Abnormalities of the external genitalia.

This article discusses the general nuances of hypospadias, exstrophy/epispadias, and ambiguous genitalia. Embryologic considerations, etiologic factors, anatomy, associated anomalies, and timing of referrals and surgery are discussed.

Reduction of paraphimosis with hyaluronidase.

The use of hyaluronidase facilitates reduction of paraphimosis. It acts by dispersing extracellular edema, permitting easy reduction of the foreskin. Its use is applicable both in the hospital and outpatient setting. Hyaluronidase is widely available and keeps well if refrigerated. It is effective for children and adults.

Extracorporeal membrane oxygenation in the neonate with congenital renal disease and pulmonary hypoplasia.

Extracorporeal membrane oxygenation (ECMO) is an effective treatment modality for the newborn with refractory hypoxemia. Oligohydramnios can be associated with congenital renal disease (CRD) and can result in respiratory insufficiency from pulmonary hypoplasia, delayed lung maturation, and persistent pulmonary hypertension of the newborn. In this retrospective study, the authors reviewed the outcome of four children with CRD who required ECMO in the neonatal period. Between October 1987 and December 1995, ECMO was used in four newborns with CRD and pulmonary hypoplasia unresponsive to maximal medical management. The causes of CRD were urinary obstruction (2), renal dysplasia (1), and vesicoureteral reflux (1). Neonatal survivors of ECMO with CRD had regular follow-up with a nephrologist, urologist, and pediatrician. Developmental history, assessment of renal function, and a nutritional evaluation were recorded on each visit. The follow-up period ranged from 6 months to 5 years. All patients with CRD were successfully weaned from ECMO. One child died, at 1 month of age, because of renal failure. The estimated glomerular filtration rates in the three survivors were 20, 24, and 60 mL/min/1.73 m2. Growth and development have been delayed in two patients. Based on the author's experience, ECMO may improve the survival of neonates with pulmonary hypoplasia and CRD. Factors associated with successful long-term outcome include (1) renal disease amenable to surgical correction, (2) aggressive nutritional support, and (3) a reliable social support system.

Fertility index analysis in cryptorchidism.

We report on 226 male patients with cryptorchidism 6 months to 16 years old who underwent open testis biopsy at orchiopexy or orchiectomy at Children's Hospital and Health Center from 1986 to 1990. A total of 355 specimens was obtained. Tissues were preserved in Bouin's solution and examined on light microscopy for fertility index measurements. Several biopsies prepared using Bouin's preserved paraffinized tissue and glutaraldehyde preserved semi-thin cut tissue were found to have comparable fertility index measurements. Of 184 patients with unilateral undescended testes 87 also underwent biopsy on the contralateral descended testis. A total of 42 patients had bilateral undescended testes. Age matched comparisons were made between fertility index measurements of the undescended testes and those previously reported of normal testes. Additional case matched comparisons of fertility indexes were made in those children who underwent biopsy of the undescended testis and its descended mate. Statistical analysis was performed using the independent Student t-test. When comparing undescended to descended testes, there was no significant difference in the fertility index of patients 1 year old or younger but fertility index differences were statistically significant in all of the other age groups. Fertility index measurements were significantly decreased from normal expected values in all age groups with unilateral cryptorchidism and in all but the 13 to 18-month-old group with bilateral cryptorchidism. The fertility index in the descended testis was similar to previously reported normal testis measurements in boys between 13 months and 6 years old. Our data suggest that potential fertility in the cryptorchid testis may be significantly impaired compared to normal testicular fertility regardless of patient age at the time of discovery of the undescended testis. The fertility index of the descended mates of unilateral undescended testes may also be somewhat impaired in certain age groups. Orchiopexy in the first year of life may be indicate to preserve available fertility potential.

Pediatric urology facts and figures. Data useful in the management of pediatric urologic patients.

This article includes facts and figures useful in the management of pediatric urologic patients. A formulary of commonly used drugs as well as modified doses for use in renal insufficiency is provided. In addition, data on bowel preparations, vital signs, hypertension, creatinine clearance, Tanner stages, and stretched penile length are provided.

Fibrous hamartoma of infancy: a report of two cases in the genital region.

OBJECTIVES: Fibrous hamartoma of infancy (FHI) is an uncommon subcutaneous proliferative lesion usually found in the upper portions of the body, particularly in males during the first 2 years of life. We report 2 cases of FHI in the genital region. METHODS: We retrospectively reviewed the charts of children treated at Children's Hospital and Health Center, with FHI located in the genital region. RESULTS: We report 2 cases of FHI in the genital region, 1 involving the scrotum and 1 the labium majus. CONCLUSIONS: FHI should be considered in the differential diagnosis of genital masses in children. The histologic features are characteristic, local excision of the tumor is curative, and the local recurrence rate is low.

Is urinary tract screening necessary for patients with cerebral palsy?

Patients with cerebral palsy are prompted to seek urological evaluation when urinary tract infection, socially unacceptable incontinence or hematuria occurs. We attempted to determine the prevalence of urinary tract structural changes by prospectively screening on sonography the kidneys and bladder of 90 patients 1 to 25 years old (mean age 8 years) who had cerebral palsy with or without urological symptoms. Uncooperative patients or those who would require sedation were excluded. Of the patients 66 were incontinent and used diapers, 18 were completely dry and 6 had nocturnal enuresis with daytime dryness. Sonographic abnormalities were detected in 7 patients, including renal size discrepancy in 2 (1 with severe scoliosis and 1 with a history of renal artery thrombosis), mild to moderate hydronephrosis with thickened bladders suggestive of neurogenic bladder dysfunction in 3 and a nonvisualized kidney in 2. However, repeat sonography confirmed 2 normal kidneys in the latter patients. Thus, urinary tract abnormalities were detected unexpectedly in 2% of patients studied. This relatively low proportion suggests that routine urinary tract screening in cerebral palsy patients may not be warranted.

An alternative approach to myelodysplasia management: aggressive observation and prompt intervention.

We have adopted an approach to the management of myelodysplasia patients which is contrary to that most commonly supported by the existing literature. We screen patients shortly after birth with ultrasound, urine culture and serum creatinine, and then follow patients at 3 to 6-month intervals with similar studies. Cystography and urodynamics are done only as required clinically or by a change in the sonogram. Clean intermittent catheterization is indicated for continence or medical reasons. With this approach of aggressive observation and prompt intervention, we observed a rate of renal deterioration (diminished function on renal scan or parenchymal loss on imaging studies) of 5%. Renal deterioration was associated statistically with urinary tract infections and reflux. Patients started on clean intermittent catheterization for medical indications had greater risk for renal deterioration than those started on it for continence. Renal deterioration occurred with equal frequency between patients with abnormal and normal urodynamic studies, that is urodynamics did not predict renal deterioration. Our data show a rate of renal deterioration similar to other reports. We believe that aggressive observation with prompt intervention for problems once identified represents a reasonable alternative to managing patients with myelodysplasia.

Preoperative laparoscopic localization of the nonpalpable testis: a critical analysis of a 10-year experience.

Laparoscopy was performed on 107 patients 8 months to 18.5 years old (mean age 46.9 months) with 119 nonpalpable testes. The procedure accurately determined the location of the gonad in 95.3% of the patients, while 16 were spared formal surgical exploration with the laparoscopic visualization of blind-ending spermatic vessels and vasa deferentia. Additionally, patency of the internal inguinal ring, determined laparoscopically, was an accurate predictor of testicular salvageability. We advocate the use of laparoscopy for all boys with a nonpalpable gonad in conjunction with operative intervention if necessary.

Genitourinary trauma in the pediatric patient.

Trauma is the major source of mortality in the pediatric population. A retrospective review was performed on patients admitted to the Children's Hospital and Health Center Trauma Program, San Diego, California, from August 1984 to May 1990. The purpose of this review was to evaluate pediatric trauma and to determine the best treatment and evaluation for genitourinary injuries. Blunt trauma was responsible for 98 percent of the injuries, with renal injuries being the most common. Bladder (7) and male urethral (2) injuries, and vaginal lacerations (8) also occurred. The most severe renal injuries (70%) and all significant bladder and urethral injuries were associated with gross hematuria. Hypotension was present in 31 percent of patients but rarely required surgical exploration for correction. Eighty-six patients underwent radiographic imaging. Computerized tomography (CT) scans demonstrated the most information about intra-abdominal solid organ injuries but was inaccurate in detecting bladder or urethral injuries. Genitourinary injury is common in children but rarely requires surgical management. CT scan is the best study to determine extent of solid-organ injury but is inferior to cystourethrography to diagnose bladder or urethral injuries.

Surgical approach to male pseudohermaphroditism.

Male pseudohermaphroditism (MPH) is defined as incomplete masculinization in patients with normal male karyotype (XY) and testicular histology. MPH encompasses a spectrum of female to male phenotypes and presents both diagnostic and technical challenges to the surgeon. Once gender is assigned, based on phenotype, adequacy of the phallus, and other considerations, the surgeon can plan appropriate genital reconstruction.

Diagnostic pneumoperitoneum for the detection of the clinically occult contralateral hernia in children.

The detection and management of occult contralateral hernia in children who present with a clinically evident unilateral hernia have evoked controversy. Routine use of herniography, intraoperative probing of the contralateral inguinal area and routine or selective exploration of the contralateral groin all have their advocates and detractors. During the last 5 years we have used intraoperative pneumoperitoneum and we report our experience in 64 patients 3 months to 9 years old. A retrospective analysis of the data revealed that pneumoperitoneum was negative in demonstrating a contralateral inguinal hernia in 59 of 64 patients (92%). Contralateral exploration was not performed in patients in whom pneumoperitoneum was negative. All 5 patients who tested positive had an indirect inguinal hernia upon contralateral exploration and all 5 were less than 3 years old. Patients who had a negative pneumoperitoneum were followed for up to 5 years and only 1 (1.8%) false negative examination was discovered. Pneumoperitoneum is a safe, effective means to evaluate the contralateral groin for occult hernia at the time of unilateral hernia repair in children.

Fascia lata and early spica casting as adjuncts in closure of bladder exstrophy.

Correction of the skeletal defect seen in the exstrophy complex consists of reconstitution of the pelvic ring. Long-term success depends upon the formation of a fibrous union between the pubes. When this union does not occur diastasis of the pubis results. Long-term urological success appears to be related to adequate pubic approximation. We used fascia lata to construct an anterior pelvic ligament between the pubic bones in 7 patients. In the 6 patients for whom followup is available the fascia lata has persisted and appears to be viable histologically on biopsy specimens obtained at a subsequent operation. Because the fascia lata holds the pubis together so well we have been able to cease Bryant's traction in children by 2 weeks and place them into a cast. This has resulted in early discharge from the hospital in all patients. For these reasons we believe that fascia lata is a useful adjunct in the armamentarium of materials used to reapproximate the pubis.

Microvascular autotransplantation of the intra-abdominal testis.

The operative management of intra-abdominal testes is controversial, largely because there is no method of orchiopexy that can reliably produce good results. We report our results on the use of microvascular autotransplantation of intra-abdominal testes. We performed 12 autotransplants in 10 boys ranging in age from 10 months to 14 years (median age 30 months). In each instance the testis had been localized previously by laparoscopy or it was known to be intra-abdominal and was believed to be grossly normal in appearance. The vascular anastomoses of the spermatic to the inferior epigastric vessels were performed by an experienced microvascular surgeon using 10-zero nylon suture and 40 times magnification. Biopsies were taken at operation in 9 patients. Followup of 6 to 30 months is available in 7 patients and all 8 testes are palpably normal and in good scrotal position. This approach is feasible even in small children and the results seem to be superior to the expected results reported with division of the spermatic vessels.

Follow-up of infants with obstructive uropathy detected in utero and treated surgically postnatally.

Thirty-four infants with obstructive uropathy detected by prenatal ultrasonography at 34 +/- 0.56 weeks (SE) had surgery during the first 2 years of life. The growth and renal function of the 25 children after being followed for over 1 year are described. Twelve children had unilateral disease, and 13 had bilateral disease. All 12 children with unilateral disease grew at or above the fifth percentile and had normal renal function (glomerular filtration rate, 101 +/- 7 mL/min/1.73 m2). Thirteen children with bilateral disease were followed for 26.1 +/- 3.4 months. Growth was good: 10 of the 13 grew at or above the fifth percentile. The serum creatinine was 0.7 +/- 0.2 mg/dL, and the glomerular filtration rate was 91 +/- 10 mL/min/1.73 m2. One child required chronic dialysis. The prenatal diagnosis of urinary tract anomalies followed by early intervention may improve the long-term outcome of children with obstructive uropathy.

Ectopic ureteroceles: surgical management with preservation of continence--review of 60 cases.

During the last 19 years we treated 60 children with ectopic ureteroceles. There was a 4 to 1 female-to-male predominance. Of the patients 47 per cent presented before age 6 months, 66 per cent were diagnosed after a urinary tract infection and 6 were identified antenatally. Based upon a review of the literature and our data, we believe that "total correction" (a single operation designed to correct abnormalities of the upper and lower urinary tract) is superior in most instances to an approach in which just the upper urinary tract pathological condition is treated. Only 4 (14 per cent) of 28 patients in whom "total correction" was used required a second operation (all because of persistent reflux) while 14 of 19 patients (74 per cent) treated with an upper urinary tract procedure intended as definitive therapy have either undergone further surgery or have a residual abnormality that may require further surgery. Marsupialization rather than enucleation of the ectopic ureterocele is effective and safe, risking neither injury to the bladder neck or sphincteric mechanism nor resulting in a vesicovaginal fistula.

Modified meatal advancement and glanuloplasty (Arap hypospadias repair): experience in 31 patients.

We used the Arap hypospadias repair in 31 patients with distal hypospadias with considerable success, achieving an excellent result in 93 per cent of our patients. Improved cosmesis, extension of the scope of the problems that can be addressed with this repair (including treatment of a distal urethrocutaneous fistula) and the ease with which the Arap procedure can be performed are the advantages that this operation has over other 1-stage distal hypospadias repairs.

Urethral strictures after hypospadias repair.

Over a 16 years period, we have treated 34 cases of urethral stricture after hypospadias repair. Seventeen patients were successfully treated with one procedure, while 16 patients required two or more procedures; one patient was lost to follow-up. Eighteen patients who presented with symptoms attributable to their stricture within 3 months of their original operation for hypospadias were designated as having early strictures. Those strictures presenting later than 3 months were termed late strictures. Manipulative therapy (dilation, direct vision internal urethrotomy) was successful in 46% of early strictures, but in only 16% of late strictures. Open surgical procedures were employed primarily in some cases or after failed manipulation in others and were successful in 82% of cases. All 4 failures of open procedures were salvaged with manipulative therapy. We conclude that treatment of post hypospadias repair urethral strictures should initially be manipulative. Open surgical repair will usually be successful but should be reserved for difficult strictures, late strictures, or failures of manipulation.

Detection of testicular torsion by magnetic resonance imaging in a rat model.

Testicular torsion is one of the most common pediatric urological emergencies. Incorrect or delayed diagnosis contributes significantly to morbidity. We previously have shown that magnetic resonance displays scrotal contents with great detail using hydrogen concentration weighted and T2 weighted images. Sprague-Dawley rats underwent either unilateral 720-degree testicular torsion or a sham procedure. Magnetic resonance images were obtained at intervals with a 3 or 5-inch surface coil. Scans after surgical torsion showed a characteristic spiral distortion of the fascial planes of the spermatic cord, not seen in the sham animals, as well as a decrease in testicular size with prolonged torsion.