bladder calculi have been reported in a third to a half of pediatric patients after bladder augmentation. We identify the incidence of bladder calculi and risk factors for stone formation in a large series of pediatric patients after bladder augmentation. MATERIALS AND METHODS We reviewed the records of 286 patients who underwent bladder augmentation between 1978 and 1994, assessed the incidence of and risk factors for bladder calculi, and reviewed treatment methods. RESULTS Bladder calculi developed in 29 of the 286 patients (10%) who underwent bladder augmentation. The type of bowel used for augmentation did not affect the rate of stone formation except stomach, which did not lead to stone formation in any case. Stones formed more commonly after bladder outlet resistance procedures and in patients with catheterizable abdominal wall stomas. Patients underwent open cystolithotomy or cystolitholapaxy with an overall 44% recurrence rate and no statistically significant difference between treatment methods. CONCLUSIONS Bladder calculi are a known complication of bladder augmentation. An increased risk of stone formation is associated with bladder outlet resistance procedures and catheterizable abdominal wall stomas. Daily irrigations to clear mucus and crystals as well as complete emptying of the augmented bladder may have important roles in decreasing stone formation.

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.

Spontaneous bladder perforations: a report of 500 augmentations in children and analysis of risk.

PURPOSE The spontaneous perforation of an augmented bladder is an uncommon but serious complication. To our knowledge our institution has the largest reported series of bladder augmentations. We examined our data to determine the incidence of spontaneous bladder perforation and to delineate associated risk factors. MATERIALS AND METHODS We performed a retrospective chart review of 500 bladder augmentation procedures performed during the preceding 25 years with a minimum followup of 2 years. RESULTS Spontaneous perforations occurred in 43 patients (8.6%), for a total of 54 events. The calculated risk was 0.0066 perforations per augmentation-year at risk. Approximately a third of the cases had perforated within 2 years of surgery, a third between 2 and 6 years postoperatively, and a third at more than 6 years after augmentation. Patients who underwent augmentation between 1997 and 2003 had a higher rate of perforation within 2 years of surgery than those operated on between 1978 and 1987. Increased risk of perforation was observed with the use of sigmoid colon and bladder neck surgery. A decreased risk was associated with the presence of a continent catheterizable channel. CONCLUSIONS We believe that this large and comprehensive series gives valuable insight into this serious complication. The delineation of these potential risk factors serves as a guide for further discussion and investigation.

A long continent ileovesicostomy using a single piece of bowel

PURPOSE In 1981 Mitrofanoff presented a procedure to create a continent urinary stoma for intermittent catheterization. Since then, several other methods have been introduced, including the Yang-Monti ileovesicostomy. The length of these ileovesicostomies is limited by the circumference of the bowel segment used, which is inadequate in some cases. We developed a procedure to double the length of the Yang-Monti ileovesicostomy using a single section of bowel. MATERIALS AND METHODS A 3.5 cm. section of ileum is isolated on its mesentery. The bowel is divided into 2 segments for 80% of its circumference, leaving the bowel intact over the mesentery. Each ring of bowel is then divided adjacent to the mesentery but on opposite sides, allowing the bowel to be unfolded and reconfigured in a single long strip that may then be tubularized. The blood supply to the tube is excellent and it is in the center of the reconfigured ileum. The ends may be trimmed or widely spatulated as necessary. RESULTS We have performed this procedure in 8 patients. The resulting ileovesicostomy created from a 3.5 cm. section ofileum is 10 to 14 cm. long and accepts a 12F catheter. A larger tube may be created from a longer piece of ileum. All patients are dry and they perform catheterization easily. CONCLUSIONS This form of ileovesicostomy allows the creation of a long bowel tube that is easily catheterized. The longer length of the tube increases application of the continent stoma principle to more patients and enables reconstruction to be performed with optimal placement and without tension.

Management of Ectopic Ureters: Experience With the Upper Tract Approach

PURPOSE The necessity of removing the ureteral stump after upper tract surgery for an ectopic ureter has been debated. We reviewed the records of patients initially treated at the kidney level to evaluate indications for later stump removal. MATERIALS AND METHODS We reviewed the medical records of 32 patients with 33 ectopic ureters treated at the kidney level during the last 10 years. RESULTS Ectopic ureters were associated with duplicated collecting systems in 31 cases and with single systems in 2. Upper pole heminephrectomy and partial ureterectomy were performed in 23 units and upper tract reconstruction was done in 8. Both patients with single systems underwent nephrectomy. Four patients (12%) required repeat surgery at the bladder level, including 1 who underwent ureteral reimplantation for persistent ipsilateral lower pole reflux and simultaneous upper pole stump removal. Preoperative voiding cystourethrography revealed reflux into the ectopic ureter in 1 patient with postoperative reflux and infections. The remaining 2 patients required a repeat operation to remove the stump due to recurrent urinary tract infections and newly detected reflux into the stump, respectively. CONCLUSIONS The majority of patients with ectopic ureters can be treated by addressing only the upper urinary tract. No patient who presented with incontinence required ureteral stump removal. Whether noted preoperatively or postoperatively, reflux into the ectopic ureter necessitated ureteral stump removal. Three of the 6 patients (50%) who had reflux to the ipsilateral kidney required lower tract surgery.

CONCEALED PENIS IN CHILDHOOD: A SPECTRUM OF ETIOLOGY AND TREATMENT

PURPOSE Concealed penis is an uncommon condition due to poor skin fixation at the base of the penis, cicatricial scarring after penile surgery and excessive obesity. The condition varies in severity and several surgical options are available, such as excision of previous scarring, degloving the penile shaft, reconstructing the penile shaft skin with flaps, fixing the penile skin at the penopubic and penoscrotal angles, and removing excess suprapubic fat. MATERIALS AND METHODS We reviewed the records of 43 patients treated for concealed penis from 1993 to 1998. We categorized the cases as type 1-congenital concealed penis, type 2-concealed penis due to scarring from previous surgery and type 3-complex cases involving excessive obesity. Cases were reviewed in regard to surgical techniques and outcomes. We identified 18 type 1, 18 type 2 and 7 type 3 cases. Mean age of type 1 patients at surgery was 12.4 months with 1 patient presented at age 7 years. None had previously undergone penile surgery. All patients underwent complete penile degloving. To reconstruct the penile shaft flaps or Z-plasties with penile skin were used in 12 patients and scrotal skin flaps were used in 2. In 12 patients the penile skin was fixed at the penoscrotal and penopubic angles to maintain penile length and in 2 excess fat was excised. Mean age of type 2 patients at surgery was 19.8 months. All had previously undergone surgery, including hypospadias in 1 and circumcision in 17. All patients underwent complete penile degloving and the cicatricial scar that trapped the penis was excised. Penile skin flaps and Z-plasties were used in 12 cases, scrotal skin flaps were used for reconstruction in 2 and skin grafting was done in 1. In 10 patients the penile skin was fixed with sutures to maintain penile length. Mean age of type 3 patients at surgery was 15.8 years. Of the 7 boys 6 had previously undergone penile surgery. All required extensive scar excision and complex reconstruction involving penile skin flaps in 3, scrotal flaps in 5 and penile skin fixation in 6. Excessive suprapubic fat was removed in 5 patients, of whom 3 underwent liposuction. RESULTS Surgical results were uniformly good in type 1 patients except in 1 who was believed to have excessive suprapubic fat. Results were good in 14 of the 18 type 2 patients, although 2 retained excessive suprapubic fat and 2 had some unsightly scarring. No type 1 or 2 patient required additional surgery. Of the 7 type 3 patients 6 had a good result and required no additional surgery. One patient has recurrent concealed penis after 2 procedures and awaits additional surgery. CONCLUSIONS Concealed penis has a varied etiology and requires a flexible surgical approach. The common surgical options in all cases include complete penile degloving, excising the scarring due to previous surgery, removing excess suprapubic fat, reconstructing the penile skin with local flaps, and fixing the penile skin at the penopubic and penoscrotal angles.

Partial urogenital mobilization: A limited proximal dissection

INTRODUCTION The treatment of urogenital sinus malformations is complex and controversial. Despite numerous and significant contemporary surgical advances, the dissection of the urogenital sinus remains technically challenging. METHODS Based on total urogenital mobilization, we describe a technique whereby this dissection is limited to the pubourethral ligament. Our short-term results with partial urogenital mobilization (PUM) performed on 15 patients are retrospectively reviewed. RESULTS There were no intraoperative complications and the short-term cosmetic results are excellent. No patients have developed voiding dysfunction or urinary tract complications. CONCLUSIONS While total urogenital mobilization is a very effective procedure, we believe that the PUM approach limits potential morbidity in the reconstruction of these complex problems.

The Indiana Experience With Artificial Urinary Sphincters in Children and Young Adults

PURPOSE We reviewed a 22-year single institutional experience with the artificial urinary sphincter in children and adolescents. To our knowledge this report represents the largest series in the world in children. MATERIALS AND METHODS Between 1980 and 2002, 142 patients underwent implantation of an artificial urinary sphincter, of whom 93 males and 41 females with a median age of 10 years (range 3 to 39) were available for analysis. A total of 59 patients initially received an AMS 742/792 (American Medical Systems, Inc., Minnetonka, Minnesota) artificial urinary sphincter, of whom 33 were subsequently changed to an AMS 800, while 75 initially received an AMS 800 model. Sphincter followup was terminated at device removal or at the last documented contact. The etiology of incontinence was neuropathic bladder in 107 cases (80%), the exstrophy/epispadias complex in 21 (16%) and other in 6 (4%). Outcome measures included continence, mechanical complications (leakage, tube kink and pump malfunction), functioning sphincter revisions (change in cuff size, pump repositioning and bulbar cuff placement), surgical complications (erosion, infection and misplacement) and associated surgical procedures. Mean followup of the pre-800 and 800 models was 6.9 (range 0.2 to 21.5) and 7.5 years (range 0.1 to 17.1), respectively. Fishers exact test, Kaplan-Meier life analysis and the chi-square test were used for statistical analysis. RESULTS After artificial urinary sphincter placement in the 134 patients continence was achieved in 86%, improved in 4% and not achieved in 10%. Of those with a sphincter in place 92% were continent. In terms of bladder emptying after artificial urinary sphincter insertion 22% of patients voided, 11% voided combined with clean intermittent catheterization, 48% performed clean intermittent catheterization only via the urethra, 16% performed it via a catherizable channel and 3% used urinary diversion. A mechanical complication developed in 38 of the 59 patients (64%) with pre-800 model compared with 33 of the 109 (30%) with the 800 model (p <0.0001). A mechanical complication occurred every 7.6 versus 16 patient-years for the pre-800 versus 800 models (p = 0.0001). Revision was required in 15 of the 59 patients (25%) with a pre-800 model versus 17 of the 109 (16%) with the 800 model (p = 0.103). Revision was performed every 22.7 versus 44.3 patient-years for the pre-800 versus the 800 model (p = 0.023). The artificial urinary sphincter eroded in 11 of the 59 patients (19%) with the pre-800 versus 17 of the 109 (16%) with the 800 model (p = 0.52). Ten patients experienced a total of 12 perforations of the augmented bladder after artificial urinary sphincter implantation. A total of 164 secondary surgical procedures were performed, including 38 of 134 bladder augmentations (28%). A total of 30 sphincters were permanently removed. CONCLUSIONS The artificial urinary sphincter is the only bladder neck procedure that allows spontaneous voiding in the neuropathic population, obviates the need for clean intermittent catheterization and yet is compatible with it when necessary. It is also equally versatile in the 2 genders. Mechanical complications occur but they were dramatically decreased by the modifications of the AMS 800 model. In addition, secondary bladder augmentation was required in 28% of our patients. Lifelong followup is mandatory in all patients with an artificial urinary sphincter.

Long-Term Followup and Outcome of Continent Catheterizable Vesicostomy Using the Rink Modification

PURPOSE Multiple techniques have been described to create a Mitrofanoff channel in the pediatric population. A small subset of patients only requires creation of a catheterizable channel without bladder augmentation. These patients are ideal candidates for a procedure that avoids the use of intestine, especially in the absence of a suitable appendix. We used a modification of the Casale vesicostomy, as described by Rink, to create a continent vesicostomy in these children. We report our long-term experience with this technique. MATERIALS AND METHODS We retrospectively reviewed the medical records of all patients who underwent continent vesicostomy at our institution between 1992 and 2000. Patient diagnosis, stomal site, associated bladder procedures, stomal continence, followup and complications associated with continent vesicostomy were documented. RESULTS Of the 31 patients who underwent continent vesicostomy, as described by Rink, 14 were female and 17 were male. Average age was 9 years (range 2.5 to 22). Primary diagnosis included neuropathic bladder in 15 cases, the prune-belly syndrome in 6, cloacal exstrophy/anomaly in 5 and other in 5. The stoma was placed in the lower abdomen in 17 patients, in the umbilicus in 7 and in a neoumbilicus in 7. Simultaneous procedures included ureteral reimplantation in 8 cases, bladder augmentation in 5, bladder neck surgery in 4 and reduction cystoplasty in 2. Mean followup was 41 months. All patients achieved excellent stomal continence. Complications included stomal stenosis requiring revision in 14 cases (45%). Stenosis developed in 60% of the patients with neuropathic bladder and in 86% with an umbilical stoma. Eventually 6 patients underwent conversion to an alternative catheterizable channel. CONCLUSIONS Continent vesicostomy can be performed successfully when there is any underlying bladder pathology with 100% stomal continence. Despite the higher rate of stomal problems with this type of Mitrofanoff channel we think that continent vesicostomy is a reasonable alternative in patients with a large bladder requiring only catheterizable channel creation. Because of the excellent results reported with the Monti-Yang technique, we would currently recommend this procedure over continent vesicostomy when bowel is used for bladder reconstruction.