Joel M. Sumfest

The Mitrofanoff Principle in Urinary Reconstruction

We report the use of the Mitrofanoff principle to achieve urinary continence in 47 patients. A catheterizable channel can be used as the primary continence mechanism, or as an adjunct to ensure complete bladder emptying if urethral catheterization or voiding is inadequate. Appendico-vesicostomy was performed in 25 of 47 patients (55%). Satisfactory continence was achieved in 45 patients (96%). Bladder emptying by clean intermittent catheterization was performed in all patients. Early complications included peristomal abscess (1 patient) and small bowel obstruction (1). Late complications included difficulty with catheterization (5 patients), stomal stenosis (9) and persistent incontinence (2). Our results support use of the appendix as the catheterizable segment of choice. We describe the technique and discuss the management of complications.

Reconstruction of the umbilicus in exstrophy

Reconstruction of the umbilicus has cosmetic and functional value in the surgical treatment of the child with bladder exstrophy. We report on 12 patients who underwent creation of a neoumbilicus as part of genitourinary reconstruction.

Aggressive surgical and medical management of autosomal recessive polycystic kidney disease

The treatment of autosomal recessive polycystic kidney disease (ARPKD) is evolving. Overall prognosis is difficult to determine, but most neonates without severe pulmonary hypoplasia will survive. It is difficult to predict which neonates with ARPKD requiring immediate ventilatory support have pulmonary hypoplasia incompatible with survival. Massively enlarged kidneys restricting diaphragmatic excursion can cause respiratory distress in addition to pulmonary hypoplasia. Three infants with ARPKD underwent bilateral nephrectomies and institution of peritoneal dialysis in an attempt to improve ventilatory status and assess long-term pulmonary function. Two children are alive at twelve and thirty months postoperatively and awaiting renal transplantation. One infant died of acute cardiorespiratory complications.

Gastrocystoplasty in children

The use of stomach for bladder reconstruction is an important tool for the urologic surgeon. Just as one would not perform hypospadias repair knowing only one technique, one should not consider bladder reconstruction with only one option for augmentation available. The advantages, disadvantages and a brief overview of our operative technique are presented. We have been very pleased with our experience with the use of stomach in the appropriate clinical situation.

The Use of the AMS800 Artificial Urinary Sphincter in Combination with the Gastric Tube for Continence in the Canine Model

An experimental canine model was designed to examine the potential use of the artificial urinary sphincter around a gastric tube. The artificial urinary sphincter was placed around a tubularized gastric flap as part of a continent gastric reservoir in 4 dogs and in 2 additional dogs the gastric tube was anastomosed to the native bladder. Two dogs underwent placement of the artificial urinary sphincter around the gastric tube 4 weeks postoperatively and the remainder had the sphincter placed simultaneously with creation of the gastric tube. All dogs with the gastric reservoir underwent urodynamics before and after activation of the sphincter. Only 61 to 70 cm. water pressure balloons were used. All dogs were continent postoperatively on clean intermittent catheterization every 8 hours. There were no erosions or problems with catheterization. Urodynamics confirmed a complaint system and an average increase of capacity of 410% after artificial urinary sphincter activation (4 dogs). There was no leakage at capacity. Histology of the artificial urinary sphincter and neighboring (control) regions, and of the reservoir at 1 (2 dogs), 3 (3 dogs) and 6 months (1 dog) was obtained. Microscopic examination of the cuff site showed mild serosal hyperplasia and fibrosis, a well preserved muscularis and mild to moderate focal mucosal atrophy. These changes were slightly more evident at 6 months. Mucosal folds were well preserved with normal submucosa and lamina propria. In the control region histology was well preserved and similar to native stomach. We conclude that the artificial urinary sphincter around a gastric tube can provide urinary continence. The minimal changes in histology under the cuff are encouraging and support the potential for use of the gastric tube with the artificial urinary sphincter, although longer term effects are unknown.

Renal Duplication Anomalies

Renal duplication anomalies are common in the pediatric population, but are frequently clinically silent. They will come to the attention of a medical provider if they develop symptoms of infection or obstruction.

Ureteropelvic Junction Obstruction

Pediatric ureteropelvic junction (UPJ) obstruction can occur in all age groups; increased detection in neonates due to prenatal hydronephrosis can be seen on ultrasonography. It is more common in boys than girls and more common on the left side.

Disorders of Sexual Differentiation

Disorders of sexual differentiation (DSD) are a variety of complex malformations which require a multidisciplinary approach to guide the best therapy for each individual patient.

Partial resection of symphysis pubis

i Resection of the symphysis pubis has been recas an aid to exposure for the repair disruption from pelvic trauma and f the membranous urethra. 1 Resec~mplete segment of the symphysis is ime consuming and labor intensive accompanied by troublesome bleedion of the posterior lip of the pubis ~ves an equal degree of exposure and avoids these problems. 2-~ We have used this technique ~for the past two years and believe it deserves Wider application including use in managing benign and malignant conditions of the prostate, strictures of the sphincter-active urethra not amenable to a perineal approach, and re~0nstructive procedures of the bladder neck.