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We previously reported that chronic urinary diversion through intestinal segments may have adverse effects on bone and mineral metabolism. This study examined the long-term health of patients managed by urinary diversion (94% by ileal conduit) for neuropathic bladders secondary to myelomeningocele defects and compared them to a control population of myelomeningocele patients managed by intermittent catheterization. Of the patients 93 were studied by personal interview, chart review, morphometric analysis, serum studies and dual-photon bone density determination. Average followup was 23 +/- 6 years in the urinary diversion group and 17 +/- 5 years in the intermittent catheterization group. Fractures occurred in 40% of the patients in both groups. Patients with a urinary diversion had an increased need for surgery to correct spinal curvature (57% versus 40%) and a significantly increased incidence of complications resulting from orthopedic procedures (17% versus 3%, p less than or equal to 0.05). There was also an adverse effect on renal function. The urinary diversion group had an increased incidence of radiographic renal deterioration (57% versus 8%, p less than 0.001), nephrolithiasis (43% versus 2%, p less than 0.001), pyelonephritis (60% versus 21%, p less than 0.001) and intermittent metabolic acidosis (20% versus 5%, p = 0.05). Surgery was required in 37% for stomal complications and in 17% for ureterointestinal stricture. Linear growth was adversely affected by urinary diversion. Patients with urinary diversion had decreased lengths for all morphometric parameters and a greater percentage of them were at or below the 10th percentile standards. Serum electrolytes, liver function studies, vitamin D3 and parahormone showed no differences in the 2 groups. No patient had a significant metabolic acidosis at the time of study. Bone densities were significantly diminished in both groups and not significantly different. This study strongly suggests that urinary diversion through intestinal segments is associated with adverse effects on bone health.

Answering the need for a simple, nonenteric method of increasing bladder capacity, this article offers a new laparoscopic procedure for autoaugmentation of the bladder in children. An outline of the technical design is given. Experience with seven children is delineated. Postoperative voiding control and function capacity were greatly improved; this procedure is judged worthy of addition to the surgical armamentarium.

Using a previously reported rat model, we examined the effects of intestinal perfusion with hemiacidrin on calcium and magnesium homeostasis, and inspected the intestinal segments for histological alterations following exposure to the solution. Intestinal irrigation with hemiacidrin resulted in a significant increase in serum magnesium. Urinary excretion rates of calcium increased 8-fold and magnesium excretion rates increased 5-fold over control values. Hemiacidrin appeared to have detrimental effects on the integrity of the intestinal mucosa, and irrigation should be done with caution in patients whose urinary tract has been reconstructed with intestinal segments.

The testicle is a prime initial target for infiltration during relapse in male children with acute lymphocytic leukemia. Herein we report our experience with management of this entity in 8 children. It is stressed that a biopsy is essential to the diagnosis. The differential diagnosis is usually straightforward. One is admonished not to make presumptive diagnosis by palpation. Orchiectomy is unwarranted. The treatment of choice is testicular radiation with 2,000 rads in ten fractions in a twelve-day course plus reinstitution of high-dose adjunctive chemotherapy in those children off chemotherapy, or reinduction therapy for children who relapse while still on chemotherapy. Prognosis of male children who undergo a bout of testicular infiltration is guarded.

Our group has undertaken the determination of serum levels of beta-2-microglobulin in 250 patients. We have compared this diagnostic entity to radioisotopic glomerular filtration rate, serum creatinine, and blood urea nitrogen. We have also analyzed the cost effectiveness of each of these diagnostic entities and combinations thereof. We conclude that beta-2-microglobulin is a simple test to perform; it is specific, sensitive, and accurate. Its cost effectiveness is excellent. We advocate its wide clinical usage.

Eight previously unreported cases of true hermaphroditism are presented, and their basic characteristics are described and discussed with regard to diagnosis and management. Two basic groups are identified: those diagnosed prior to establishment of gender role and those diagnosed at a later date. The latter group should benefit from therapy designed to enhance and maintain their sex of rearing, while the former group should be reconstructed according to functional considerations first and fertility considerations second. Diagnostic criteria are evaluated, and the value of the exploratory laparotomy is stressed.

We believe that the confusion regarding abnormal renal development could be reduced by more precise terminology. Therefore, we suggest precise definitions for dysgenesis, hypoplasia, dysplasia, hypodysplasia, aplasia and agenesis of the kidney. We suggest the term reflux nephropathy be a generic label for any instance of abnormal renal morphology (gross or microscopic) associated with vesicoureteral reflux. Hypoplasia and hypodysplasia can be subclassified on the basis of associated urological criteria.There have been many previous attempts to classify cystic disease of the kidney but none has been accepted collectively by pathologists, urologists, nephrologists and radiologists. On the basis of known patterns of inheritance, a classification is outlined in which renal cystic disease is divided into 2 major groups: genetic and nongenetic. Each entity is discussed.

Rhabdomyosarcoma is the common malignant soft tissue tumor of childhood. Twelve of 67 cases recently reviewed from this institution arose in the bladder or prostate (6 are surviving). Until recently and regardless of the primary site, the reports of successful treatment of this aggressive embryonal neoplasm have been limited to very small series or to isolated cases managed by radical mutilative surgery. Now, combined therapy, as has proven so effective in Wilms' tumor, and utilizing “reasonable surgery” (excision of all gross tumor), tolerable x-ray therapy (Cobalt 60 2-4000 R according to age in 3–4 weeks), and repeated courses of Actinomycin D and Vincristine (9-week cycles for at least a year), is recommended. At Ohio State University the curability rate in all types of rhabdomyosarcoma including all bladder and prostatic lesions has improved spectacularly from 14% prior to 1967 to 71% (corrected survival by life table method) in the last 38 cases treated.

Extended biopsy followup of 17 relatively young men with resectable prostatic cancer treated by external supervoltage radiotherapy is presented. Although six of the eight surviving patients now have negative biopsies, the over- all survival rate and the over-all incidence of negative biopsies are not so encouraging, with only 35 percent rendered free of tumor. Thus, the role of radiotherapy in resectable prostatic carcinoma should be critically scrutinized. More accurate staging is needed. Hopefully, more sophisticated studies of diagnostic and prognostic value will be available in the near future. (auth)

Urethrocutaneous fistulas are one of the major causes of morbidity following hypospadias repair. No matter how well designed the initial hypospadias procedure is, how gently the tissues are handled nor how expertly the procedure is done, fistulas continue to occur at an unacceptably high rate. We review our experience with hypospadias repair, with emphasis directed to the management of urethrocutaneous fistulas. Some tiny fistulas will close on prolonged catheter drainage but our experience shows that most need a definitive closure procedure. Although many procedures have been described for the closure of urethrocutaneous fistulas we advocate use of an advancement flap. Our success rate has been excellent with this procedure. Particular attention to well established techniques of hypospadias repair will decrease the incidence of urethrocutaneous fistulas.

The ureteral pressure/flow perfusion study has lately been repopularized and its use demonstrated in many forms of suspected obstructive uropathy. The present study was undertaken to compare the ureteral pressure/flow perfusion study with a radioisotopic method of investigating ureteral obstruction. The 2 methods were evaluated with a mongrel canine laboratory model. Both diagnostic modalities were employed concomitantly on intact renoureteral units; first with the ureter unobstructed, then after submission to varying degrees of ureteral occlusion. Analysis of data from these studies shows that the curve pattern produced by the computer generated 99m Tc-DTPA renogram is as accurate as ureteral pressure/flow perfusion study in determining the presence and degree of ureteral obstruction.

Ninety-eight children have been treated with clean intermittent catheterization (CIC) over a five-year period. Follow-up in 73 of these is presented herin. The complication rate encountered with the technique is low (7 per cent major complications). Only 21 per cent of the patients were able to maintain persistently sterile urine, although no new bouts of pyelonephritis were encountered in the group since our report of three years ago. Six of 44 (14 per cent) refluxing renal units stopped refluxing on CIC, but in two units reflux de novo developed while on treatment. Most patients (79 per cent) demonstrated stable upper tracts on CIC, while 14 per cent showed increased calicectasis with 7 per cent showing improvement. Thirty-one of the patients were less than three years of age when begun on clean intermittent catheterization. We recommend CIC as the treatment of choice in neurogenic vesical dysfunction where total bladder emptying is a problem and advise that it be started as early as possible.

Since June 1, 1971 patients undergoing evaluation for hypertension have been evaluated with rapid sequence excretory urography, abdominal aortography and selective renal arteriography. Renal venous assays have been done in selected patients. Through July 31, 1974, 812 arteriographic studies in new hypertensive patients have been done. In 6 of these patients renal tumors were discovered by arteriography only and were not suggested by symptoms, urinalysis nor diagnosed on hypertensive excretory urography. This high incidence surprised us and we hope others will review their arteriographic series of hypertensive patients.

Problems encountered in attempts to construct a normal vaginal orifice in girls with congenital adrenal hyperplasia have met with great difficulty. Many procedures are complicated by failure to achieve adequate patency at the time of operation and/or by postoperative stenosis. The usual method of direct posterior Y-V vaginoplasty has been altered by bringing the V flap in from the side of the perineum, between the inferior aspect of the labium majus and the median perineal raphe. Thereby, a thicker, larger, more pliable and more movable flap is obtained. The 11 children on whom this method was done are presented. Followup is from 6 months to 5½ years. The importance of teaching the parents or child the use of home vaginal dilation with Hegar dilators is stressed. The use of this surgical technique plus home dilation will overcome the problems of restenosis in this group of children.

Extramammary Paget’s disease is a malignant condition. The clinical presentation, treatment and prognosis of this lesion in the scroto-inguinal area are discussed.

Our experience with a microtechnique using 99mTc-DTPA for determination of glomerular filtration rate in 172 children is presented. It is a simple finger-stick method carried out in a four-hour time period by trained nuclear medical per sonnel. The procedure meets all criteria for precise measurement of glomerular filtration rate, and the radiation dose is low. Proof of accuracy was obtained by comparison of this technique with concomitant 24-hour urinary creatinine clear ance in a subset of children.

The results of the application of clean intermittent catheterization to children are discussed. Of 34 patients only 7 maintained persistently sterile urine and 52 per cent had more than 1 positive culture in the followup period. Complications of the application of this technique are discussed. The major complication rate was 15 per cent and 6 per cent were considered failures of therapy. Only 1 of the 34 patients demonstrated progression of upper tract disease by excretory urography during the followup period. Intermittent catheterization was found to be effective in preserving renal function as well as helping to improve patient social acceptability.

We report 3 cases of a series of 372 (0.8 per cent) renal transplant recipients in whom urinary tract calculi developed as a complication of the procedure. In each patient symptoms were minimal and not classic of calculous disease. We disagree with the contention that all such patients have either hypercalcemia, infection, or tubular acidosis, although thorough evaluation is indicated. We believe this entity should be considered in problematic renal transplantation patients. Conservative therapy is advocated when the situation permits.

We report the second case of a cake kidney with a single ureter accompanied by caudal regression. The embryology, presentation and management of this malformation are delineated.

Large intravesical prostates in relatively young men are unusual and present a diagnostic challenge. The anatomy and pathology of such a lesion are presented and various other diagnostic possibilities are discussed.

A case of biopsy-proved postnephrectomy regression of a metastatic skin lesion of renal carcinoma is presented. The incidence of postnephrectomy regression, occurrence of other pulmonary entities resembling metastases, and the possible mechanisms of postnephrectomy regression are discussed. Biopsy of suspected metastatic lesions in renal carcinoma is advocated. We further advocate nephrectomy in the face of distant metastasis, and also consider excision of single metastases, and chemotherapy and/or irradiation when indicated.

A long-term study of 17 patients with paroxysmal nocturnal hemoglobinuria revealed an unexpectedly high incidence of functional and anatomic renal abormalities. All patients demonstrated varying degrees of hematuria and proteinuria distinct from hemoglobinuria. All patients also had granular casts in multiple urinalyses. Evaluation of renal function revealed hyposthenuria, abnormal tubular function and declining creatinine clearance. Radiologically, one or more of these demonstrated enlarged kidneys, renal cortical infarcts and thinning, papillary necrosis, acute renal atrophy, retroperitoneal hematoma and ureteral infarction, which were confirmed by autopsy studies. Hypertension developed in 7 patients. Urinary tract infection was uncommon and no patient had a clinical history compatible with chronic or acute pyelonephritis. Contrary to usual opinion our compatible clearly showed evidence of frequent and widespread renal pathology in paroxysmal nocturnal hemoglobinuria most likely due to repeated microvascular thromboses similar to the venous trombosis involving other organs in this disorder. Since most of these patients present initially to urologists knowledge of this entity is mandatory.

The case reports of 3 patients who underwent anterior transposition of the urethra are presented. Our modification is believed to shorten the operative procedure and to be equally satisfactory compared to the results presented by previous urologic surgeons. We join those who recommend this operation in the thin female patient who has a neurogenic bladder with constant leakage around the indwelling urethral catheter despite use of larger size tubes.

Recommendations are made for the standardization of nomenclature describing duplex systems, ectopic ureters and ureteroceles. The elimination of some terms and redefinition of others are proposed in the hope to eliminate the ambiguity and confusion that exist currently.

During a ten-year period, 35 children presenting with vesicoureteral reflux, ureteropelvic junction obstruction, or a “small kidney” were found to be hypertensive. Of these, 15 subsequently underwent surgical procedures for relief of hypertension. Seven were “cured,” six were “improved,” and two were “unchanged.” The severity of hypertension could not be correlated with the degree of reflux nor with the degree of obstructive uropathy. However, all children with reflux in our study who were hypertensive had some degree of calicectasis noted preoperatively on intravenous pyelogram. Also it was noted that hypertension may occur several years after successful antireflux surgery. Children with vesicoureteral reflux, ureteropelvic junction obstruction, or a small kidney need to have blood pressure determinations at regular intervals, even if all previous readings had been in the normotensive range and whether or not they were followed up medically or post surgically. We suggest that blood pressure determinations be made every three months for the first year after diagnosis of reflux or ureteropelvic junction obstruction, and at least once a year thereafter.

Saralasin (S), a competetive antagonist of angiotensin II, has been used to evaluate renin-angiotensin dependent hypertension in adults. The purposes of this study were to determine 1) if S might be useful in the evaluation of hypertension in children and 2) if it would provide information about the involvement of the renin-angiotensin system in various forms of hypertension in the young. An IV infusion of S (0.4 to 0.8mg/min) was given to 21 children (4 to 18 years) with sustained hypertension (mean admission BP=162/114). All pats had complete diagnostic evaluation including renal arteriography. Pats were divided into 3 groups: hypertension secondary to renal parenchymal disease (RPH), reno-vascular hypertension (RVH) and essential hypertension (EH). The S test was considered to be positive when systolic BP decreased 10mmHg and diastolic BP decreased 6mmHg. A positive S test was obtained in 4 of 8 pats with RPH, 4 of 7 pats with RVH and 1 of 6 pats with EH. All pats with a positive S test had increased peripheral renin activity (PRA). Two pats with EH and 1 with RVH had increased PRA but negative S tests.. Four pats with RPH (3+ and 1-S test) and all 7 pats with RVH have had a surgical procedure to help control their hypertension. These results suggest that S may be useful in the diagnostic evaluation of hypertension in children. More experience and time are required to determine whether S can help predict surgical success for RVH and RPH in the young.