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Squamous cell carcinoma arising from a urinary stoma is exceedingly rare, and none so far is reported from a cutaneous ureterovesical stoma. Squamous cell carcinoma usually occurs as a late complication of urinary diversion, and we report the first such case in a cutaneous ureterovesical stoma with a review of published literature., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: We further define the natural history and management of minimal fetal hydronephrosis., Materials and Methods: Experience with minimal fetal hydronephrosis (renal pelvic diameter 4 to 10 mm. before 20 weeks of gestation and 5 to 10 mm. from 20 to 24 weeks) was reviewed for 18 months at New England Medical Center., Results: The incidence of minimal fetal hydronephrosis was 2.2% in the 5,900 fetuses screened by ultrasound. In 63% of cases prenatal ultrasound was done more than once, and revealed that dilatation of the urinary tract was stable during gestation in 31 (25%), and became normal in 35 (29%) and worse in 11 (9%). A total of 63 patients (103 renal units) followed for up to 1 year underwent postnatal sonography. Those with abnormalities were screened with a voiding cystourethrogram and renal scan, including 40 with normal postnatal ultrasound and no changes on subsequent evaluation. Of the 23 patients with abnormal findings 4 had functionally significant ureteropelvic junction obstruction and 1 had severe vesicoureteral reflux. A retrospective analysis of the sonograms of patients with minimal fetal hydronephrosis and persistence or progression of hydronephrosis revealed calicectasis, progression of minimal fetal hydronephrosis in utero and/or abnormal renal echogenicity in each fetus., Conclusions: Our study shows that in a small but significant number of fetuses minimal fetal hydronephrosis will progress, whereas in most it will resolve.

Tuberous sclerosis, a genetically transmitted multisystem neurocutaneous disorder, is associated with renal lesions in 50% of cases. The expected lesions are angiomyolipomas or renal cysts; renal cell carcinoma has been encountered in adults who have tuberous sclerosis, but is very rare in children. The authors report the case of a 5-year-old girl with tuberous sclerosis for whom atypical computed tomography findings led to the diagnosis of renal carcinoma at an early age. This experience suggests that children with tuberous sclerosis may need earlier screening.

Purpose: Pediatric surgeons are increasingly called on by obstetrical colleagues to counsel parents about the implications of a prenatal ultrasound finding. Our understanding of the natural history of many prenatally diagnosed surgical conditions has grown significantly in recent years. Whether prenatal surgical consultation can influence perinatal course had not been investigated., Methods: During an 21-month period, 12,865 prenatal ultrasound studies were performed on a total of 4,551 patients, and 221 prenatal surgical consultations were obtained through a newly established fetal treatment program at a tertiary care prenatal diagnostic center. To evaluate the impact of prenatal pediatric surgical consultation on perinatal course, the authors reviewed changes in management including termination of pregnancy, in utero intervention, and altered site, mode, or timing of delivery., Results: Two hundred twenty-one fetuses were referred for consultation; their 234 congenital anomalies included genitourinary (36%), thoracic (16%), intraabdominal (14.5%), abdominal wall (10.6%), neurological (9%), skeletal (6%), and head and neck (2.5%) defects; 2.5% had tumors and 2.5% were twin pregnancies. Pregnancy was terminated in 9.5% of cases, because of patient request, chromosomal abnormality, or dismal prognosis. In 3.6%, the decision to terminate was changed as a result of consultation. Site of delivery was changed as a result of consultation in 37% to facilitate postnatal evaluation and initiate immediate treatment. Mode of delivery was changed in 6.8% to prevent dystocia, hemorrhage into a tumor, as in sacrococcygeal teratoma, or to provide an emergency airway, as in cervical teratoma. The timing of delivery was changed in 4.5% to avoid further damage to fetal organs in cases of obstructive uropathy, gastroschisis, sacrococcygeal teratoma with high-output failure, and hydrocephalus. Five percent (11) underwent treatment in utero for fetal hydrothorax, obstructive uropathy, twin-twin transfusion syndrome, or lymphangioma. The overall perinatal mortality rate was 2.5%., Conclusion: Prenatal pediatric surgical consultation may have a significant impact on the perinatal management of the fetus with a surgically correctable congenital anomaly. Providing obstetric colleagues and families with valuable insight into the surgical management of anomalies allows fetal intervention when appropriate, and delivery in an appropriate setting, by the safest mode of delivery, and at the gestational age appropriate to minimize effects of the anomaly.

Growing interest in the endoscopic treatment of vesicoureteral reflux and urinary incontinence has led to the development of safe reliable techniques that use an injectable bulking agent. A variety of injectable materials has been studied, such as polytetrafluoroethylene, fat and bovine collagen. We evaluated autologous collagen extracted from the dermis of skin for potential use in the urinary tract. Therefore, an animal model was developed to study the biological behavior of autologous collagen within the bladder. Collagen was extracted from the skin of rabbits and radiolabeled with 125iodine. An aliquot of radiolabeled autologous collagen was injected submucosally in the bladder. The animals were monitored weekly under a gamma camera. The decay of the radiolabeled collagen was recorded during 50 days. The animals were then sacrificed and the bladder wall at the site of injection was studied histologically and on electron microscopy. Decay of the injected collagen within the urinary tract was similar to control material, indicative of good stability within the bladder. A mild inflammatory response with fibroblastic and vascular invasion of the collagen implant was observed. These preliminary data suggest that autologous collagen injected endoscopically can provide a safe reliable bulking material that could be used to treat urinary incontinence and lower grades of vesicoureteral reflux in children.

The authors report the first case of a simple testicular cyst presenting in a neonate. Diagnostic criteria are outlined and theories concerning etiology are discussed. The authors show an absence of elastin staining in the specimen, and suggest this precludes germinal tubules as a source. The rationale for testicular-sparing surgery is discussed.

The authors report a case of microcolon-intestinal hypoperistalsis and prune-belly syndrome, with a huge adynamic bladder and a vesicostomy. The patient was treated surgically; a continent stoma was created through which urine is drained by intermittent clean catheterization via a catheterizable conduit constructed from a tubularized strip of posterior bladder wall.

To our knowledge intra-abdominal desmoplastic round cell tumor has not previously been described in the pediatric urological literature. This lesion has only recently been recognized as a clinicopathological entity with predilection for adolescent boys. We report on 2 patients who presented with urological symptoms and a large abdominal or pelvic tumor that was biopsied at exploratory laparotomy. Complete surgical excision was impossible in both patients, who subsequently underwent multiagent chemotherapy. Histological and immunohistochemical staining are distinctive in this condition. The tumor is associated with a poor prognosis, despite multidisciplinary, multimodal therapy.

Von Hippel-Lindau disease is a rare autosomal dominant disorder. Kidney lesions occur in the majority of cases, with renal cell carcinoma noted in 40% and renal cysts in 60%. Renal cell carcinoma in von Hippel-Lindau disease is usually bilateral and occurs at an earlier age than in patients with sporadic renal cell carcinoma. We report on a 16-year-old boy who, to our knowledge, is the youngest patient to present with von Hippel-Lindau disease and renal cell carcinoma. Controversy currently exists regarding the nature of renal cysts in von Hippel-Lindau disease and the optimal therapeutic approach (that is radical versus parenchymal sparing surgery). We review the histology of renal cysts and carcinoma, and discuss the rationale for selecting parenchymal sparing surgery.

Desmopressin acetate, a synthetic antidiuretic drug, was evaluated in two US multicenter studies involving a total of 176 children with severe primary nocturnal enuresis. Patients who qualified during the screening period, entered a double-blind treatment phase during which they received 20 micrograms or 40 micrograms of desmopressin acetate or placebo at bedtime for four weeks. This phase was followed by a four-week open-label period, during which all patients received 20 or 40 micrograms of desmopressin acetate. In both studies, children given desmopressin acetate had significantly fewer wet nights after two and four weeks of therapy than did children given placebo. During the open-label period, patients crossed over from placebo to desmopressin acetate showed similar reductions in enuresis. Global evaluations by the patients, their parents, or the investigators further confirmed the good results with desmopressin acetate. No serious adverse experiences were reported. Thus the results of these studies corroborate previously published reports of the efficacy and safety of desmopressin acetate in the treatment of primary nocturnal enuresis.

This workshop was conducted in an attempt to analyze critically the role of reconstruction of the myelodysplastic patient who had undergone urinary diversion and to develop guidelines for selecting those patients in whom urinary undiversion might be undertaken safely. The collective experience initially seems to be acceptable; however, the authors emphasize the gravity of the decision and the complexity of the evaluation which must be undertaken prior to embarking on such reconstructive surgery. Contrary to some reports, we believe that the defunctionalized bladder frequently can be evaluated. Further, many of the contraindications to urinary undiversion have been identified and several of the hazards involved therein can be avoided. We believe that the neurogenic bladder is no longer an absolute contraindication to undiversion. Our experience suggests that undiversion is a reasonable surgical treatment in select patient with neurogenic bladder dysfunction. But, the decision to remove a satisfactorily functioning conduit must not be undertaken lightly. Patients should be selected only after a thorough, detailed, and properly conducted evaluation. A protocol has been developed which will hopefully assist in this evaluation. Perhaps additional shared experience will further refine and delineate the circumstances appropriate for reconstruction of these patients.

Sacral agenesis is a rare congenital anomaly of the spine and is usually associated with urologic abnormalities. Twenty-two patients are reviewed. Twenty-one had other congenital and developmental abnormalities in addition to sacral agenesis. Physical examination showed flattening of the buttocks, loss of the gluteal cleft, widely spaced buttock dimples, and a palpable sacral defect. Lateral views of the spine are essential when anteroposterior views suggest an absence of sacral segments. Seven patients had a significant familial history of diabetes mellitus. All patients with involvement of more than one sacral segment had neurogenic bladder dysfunction with incontinence. The degree of neurologic deficit could not be predicted from the radiologic appearance of the sacrum.

The efficacy of Credé's maneuver to promote voiding was evaluated prospectively in 207 patients with a variety of lower urinary tract disorders. The normal response to Credé's maneuver was a contraction of the external urethral sphincter (the Credé effect), an increase in urethral pressure and maintained closure of the vesical neck. Relaxation of the external urethral sphincter and synchronous opening of the bladder neck during Credé's maneuver were seen in only 4 patients (2 per cent). We conclude that Credé's maneuver is an inefficient method of bladder emptying in the majority of patients and even when voiding is accomplished with Credé's maneuver it usually is grossly inefficient and associated with significant urethral obstruction.

The first documented case of prolapse of an ectopic ureterocele and bladder trigone is presented. The differential diagnosis and therapy are discussed.

Hypospadias with chordee and associated torsion can be corrected by a simple 1-step procedure. The repair is based on the procedure described by Allen and Spence but is modified to correct more severe degrees of hypospadias and chordee below skin level. The procedure is simple, involves minimal complications and justifies the repair of marginal defects.

Four children with previously repaired myelomeningoceles presented toward the end of the first decade or early in the second decade of life with deteriorating lower-extremity and bladder function. Myelography and computerized tomography scanning demonstrated irregular filling defects at the area of the myelomeningocele repair, and surgical exploration disclosed dermoid tumors that were adherent to the placode and adjacent roots. Dermoid tumors should be considered in the differential diagnosis of neurological deterioration in children with a repaired myelomeningocele.

A case report of vaginal calculus formation in a nine-year-old girl with myelodysplasia is presented. Etiologic factors in the formation of such calculi include fistulous communication between the vagina and the bladder, neuropathic urethrovesical dysfunction, anatomic conditions causing vaginal outlet obstruction, and/or vaginal pooling of urine and urease-producing bacterial infection. A correct preoperative diagnosis can be made by using oblique x-ray films and cystoscopy. Surgical treatment is simple and successful.

Congenital ureteral valves are a rare cause of obstructive uropathy and the majority of cases are diagnosed only at surgery or autopsy. A case of congenital ureteral valve associated with an incompletely duplicated kidney is reported and the literature on ureteral valves is reviewed. Modern uroradiological and endoscopic diagnostic techniques should result in a more precise preoperative diagnosis. The high incidence of associated genitourinary anomalies, particularly duplication anomalies, suggests abnormal ureteral embryogenesis as an etiological factor in congenital ureteral valve formation.

A female child, born at term to a mother who contracted varicella in early pregnancy, presented with multiple congenital defects. These included mental retardation, numerous skeletal anomalies, and absent uterus and vagina. Urologic anomalies included bilateral chronic pyelonephritis secondary to vesicoureteric reflux. This pattern of congenital abnormalities has not been reported previously.

Intermittent catheterization is a valid treatment modality in the management of the neuropathic bladder. Numerous adjunctive surgical procedures have been tried to improve continence and to improve or preserve renal function. These procedures, performed on 38 of 82 patients on long-term intermittent catheterization, are preferable to urinary diversion and possibly to the implantation of prosthetic or artificial urinary sphincters. Uretero-ileocecocysto-urethroplasties have not been successful in our hands, but the other procedures certainly offer a preferable alternative to the treatment modalities in use up to only 4 years before this bicentennial year!

Contemporary therapy of acute leukemia frequently achieves long-term continued complete remission (CCR) of bone marrow disease and prevents central nervous system relapse. However, accompanying improved survival is an increasing incidence of overt testicular relapse either during CCR or associated with bone marrow relapse. In 7 boys testicular abnormalities developed during CCR, 6 had open biopsy and 5 had histologically confirmed leukemic infiltration. Despite local therapy of orchiectomy or irradiation and chemotherapy reinduction, 2 of 6 had testicular relapse and 4 of 6 died. Three boys with coexistent overt testicular and systemic relapse died. Nine boys with normal testes had testicular biopsy during CCR prior to discontinuation of chemotherapy. Results of all biopsies were benign, but one boy had a relapse. The diagnosis of occult testicular leukemia prior to discontinuation of chemotherapy allows selection of high-risk boys requiring prolonged, intensive, and possibly alternative therapy. The indication for testicular biopsy in boys with acute leukemia is documented, and appropriate clinical management of testicular leukemia is presented.

One hundred sixty-five infants of both sexes, 2 weeks to 2 years of age, were screened by their parents at home for bacteriuria. Parents were instructed during a single brief period during the initial office visit to collect and to culture their infants' urine at home. Urine was collected by specimen bag and cultured promptly at cribside using a newly developed culture kit and inoculating swab. The same specimen was cultured by the office laboratory staff by kit and swab and, as a reference standard, by the loop-MacConkey method. Infants were screened at home after three consecutive office visits. Then, 870 specimens were processed. Urinary tract infection (UTI) was diagnosed in 3.6% of the study population. Results show that home screening of infants for UTI by the parent compares favorably with screening by the office laboratory.

We have studied the ultrastructure of glycocalyx at the luminal surface of normal and diseased urothelium from humans and rats with ruthenium red staining. A correlation between the thickness and staining intensity of the glycocalyx and the surface topography of the luminal surface was observed. An intensely stained thick glycocalyx was associated with prominent surface microvilli seen in the following conditions in humans: some control urothelium, inverted papilloma, well and moderately differentiated transitional cell carcinomas and mucin producing adenocarcinomas. These changes were also present in rats with FANFT-induced preneoplastic and neoplastic changes. A thin glycocalyx was associated with a scalloped luminal surface containing asymmetric unit membrane plaques and was found in some control humans urothelium and in normal rat urothelium. A thin glycocalyx was also associated with the relatively smooth surface seen in poorly differentiated transitional cell carcinomas as well as in some mucin producing adenocarcinomas. We suggest that urothelial glycocalyx, as demonstrated by ruthenium red staining, correlates with the luminal surface topography rather than specific pathological conditions of the bladder.

Surgical correction of the undescended testis is frequently postponed beyond the optimal time, namely, 6 years of age.An accurate diagnosis of undescended testis may be made during the first year of life. Complications and mistakes arising from misdiagnosis of undescended testis and retracted testis may, therefore, be prevented by recording findings.The purpose of this article is to present the arguments in favour of early diagnosis and operative treatment of undescended testis, and to correct possible misconceptions.