Antenatal hydronephrosis with postnatal resolution: how long are postnatal studies warranted?

We present 2 cases of antenatal hydronephrosis with initial normalization of postnatal studies. Both patients experienced late-onset (6 and 22 months) hydronephrosis secondary to ureteropelvic junction obstruction, necessitating surgical intervention. These cases raise questions about the need for late follow-up imaging in patients with apparent resolution of hydronephrosis diagnosed antenatally.

Acute urinary retention in children.

PURPOSE: Acute urinary retention is relatively infrequent in children. There are a variety of causes that are poorly defined in the literature, and they differ greatly from those seen most frequently in adults. We review our experience with pediatric patients presenting with urinary retention to 2 major metropolitan children's hospitals. MATERIALS AND METHODS: Records from Egleston and Scottish Rite Hospitals for Children were reviewed for a 6-year period for the diagnosis of urinary retention. Urinary retention was defined as inability to empty the bladder volitionally for greater than 12 hours with a volume of urine greater than expected for age ([age in years + 2] x 30 cc) or a palpably distended bladder. All cases resulting postoperatively and believed secondary to surgical dissection, narcotic use or immobility, and children previously diagnosed with chronic neurological disorders and voiding dysfunction or with reduced mental status were excluded from study. The majority of these encounters were emergency department visits specifically for acute urinary retention. RESULTS: We identified 53 children meeting these criteria, including 37 boys 6 months to 17 years old and 16 girls 1 to 17 years old. Etiologies included neurological processes in 17%, severe voiding dysfunction in 15%, urinary tract infection in 13%, constipation in 13%, adverse drug effect in 13%, local inflammatory causes in 7%, locally invading neoplasms in 6%, benign obstructing lesions in 6%, idiopathic in 6%, combined urinary tract infection and constipation in 2%, and incarcerated inguinal hernia in 2% of cases. Adverse drug effects and dysfunctional voiding were implicated 3 times as often in males than females (16% versus 6% and 19% versus 6%, respectively). Urinary tract infections were 6 times more common in females (31% versus 5%). Constipation and local inflammatory processes were twice as common in females than males (19% versus 11% and 12% versus 5%, respectively). Local neoplasms, benign obstruction and idiopathic causes were found exclusively in males. CONCLUSIONS: Urinary retention in children is a relatively rare entity but there is a significant incidence of neurological abnormalities in this population. If a clear reason for this condition cannot be delineated based on history, physical examination and laboratory assessment, these patients should undergo prompt neurological evaluation and appropriate imaging studies.

Voiding function in patients with the prune-belly syndrome after Monfort abdominoplasty.

PURPOSE: We reviewed our experience with patients with the prune-belly syndrome who had undergone Monfort abdominoplasty to assess whether our clinical impression of improved voiding efficiency could be demonstrated objectively. MATERIALS AND METHODS: From 1990 to 1993, 12 patients with the prune-belly syndrome underwent Monfort abdominoplasty with or without concomitant genitourinary reconstruction. All patients completed questionnaires on voiding before and after abdominoplasty, incontinence, bladder sensation, urinary flow, history of urinary tract infections and the ability to defecate. Urodynamic testing was performed in 8 patients before and after abdominoplasty. RESULTS: Subjective changes that occurred after abdominoplasty included resolution of or less double voiding in 9 patients, improved urinary continence in 7, improved bladder fullness sensation in 11, improved urinary flow in 10 and improved defecation in 5. The incidence of urinary tract infections decreased from a preoperative average of 5.7 per patient per year to 1.2 per patient per year postoperatively. Urodynamics demonstrated no significant changes in the measured urinary flow, capacity or maximal detrusor pressure. Compliance decreased on average but it remained in the compliant range. However, post-void residual volumes did significantly decrease from a preoperative average of 40.3% of bladder capacity to 13% after abdominoplasty. If the 5 patients who underwent concomitant urinary reconstruction were excluded, the reduction in average post-void residuals remained approximately the same, which was 40% of bladder capacity preoperatively to 14.3% after abdominoplasty. These data suggest that abdominoplasty alone was responsible for improved micturition. CONCLUSIONS: In addition to the cosmetic benefits and exposure provided for genitourinary reconstruction Monfort abdominoplasty seems to improve voiding efficiency.

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.

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.

Outcome of sling cystourethropexy in the pediatric population: a critical review.

PURPOSE: We performed a critical retrospective analysis of the records of all consecutive children who underwent sling cystourethropexy at our institutions. We particularly assessed the incidence and outcome in children who underwent enterocystoplasty in addition to a sling procedure. MATERIALS AND METHODS: Between May 1992 and August 1994, 24 girls and 15 boys 4 to 17 years old (mean age 9) consecutively underwent 1 of 4 techniques of sling cystourethropexy for stress urinary incontinence using rectus fascia via an abdominal approach. Diagnoses included spinal dysraphism in 34 patients, spinal trauma in 2, cecoureterocele in 1, epispadias in 1 and classic bladder exstrophy in 1. All children underwent preoperative video urodynamics. In 4 patients only a sling procedure was performed (group 1), while in 9 others a sling procedure was performed after enterocystoplasty (group 2). In 26 patients a sling procedure and concomitant enterocystoplasty were performed (group 3). RESULTS: Patients who underwent concomitant enterocystoplasty had more hyperactive preoperative urodynamics than those who did not. Postoperative continence was subcategorized in terms of patient age, sex, diagnosis (neurogenic versus nonneurogenic), preoperative video-urodynamics, surgeon technique, group (that is with or without cystoplasty) and type of enterocystoplasty (that is stomach versus ileum). Of these factors only concomitant enterocystoplasty was predictive of postoperative dry continence (73% of group 3, 33% of group 2 and 25% of group 1). In most patients who were not dry postoperative video urodynamics suggested suboptimal outlet resistance overcome by a hyperactive detrusor. No patient had postoperative upper tract deterioration. CONCLUSIONS: These results suggest that concomitant enterocystoplasty should be critically considered for pediatric patients undergoing sling cystourethropexy.

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 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.

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.

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)

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.

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.

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.

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.

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.

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.

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.

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.

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.