Prolapse of ectopic ureterocele and bladder trigone.

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

Sacral agenesis. Analysis of 22 cases.

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.

Acute leukemia: diagnosis and management of testicular involvement.

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.

Vaginal calculus in female with myelodysplasia.

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 varicella syndrome with genitourinary anomalies.

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.

Urinary undiversion in patients with myelodysplasia and neurogenic bladder dysfunction. Report of a workshop.

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.

Desmopressin acetate in children with severe primary nocturnal enuresis.

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.

Dermoid tumors occurring at the site of previous myelomeningocele repair.

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.

Continent vesicostomy using a catheterizable posterior bladder tube: modification of the Mitrofanoff principle.

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.

Simple testicular cyst in the neonate.

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.

Renal cell carcinoma in association with tuberous sclerosis in children.

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.

Prenatal diagnosis and the pediatric surgeon: the impact of prenatal consultation on perinatal management.

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.