Hjalmar W. Johnson

Gonadal tumors in disorders of sexual differentiation

OBJECTIVE To study the prevalence and distribution of gonadal tumors in patients with disorders of sexual differentiation. METHODS Retrospective review of pathologic materials and clinical data on all patients diagnosed with mixed gonadal dysgenesis, pure gonadal dysgenesis, androgen insensitivity, and true hermaphroditism between 1982 and 1990. RESULTS Twenty-one patients were identified and all underwent bilateral gonadectomy at the time of diagnosis. Nine of 21 patients had a gonadal tumor for a prevalence of 44 percent. Those at greatest risk for tumor were patients with mixed gonadal dysgenesis (6 of 11 patients) and pure gonadal dysgenesis (2 of 3 patients). There were four gonadoblastomas, two dysgerminomas, and one each of teratocarcinoma, seminoma, cystadenofibroma, and juvenile granulosa cell tumor. CONCLUSIONS The high prevalence of gonadal tumors in children with mixed and pure gonadal dysgenesis warrants consideration of early, bilateral, prophylactic gonadectomy once the diagnosis is established with certainty.

Bladder autoaugmentation byvesicomyotomy in the pediatric neurogenic bladder

OBJECTIVES The authors describe the procedure of bladder autoaugmentation by vesicomyotomy in 12 pediatric patients with neurogenic bladders. METHODS Indications for augmentation included low-capacity, high-pressure bladders with incontinence despite maximal anticholinergic therapy. Clean intermittent catheterization was successfully reinstituted postoperatively and no patient has subsequently required enterocystoplasty. RESULTS There were no major complications and eight patients underwent concurrent procedures on the bladder. Urodynamic studies revealed a mean increase in capacity of 40% and a mean decrease in leak point pressure of 33% compared with preoperative values. CONCLUSIONS Early clinical experience would suggest that vesicomyectomy (excision of released detrusor) offers no advantages over vesicomyotomy in pediatric patients. Vesicomyotomy (simple incision into detrusor) proved to be a simple technique that could be safely performed in pediatric patients.

TREATMENT OF URINARY INCONTINENCE IN CHILDREN BY ENDOSCOPICALLY DIRECTED BLADDER NECK INJECTION OF COLLAGEN

PURPOSE We assessed optimal selection criteria for and expected outcome of bladder neck injection of collagen in children with intrinsic sphincteric deficiency. MATERIALS AND METHODS We retrospectively reviewed the charts of children with intrinsic sphincteric deficiency treated with bladder neck injection of collagen at 3 pediatric urology centers. RESULTS We reviewed the records of 12 boys and 6 girls 6 to 18 years old (median age 10.5). The underlying etiology of incontinence was neurogenic bladder in 10 patients, the epispadias-exstrophy complex in 6, ureterocele in 1 and trauma in 1. Previous surgery included bladder augmentation in 4 cases, bladder neck plasty in 7 and epispadias repair in 4, and concurrent medical management comprised anticholinergics in 8, alpha-agonists in 1 and intermittent catheterization in 9. Skin tests in all patients were negative for collagen. Seven patients had 1 injection, 9 had 2 and 2 had 3. The volume of collagen injected ranged from 2.4 to 13 cc (median 5) per treatment and 3 to 28 cc (median 7) per patient. Followup ranged from 5 to 21 months (median 15). Of the 14 patients evaluated 5 are dry, 4 are improved, 1 is unchanged and 4 underwent further continence surgery. No treatment related morbidity was seen. CONCLUSIONS Endoscopically directed injection of collagen around the bladder neck is successful in 64% of children with intrinsic sphincteric deficiency. It is an outpatient treatment with no significant morbidity. Further continence surgery is not precluded when necessary. Patients with adequate capacity and compliant neurogenic bladders, and those with the exstrophy-epispadias complex and failed bladder neck plasty seem to be optimal candidates for collagen injection.

Microdissection and radiographic studies of the arterial vasculature of the human testes

Conflicting and sparsely detailed reports concerning the arterial vasculature of the testes prompted us to study 51 human cadaveric testicles radiographically in order to document any interarterial communications. Twelve specimens were harvested from the pediatric age group and 39 from adult cadavers. Vascular communication was demonstrated between the testicular, cremasteric, and vasal arteries in all specimens studied. Large-caliber anastomotic channels were demonstrated between the testicular and vasal arteries in 87% of specimens. No discernible differences in these vascular communications were documented among adult, pediatric, and undescended testes.

Wilms Tumorlet, Nodular Renal Blastema and Multicystic Renal Dysplasia

We reviewed 60 cases of multicystic renal dysplasia collected during a 10-year period. Differentiated nonproliferative nodular renal blastema occurred in 6.7 per cent of the cases, which is similar to the incidence of nodular renal blastema in kidneys of other infants with congenital obstructive uropathy. A unique case of cystic dysplasia containing nodular renal blastema and Wilms tumorlet indicates the possibility that a persistently proliferative nephroblastomatous lesion may rarely occur.

Endoscopic Treatment of Vesicoureteral Reflux with Collagen: Preliminary Report and Cost Analysis

PURPOSE We determined therapeutic and operative cost efficacy of endoscopic collagen injection for vesicoureteral reflux. MATERIALS AND METHODS We performed a multicenter study of 10 male and 23 female sequential patients (45 renal units) with vesicoureteral reflux and a surgical indication. Patients were skin tested with collagen and when negative, they underwent endoscopic injection. Renal ultrasound/cystography was done 3 months after treatment. Cost analysis was performed at 1 institution. RESULTS Reflux was grade I in 2 renal units, II in 18, III in 15 and IV in 10. The majority of cases were single systems with primary reflux. We performed 1 treatment in 32 renal units, 2 in 6, 3 in 6 and 4 in 1. Cure was evident in 31 of 38 renal units (81.6%) and 21 of 27 patients (78%) 3 months after the last injection. Patient morbidity was minimal. Operative cost per renal unit was

Experiences with Priapism

1,599.68 for collagen injection and

Cutaneous vesicostomy in the young child: Indications and results

9,144.47 for reimplantation. CONCLUSIONS Endoscopic injection of collagen is effective treatment for vesicoureteral reflux. Furthermore, it causes minimal morbidity and may effect cost savings in health care management.

Laboratory variables of bladderautoaugmentation in an animal model

During a 10-year period 11 cases of priapism were treated with a saphenocavernous bypass. Uniformly good functional results were obtained if the procedure was done within 36 hours of the commencement of priapism. Prior aspiration and irrigation appear to lengthen this interval. The formation of temporary cavernovenous communication with the superficial venous system of the penis by aspiration and irrigation with large needles is demonstrated and is postulated as a mechanism by which this method of treatment alone is occasionally successful. Persisting graft patency has been associated with impotence. If erection does not occur within 3 months of the saphenous bypass the shunt should be ligated.

Congenital Obstructive Uropathy and Nodular Renal Blastema

With newer methods of managing lower urinary tract pathology in the young child, the role of cutaneous vesicostomy may be changing. This prompted a review of 50 consecutive patients treated with initial vesicostomy at our center over ten-year period. These children underwent vesicostomy diversion at a median age of 5.8 months and, of the 34 vesicostomies which have been subsequently closed, for a median duration of twenty-five months. Our indications agree with series reported previously and include patients with meningomyelocele, posterior urethral valves, or other forms of congenital or acquired lower urinary tract anomaly or dysfunction, along with complicating factors such as vesicoureteral reflux, recurrent infections, and/or renal deterioration. However, we also have identified a major group--those with primary gross vesicoureteral reflux--not previously included in detail. Follow-up averaged thirty-eight months. Improvement or stabilization of upper urinary tracts was achieved in over 90 percent of cases, and this trend continued after vesicostomy closure. As well, cutaneous vesicostomy allowed ureteral dilation to normalize, decreasing the degree of reflux and need for subsequent ureteral tapering and reimplantation at closure. Finally, though our stomal revision rate of 20 percent is high, modified techniques are being pursued.