High-flow priapism associated with Fabry's disease in a child: a case report and review of the literature.

We report a case of priapism in an 11-year-old boy known to have Fabry's disease. High flow was confirmed by color flow Doppler ultrasound and intracorporal blood gas measurements. Successful treatment was achieved by unilateral percutaneous Gelfoam embolization of the left internal pudendal artery. Previously reported cases of priapism associated with Fabry's disease were identified through a MEDLINE search of the English literature and review of the publications. Five reported cases of priapism associated with Fabry's disease were found, including those of 3 children. A report of a child with high-flow priapism that did not respond to conventional treatment including cavernovenous shunting was noted. Priapsim associated with Fabry's disease may be caused by unregulated high arterial inflow. Early recognition of the underlying pathophysiology may identify those cases that would be amenable to percutaneous embolization therapy and may obviate the need to pursue other ineffective conventional treatments.

A minipig model for urodynamic evaluation of infravesical obstruction and its possible reversibility.

PURPOSE: The aim of our study was to create reproducible partial bladder outlet obstruction and to evaluate the resulting urodynamic changes. MATERIALS AND METHODS: Thirty-one female Yucatan microswine were randomly divided into obstructed and control groups. In the obstructed group of 21 animals, an artificial 4.5-cm. sphincter cuff was placed around the bladder neck. Fourteen of these minipigs were eventually deobstructed. In the controls (unobstructed sham-operated group), the sphincter cuff was removed prior to closure. Urodynamic measurements were conducted at 2, 4, 8 and 12 weeks postobstruction in conscious animals restrained in specially designed adjustable cages. RESULTS: (with all pressures in cm.H2O): 1) Unobstructed sham controls: Pdet,open 28.8 Pdet (Qmax) 22.5, Qmax 30.8 ml. per second, Pdet,isv 61.7, urethral resistance relation (URA) 7.7 cm.H2O, and Watts factor (WF) 4.7 W/m.2; 2) Obstructed group: Pdet,open 85.2; Pdet (Qmax) 94.4, Qmax 5.6 ml. per second, Pdet,isv 135.2, mean URA 74.3 cm.H2O and WF 13.5 W/m.2; 3) Deobstructed group: Pdet,open 39.9, Pdet (Qmax) 25.9, Qmax 25.0 ml. per second, Pdet,isv 58.0, mean URA 8.9 cm.H2O and WF 5.4 W/m.2. CONCLUSION: This animal model of reversible infravesical obstruction has given consistent and reproducible pressure flow data in the conscious, upright state.

Gastric muscularis as a urinary sphincter.

OBJECTIVE: To test the ability of gastric muscularis to act as a urinary sphincter in a rat model system. METHODS: Fourteen Long-Evans rats had an ileal conduit constructed joining the bladder to the ventral skin, creating urinary incontinence. A segment of gastric muscle maintained on its vascular pedicle was encircled around the conduit. The first 7 animals (group A) had both conduit and sphincter constructed simultaneously, whereas the remaining 7 animals had the conduit constructed first, followed by a two-week period of observation, after which gastric sphincters were added (group B). All animals were observed for urine leakage via the cutaneous stoma. At two weeks after sphincter placement, all animals underwent surgical exploration, pressure profilometry of the conduit, and histologic examination of the sphincter zone. RESULTS: Pressure profilometry demonstrated elevated pressures in the sphincter zone relative to baseline pressures in the conduit of all animals (group A: mean baseline, 4.8 cm water, mean maximum, 20.6 cm water; group B: mean baseline, 5.2 cm water, mean maximum, 18.1 cm water). At exploration, no intra-abdominal complications were noted in 13 of 14 animals. All conduits were easily catheterizeable and were found to be continent after they had been filled to capacity. The ileum and gastric segments were histologically found to be viable with no evidence of necrosis or ischemia. CONCLUSIONS: These preliminary results suggest that gastric muscularis may be a potential substitute urinary sphincter in the management of sphincteric urinary incontinence.

Conscious minipig model for evaluating the lower urinary tract.

An animal model using minipigs to study urodynamic pressure flow relationships in the conscious animal is described. Port-catheters were implanted subcutaneously in the backs of 22 minipigs with access to the bladder (to measure intravesical pressure Pves) and to the peritoneal cavity (to measure abdominal pressure Pabd). The subtracted pressure (Pdet = Pves = Pabd) was recorded during the storage phase following Furosemide diuresis. Simultaneously, measurement of urinary flow (Q) during voiding was also recorded. Urodynamic studies were performed satisfactorily in all animals, and normal urodynamic profiles in minipigs were established. The minipig model allows reliable and reproducible measurement of pressure-flow parameters in a conscious state and in the natural posture.

Leak point of incontinence: a measure of the interaction between outlet resistance and bladder capacity.

A total of 48 consecutive spina bifida patients undergoing routine urodynamic studies was classified into 2 groups depending upon whether they demonstrated uninhibited bladder contractions. Of the patients 34 demonstrated detrusor hyperreflexia and 14 had detrusor areflexia. These 2 groups were then subdivided depending on the continence status. Of the areflexic and hyperreflexic groups, 6 and 7 patients, respectively, were wet despite at least 2 years of conservative medical management. Leak point pressure and leak point volume were determined in all patients and results were compared in both subgroups. In the hyperreflexic groups leak point volume but not leak point pressure was significantly different between the wet and dry patients. The areflexic group demonstrated the opposite finding, that is the leak point pressure but not the leak point volume was significantly different between wet and dry patients. From these studies we determined that in the areflexic group leak point pressure was useful to predict incontinence, whereas in the hyperreflexic group leak point volume was more useful. The addition of the measurement of leak point pressure and leak point volume during routine urodynamic studies in myelodysplastic patients enhances accurate diagnosis and may select those who will best benefit from bladder augmentation and/or a procedure to increase outlet resistance.

Congenital urethral fistula with normal anus: a report of two cases.

Congenital rectourethral or anourethral fistulae without imperforate anus in males are rare, representing less than 1% of anorectal malformations. We report our experience with two males with "N type" urethral fistulae. One, a 5-year-old boy, presented with recurrent urinary tract infections (UTIs) and passage of urine per anus. Investigations included a voiding cystourethrogram (VCUG), which demonstrated a fistula from the urethra to the anus. On physical examination, a small perianal opening was noted just outside the anus, which drained a small amount of urine after voiding. The fistula was excised via a perineal approach. The second patient is a 5-year-old boy with a long history of recurrent UTI requiring multiple hospitalizations since the newborn period. Chronic renal failure developed as a complication of repeated urinary tract infections. Investigations showed a single hydronephrotic pelvic kidney and a small bladder. He underwent numerous diagnostic and reconstructive procedures including cystoscopy and augmentation cystoplasty. Recurrent infections continued and an N type anourethral fistula was eventually diagnosed. The fistula was located between the anal canal and the membranous urethra. An anterior perineal approach was also used. Both fistulae were easily located, and reconstructive surgery of the urethra was not required. Postoperative VCUGs in both patients were normal. They have been free of infection with normal urinary continence since resection of the fistula. Congenital N type anourethral fistulae are rare, but should be considered in cases of recurrent urinary tract infections. The diagnosis may be missed by endoscopic procedures, but VCUG should demonstrate the fistulous tract.

Experience with the AS-800 artificial urinary sphincter in myelodysplastic children.

The authors describe their experience with an artificial urinary sphincter (model AS-800; American Medical Systems, Minnetonka, Minn.) in treating urinary incontinence in children. Twenty-eight sphincters were implanted in 27 boys between May 1986 and June 1989. All the boys had neurogenic bladders secondary to myelomeningocele or sacral agenesis. The mean age at the time of initial implantation was 14 years (range from 9 to 19 years), and the median follow-up was 12 months (range from 6 to 31 months). The sphincters were implanted initially around the bladder neck in 25 cases. Three required reimplantation around the bulbous urethra. The complication rate was 39% (11 of 28 cases). There were two cases of erosion, two cases of infection without erosion and seven cases of device-related problems. The artificial sphincter had to be removed in four cases. There were no deaths. The revision rate was 25% (7 of 28 cases). Continence was evaluated in 22 (88%) of 25 boys who had functional sphincters in place. Five boys required oxybutinin chloride to maintain continence. Ten boys required augmentation cystoplasty before (3), after (6) and combined with (1) sphincter implantation. Eight of these 10 children were able to void spontaneously and were continent. One required intermittent catheterization twice a day and another six times a day. The authors conclude from their experience that the artificial urinary sphincter (model AS-800) can establish continence in boys with neurogenic bladders. Proper selection of the ideal patient for the artificial sphincter is essential to avoid complications.

Management of an impalpable testis: the role of laparoscopy.

The use of laparoscopy through a small infraumbilical incision permits noninvasive exploration of the pelvic fossae. The vas deferens as well as the spermatic vessels can be identified either with the testicle or as a blind ending. Twenty-six laparoscopies were performed on 19 patients with unilateral impalpable testis and 7 with bilateral impalpable testis. In this small series, laparoscopy was found to be the best method of locating the impalpable testis, and, depending on the laparoscopic findings, no further surgery or a simple inguinal exploration or abdominal incision is needed for the management of this condition.

Use of a urethral splent in one-stage hypospadias repair.

Over a 9-month period, 28 patients with distal penile or more proximal hypospadias underwent one-stage surgical repair. Bladder drainage was achieved in the traditional fashion with either an indwelling Foley catheter or suprapubic catheter or by using a modified urethral silicone stent ("splent"). Twenty-two patients had repair with a perimeatal skin flap, and the remaining six patients had major urethral reconstruction with a vascularized preputial island flap. Use of the urethral splent was associated with shorter postoperative hospitalization and minimal short-term complications. The authors' experience has shown that use of a urethral splent for urinary drainage is efficient and effective in postoperative management after hypospadias repair.

The surgical management of urinary incontinence in myelodysplastic children.

Over a 4-year period, 69 patients with intractable urinary incontinence secondary to myelodysplasia have undergone surgical therapy to try to achieve continence. Preoperative evaluation used uroradiological and urodynamic studies, including measurement of leak point pressure and leak point volume. Twenty-one patients had a procedure to increase outlet resistance, 30 patients had bladder augmentation, and 18 patients had both procedures performed. Fifty-seven of the 69 patients have achieved total continence for a success rate of 83%.

Augmentation cystoplasty in rats: development of an animal model.

An animal model of the Long-Evans species of rats was developed to study the short-term and long-term effects of enterocystoplasty. Various enterocystoplasties were performed in 39 rats, including ileal in 29, colonic in 5 and gastric in 5. The followup period was 3 months. Frequency and pattern of voiding, 24-hour urinary collection for mucus production, and blood and urinary electrolytes were analyzed. All voiding parameters, renal function and biochemical studies remained normal. Mucus production was higher in the gastrocystoplasty and colocystoplasty than in ileocystoplasty cases. Postmortem histopathological examination of the enteropatch was performed, which showed urothelialization of the graft with native transitional epithelium extending over the junctional margin of the graft and covering the enteropatch mucosa. The enteropatch muscle orientation was maintained in all 3 types of grafts. We believe that the Long-Evans species of rats is a good model for the study of enterocystoplasty.

The evolution of artificial sphincter cuff in growing animals.

An animal model was developed to simulate the effect of implantation of artificial sphincter cuff on the urethra in children. The study was conducted on 28 pigs, 15 castrated males, four uncastrated males and nine females, divided into four groups: control unoperated, and three operated groups. Group I contained young piglets (castrated males, uncastrated males and females), group II contained adult animals and group III contained sham operated animals. An AS 800 belt occlusion cuff was implanted in the deflated state distal to the bladder neck around the urethra in the young and adult groups, while no sphincter was implanted in the sham group. Neither primary nor secondary activation was done. The pigs were followed for a period of six to eight weeks and then sacrificed. The morphological and histological observations on the effects of the artificial sphincter cuff on the underlying urethral tissue showed significant transmural atrophy of the urethral and prostatic segment underlying the cuff in the young growing castrated and uncastrated male group with mild changes in the young female group. Bladder rupture occurred in three uncastrated males. There were no changes encountered in the adult or sham operated groups. Variable degrees of upper tract changes and renal deterioration were seen in the young group. These changes occurred mainly in the male piglets while a lesser degree occurred in the female piglets. No changes were demonstrated in the adult and sham groups. This study suggests the possibility of similar changes occurring in young male children who have undergone artificial sphincter implantation.

Acute renal failure in an infant with congenital neuroblastoma - successful treatment by hemodialysis.

Acute renal failure developed in an infant aged 30 days during chemotherapy and irradiation for hepatic neuroblastoma (IV-S). Acute hemodialysis via a profunda femoris - long saphenous vein shunt was accomplished on 9 occasions in this 4 kg infant. The complications of septicemia and acute respiratory embarrassment caused by hepatomegaly were treated by appropriate antibiotic treatment, creation of a temporary ventral hernia and assisted ventilation. Careful attention to correct pediatric hemodialysis technique, as described, can permit long-term survival if the underlying disorder is also amenable to treatment.

Hormones in the treatment of metastatic renal cancer.

Metastatic renal adenocarcinoma has been highly resistant to most therapeutic approaches. However, hormonal treatment has been reported to induce significant subjective or objective improvement. Two groups of patients receiving either androgens or progestogens were evaluated. The number of responses was disappointing. Furthermore, neither of the 2 regimens appeared to increase the mean surival between detection of metastases and fatal outcome.

Response of renal adenocarcinoma to cyclophosphamide.

A group of patients with metastatic renal adenocarcinoma was treated with cyclophosphamide. No antitumor effect was noted and, probably, enhancement of neoplastic growth was produced in some cases. Compared with other available therapies, a shorter survival rate was noted.