Cadaveric renal transplants in children under 6 years of age.

Thirty-nine cadaveric renal allografts were performed in 28 children under 6 years of age. Common primary renal diseases were glomerulonephritis, dysplasia/hypoplasia, and reflux/obstructive nephropathy. After a mean follow-up of 40 months of patients with surviving grafts, 19 patients had functioning grafts, 3 had returned to dialysis, and 6 had died. These children required an extraordinary amount of care postoperatively because of anorexia, diarrhea, and ileus. Their psychomotor and physical development was retarded prior to transplant; this reversed dramatically after transplant, but catch-up growth occurred in only 4 patients. Many patients were noticeably more active and distractible for 1 to 2 years post-transplant. Major causes of graft failure were primary nonfunction of 5 donor kidneys (4 from donors under 1 year old) and renal vessel thrombosis in 5 recipients (3 with native kidneys in place who received kidneys from donors over 10 years old). Other causes were recurrence of hemolytic uremic syndrome and Wilms tumor, rejection, and sepsis. Kidneys from donors under 1 year old proved unsatisfactory, and large donor kidneys in small children tended to thrombose, especially when native kidneys with high urine output were left in situ.

Megalourethra with posterior urethral valves.

A case of posterior urethral valves and scaphoid megalourethra is presented. We believe this is the first report to document the association of these two anomalies, although urethral valves have been reported in at least 2 patients with saccular anterior urethral diverticula. Although fusiform megalourethra is often associated with other genitourinary anomalies, the scaphoid type is thought to be an isolated defect. This report would suggest that patients with either type of megalourethra should have complete urologic investigation.

Magnetic resonance imaging of cystic, partially differentiated nephroblastoma.

The magnetic resonance imaging (MRI) appearance of a cystic, partially differentiated nephroblastoma is described, together with pathologic correlation. The difficulty in reaching a correct preoperative diagnosis even with multimodal imaging techniques is emphasized. MRI is an adjunct to ultrasonography and may be superior to computerized tomography (CT) scan in the evaluation of a child with multiloculated cystic renal mass.

A comparative study of intravenous urograms and radionuclide renal scans in diagnosis of renal trauma.

We analyzed 31 patients who underwent intravenous urograms (IVU) and radionuclide renal scans (RNRS) in the investigation of blunt abdominal trauma, and compared the IVU to the RNRS in the definition of blunt renal injuries, and their correlation with patients' symptoms and signs. Thirteen patients had abnormal IVU and 12 had abnormal RNRS (42% of the 31 patients). In 8 patients findings on IVU correlated with those on RNRS (62%), and in 5 patients findings were different (38%). Only 2 patients (6.5%) had significant differences which may have influenced further treatment. Only 1 patient required operative management. Follow-up IVUs were performed on 6 of the 13 patients who had abnormal radiologic studies. The radionuclide renal scan was shown to be as accurate as the intravenous urogram in defining renal trauma. Patients who undergo liver-spleen scans for investigation of blunt abdominal trauma, and who may have renal trauma, may well undergo RNRS at the same time and obviate the need for an IVU, without any diagnostic compromise.

Acquired vulvar lymphangiectasis in a child.

A unique case of extensive acquired vulvar lymphangiectasis without lymphedema in a child is described. This lesion developed after pelvic exenteration, lymphadenectomy, and radiation therapy for rhabdomyosarcoma of the bladder. These acquired etiologic factors simulated the pathophysiology of congenital lymphangioma. She was successfully treated with surgical excision and vulvar reconstruction.

Gender assignment in male pseudohermaphrodite children.

Experience with male pseudohermaphrodites at The Hospital for Sick Children, Toronto, from 1976 to 1981 was reviewed to identify factors that had influenced gender assignment. Seventeen children with microphallus, ambiguous genitalia, and 46 XY karoytype were studied. Five had been raised as females and 12 as males. Of those raised as females, 3 were from families with prior experience of androgen insensitivity. The other 2 had gross perineal abnormalities in addition to genital ambiguity. Of those raised as males 6 were not considered for gender reassignment, 4 because they were too old at presentation, and the other 2 because of parental refusal. In this series the parents were not prepared to accept gender reassignment on the basis of a small phallus alone. Androgen stimulation so far has been a reliable indicator of phallic growth potential, but long-term follow-up is necessary to determine its true value.

Neoplastic potential in patients with disorders of sexual differentiation.

The occurrence of tumors was determined in 157 patients with disorders of sexual differentiation at a major pediatric hospital from 1960 to 1980. Gonadoblastoma developed in 2 of the 4 patients with mixed gonadal dysgenesis (MGD) who had not had bilateral gonadectomy; this indicates the need for early gonadectomy in such cases. Gonadal embryonal carcinoma developed in 1 child with Turner's syndrome (1 of 71). Four cases of nephroblastoma occurred, 1 in a child with MGD (1 of 18) and 3 in male pseudohermaphrodites (3 of 35). The nature of the defect linking male pseudohermaphroditism with Wilms tumor remains to be established.

Renal transplantation in children following augmentation ureterocystoplasty.

OBJECTIVES: To assess the impact of augmentation ureterocystoplasty on the success of cadaveric renal transplantation in children with dysfunctional bladders. METHODS: Two patients with end-stage renal failure secondary to dysfunctional bladders (one myelodysplasia and one posterior urethral valves) underwent augmentation ureterocystoplasty prior to renal transplantation in order to increase bladder capacity and improve compliance. RESULTS: Significant improvement of bladder storage function was achieved in both patients. By the use of megaureter for augmentation, untoward sequelae of enteric or gastric augmentation were obviated. Renal transplantation was successful in both patients. Both have normal renal function 4 and 3 years after transplantation. CONCLUSIONS: Renal transplantation into bladders previously augmented with megaureters is successful. The use of urothelial-lined biomaterial for augmentation avoids the potential complications of gastro- or enterocystoplasty, which are especially dangerous in transplant patients.

Bilateral congenital midureteral adynamic segments.

Two cases of congenital midureteral adynamic segments are presented. Both children were successfully managed with excision of the lesions and primary reanastomosis. Pathologic examination revealed probe-patent ureters with muscular disarray, suggesting functional obstruction. Appropriate management of the anomaly was dependent on precise radiographic localization of the area of narrowing, and for this retrograde urography was essential. Primary ureteroureterostomy was successful in both cases. In this report we discuss the diagnosis, embryology, radiographic evaluation, and management of this rate situation.

Extravesical detrusorrhaphy for refluxing ureters associated with paraureteral diverticula.

OBJECTIVES: To demonstrate the effectiveness of an extravesical approach to refluxing ureters associated with paraureteral diverticula. METHODS: Over a 39-month period, 23 children underwent repair of Hutch diverticula with refluxing ureters using an extravesical approach. Seventeen had unilateral reflux with an ipsilateral diverticulum, 4 had bilateral reflux with a unilateral diverticulum, and 2 had bilateral reflux with bilateral diverticula. Overall, 25 ureters with associated diverticula were repaired. RESULTS: Twenty ureters were repaired with a nondismembered technique and 5 with a dismembered technique. Twenty-two of the 23 patients (96%) were successfully repaired with this approach. Three patients had transient reflux postoperatively, which resolved spontaneously within 6 months. One patient who underwent bilateral dismembered procedures for bilateral diverticula has persistent unilateral grade II reflux postoperatively. No patient developed ureteral obstruction. CONCLUSIONS: The major advantage of this technique is seen in the minimal postoperative morbidity. The extravesical approach is a safe, simple, and effective method for the management of a refluxing ureter with an associated diverticulum.

External genital abnormalities associated with Wilms tumor.

The records of 170 patients with unilateral and 18 patients with bilateral Wilms tumor and 6 patients with congenital mesoblastic nephroma were reviewed for abnormalities of the external genitalia. There were 4 patients with cryptorchism, 1 with hypospadias, 1 with mixed gonadal dysgenesis, and 3 with male pseudohermaphroditism. Of the group, these 9 patients had earlier symptoms; 6 of them were under two years old. Five patients (27.7%) with bilateral Wilms tumors had external genital anomalies in contrast to only 4 (2.3%) of those with unilateral tumors. One of the 9 children with genital anomalies had unilateral congenital mesoblastic nephroma; the others had Wilms tumor. In all cases the histologic subtypes were unusual. These observations substantiate the previously reported association between external genital abnormalities and Wilms tumor and also represent a somewhat higher than expected incidence. The children with this association are remarkable for the high frequency of a particular histologic type of tumor and for the high incidence of bilaterality. The high incidence of Wilms tumors in the male pseudohermaphrodite population presented suggests that such patients should be monitored for development of renal tumors.

Intestinal metaplasia is not a strong risk factor for bladder cancer: study of 53 cases with long-term follow-up.

OBJECTIVES: Intestinal metaplasia often coexists with adenocarcinoma of the urinary bladder, suggesting to some investigators that it is premalignant. However, the natural history and long-term outcome of intestinal metaplasia in isolation are unknown. We report 53 cases of intestinal metaplasia of the urinary bladder followed for more than 10 years. METHODS: We reviewed the Mayo Clinic surgical pathology files between 1926 and 1996 and all patients with exstrophic bladder recorded in the files of the Hospital for Sick Children (Toronto, Ontario, Canada) and Dallas Children's Hospital (Dallas, Texas) between 1953 and 1987, and identified all patients with intestinal metaplasia of the bladder. RESULTS: A total of 53 cases were identified from both series, and none of the patients developed adenocarcinoma of the bladder. The Mayo Clinic series consisted of 24 patients. Nineteen of the 24 (79.1%) were alive without evidence of cancer (median follow-up 14 years, range 0.9 to 53), and 5 patients died of intercurrent disease (at 0.9, 4, 8, 11, and 53 years after diagnosis) without evidence of bladder cancer. The Dallas Children's Hospital and the Hospital for Sick Children series consisted of 29 patients. Twenty-seven of the 29 (93.1%) were alive without evidence of cancer (median follow-up 13 years, range 3 to 23.9). Two patients died of trauma (at 10.9 and 12 years after diagnosis) and at autopsy had no evidence of bladder cancer. CONCLUSIONS: Intestinal metaplasia of the urinary bladder is not a strong risk factor for adenocarcinoma or urothelial cancer.

Growth in boys and posterior urethral valves. Primary valve resection vs upper tract diversion.

Seventy-four patients with posterior urethral valves were evaluated for long-term growth potential and renal function. In those boy diagnosed and treated within the first year of life, initial treatment by temporary supravesical diversion (prior to valve resection) was associated with better growth potential and renal function than was treatment by primary valve resection alone.

Emergency treatment and long-term follow-up of posterior urethral valves.

Posterior urethral valves have a broad spectrum of clinical severity determined by the degree and reversibility of the long stepwise sequence of secondary pathology. Neonatal azotemia and severe bilateral reflux are particularly important negative prognostic factors. In the mild cases, valve ablation with or without delayed reconstruction is good therapy. In the very severe cases, our interpretation of all the clinical and experimental information now available suggests that the time interval and the level of decompression are extremely important. Achievement of consistent low caliceal pressure without stasis and infections should be achieved as soon as possible. We do not agree with the philosophy of "valve ablation and wait and see" for secondary reconstruction as applied to the severe cases. An analogous philosophy would be treating all respiratory infections as upper respiratory infections and applying aggressive appropriate therapy for pneumonia only if the patient does not respond clinically. However, all controversy aside, the management of neonatal infants with posterior urethral valves remains a difficult and challenging problem for us all. The real challenge will be to improve published management results to the point that the family faced with decisions regarding an in utero diagnosis of posterior urethral valves will have enough hope to continue the pregnancy.

Complications of posterior urethral valve surgery and their prevention.

The treatment of patients with posterior urethral valves has improved dramatically in recent times. Our personal experience with 207 patients with posterior urethral valves between 1957 and 1978 reveals a striking reduction in both mortality and morbidity during the latter decade. We have attempted to identify clearly the incidence, etiologic factors, preventive measures, and surgical treatment of complications arising in these patients. The overall improvement in management is certainly attributable to a growing experience and the constant advancements in the medical and surgical armamentarium. Our next goal at this time is to maximize long-term renal function and growth potential in these patients.

Primary hydronephrosis. Assessment of diuretic renography, pelvis perfusion pressure, operative findings, and renal and ureteral histology.

Hydronephrosis generally implies ureteropelvic junction obstruction, but may be mimicked by a variety of other disorders. The authors have attempted to determine the relative diagnostic value of diuretic renography and the pelvis perfusion test in children with hydronephrosis by correlating the results with operative findings, renal and ureteral histology, and postoperative results.

Allograft renal-artery stenosis: increased peripheral plasma renin activity as an early indicator of uncontrollable hypertension.

We measured indices of the renin-aldosterone system and body-fluid spaces in 11 adolescents who had received a renal transplant after removal of their own diseased kidneys. None had hypervolemia but 6 had hypertension. Renal angiography revealed greater than 50% luminal occlusion by allograft renal-artery stenosis (RAS) in only the 3 patients who had severe hypertension refractory to conventional medical therapy. Excessive peripheral plasma renin activity (PRA) distinguished these patients from those who had less severe stenosis or normal angiogram, and diuretic stimulation heightened the PRA differences. We conclude that significant allograft RAS does not necessarily act like a typical single-kidney Goldblatt model until after volume depletion. Our findings indicate that peripheral PRA values can be used to assess the degree of graft ischemia clinically. This permits early identification of patients who have severe RAS that probably will be difficult to control medically, and, therefore, should be followed closely with a view of reconstructive vascular surgery before further deterioration of renal function.

Prototype system for enhancing cystometric analysis with special emphasis on the pediatric population.

BACKGROUND AND PURPOSE: A urodynamic test system of improved accuracy and reliability was developed and implemented for enhancing cystometry. This system integrates known medical information, including the specialized problems of pediatric urodynamics, with the cystometric and imaging data. METHODS: After the requirements for the ideal cystometrogram test unit were established, a system was constructed, calibrated, and implemented in clinical practice. The patient's age, size, and sex are used to produce a patient-specific pressure-volume template for the cystometrogram test. RESULTS: This template showed the minimal and normal bladder capacities and the physiologically safe, equivocal, and dangerous pressure fields coded with symbolic colors. Different time averages of the pressure data were used to show bladder factors such as compliance and instability. The templates with data were presented automatically (therefore objectively) without operator intervention on monitors during testing and as printed copies on completion. CONCLUSIONS: The presentation of data in an easily understood format facilitates effective communication between the urologist, referring physician, and patient. Some of the physiological and statistical problems in pediatric urodynamic testing are efficiently and accurately resolved by this system, resulting in better analysis and diagnostic capabilities.

The artificial urinary sphincter.

Urinary incontinence has many etiologies and a similarly varied and wide-ranging spectrum of therapeutic alternatives. The AMS artificial sphincter represents one of these options that has a distinct, albeit limited, place in certain patients. The authors outline the mechanical details of these devices and present their own clinical experience in a large pediatric population.

Pediatric urine testing.

Today, urinalysis is one of the most common clinical tests ordered for adult and pediatric patients. Because urine specimens are usually readily available and are obtained noninvasively, the urine testing is well suited for children. This article discusses the most common urine tests performed in children for screening purposes and also less common tests for diagnosis of specific disorders. Special considerations regarding urine specimen collection in children are discussed. Some simple tests that are underused by clinicians are mentioned, as are some exciting new molecular applications of urine testing.