Childhood urolithiasis: experiences and advances.

Between June 1979 and June 1989, 54 children with urolithiasis were evaluated and treated at the Johns Hopkins Children's Center. The most common symptoms were flank or abdominal pain (58%) and gross hematuria (28%). In 46 children (86%), stones were secondary to a preexisting condition and in only 8 (14%) no apparent cause of stone formation could be found. Thirty-six patients (66%) had a solitary stone, most commonly found in the kidney. Urinary tract infections were present in 25 (47%) of the patients who had stones. Stones composed either of calcium oxalate or struvite were the most frequently recovered in these patients with infections. Twenty-one patients (39%) spontaneously passed their stones whereas 23 (43%) required either surgery or extracorporeal shock-wave lithotripsy to resolve stones. Ten (20%) showed recurrence of their urolithiasis, with follow-up examination periods ranging from 1 month to 10 years. Recent advances in the management of urolithiasis and their applicability to the pediatric population are discussed.

Fetal genitourinary tract anomalies: evaluation, operative correction, and follow-up.

The prenatal diagnosis of a genitourinary anomaly was made in 53 fetuses. Sonographic findings, antenatal course, and postnatal treatment and outcomes were examined. No interventional therapy was undertaken in utero, and the natural history could be examined in the 44 of 53 (83%) who did not electively terminate their pregnancies. Twenty-three of the total 53 (43%) had unilateral disease and 30 (57%) had bilateral involvement. Thirty-five of 53 (66%) survived, with 22 undergoing operative therapy postnatally. In all but one of the 35 survivors, the anomaly was isolated to the genitourinary tract, and the majority of surviving fetuses had unilateral disease. Oligohydramnios was present in only three of 35 survivors, and none had severe oligohydramnios. Nine of 53 women underwent termination of pregnancy and nine others experienced a neonatal death. All 18 of their fetuses had bilateral disease, with oligohydramnios present in 14 (78%). All five fetuses with chromosomal abnormalities were found in this group of 18. The majority of fetuses with a genitourinary anomaly will do well postnatally. Pulmonary hypoplasia, extrarenal anomalies, and chromosomal defects are frequent findings in the minority with poor outcomes. A multidisciplinary approach to management of the pregnancy with a fetal genitourinary tract anomaly is essential to optimize outcome.

Criteria for the prenatal diagnosis of classic bladder exstrophy.

OBJECTIVE: To define ultrasonographic criteria for the prenatal diagnosis of classic bladder exstrophy. METHODS: Forty-three prenatal ultrasound scans were studied from 25 pregnancies in which live delivery of an infant with classic bladder exstrophy occurred. The diagnosis of bladder exstrophy could be made retrospectively in 29 prenatal studies from 17 pregnancies. The time of the fetal ultrasound varied from 14-36 weeks' gestation (mean 23). The diagnosis of bladder exstrophy was made before delivery in only three cases. RESULTS: Five factors associated with bladder exstrophy were identified: 1) The bladder was not visualized on ultrasound in 12 of 17 cases (71%); 2) a lower abdominal bulge representing the exstrophied bladder was seen in eight of 17 cases (47%); 3) a small penis with anteriorly displaced scrotum was identified in eight of 14 males (57%); 4) the umbilical insertion was low set in five of 17 cases (29%); and 5) abnormal widening of the iliac crests was seen in three of 17 cases (18%). CONCLUSION: The prenatal diagnosis of bladder exstrophy should be considered any time the bladder is not visualized or any of the aforementioned factors are noted.

Metachronous bilateral Wilms tumor. Report of longest-known survivor and guidelines for conservative management.

In a one-year-old black male patient a classic Wilms tumor of the left kidney was treated with left nephrectomy, chemotherapy, and irradiation to the entire abdomen. Two years later a mature Wilms tumor, consisting predominantly of rhabdomyomatous elements, was discovered in the right kidney. Although a second course of chemotherapy was given and 2,000 rad were delivered to the right flank, the mass continued to enlarge with progressive deterioration of renal function. A nephron-sparing procedure was performed, in which a 3,400-g tumor measuring 19 cm X 16 cm X 9 cm was enucleated from the right kidney without compromise to the remaining normal tissue. Pathologic examination of the surgical specimen revealed a mature Wilms tumor with a malignant anaplastic sarcoma arising in the central portion. Currently, he is disease-free with normal renal function more than twenty years after diagnosis of the metachronous bilateral Wilms tumor. We believe he is the longest known surviving metachronous Wilms tumor patient and emphasizes the importance of conservative, meticulous surgery in the management of both unilateral and bilateral Wilms tumors. The role of multimodal therapy (chemotherapy, irradiation, and surgery) as well as the current guidelines for the management of bilateral Wilms tumors as proposed by the National Wilms Tumor Study are reviewed.

Incidental ganglioneuroblastoma.

A case is described in which an incidental ganglioneuroblastoma was discovered at the time of surgical correction of upper pole hydronephrosis secondary to an obstructing ectopic ureterocele in a duplicated system. We believe this is the first case of incidental ganglioneuroblastoma reported in the urologic literature. The management of this patient's disease is discussed as is the entity of incidental neuroblastoma.

Primary obstructive megaureter in children.

Sixty-five cases of primary obstructive megaureter were retrospectively evaluated. The clinicoradiologic features of the abnormality are characteristic; however, a complete urologic workup is necessary to exclude the more common causes of ureteral dilatation. Treatment must be planned according to status of renal and ureteral functions. Surgical excision of the obstructive segment, reduction of ureteral caliber when indicated, and reimplantation achieved the best results in this series. A brief period of nephrostomy drainage prior to surgical remodeling of the lower ureter can be useful in selected cases. Conservative treatment of children with relatively few symptoms and slight ureteral dilatation is worthwhile.

Urinary diversion in hypospadias surgery 1987.

To determine which form of urinary diversion is most effective for each type of hypospadias, we polled all full-time practicing pediatric urologists in the United States and Canada. Each was asked to complete a questionnaire, specifying the method of urinary diversion used, based on the severity of the hypospadias. Ninety-eight per cent (84 of 86) of the urologists contacted replied to the survey. Our findings suggest that: Urinary diversion is usually not necessary for very mild types of hypospadias. Intraurethral drainage is the preferred form of diversion for moderate types of hypospadias. Suprapubic drainage with an intraurethral stent is the favored means of diversion for severe types of hypospadias. Perineal urethrostomy is rarely used today in hypospadias surgery.

Long-term nutritional consequences of bowel segment use for lower urinary tract reconstruction in pediatric patients.

OBJECTIVES: To identify whether nutritional abnormalities, specifically vitamin B12 deficiency, folate deficiency, and low carotene levels, are long-term sequelae of the use of ileum and/or colon for lower urinary tract reconstruction in pediatric patients. METHODS: Serum levels of vitamin B12, methyl malonic acid (MMA), carotene, and folate were measured 3 to 13 years (mean 6) after surgery in patients less than 18 years old at the time of operation. RESULTS: Thirteen (44.8%) of 29 patients had abnormal nutritional serum levels, with 5 (38.5%) of 13 having more than one abnormal value. Vitamin B12 was low in 4 (14%) of 29 patients; MMA was abnormally high in 7 (26%) of 27; and both folate and carotene levels were low in 4 (14.8%) of 27. No patient with reconstruction with colon alone (n = 3) had abnormal values, and no patient had any clinical signs or symptoms of vitamin B12 deficiency or fat malabsorption. CONCLUSIONS: Nutritional abnormalities can occur in patients after lower urinary tract reconstruction with ileum. No patient in this study had symptomatic abnormalities; however, long-term clinical effects may be significant. The implications of low folate levels in women of childbearing age must also be taken into consideration because of the potential association with congenital anomalies.

Metabolic complications secondary to obstruction of a Kock pouch afferent limb.

Metabolic complications are frequently seen following urinary diversion with an intestinal segment. Afferent limb complications are noted in 10% of patients undergoing continent Kock urinary diversion. We present a patient with cloacal exstrophy with developed significant hypocalcemic tetany and metabolic acidosis related to a nondraining afferent limb of a Kock pouch created to achieve continence.

Control of prolonged, benign, renal hematuria by silver nitrate instillation.

Four patients with protracted benign hematuria underwent silver nitrate instillation of the upper collecting system. The doses varied from 10 ml. of 0.25 per cent silver nitrate to 14 ml. of 1 per cent silver nitrate. In all 4 cases the hematuria resolved within four days without significant complications. At follow-up extending from three to twenty-four months there have been no recurrences of gross hematuria. Silver nitrate instillation appears to be a safe and effective method of treating benign renal hematuria, thus obviating the need for extirpative surgery.

Pediatric testicular tumors: the Johns Hopkins experience.

Testicular neoplasms constitute 1 percent of all childhood malignancies and rank eighth in childhood cancer mortality. From 1970 to 1988, 25 testicular tumors in children eighteen years and under were seen. The majority of the patients were white (88%). Pathologic analysis of the tumors revealed that 68 percent were germinal and 32 percent were nongerminal. Staging was undertaken in all patients with serum markers, chest x-ray film, and computerized tomography scans or lymphangiography. All patients underwent radical orchiectomy, and further therapy was given dependent on tumor type and stage. The survival among this cohort was excellent, with only 3 patients succumbing to their disease. Detailed results of treatment, and approaches to avoid excess treatment morbidity are reviewed.

Epispadias with phimosis: an unusual variant of the concealed penis.

An unusual variant of concealed penis is described. Two neonates with epispadias and complete phimosis were diagnosed at birth and underwent delayed repair. These 2 cases are the 5th and 6th such reports in the literature. This anomaly's possible embryogenesis is reviewed.

Ureteral bladder augmentation using the lower pole ureter of a duplicated system.

Megaureters of nonfunctioning renal segments in a duplex system are commonly available for use in reconstructive procedures secondary to high-grade reflux, ectopic ureter, ectopic ureterocele, or obstructive megaureter. The use of megaureters subtending a nonfunctioning lower pole renal segment for bladder augmentation in 2 patients is reported.

Urinary tract reconstruction in prune belly syndrome.

Fifteen boys with prune belly syndrome cared for over a ten-year period required surgical treatment because of uncontrolled infection and/or progressive azotemia. Five died of renal dysplasia. Primary or staged reconstruction resulted in significant improvement when ureteral reimplantation was combined with tailoring of ureteral caliber.

Idiopathic anterior urethritis in prepubertal and pubertal boys: pathology and clues to etiology.

OBJECTIVES: Records were reviewed to examine the common features of boys with noninfectious anterior urethritis, and to describe the underlying pathology. METHODS: Five cases of anterior urethritis with features typical of this syndrome are reviewed. RESULTS: These cases demonstrate the typical appearance and in 1 case the pathologic diagnosis of squamous metaplasia of the bulbar urethra. CONCLUSIONS: Prepubertal urethrorrhagia is associated with squamous metaplasia of the urethra. The tendency to stricture formation may precede instrumentation of the urinary tract.

Primary massive reflux in children.

Over a ten-year period, of more than 1,000 children whose primary vesicoureteral reflux was treated surgically, there were 54 children with 80 single ureters which were massively dilated. Primary reconstruction with ureteral tailoring was done in 25 ureters with a success rate of 92 per cent. Forty-five ureters were temporarily diverted because of uncontrolled infection and/or azotemia. In almost half of these instances, temporary diversion resulted in improvement in ureteral caliber to such an extent that nontailored reimplantation could be performed with a success rate of 87 per cent. Six kidneys were removed and four have been maintained in the diverted state with severe renal dysplasia. Eighty-eight per cent of the refluxing megaureters were reconstructed with a success rate of 89 per cent.

The Cantwell-Ransley epispadias repair in exstrophy and epispadias: lessons learned.

OBJECTIVES: We evaluated our experience with the Cantwell-Ransley epispadias repair to determine the lessons that have been learned with the increased experience and follow-up. METHODS: A total of 75 boys (60 with bladder exstrophy and 15 with complete epispadias) underwent a Cantwell-Ransley epispadias repair at our institute in the last 6 years. Primary repair was performed in 58 boys (45 with exstrophy and 13 with epispadias), and secondary repair was performed after prior failed closure in 17 boys (12 at the secondary exstrophy closure, 3 with exstrophy, and 2 with complete epispadias). RESULTS: At a mean follow-up of 28 months, all patients had a horizontal or downward angled penis while standing. The incidence of urethrocutaneous fistulas in the immediate postoperative state was 21% and at 3 months was 15%. The incidence of urethrocutaneous fistulas was no more in those patients in whom paraexstrophy skin flaps were used at anterior closure than in those in whom the urethral plate was left intact. Two patients developed a urethral stricture at the proximal anastomotic area, and 4 patients had minor skin separation of the dorsal penile skin closure. Catheterization or cystoscopy, or both, has been performed in 60 patients and revealed an easily negotiable urethral channel in all. CONCLUSIONS: The Cantwell-Ransley epispadias repair offers a straighter urethra, better correction of chordee and cosmesis, and a lower fistula rate in the exstrophy or epispadias patient.

The multiple reoperative bladder exstrophy closure: what affects the potential of the bladder?

OBJECTIVES: To define the possible cause of failure and the eventual potential of the bladder in 23 exstrophy patients, who underwent more than two failed prior attempts at closure. METHODS: Twenty-three patients were selected from the exstrophy data base who had two or more prior closures. Eighteen patients had undergone 2 previous closures and 5 patients 3 previous closures for either complete dehiscence or significant prolapse. At the time of initial closure, 19 patients did not have an osteotomy. At secondary closure, 10 underwent osteotomy while at third closure 5 had an osteotomy. At the time of reclosure at our institution all underwent an osteotomy. RESULTS: Reoperative repair at our institution was successful in all patients. Six patients achieved a bladder size suitable for bladder neck reconstruction and of them 3 are dry. The bladder size was inadequate in 9 patients and 8 are being monitored for possible bladder growth. CONCLUSIONS: Tension-free closure with osteotomy and immobilization are important factors both in an initial or any subsequent closure. The chance of obtaining an adequate bladder capacity for bladder neck plasty and eventual continence, following multiple reclosures, is markedly diminished.

Prostate-specific antigen in men born with bladder exstrophy.

OBJECTIVES: To determine serum levels of prostate-specific antigen (PSA) in men born with bladder exstrophy and to investigate the clinical utility of this test in the bladder exstrophy population. METHODS: Ten men aged 19 to 45 years with a history of bladder exstrophy underwent digital rectal examination and free and total serum PSA determinations. Immunohistochemistry for PSA and prostate-specific acid phosphatase (PSAP) was performed on archival prostate tissue from 1 man with bladder exstrophy. RESULTS: Free and total serum PSA levels for all patients were measurable and below the upper limit for established age-specific reference ranges for normal men. Both PSA and PSAP were detectable by immunohistochemistry in prostate epithelial cells of a man with bladder exstrophy. CONCLUSIONS: Men born with bladder exstrophy have detectable serum PSA levels. However, because prostate cancer in men with bladder exstrophy has not been reported, whether PSA will have a clinical role as a serum tumor marker for prostate cancer in this population remains to be determined. If so, the PSA reference range for men with the exstrophy condition will need to be defined. Because prostate histology in men with exstrophy resembles that of normal men, careful clinical monitoring for benign and malignant tumors should not be overlooked in this particular population.

Exstrophy and cloacal exstrophy.

Classic bladder exstrophy and cloacal exstrophy are grotesque anomalies in the eyes of young parents. An immediate evaluation by an experienced team is required to assess the variations in the condition and to devise a plan of treatment that can reassure the parents as to eventual prognosis and rehabilitation. This plan of treatment must include provisions for renal preservation, urinary control, cosmetic appearance, and sexual function. The newborn is usually normal in respect to nutrition and its cardiopulmonary state, and will tolerate surgery well. Passive parental immunity provides better resistance to intercurrent illness at this time than will be present in the later months of infancy. When applicable, the initial stages of surgery should be undertaken at birth when the pelvic ring can be approximated without osteotomy and the bladder mucosa has not deteriorated from inflammatory changes. Parental attitudes toward the child as well as successful reconstruction may both be best served by immediate surgery to begin reconstruction and reduce the visible defect.