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Purpose: To evaluate the effect of conventional and standard (ST) versus pulse-intensive (PI) chemotherapy and short-duration versus long-duration chemotherapy on relapse-free survival (RFS) and overall survival rates of patients with clear-cell sarcoma of the kidney (CCSK) entered onto the National Wilms' Tumor Study (NWTS)-4., Patients and Methods: The 5-year and 8-year RFS rates were determined for patients with CCSK treated on the NWTS-4. After August 6, 1986, 40 previously untreated children younger than 16 years with CCSK were randomly assigned, after the completion of 6 months of chemotherapy, to discontinue (short) or continue 9 additional months (long) of treatment with chemotherapy regimens that included vincristine and either divided-dose (ST) courses (5 days) or single-dose (PI) treatment with dactinomycin and divided-dose (ST) courses (3 days) or single-dose (PI) treatment with doxorubicin., Results: For patients with CCSK, the 5- and 8-year RFS rates were 65.2% and 60.6%, respectively, for patients randomly assigned to the short chemotherapy and 87.8% (both 5- and 8-year RFS) for patients randomly assigned to the long chemotherapy (P =.08). The overall survival rates for patients at 5 and 8 years were 95.5% and 85.9%, respectively, for the short chemotherapy and 87.5% (both 5- and 8-year overall survival) for the long chemotherapy (P =.99). In NWTS-4, the overall survival rates for patients with CCSK improved from NWTS-3 (83% v 66.9% at 8 years, respectively; P <.01)., Conclusion: CCSK patients exhibit an improved RFS from a longer course of therapy when using vincristine, doxorubicin, and dactinomycin, but their long-term survival is unchanged compared with patients receiving 6 months of therapy. The overall survival rates for patients with CCSK have improved from NWTS-3.

Purpose: The National Wilms' Tumor Study (NWTS)-4 was designed to evaluate the efficacy, toxicity, and cost of administration of different regimens for the treatment of Wilms' tumor (WT)., Patients and Methods: Between August 6, 1986 and September 1, 1994, 1,687 previously untreated children less than 16 years of age with stages I to II/favorable histology (FH) or stage I/anaplastic histology WT (low-risk [LR] group) or stages III to IV/FH WT or stages I to IV/clear cell sarcoma of the kidney (high-risk [HR] group) were randomized to treatment that included vincristine and either divided-dose (standard [STD]) courses (5 days) or single-dose (pulse-intensive [PI]) treatment with dactinomycin. HR patients also received either STD courses (3 days) or PI treatment with doxorubicin., Results: The 2-year relapse-free survival (RFS) rates for LR patients were 91.3% for 544 randomized to treatment with PI and 91.4% for 556 randomized to treatment with STD chemotherapy (P = .988). The 2-year RFS rates for HR patients were 87.3% for 299 randomized to treatment with PI and 90.0% for 288 randomized to treatment with STD chemotherapy (P = .865)., Conclusion: We conclude that patients treated with PI combination chemotherapy for LR or HR WT or clear cell sarcoma of the kidney have equivalent 2-year RFS to those treated with STD regimens. PI drug administration is recommended as the new standard based on demonstrated efficacy, greater administered dose-intensity, less severe hematologic toxicity, and the requirement for fewer physician and hospital encounters.

Methods: The authors reviewed 131 children enrolled in National Wilms Tumor Study-3 (NWTS-3) who received preoperative treatment for tumors unable to be resected at surgery or judged inoperable by imaging evaluation. Preoperative biopsies were performed on 103 patients. Patients were assigned a pretreatment stage: stage II (11 patients), stage III (39 patients), stage IV (66 patients), and unknown (15 patients). The chemotherapy regimen included dactinomycin and vincristine (81 patients), dactinomycin, vincristine, and doxorubicin (30 patients), dactinomycin, vincristine, doxorubicin, and cyclophosphamide (10 patients), and other (8 patients). Preoperative radiation therapy was started concurrently with chemotherapy (27 patients) or because of lack of response (14 patients). Two patients were given preoperative irradiation without chemotherapy., Results: Response to therapy was assessed after the first trial of chemotherapy. Partial responses were noted in 110 patients (85%), 3 had complete responses, 13 had no response or progression of disease, and 5 patients were not able to be evaluated. There were no significant differences in preoperative response to the different chemotherapy regimens. Median time interval from diagnosis to nephrectomy was 58.5 days. When compared with NWTS-3 patients not receiving preoperative treatment, survival was reduced for patients treated preoperatively (88% vs. 74%, respectively, 4-year survival), which was only partially explained by differences in stage distribution. Median duration of follow-up was 5.9 years. Lack of response to the preoperative treatment was associated with a poor prognosis. Eight children died before removal of the primary tumor. All eight had either progressive disease or no response to the preoperative treatment., Conclusions: The use of preoperative treatment can facilitate subsequent surgical resection in selected patients with inoperable Wilms tumors. Although these very large tumors--judged unable to be resected--have a somewhat worse prognosis, nephrectomy was completed in 93% of patients after preoperative treatment. However, preoperative treatment will lead to less accurate surgical and pathologic staging, and undertreatment should be avoided in these high-risk patients.

The incidence, cause and significance of hydronephrosis in patients with renal ectopia are unknown. Therefore, we conducted a retrospective review of 77 patients with 82 ectopic kidneys. Of 82 kidneys 46 (56%) had hydronephrosis. Dilatation of the renal pelvis was the result of primary ureteropelvic or ureterovesical obstruction in 52%, grade 3, 4 or 5 vesicoureteral reflux in 26% and extrarenal collecting systems with malrotation that produced apparent ureteropelvic junction obstruction in 22%. Surgery was performed in 41 patients, including primary nephrectomy in 18, ureteral reimplantation in 14 and ureteropelvic junction repair in 8. Hydronephrosis was present in 15 of 58 contralateral nonectopic kidneys, and 11 required surgery mostly for vesicoureteral reflux. Solitary ectopic kidneys with no associated obstruction or reflux demonstrated normal renal function. Our data indicate that the prognosis in renal ectopia relates directly to the associated urological disease and timely surgical intervention, not to ectopia alone.

Objective: This study was undertaken to define the incidence and etiology of small bowel obstruction (SBO) after nephrectomy for Wilms' tumor., Summary Background Data: Intestinal obstruction is one of the most common postoperative complications after nephrectomy for nephroblastoma. However, few reports have evaluated risk factors for SBO. Radiation therapy has been associated with increased intestinal complications in some adult cancer patients, but this has not been reported in children undergoing cancer surgery., Methods: Postoperative SBO occurred in 131 of 1,910 children (6.9%) enrolled in the Third National Wilms' Tumor Study (NWTS). The etiology of the SBO was bowel adhesions in 104 cases, intussusception in 17, internal hernia in 2, and uncertain in the remaining 8 children., Results: The factors found to be of potential importance in explaining the incidence of SBO were higher local tumor stage, extrarenal intravascular involvement, and en bloc resection of other organs at the time of nephrectomy. The incidence of postoperative SBO was not increased in children who received postoperative radiation therapy., Conclusions: Although the overall incidence of SBO after nephrectomy for Wilms' tumor is comparable to that after other major abdominal operations in children, it can be responsible for significant morbidity. There were 4 children among the 1,910 patients with infectious complications of SBO, which contributed to their death.

Background: Primary surgical management of Wilms tumor with intravascular tumor extension is associated with significant morbidity. Retrospective analysis of a group of Wilms tumor patients with intravascular tumor extension treated with preoperative therapy was performed to evaluate the feasibility of this approach., Methods: Thirty children with caval (15 patients) or atrial (15 patients) extension of Wilms tumor who received preoperative therapy were reviewed. Patients were classified according to the stage of the disease as follows: (1) Stage III, 7 patients; (2) Stage IV, 18 patients; and (3) Stage V, 5 patients. The histologic type was favorable in 20 patients, anaplastic Wilms tumor in 7, and unknown in 3. All of the children received preoperative chemotherapy, ranging from 1-16 weeks with an average of 7.2 weeks., Results: One child died of progressive disease and another from toxicity before surgery. In 23 patients, a decrease in the size of the intravascular extension was noted preoperatively, without tumor embolization in any case. There was complete resolution of tumor thrombus in seven patients. Two-year survival for the entire group is 70%, with a median follow-up period of 21 months., Conclusions: The use of preoperative therapy is appropriate in selected patients with extensive intravascular tumor involvement. Surgical management was facilitated in the majority of cases due to shrinkage of the primary tumor and reduction in the size of the tumor thrombus.

Incidental appendectomy is frequently performed during nephrectomy for Wilms' tumor. The rationale for this approach is that the onset of appendicitis during a period of neutropenia after chemotherapy may cause increased morbidity. Additionally, children receiving chemotherapy or radiation therapy, or both, may experience abdominal pain, producing a diagnostic dilemma if the appendix is still present. We reviewed the charts of 1,910 children enrolled in the Third National Wilms' Tumor Study who underwent primary nephrectomy. Four hundred and fifty-five patients (23.8 percent) had appendectomy at the time of nephrectomy. An inversion appendectomy was performed upon 188 patients, and the appendix was excised in 267. Infectious complications and postoperative intestinal obstructions occurred with equal frequency in children who did or did not undergo appendectomy. The median follow-up period for the entire group of patients was 5.1 years. Only three of the 1,455 children (0.2 percent) who did not have an incidental appendectomy had appendicitis at two, seven and ten months after nephrectomy. None of the three children experienced any perioperative morbidity after appendectomy. These data suggest that incidental removal of the appendix is not essential for children undergoing removal of a Wilms' tumor.

We reviewed the charts of 1,910 children enrolled in the Third National Wilms' Tumor Study who underwent primary nephrectomy. Four hundred and ninety-five surgical complications occurred in 379 children (19.8 percent). Patients with inoperable tumors, bilateral renal tumors, peroperative therapy and those who refused treatment were excluded from this review. The most common complication was intestinal obstruction, which occurred in 132 patients (6.9 percent). This was followed by extensive intraoperative hemorrhage (112 patients), defined as blood loss exceeding 50 milliliters per kilogram of body weight. Intraoperative injuries to other visceral organs (including intestine, liver and spleen) occurred in 21 children and extensive vascular injuries were reported in 27 patients. There were nine deaths attributed to surgical complications (0.5 percent), only one of which was intraoperative. Survival of patients with complications was similar to patients without complications when stratified by histologic study and stage. Factors associated with the development of surgical complications included advanced local tumor stage at diagnosis, intravascular tumor extension and resection of other visceral organs at the time of nephrectomy. Complete removal of the tumor is important, but not at the expense of radical removal of adjacent structures, because of gross appearances at operation. They are often not invaded by the tumor, but rather are compressed, distorted or adherent without tumor infiltration. Identification of these groups will aid the surgeon in choosing the appropriate management for these patients at high risk. When initial exploration and precise surgical staging indicate that only a formidable operation will achieve total excision, shrinkage of the tumor with selective use of chemotherapy or radiotherapy, or both, may facilitate removal and decrease surgical morbidity. Preoperative therapy may also be the preferred approach for children with extensive intravascular tumor.

Extrarenal Wilms' tumor is extremely rare and occurs predominantly in children. Eight cases of extrarenal Wilms' tumor were reported to the National Wilms' Tumor Study from 1980 to 1986. Patients were followed in the study and not randomized to a particular treatment protocol. Seven patients had a favorable histology. One tumor located in the sacrococcygeal region showed immature teratoma with nephroblastic tissue. The embryogenesis of extrarenal Wilms' tumor is controversial; however, tumor containing teratomatous elements most likely represents a different embryologic origin and, therefore, should be classified separately. All eight patients were treated with operative excision and chemotherapy. Seven of the eight patients were disease-free with a mean follow-up of 34.3 months. It can be inferred from this small group of patients that the prognosis is comparable to intrarenal Wilms' tumor in the National Wilms' Tumor Study.

Iatrogenic injury to the aorta or its major branches during nephrectomy for Wilms' tumor in children is rarely reported but may be more common than is currently acknowledged. We identified four patients with ligation of the superior mesenteric artery (SMA) that occurred during nephrectomy for nephroblastoma and another child in whom SMA thrombosis developed postoperatively. All of the tumors were on the left side. Interruption of the SMA was recognized intraoperatively in all four children, and primary repair was done. In three patients, appearance of the bowel remained normal before repair of the injury. Three of the arteries were repaired by primary reanastomosis, and one was joined with an interpositioned hypogastric artery graft. None of these patients had gastrointestinal complications postoperatively. In the fifth patient, SMA thrombosis developed after repair of an aortic tear during nephrectomy. This patient required subsequent small bowel resection for bowel infarction and died in the perioperative period. Every surgeon treating children with Wilms' tumor should be aware of the possibly distorted vascular anatomy and take precautions to avoid such a significant injury. Attempts at early ligation of the vessels may not be justified until the renal vasculature is clearly identified.

A comprehensive statistical analysis of relapses to lung and abdomen and of deaths due to tumor that occurred among 1466 patients with nonmetastatic, favorable histology (FH) Wilms' tumor entered on the Third National Wilms' Tumor Study (NWTS-3) was undertaken. In addition to lymph node involvement, age at diagnosis and tumor size as measured by the weight of the excised specimen were the most important determinants of outcome. The effects of tumor size were most apparent for patients with localized (Stage I) disease; age effects were found for patients with regional (Stage II or III) disease. The presence of microscopic tumor in the margin of surgical resection was an independent predictor of abdominal recurrence and death in the latter group. Although the report of the surgeon of diffuse soilage of the peritoneal cavity from tumor spilled or cut across in the course of nephrectomy was highly correlated with outcome, it was not possible to establish an independent prognostic role for such a finding after adjustment for the aforementioned factors. Patients with intralobar nephrogenic rests (ILNR) had a favorable survival outlook even after accounting for their generally younger ages and lower stages.

The Cohen technique for antireflux surgery is well established and provides excellent results. A major drawback with this technique is the difficulty in catheterizing the ureter postoperatively. We describe different strategies that can be used to catheterize the ureter successfully.

The long-term survival of patients with synchronous bilateral Wilms tumor is not well defined. Retrospective review of 185 patients registered with the National Wilms Tumor Study from January 1974 to July 1986 with stage V tumors suggests that the long-term outcome remains good. Over-all survival is 83%, 73% and 70% at 2, 5 and 10 years, respectively. Unfavorable histology, age at diagnosis and the most advanced stage of the individual tumors remain the most important prognostic variables. No significant difference in survival was noted between patients undergoing initial surgical resection of the tumor and those managed with initial tumor biopsy followed by chemotherapy with or without radiotherapy and subsequent surgical resection. Survival does not appear to be compromised by attempting to conserve native renal function with renal-sparing operations.

A follow-up study of 224 male patients from Rochester, Minnesota, diagnosed with cryptorchidism during the period from 1935 through 1974 was conducted to assess the frequency of subsequent testicular neoplasms. Age-specific rates for testicular neoplasm in this community were used to estimate an expected number for comparison with that observed. Two malignant testicular neoplasms have occurred in this cohort; thus, the standardized morbidity ratio was 11.4 (95% confidence interval, 1.4 to 41.1). Of the 158 patients born in the community, 11% were known to have birth weights of less than 2,500 g, approximately twice the expected occurrence. Increased risks were also noted for inguinal hernia, hypospadias, Down's syndrome, cleft lip or palate (or both), clubfoot, and congenital heart disease, for which the standardized morbidity ratios were 3.6, 4.7, 17.8, 11.3, 11.7, and 2.7, respectively. The increased risk for malignant neoplasms observed in this cohort of patients with cryptorchidism is consistent with that found in earlier studies.

Intraoperative radiotherapy with variable energy electrons has been used as a supplemental boost to treat 6 children with locally advanced retroperitoneal malignancies. Of the patients 4 had treatment-related injuries to portions of the urinary tract within the intraoperative and external radiation fields. Three patients had significant renal impairment requiring surgical correction. The pathogenesis and management of treatment-induced injury to the urinary tract are discussed.

The records of children enrolled in the National Wilms' Tumor Study-3 who had Wilms' tumor and thrombosis into the renal vein were reviewed. There were 164 patients with gross involvement of the extrarenal vein and 47 patients with microscopic involvement, an incidence of 11.3% (211 of 1,865 evaluable patients). Two-year survival rates were 90, 79 and 72% for stages II, III and IV, respectively. The most important predictors of survival were histologic pattern and stage. Complete en bloc excision of the tumor and thrombus continues to be the most effective initial management.

Simple renal cysts in childhood are uncommon and often are confused with duplication anomalies or renal malignancy. A review of 20 children with simple renal cysts at our institution has documented the spectrum of clinical presentations and radiographic findings of this unusual renal mass. Surgical exploration and marsupialization of the cyst wall were done in 12 patients, 4 underwent nephrectomy earlier in our series and 4 have been followed conservatively by ultrasonography or computerized tomography scanning. Although equivocal cases necessitate surgical intervention, we advocate conservative therapy with diagnostic ultrasonography with or without cyst puncture in asymptomatic and normotensive children with simple renal cysts.

Recent technological advances in prenatal ultrasonography have been responsible for the detection of a variety of congenital abnormalities in utero. The widespread use of this study during pregnancy has led to an increased recognition of foetal hydronephrosis. It has been postulated that unrelieved, high-grade urinary obstruction in utero may produce progressive renal damage. This supposition has resulted in the development of techniques for foetal intervention in attempts to correct obstructive uropathy and arrest renal dysplasia. Herein we review the techniques for assessment of foetal renal function and treatment options available for the foetus with hydronephrosis in utero. Most foetuses with hydronephrosis have dilated, low pressure systems with adequate urinary output and amniotic fluid volume and can be followed safely to term delivery. The accumulated experience to date indicates that the foetus with congenital hydronephrosis detected in utero should be managed expectantly with serial ultrasound examinations after delivery to establish an accurate diagnosis and to evaluate the status of the contralateral kidney. Functional tests after birth, such as renal scans and excretory urography, are mandatory to establish the proper diagnosis and assess the need for operative intervention.

Single-stage reconstruction of female epispadias produced gratifying functional and cosmetic results and complete urinary control in 10 out of 12 patients (83%). Factors critical to the achievement of complete continence in these children include deferment of surgery until the patient is at least 3 years of age and a well-developed bladder with adequate capacity and musculature. Because complete continence is often delayed several weeks to months postoperatively in these children, expectant management and observation are necessary to allow enlargement of bladder capacity, which enhances successful results.

Vesical neck reconstruction was performed in 50 male and 12 female patients with the epispadias-exstrophy complex. Of these patients 45 had epispadias and 17 had classical exstrophy. Patient age ranged from 3 to 27 years, with a mean age of 12.6 years. Followup after vesical neck reconstruction averaged 11.6 years. Of the 45 patients with epispadias 35 (78 per cent) and of the 17 with bladder exstrophy 13 (76 per cent) are continent, for an over-all continence rate of 77 per cent. An adequate bladder capacity was one of the most important determinants of continence. In 11 patients with a small capacity or poorly compliant bladder augmentation cystoplasty was combined with vesical neck reconstruction to increase vesical capacity and to produce complete urinary continence.

Ureterocalicostomy was performed in 21 patients for ureteropelvic junction obstruction. Ten patients had failed pyeloplasty, 10 had anomalies of renal fusion, ascent or rotation in conjunction with ureteropelvic junction obstruction and 1 had failed prior ureterocalicostomy. Patient age at operation ranged from 6 months to 17 years and averaged 9 years. Of the 21 patients in this series 19 had excellent results after ureterocalicostomy with decrease or total elimination of hydronephrosis. There were 2 postoperative complications in 2 patients: prolonged anastomotic urinary leakage in 1 and a Candida perinephric abscess in 1. Ureterocalicostomy should be considered in selected patients with previous unsuccessful pyeloplasty, ureteropelvic junction obstruction associated with anomalies of renal fusion, rotation or ascent, an intrarenal pelvis or a short ureter. Although endopyelotomy or ureteropelvic junction dilation should be considered in patients who fail pyeloplasty, ureterocalicostomy continues to be a reliable salvage procedure in these patients; it bypasses extensive peripelvic scarring, provides for dependent drainage and compensates for lack of adequate ureteral length.

Our experience with children who require supravesical diversion in the face of a compromised urinary tract indicates that in selected cases transureteroureterostomy with cutaneous ureterostomy will lead to a stabilized urinary tract that will be free from infection and postoperative complications.

The karyotype 46,XX/69,XXY was found in a 13-year-old mentally subnormal patient with club feet, strabismus, eunuchoid habitus, small penis, midscrotal urethrovaginal opening, small descended left testis, and small undescended right testis; no ovarian tissue could be found at laparotomy. Triploid:diploid cell ratios were 60:40 and 4:96 in skin fibroblasts and curculating lymphocytes, respectively. In the triploid line, two of the no. 13 chromosomes had unusually large satellites and one of the no. 22 chromosomes had a brightly fluorescent zone on its short arms. The patient's father was heterozygous for both these autosomal markers; the mother carried neither marker. This, together with the single Y, indicated that the extra haploid set was derived from the father. Of several possible mechanisms, we favor the suggestion that double fertilization occurred; one sperm nucleus immediately fused with the egg nucleus producing the diploid line; the second sperm nucleus was incorporated later into one of the two cells resulting from the first division of the zygote, producing the triploid line.

A spectrum of roentgenographically dysmorphic kidneys in 8 children is described. The clinical significance of these lesions lies in the need to differentiate them from renal malignancy and the more usual forms of hydronephrosis. It also reflects their effects on renal function, superimposed infection and poor drainage. It would appear that infundibulopelvic dysmorphism is a link in the clinical spectrum extending from cystic dysplasia of the kidney to hydronephrosis.

In summary, an acute disorder of the scrotum in a child without previous urologic history must be viewed as torsion until proved otherwise by surgical exploration. No deleterious effects have been reported from such an approach, which requires a minimal period of hospitalization and little discomfort to the child. The shorter the interval between the onset of symptoms and surgical exploration, the greater the likelihood that the testicle will be viable later. The diagnosis of epididymo-orchitis in a healthy child without a history of urologic abnormality or evidence of lower urinary tract infection has no place in the differential diagnosis of the child with the painful scrotum.

This report describes an infant with bilateral diffuse nephroblastomatosis, illustrating the typical clinical and pathologic characteristics that allow it to be differentiated from true bilateral Wilms' tumor.

An operation was required in 78 children with vesical diverticula in the absence of bladder outlet obstruction. Virtually all diverticula were located in juxtaposition to the ureteral orifice and were associated with vesicoureteral reflux in all but 6 of 89 diverticulum-ureteral units. Upper tract changes were seen on excretory urography in 78 per cent of the refluxing units. The surgical procedure of choice was diverticulectomy and ureteroneocystostomy, which yielded a high degree of success. Diverticulectomy alone and vesical neck revision were rarely indicated.

We reviewed 103 patients with exstrophy of the bladder. Followup was more than 15 years in 51 patients. Initial management consisted of primary bladder closure in 32 patients and urinary diversion in 71. Urinary continence, renal function, urinary tract infections and development of malignant lesions were evaluated. Factors leading to success or failure were analyzed. Although the highest continence rate (83 per cent) was achieved in 40 patients with ureterosigmoidostomy, renal functional deterioration was highest in this group, with 70 per cent of the evaluable renal units being abnormal. Furthermore, 10 per cent of this group died of renal failure and 23 per cent lost 1 kidney each. In 26 patients with an ileal conduit 69 per cent of the renal units evaluated were abnormal. Only 1 patient died of renal failure but 27 per cent lost 1 kidney each. Of 32 patients with primary bladder closure 31 had preservation of renal function. Twelve of 18 patients (67 per cent) in whom vesical neck reconstruction had been completed had total urinary continence and 3 (17 per cent) had partial continence. The incidence of significant urinary tract infections was highest in the ureterosigmoidostomy group (63 per cent) and next to the highest in the ileal conduit group (48 per cent). Malignant lesions developed in 8 patients (8 per cent).

In 12 children with membranous urethral strictures transpubic urethroplasty has been performed successfully with minimal morbidity and no mortality. Of the 12 patients 8 are completely continent after transpubic urethroplasty, 2 have partial continence and 2 are incontinent. Wedge resection of the pubic symphysis afforded satisfactory exposure to the prostatic urethra, and none of the 12 patients experienced orthopedic instability, abnormal gaits or pelvic girdle pain. In 3 patients there was loss of erectile function after pelvic injury, which reflects the significant risk of impotence after crush injury to the pelvis with rupture of the posterior urethra.

The chromosomes of undescended tests from 13 boys were analyzed. In 12 of these patients the peripheral blood chromosomes were examined and in 7 the chromosomes of normal skin cultures were examined. In addition to standard techniques the chromosomes also were analyzed by G and Q-banding methods. We were unable to detect any significant abnormalities of either chromosome number of structure in the undescended testes. Thus, our investigation failed to demonstrate a chromosomal lesions that may be responsible for maldescent of the testes. Our investigation did not reveal any changes in the chromosomes of these undescended testes that could be interpreted as indicating a potentially malignant state.

This case report and reports from the literature emphasize that the so-called adult form of polycystic renal disease can be diagnosed in infancy. The tern "adult form of polycystic disease" probably should be abandoned and the disease considered the autosomal dominant form of polycystic kidney disease.

The records of 87 children with bladder exstrophy seen between 1912 and 1935 are reviewed. We believe that ureterosigmoidostomy with cystectomy can provide a satisfactory solution to the problem of bladder exstrophy. Furthermore, ureterosigmoidostomy appears suitable from the standpoint of preservation of upper tracts and provides a socially acceptable solution of the question of disposal of the diverted urine.

The management of 78 children with upper urinary calculi is described. Boys outnumbered girls by a ratio of 2 to 1. Two-thirds of the patients had identifiable metabolic causes, while the remaining third had infected renal lithiasis. In this latter group, all patients had had multiple urologic procedures, urinary infection, and stasis with diversionary and indwelling drainage devices. Contrary to earlier views, idiopathic renal lithiasis with or without hypercalciuria was the most common metabolic form of nephrolithiasis in children. Sixty-seven patients (86 per cent) were followed for an average of 7 1/2 years. With appropriate therapy, stone disease became inactive in 70 per cent of the children. The remaining 30 per cent continued with active disease--5 died of renal failure and 1 has received a renal allograft. Stone formation may be regarded as a solitary complication or one of several manifestations of a large number of underlying disorders. Along with a thorough search for etiologic factors there must be an equally aggressive therapeutic effort. Because the disease is ofter sporadic, careful long-term followup of the patients with active as well as those with inactive stone disease is mandatory.

In male patients who undergo surgery for epispadias the major postoperative complaint often concerns the cosmetic appearance of the penis and the failure to correct the dorsal chordee rather than the urinary incontinence. In the past straightening and lengthening of the penis were not given adequate consideration, and penile elongation was limited to release of dorsal skin chordee only. Recently, however, additional penile length has been achieved by partial mobilization of the crura of the corpora cavernosa from the pubic rami. In a series of 70 patients penile lengthening and urethroplasty were performed separately or were combined as a 1-stage procedure with full thickness grafts or preputial flaps. The recent use of hair-bearing groin flaps has provided excellent skin coverage for the large dorsal defect at the penoabdominal angle. A satisfactory cosmetic appearance of the external genitalia, with a straight penis angulated downward in the standing position, was achieved in 67 per cent of the patients. Normal erectile function was preserved in all patients, 80 per cent of whom have had a straight penis with erection and satisfactory intercourse. Of the patients 29 are married and 19 have fathered children. Meticulous attention to the technical aspects of reconstructive surgery usually can result in a gratifying cosmetic appearance, normal genital function and preservation of fertility potential in most patients.

We evaluated 36 patients for müllerian duct remnants that were manifest in several forms. The most common type was an enlarged prostatic utricle communicating with the urethra in 22 younger patients, which was associated with hypospadias or intersex disorders in more than 90 per cent of the cases. A cystic müllerian duct remnant was the other configuration noted in 14 older patients who had normal external genitalia and often presented with a rectal mass. The diagnostic evaluation consists of voiding cystourethrography, retrograde urethrography and cystoscopy. Other useful imaging techniques are ultrasonography and computerized tomography. Careful delineation of the anatomy of the lower urinary and genital tracts will help to plan surgical therapy. Suprapubic excision was the most frequent operation and it was successful in all 18 patients in whom it was performed.

Surgical management of 100 children with abnormalities related to ureteral duplication is reviewed. In general, separation of the distal portions of the double ureters is avoided, at least during the initial procedure, because this maneuver represents a significant risk to the blood supply and hence to the integrity of the remaining ipsilateral ureter. Complete ureteral duplication with vesicoureteral reflux is best managed initially by reimplantation of the duplex unit. On the other hand, upper segment heminephrectomy with subtotal ureterectomy is the initial treatment of choice for ureters ending ectopically with or without ureterocele.

Hypospadias represents a spectrum of disorders known as androgen insensitivity syndromes, which are characterized by a nonresponsiveness of an androgen target tissue to the steroid. The basic defect in this disorder is unknown. This article presents results of studies of the androgen receptor with use of human foreskin from normal neonates undergoing circumcision and from patients undergoing urethroplasty necessitated by hypospadias. It is speculated that a defect in receptor level or function might explain this disorder. The androgen receptors from both normal and hypospadiac tissue were stabilized with a buffer containing 50 mM Tris hydrochloride, 1 mM ethylenediaminetetraacetic acid, 12 mM monothioglycerol, 10% (v/v) glycerol, and 10 mM sodium molybdate plus 1 mM phenylmethylsulfonylfluoride (pH 7.5). The receptors were characterized by Scatchard analysis, competitive binding assays, sedimentation analysis, molecular sieve chromatography, and isoelectric focusing techniques. Specific R 1881 binding to soluble proteins from normal human foreskin was shown to be high affinity (dissociation constant = 0.5 nM), low capacity (6 fmol/mg of protein), and steroid specific. Sedimentation analysis by sucrose density gradients demonstrated the 8S-to-4S shift from low- to high-salt treatments. The receptor from the foreskins of hypospadiac patients also contained androgen receptor in a concentration similar to that in the foreskins of normal persons. The affinity for steroid binding and sedimentation profiles or sucrose gradients of the androgen receptor between normal persons and hypospadiac patients were also similar. However, the androgen receptor from hypospadiac patients displayed different patterns of molecular sieve chromatography from those displayed by the receptor of normal persons. The reason for this difference is not known but may reflect a defective receptor and explain the basic mechanism of this disorder.

Between 1946 and 1980, 54 patients with epispadias and total urinary incontinence were evaluated at our institution. Factors critical to the achievement of complete continence include deferral of surgery until at least 3 yr of age, the patient having a well-developed bladder with adequate capacity and musculature, and maturation of the prostate at puberty in males. Complete continence was obtained in 9 of 11 female patients (82%). Fourteen of 35 male patients obtained complete urinary continence in the initial postoperative period. Complete urinary control was delayed until puberty in 11 additional patients, for an overall success rate of 25 of 35 males (71%). The significant increase in continence with the onset of puberty in males demonstrates the importance of expectant management of the child's progress in relation to the potential contribution of prostatic maturation in the development of urinary control.

We reviewed the records of 77 children enrolled in the National Wilms Tumor Study-3 who had involvement of the inferior vena cava with tumor thrombus. None of these patients received preoperative chemotherapy or radiotherapy. Ultrasonography and inferior venacavography were more helpful than computerized tomography in the preoperative diagnosis. The median followup for the group was 2.7 years. The 3-year survival rates for patients with intracaval involvement were determined by stage (88, 89 and 62 per cent for stages II, III and IV, respectively). The most important prognostic factor was histological type. The level of vena caval involvement had no effect on survival; all 16 patients with atrial involvement survived 3 or more years. We continue to recommend appropriate surgical excision of the tumor and thrombus when it is technically feasible.

In 1980 the Section on Urology of the American Academy of Pediatrics established a registry of prepubertal testicular tumors. A total of 181 yolk sac tumors has been reported to the registry. Complete followup is available for 154 of these 181 patients. Most of the patients presented with low stage disease. Radical orchiectomy without adjunctive retroperitoneal lymphadenectomy, chemotherapy or radiotherapy was sufficient therapy for most stage 1 cancer patients. alpha-Fetoprotein is a reliable marker for this tumor. Chest x-rays and abdominal computerized tomography are reliable for staging. When metastases appeared they did so within 14 months of presentation so that 2 years of followup after any evidence of disease seems to be adequate. Approximately two-thirds of the patients with metastases were salvaged by chemotherapy, radiotherapy and/or an operation.

The author, who's considerable personal experience is based on 411 cases of hypospadias in children operated at the Mayo Clinic (Rochester, Minnesota, U.S.A.), emphasizes: 1 The importance of a strict assessment of results, considering not only fistulas and apparent cosmetic results, but also stenoses of urethroplasty, sacculations, meatic stenoses and stricture, and insufficient correction of the penile curve. 2 The capacity to choose, in each particular case, between several procedures. 3 The unquestionable security offered by correction in three separate stages: straightening of the penis, urethroplasty by burrowing in the scrotum then resurfacing. 4 The difficulty of re-operation which leads to 60% of complications and needs several surgical stages. In short, you can't improvise the treatment of hypospadias.

Among renal stone-forming children 6 per cent suffer from primary hyperparathyroidism. Urologic complications were noted in 7 of 9 children (78 per cent) with surgically proved hyperparathyroidism seen in a 24-year period. These included nephrolithiasis and nephrocalcinosis (6), defective urinary concentration or acidification (5) and mild impairment of renal function (3). All children responded favorably to parathyroidectomy, which should be performed before treatment of stone disease is undertaken. The nature of urologic manifestations of hyperparathyroidism and their management are discussed.

Postoperative renal growth was determined in 22 patients with unilateral atrophic kidney and vesicoureteral reflux. Atrophic kidney growth was compared to the contralateral kidney and growth tables. Significant growth of the atrophic kidney was observed in 68 per cent of the patients after reimplantation. Ureteroneocystostomy enhances growth in atrophic kidneys and is important in salvaging renal function.

Despite institutional variation in the treatment of synchronous bilateral Wilms tumor, a 76 per cent over-all 3-year survival rate has been achieved. We reviewed 145 patients with stage V disease (synchronous bilateral Wilms tumor) registered with National Wilms Tumor Studies 2 and 3. The majority of the patients (94, or 65 per cent) underwent initial surgical resection followed by chemotherapy with or without radiation, with a 3-year survival rate of 82 per cent. However, survival of 45 patients who underwent initial biopsy, postoperative chemotherapy and second-look surgery was 57 per cent at 3 years, a result not significantly different statistically from the 82 per cent figure. Only 38 per cent of the patients had all tumor resected at 1 or more operations. Therefore, emphasis is being placed on chemotherapy as the predominant treatment modality for this disease. Unfavorable histology was found in 10 per cent of the patients and 6 patients (4 per cent) had discordant histology, that is unfavorable histology on 1 side with contralateral favorable histology. Better prognostic factors were patient age less than 3 years at diagnosis, lower stage of the most advanced lesions, favorable histology and negative nodal involvement. Currently, routine bilateral biopsy of tumors is recommended, followed by chemotherapy according to the stage of the most advanced lesion. Impressive shrinkage of tumor can be expected with the hope that surgery will allow renal preservation (excisional biopsy or partial nephrectomy) rather than nephrectomy.

Seven boys with fracture(s) of the bony pelvis and associated partial or complete rupture of the posterior urethra were managed by the time-honored technique of early suprapubic cystostomy and concomitant primary realignment of the urethra over a catheter. Of the 4 children who had a functionally significant urethral stricture 3 were cured within a few months by 1 or 2 simple urethral dilatations and 1 by subsequent transperineal lysis of the angulated urethra from its surrounding fibrous tissue. Followup data for 8 to 22 years (mean 14 years) indicate that all 7 patients void with an excellent stream and are continent, free of infection and potent. In fact, 3 of the 4 married boys have fathered children.

From 1970 through 1975, 198 children with hypospadias were operated upon at our clinic. Three basic types of primary urethroplasty were used: 1) a modified 2-stage Cecil urethroplasty after separate straightening, 2) a modified 1-stage Denis Browne procedure after prior correction of chordee and 3) a 1-stage combined repair of hypospadias and chordee. A comparative review of early complications is presented.

Vesicoureteral reflux was detected in 200 adults seen in a 4 year period. In group 1 (no history of urinary tract infection and negative urine cultures), all patients had abnormalities on excretory urograms that were suggestive of reflux. In patients with a history of recurrent urinary tract infection (groups 2 and 3), the excretory urograms showed an abnormality in 79 per cent of those with negative cultures and in 83 per cent of those with positive cultures. Thus, reflux should be suspected in patients with a history of recurrent urinary tract infection even if infection cannot be documented with urine culture. Urographic evidence of renal parenchymal scarring, ureteral dilatation, or mucosal striations should also suggest the possibility of vesicoureteral reflux even when the patient may have no urinary tract symptoms. Voiding cystourethrography should be performed in all adults with these findings to exclude the possibility of vesicoureteral reflux.