Richard C. Rink

Regenerative Urinary Bladder Augmentation Using Small Intestinal Submucosa: Urodynamic and Histopathologic Assessment in Long-term Canine Bladder augmentations

PURPOSE To evaluate small intestinal submucosa (SIS) as a possible bladder augmentation material. MATERIALS AND METHODS Nineteen male dogs underwent 35 to 45% partial cystectomy with immediate augmentation with SIS grafts. All dogs were evaluated pre- and postoperatively with blood chemistries, urine cultures, intravenous urograms, cystograms and cystometrograms. Postoperatively (1 to 15 months), bladders were examined with routine histology and image analysis. RESULTS All dogs survived their intended survival period without morbidity. All results were normal. Histologically, all 3 layers (mucosa, smooth muscle, serosa) of the normal bladder showed evidence of regeneration. CONCLUSIONS Small intestinal submucosa acts as a scaffold for bladder augmentation through regeneration and could be a potential option for bladder reconstruction.

Experimental assessment of small intestinal submucosa as a bladder wall substitute

OBJECTIVES This study determined the feasibility of promoting urinary bladder regeneration with porcine-derived small intestinal submucosa (SIS). METHODS Twenty-two Sprague-Dawley rats underwent partial cystectomy with immediate bladder augmentation with SIS. Bladders were harvested for histologic evaluation at 2, 4, 8, 12, 24, and 48 weeks. RESULTS Histologically at 2 weeks, there was infiltration of the graft material with viable host cells consisting of fibroblasts, macrophages, and blood vessels covered by complete mucosal urothelium comprised of transitional cells. During the next 10 weeks, collagen formation and maturation were noted, and by the end of 12 weeks, the SIS graft was comprised of a mature collagen matrix admixed with thinly scattered disorganized smooth muscle bundles and covered by normal urothelium. At 48 weeks, all three layers of the normal bladder (urothelium, smooth muscle, and serosa) were present and were grossly and microscopically indistinguishable from the normal rat urinary bladder. CONCLUSIONS This study further supports the concept of bladder regeneration and suggests that SIS may be a viable material for bladder augmentations.

Gastrocystoplasty: An alternative solution to the problem of urological reconstruction in the severely compromised patient

An isolated segment of stomach was used for bladder augmentation in 10 patients or construction of a continent urinary reservoir in 3. Diagnosis in these 13 patients included cloacal exstrophy (5), myelodysplasia (4), posterior urethral valves (2), radiation cystitis (1) and neurogenic bladder secondary to a rectal pull-through procedure (1). Indications for the use of stomach in bladder reconstruction were decreased renal function and acidosis (6 patients), insufficient large and small bowel (6) and decreased mucus production (1). Postoperative followup averaged 13 months (range 6 to 23 months). All patients have stable upper tracts radiographically and stable or improved renal function. Of 13 patients 10 require intermittent clean catheterization to empty and 11 are completely continent. Nine patients have remained free of infection, while 4 had asymptomatic bacteriuria. Mucus production is reduced relative to other intestinal segments and 10 patients require no bladder irrigations. Postoperative urodynamic evaluation is similar to that of ileocystoplasty or colocystoplasty. Use of stomach has protected these patients from the development of new or worsened hyperchloremic acidosis. Serum chloride values have decreased and serum total carbon dioxide values have increased after bladder reconstruction, particularly in patients with impaired renal function. Stomach should be considered when lower urinary tract reconstruction is necessary in such compromised patients.

Rabbit urethral regeneration using small intestinal submucosa onlay grafts

OBJECTIVES To determine if small intestinal submucosa (SIS) can evoke urethral regeneration. METHODS Twenty male white New Zealand rabbits were assigned to one of three experimental groups. Group 1 (n = 4) underwent simple urethrotomy and closure. Group 2 (n = 8), a second control group, underwent an onlay urethroplasty with a graft of full-thickness preputial skin from the host rabbit. Group 3 (n = 8) underwent an onlay urethroplasty with an SIS graft. RESULTS All eight SIS onlay grafts promoted regeneration of the normal rabbit epithelium supported by a well-vascularized collagen and smooth muscle backing. Preputial free onlay grafts maintained a keratinizing squamous cell epithelium with a poor supportive backing, which resulted in the formation of urethral diverticulum. CONCLUSIONS SIS onlay patch grafts for urethroplasty promote rabbit urethral regeneration.

Characterization of Small Intestinal Submucosa Regenerated Canine Detrusor: Assessment of Reinnervation, in Vitro Compliance and Contractility

PURPOSE We characterized small intestinal submucosa regenerated canine bladder. MATERIALS AND METHODS We subjected 15-month small intestinal submucosa regenerated canine bladder strips to in vitro muscle bath compliance, contractility testing and immunohistochemical staining. RESULTS Compliance studies demonstrated no significant difference between small intestinal submucosa regenerated and control bladders, which were 30-fold more compliant than native small intestinal submucosal graft material. Contractility studies demonstrated contractile responses and innervation similar to those of normal canine bladder. Afferent nerves were demonstrated through immunohistochemical techniques. CONCLUSIONS These characteristics further support the regenerative capacity of small intestinal submucosa and its potential use as a bladder augmentation material.

Summary of Consensus Statement on Intersex Disorders and Their Management

Advances in understanding of genetic control of sexual determination and differentiation, improvements in diagnostic testing and surgical genital repair, and the persistent controversies inherent to clinical management were all compelling factors that led to the organization of an international consensus conference. The goals were to acknowledge and discuss the more controversial issues in intersex management, provide management guidelines for intersex patients, and identify and prioritize questions that need additional investigation. This is a summary statement. Advances in molecular genetic causes of abnormal sexual development and heightened awareness of the ethical and patient-advocacy issues mandate reexamination of existing nomenclature for patients with intersex.1 Terminology such as “pseudohermaphroditism” is controversial, potentially pejorative to patients,2 and inherently confusing. Therefore, the term “disorders of sex development” (DSD) is proposed to indicate congenital conditions with atypical development of chromosomal, gonadal, or anatomic sex. Additional rationale for new classification is the need for modern categorization to integrate the modern molecular genetic aspects, to maximize precision when applying definitions and diagnostic labels,3 and to meet the need for psychologically sensitive yet descriptive medical terminology. Nomenclature should be flexible enough to incorporate new information, robust enough to maintain a consistent framework, use descriptive terms, reflect genetic etiology, accommodate phenotypic variation spectrum, and be useful for clinicians, scientists, patients, and families. Hence, we propose a new classification (see “Consensus Statement on Management of Intersex Disorders”4 in this months issue of Pediatrics Electronic Edition ). Three traditionally conceptualized domains of psychosexual development are gender identity (ones self-representation [ie, male or female]), gender role (sexually dimorphic behaviors within the general population, such as toy preferences, aggression, and spatial ability), and sexual orientation (direction[s] of erotic interest). Gender dissatisfaction denotes unhappiness with assigned sex and may result in gender self-reassignment. Psychosexual developmental factors relate to parental psychopathology, parent-child … Address correspondence to Peter A. Lee, MD, PhD, Department of Pediatrics, MC-H085, Penn State College of Medicine, Milton S. Hershey Medical Center, Box 850, 500 University Dr, Hershey, PA 17033-0850. E-mail: plee{at}psu.edu

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.

Feminizing genitoplasty: state of the art

Abstract Surgical management of intersex states continues to evolve such that todays efforts achieve near normal cosmetic and functional results. In this article we discuss the reconstruction of the female genitalia in those that have had significant androgen stimulation as well as those with near normal external genitalia but significant urogenital sinus anomalies. The initial evaluation as well as postoperative follow-up management is addressed.