Simplified coagulum pyelolithotomy using cryoprecipitate.

Coagulum pyelolithotomy has been beneficial in removing calyceal calculi when the diameter of the infundibulum and pelvis is adequate. By utilizing a simple, precise ratio of cryoprecipitate, thrombin, and calcium chloride (25:4:1), an effective coagulum was obtained in 7 consecutive cases with complete stone removal in 5.

Management of unresectable Wilms tumor.

Wilms tumor has become a model of highly curable cancer. With the addition and refinement of chemotherapy protocols, survival rates have risen from 20 per cent three decades ago to 80 per cent or greater in the past decade. Surgery remains an integral part of the management of Wilms tumor both as a diagnostic tool and for removal of tumor bulk. Few primary tumors cannot be surgically removed during the initial phase of therapy. However, bilateral Wilms tumor and large unilateral tumors may occasionally be initially unresectable without prohibitive loss of renal or nonrenal organ function. The National Wilms Tumor Study group has no protocol for management of these tumors but does offer guidelines unsupported by large numbers of patients. Two cases are presented in which the tumors were initially deemed surgically unresectable. Initial management with chemotherapy (both cases) and radiotherapy (1 case) followed by surgical removal of tumor left both patients free of gross tumor, with relapse-free survivals of two and one-half years and six years thus far. Based on our experience and that found in the literature, we believe that preoperative chemotherapy is indicated for children suspected of having Wilms tumors judged surgically unresectable.

Reversal of acute polyhydramnios after fetal renal decompression.

Polyhydramnios and premature labor at twenty-seven weeks secondary to an intrafetal renal cyst are described. Antenatal fetal cyst decompression successfully reversed the polyhydramnios and prevented premature delivery, resulting in a full-term healthy infant delivered vaginally at thirty-nine weeks. Urologic investigation postpartum revealed a probable congenital ureteropelvic junction obstruction.

Spontaneous vesicoileal fistula two years post ureteroileoneocystostomy undiversion.

The complication of spontaneous vesicoileal fistula formation in an eleven-year-old male two years after ureteroileoneocystostomy undiversion is presented.

Primary extrarenal Wilms' tumor in children.

We report three additional cases of primary extrarenal Wilms' tumor and review those cases previously documented. Analysis of the location, histopathology, treatment, and survival of these cases supports the following conclusions: Wilms' tumor may occur in an extrarenal location without primary renal involvement and must be included in the differential diagnosis of abdominal, pelvic, and inguinal masses; an extrarenal location supports a more frequent occurrence of ectopic metanephric blastema than was previously recognized or origin of Wilms' tumor from a more primitive mesodermal tissue; and the natural history and prognosis of extrarenal and renal Wilms' tumors appears similar.

Current urologic management of cloacal exstrophy: experience with 11 patients.

PURPOSE: Since 1980 the authors have treated 12 infants with cloacal exstrophy (10 classical and 2 variants). Eleven patients had repair, and are all surviving. The initial phases of management that led to improved survival have previously been reported. Quality of life is now a major focus for the cloacal exstrophy patient. During the past 10 years, nine of the 11 patients had lower urinary tract reconstructive procedures. This review evaluates experience with reconstructive efforts to achieve bowel and bladder control and to improve the quality of life in this complex group of patients. METHODS: Through review of patient charts and by patient interviews, data were collected to evaluate the ability to provide urinary and bowel control. A continence score was applied to provide a measure of success: voluntary control, 3; control with an enema program or intermittent catheterization, 2; incontinence with a well-functioning stoma, 1; and incontinence without a stoma, 0. The best continence score is 6 (genitourinary and gastrointestinal). Surgical complications, urodynamic and metabolic sequelae of continent urinary diversion were reviewed. RESULTS: At the time of the authors' previous report, eight of 11 patients had a continence score of 2 or less. Currently, eight of 11 patients have a score of 3 or better (five with enteric stoma and continent urinary diversion, two with enema program and continent urinary diversion, and one with enema program and continent bladder). Urinary-diversion procedures have included two gastric augmentations and five gastric reservoirs, two of which have required subsequent bowel augmentation. Gastric augmentations carry a definite risk of metabolic problems with three of our patients demonstrating significant episodes of metabolic alkalosis. In addition, results of urodynamic monitoring suggests that gastric reservoirs may be less compliant than reservoirs formed using other bowel segments. CONCLUSIONS: Modern principles of continent urinary diversion have been successfully applied to the cloacal exstrophy patient further improving their quality of life. Use of gastric flaps with preservation of intestinal length has been central to urologic reconstructive efforts. Use of stomach alone for formation of urinary reservoirs may produce suboptimal compliance, and composite ileogastric construction should be considered if the gastric flap is of marginal size.

Modern treatment of cloacal exstrophy.

We have treated 12 newborns with cloacal exstrophy (10 classical and 2 variants) since 1980. Eleven infants underwent repair and they are all surviving; the one nonoperated infant died of pulmonary hypoplasia. There are six genetic females and six genetic males, five of whom are being raised as females. The initial operation consisted of separating the bowel from the bladder to create an intestinal stoma; closing the omphalocele; and reapproximating (5), closing (4), or leaving the exstrophied bladder undisturbed (2). The importance of creating a "tailgutostomy" instead of an ileostomy to prevent problems with diarrhea, dehydration, and acidosis is emphasized. There have been 71 subsequent operations in these 11 patients (28 general surgical, 25 urological, 9 neurosurgical, and 9 orthopedic). Quality of life, rather than survival, is now the major issue facing patients with cloacal exstrophy. All of these patients except one are neurologically normal. The defect is closed in all patients. Three patients wear no appliances, 7 wear one (gastrointestinal [GI]), and one wears two (GI and genitourinary [GU]). We developed a scoring system to analyze bowel and bladder continence: voluntary control = 3; control with an enema program or intermittent catheterization = 2; incontinence with a well-functioning stoma = 1; and incontinence without a stoma = 0. The best continence score is 6 (GU + GI). We currently have 7 patients with a continence score of 1 (colostomy + incontinent bladder); 1 with a score of 2 (ileostomy + incontinent ureteroenterostomy); 2 with a score of 4 (enema program + continent urinary diversion); and 1 with a score of 5 (enema program + continent bladder).(ABSTRACT TRUNCATED AT 250 WORDS)

Familial torsion of the spermatic cord.

Familial spermatic cord torsion is rare. We present the fifth recorded family with this condition. Due to awareness of the condition and its consequences on the part of the family there was minimal delay in presentation and early testicular salvage occurred in all 4 patients.

Low spinal anesthesia for the prevention of autonomic dysreflexia in the spinal cord injury patient.

Twenty-five patients with traumatic spinal cord injuries above T7 have been managed with low spinal anesthesia during endoscopic urologic procedures. The technique has been completely successful in preventing autonomic dysreflexia and has not been associated with hypotension.

Cancer of the bladder in spinal cord injury patients.

Since 1963, 10 cases of bladder carcinoma have been detected in 1,052 new admissions to our center. A high percentage of these patients had squamous cell carcinoma and/or squamous elements. This relatively high incidence stimulated a prospective study of 81 spinal cord injury patients either maintained on intraurethral catheter drainage for 10 years or an external appliance for 15 years. There were changes of squamous metaplasia in 19 per cent of the cases but no cancer was detected. It remains undetermined if squamous metaplasia is a pre-malignant lesion. However, the incidence of squamous metaplasia and squamous cell carcinoma in paraplegics with chronically infected bladders is not uncommon. Any spinal cord injury patient with hematuria needs a complete bladder evaluation and any long-term paraplegic with chronic infection should undergo cystoscopy and Papanicolaou smears as part of the yearly checkup.

Neonatal vaginal cysts: diagnosis and management.

We report a 2.5 cm. vaginal cystic mass in a neonate. The differential diagnosis, evaluation and management of this lesion are discussed.

Pelvic rhabdomyosarcoma in children.

Twenty children with pelvic rhabdomyosarcoma treated between 1976 and 1983 are reviewed. The survival rate was 55% (11/20), with a bladder salvage rate of 54.5% among survivors. Systemic chemotherapy combined with radiotherapy and/or surgery was most effective in producing cure.

Renal tubular acidosis type 4 in neonatal unilateral kidney diseases.

Three neonates, two with unilateral renal vein thrombosis and one with unilateral dysplastic kidney, developed type 4 renal tubular acidosis, manifested by nonazotemic hyperkalemic metabolic acidosis with alkaline urine pH and reduced potassium excretion. Normal plasma concentrations of sodium, aldosterone, and renin activity, together with normal renal fractional excretion of sodium, supported the diagnosis of renal tubular acidosis type 4, subtype 5. Arginine HCl loading studies showed that despite their ability to bring the urine pH to less than 5.8, net acid excretion was inadequate relative to the corresponding plasma bicarbonate concentration. Treatment with oral bicarbonate resulted in sustained normalization of blood acid-base status and accelerated linear growth in the first two infants, in whom spontaneous recovery occurred by ages 8 and 15 months, respectively. At that time, the affected kidneys were extremely small with distorted collecting systems; the contralateral kidneys showed compensatory hypertrophy. In the third infant, persistent acidosis and growth failure resulted from medical noncompliance; the removal of the dysplastic kidney at 7 months of age was followed by the return to normal blood acid-base status and normalized tubular hydrogen and potassium excretion. We conclude that neonatal unilateral kidney disease can result in renal tubular subtype 5. Spontaneous recovery can be expected, presumably because of " autonephrectomy " of the affected kidney plus the compensatory hypertrophy of the contralateral kidney.

A clinical experience with dantrolene sodium for external urinary sphincter hypertonicity in spinal cord injured patients.

Significant bladder residual urine is secondary to pelvic floor skeletal muscle hypertonicity in some spinal cord injury patients with suprasacral or mixed lesions. Fifteen patients with residual urine volumes greater than 150 cc were treated with dantrolene sodium because of its ability to decrease skeletal muscle contractibility. All of the patients had urethral closure pressures greater than 100 cm. water. Of the 15 patients 8 benefited from dantrolene sodium therapy and were maintained on external condom urinary drainage. Five of these 8 patients required up to 600 mg. dantrolene sodium daily to affect this result. The residual urine volume decreased to less than 100 cc and the post-therapy decrease in urethral pressure averaged 77 cm. water (49 per cent). The patients in the failure group (residual urine greater than 150 cc) had an average decrease in urethral pressure of 21 cm. water (16 per cent). Detrusor hyporeflexia possibly contributed to the failure rate. In summary, dantrolene sodium seems to be beneficial in some patients with external urinary sphincter hypertonicity. However, it will not supplant external sphincterotomy in the more complete male spinal cord injury patient in whom reflex incontinence is of minimal concern. Dantrolene sodium could be an ideal treatment of patients with incomplete neurologic lesions in whom continence might be preserved. The drug will have to be effective at low doses to obviate the major side effect of over-all muscle weakness.

The use of ultrasound for evaluating subacute unilateral scrotal swelling.

In 15 patients with subacute (longer than 8 hours) unilateral scrotal swelling in whom the etiology was in doubt scrotal ultrasound was used to determine whether the pathological condition was intratesticular and/or extratesticular. Surgical exploration confirmed intratesticular or intratesticular and extratesticular findings in 9 patients: 8 had torsion of the spermatic cord (including a testis rupture in 1 and epididymal ruptures in 2) and 1 had a mixed germ cell carcinoma. Of the 6 patients with extratesticular findings 3 had clinical epididymitis that resolved on antibiotic therapy and 2 had what appeared to be paratesticular hematomas with normal testes presumed to be secondary to minor trauma. The condition resolved with conservative therapy in the latter 2 patients. The remaining patient required surgical drainage because of the size and an epididymal rupture suspected by the ultrasound examination. Scrotal ultrasound is a quick, noninvasive, easily applied, accurate method to diagnose scrotal pathological conditions and should be used whenever the etiology of scrotal swelling is in doubt.

The use of stomach in pediatric urinary reconstruction.

We report on 30 consecutive patients (mean age 9 years) who underwent gastrocystoplasty (21) or received a continent urinary reservoir with stomach (9). A successful outcome, defined as complete urinary continence for intervals greater than 3 hours, preservation or improvement of the upper tracts and renal function, and normal electrolyte and acid base balance, was achieved in 90% of the patients who received continent urinary reservoir and 72% of those who underwent gastrocystoplasty. Urodynamic data were obtained in 17 patients in the gastrocystoplasty group and 7 in the continent urinary reservoir group at a mean of 6 months postoperatively. Metabolic alkalosis associated with hypergastrinemia occurred in 2 children. Partial excision of the gastric segment was necessary in 1 patient refractory to medical treatment.

Metabolic complications of the use of stomach for urinary reconstruction.

A total of 34 children with normal renal function underwent either gastrocystoplasty or continent urinary reservoirs with stomach at our institutions. Severe hypochloremic hypokalemic metabolic alkalosis developed in 2 patients, manifested by intractable seizure disorder in 1 and altered mental status with respiratory depression in 1. Symptoms developed at 4 and 6 months, respectively. Despite severe alkalosis, urinary pH was less than 5.0 and fractional excretion of chloride remained high in both patients. Resuscitation with sodium chloride, arginine hydrochloride and potassium chloride restored electrolyte balance in less than 48 hours in both patients. Serum gastrin was slightly elevated in 1 patient (137 pg./ml., normal 0 to 125) who responded to long-term histamine-blocker therapy. The other patient had significant hypergastrinemia (624 pg./ml.) with secondary hyperaldosteronism. Maximum doses of histamine blockers, oral replacement of sodium chloride and potassium chloride, and the proton pump inhibitor omeprazole failed to control recurrent bouts of severe hypochloremic metabolic alkalosis. This patient ultimately underwent removal of three-quarters of the gastric augmentation and replacement with ileum. Postoperatively, serum gastrin levels and electrolytes reverted to normal. The pathophysiology of this potentially lethal complication is further discussed.

Symptomatic tumors affecting the urinary tract in children with neurofibromatosis.

PURPOSE: We determined the need for urological screening in children with neurofibromatosis. MATERIALS AND METHODS: Of 96 children with neurofibromatosis treated at our institution 6 (6.2%) had a symptomatic tumor that affected the urinary tract. RESULTS: There were 5 plexiform neurofibromas and 1 prostatic rhabdomyosarcoma. Five children had a palpable abdominal mass and 3 required urinary diversion. CONCLUSIONS: Since most morbidity resulted because tumors were advanced by the time symptoms developed, we suggest that children with neurofibromatosis be screened annually with medical history, physical examination, urinalysis and serum chemistry studies for tumors that affect the urinary tract.

The normal and abnormal scrotum in children: evaluation with color Doppler sonography.

The use of color Doppler sonography to evaluate the symptomatic testes in children with scrotal pain or swelling was prospectively studied with a fourth-generation color sonographic unit with a 7-MHz linear transducer. The 32 patients were 1 day to 18 years old (mean age, 8.6 years). Results were correlated with scintigraphic findings in 23 patients, with the final diagnosis established by surgery in 12 patients, and with clinical follow-up in all patients. Eight cases of testicular torsion, including two of acute torsion and six of late torsion, were correctly detected by color Doppler sonography and confirmed surgically. In the remaining patients, perfusion of the testis was correctly detected by color Doppler examination. The final diagnoses in these patients included torsion of the appendix testis (15 patients), epididymitis (five patients), epididymo-orchitis (one patient), yolk sac tumor of the testis (one patient), hydrocele (one patient), and local reaction to an insect bite (one patient). The ability to detect blood flow in the normal contralateral testis was also evaluated in 28 patients. Blood flow was demonstrated in normal testes larger than 1 cm3. Detection of flow in the very small normal prepubertal testis was often difficult, and no flow was identified in one testis. Flow was identified in central arteries in only six of 13 testes smaller than 1 cm3. We conclude that color Doppler sonography is helpful in the initial evaluation of pediatric testes, providing accurate evaluation of the involved hemi-scrotum in our patients and also providing the benefit of both structural and flow information. Until our sensitivity to low-velocity flow improves, we would not suggest the exclusive use of color Doppler sonography in the evaluation of testicular perfusion in the prepubertal patient. We advocate the addition of testicular scintigraphy to corroborate the presence of testicular perfusion when flow in intratesticular arteries cannot be established with certainty by color Doppler sonography.

Submucosal bladder neck injection of bovine dermal collagen for stress urinary incontinence in the pediatric population.

PURPOSE: The surgical management of stress urinary incontinence in the pediatric population includes various surgical techniques of which the simplest is outpatient transurethral or periurethral injection of a bulking agent. Currently purified bovine dermal collagen cross-linked with glutaraldehyde (Contigen) is the only Food and Drug Administration approved bulking agent available. Patients with stable detrusor function are ideal candidates. We review our initial experience with children. MATERIALS AND METHODS: Between January 1994 and June 1995, 23 boys and 9 girls 4 to 17 years old (mean age 9) consecutively underwent submucosal bladder neck injection with cross-linked bovine dermal collagen. Incontinence was secondary to spinal dysraphism in 24 patients, complete epispadias in 4, classic bladder exstrophy in 3 and neurogenic bladder secondary to a sacral teratoma in 1. Preoperative video urodynamics documented intrinsic sphincteric deficiency in all patients with leak point pressure of 15 to 60 cm. water (mean 37). Injection volumes ranged from 2.5 to 17 cc (mean 10). Continence results were defined as dry-requiring no protection while on a 4-hour clean intermittent catheterization program, good-improved but requiring 1 to 5 pads daily and failure-no postoperative improvement or still requiring diapers. RESULTS: Of the children with neurogenic bladder 20% became dry following the first injection and an additional 28% had a good result. Furthermore, of the 6 children with exstrophy or epispadias who underwent a Young-Dees-Leadbetter procedure 50% were dry and 17% had a good result after the first injection. Complications were limited to a febrile urinary tract infection associated with urinary retention in 1 patient and transiently worse continence in 2. CONCLUSIONS: Despite the limited success rate, we believe that transurethral collagen injection therapy has a viable role in the treatment of intrinsic sphincteric deficiency in select pediatric patients, particularly since the procedure has low morbidity and can be performed on an outpatient basis. Preoperative counseling should be given with realistic expectations.