Abstract Congenital mid-ureteral stenosis is a very rare entity which typically occurs on one side. This condition is usually misdiagnosed as hydronephrosis or megaureter, and only after surgery is ...the correct diagnosis made. We report the case of a bilateral mid-ureteral stenosis. No other cases of bilateral strictures have been found in the literature.
Preoperative computer tomography (CT) guidance localization utilizing a percutaneous guidewire before thoracoscopic resection is safe and beneficial in children with pulmonary nodules less than 1 cm ...in size or located deep in the pleural surface. This paper describes a successful thoracoscopic resection of a little subpleural pulmonary metastasis of a Wilm's tumor in a 5-year-old child utilizing preoperative CT-guided wire localization of the lesion. The thoracoscopic procedure was performed with the use of two ports, the nodule was easily localized,and the pulmonary wedge resection was made by the use of an endo-GIA linear stapling device after guidewire removal. The operating time was 45 minutes and the chest tube was removed after 48 hours. The postoperative course was uneventful, and the child was discharged on postoperative day 5. This technique allows the surgeon to resect little pulmonary nodules, avoiding the need of more invasive procedures as standard thoracotomy without adjunctive morbidity and with good cosmetic results.
The growing use of routine ultrasonography during pregnancy is leading to an increasing number of prenatally diagnosed neuroblastomas. Optimal strategy has not yet been defined for these patients, ...because knowledge on this particular neuroblastoma (NB) population is still limited. However, definite guidelines are needed to avoid inadequate treatment. The authors analyzed the cases of antenatally detected NB (ADNB) reported in the Italian Neuroblastoma Registry during the past 6 years to elucidate the features of this subset of NB.
The Italian Neuroblastoma Registry was reviewed for the period January 1993 to December 1998 to collect clinical, radiographic, surgical, and histopathological data on ADNB cases. NB stage was evaluated according to INSS criteria. All patients had undergone imaging (computed tomography or magnetic resonance imaging) of the primary tumor and bone marrow biopsy before surgical resection.
Seventeen patients were identified. Primary tumour site was adrenal glands in 16 cases and retroperitoneal ganglia in 1. Stage distribution was stage I, 13 cases; stage II-A, 1 case; stage II-B, 1 case; stage IV-S, 2 cases. All cases underwent primary tumour resection. Mean age at surgery was 4 weeks. Resection of primary tumor was radical in 16 cases, partial in 1. All tumors were characterised by favourable histology according to Shimada classification. N-myc gene amplification was studied in 14 patients. N-myc amplification was detected only in a newborn with stage II-A NB, who died of massive bleeding 2 days after tumor resection. DNA index and 1 p deletion were studied in 11 and 8 patients, respectively. Both diploidy and deletion of 1p were observed in a newborn who subsequently died of disease progression despite surgery, chemotherapy, and radiation therapy. Fourteen of 17 patients currently are alive and free of disease, and one with IV-S NB and short follow-up is alive with disease.
Our data give evidence that in most cases infants with ADNB represent a subset of patients with excellent outcome. Aggressive treatment may not always be necessary. Infants with ADNB with unfavorable features should undergo early surgical excision, whereas patients with favourable features could be observed awaiting spontaneous regression of the mass, reserving delayed surgery for tumors that increase in size or do not regress.
Adrenocortical tumors in children are extremely rare, accounting only for 0.3-0.4% of all neoplasms in this age. Most frequently they secrete hormones, resulting in virilization, Cushing's syndrome ...or feminization, while the non-functioning ones are unusual. The authors describe 12 cases observed in 13 years (1976-1989), with a mean age of 5 years. 9 cases showed virilization, 4 presented with Cushing's syndrome and in 5 patients an abdominal mass was palpable. One case was affected by Beckwith-Wiedemann's syndrome. I.V. urography was performed in 8 patients, arteriography in 4 and since 1982 all patients were submitted to abdominal sonography and CT scan or MR imaging. Urinary 17-ketosteroids, 17-hydroxycorticoids and serum testosterone and cortisol were tested in all children. Dexamethasone suppression test was performed in 7. All patients were treated with surgery which seems to be the most suitable treatment, while the real effectiveness of treatment by drug therapy with suppressors of steroidogenesis is not confirmed in children. Histopathological examination showed typical features of adenoma in 5 cases, of adenocarcinoma in 4, while three cases revealed border line forms classified as "atypical adenomas". At the moment 10 patients are alive with a follow-up ranging from 18 months to 14 years, while 2 children with adenocarcinoma are dead.
In 34 consecutive patients in whom the diagnosis of Hirschsprung's disease (HD) was suspected, the reliability of clinical symptoms, radiological parameters, and anorectal manometry was ...retrospectively and blindly evaluated by three independent investigators. In 19 patients the diagnosis of HD was histologically proved, while in the remaining 15 cases the diagnosis of idiopathic constipation was justified by persistent success of medical treatment at regular follow-up. Anorectal manometry was correctly diagnostic in all the patients who were examined. The recto and inhibitory reflex, recorded in all the patients with idiopathic constipation was absent in patients suffering from HD. Moreover, other manometric parameters, peculiar to HD were found: a significant lower anal resting pressure and pain threshold, as well as a decreased frequency of spontaneous rhythmic oscillations. The reliability of radiological and clinical data for these diagnoses appeared to be inadequate. Therefore, the diagnosis of HD can be confidently stated only on the basis of manometric investigations.
A case of cystic nephroma is described in a 20-month-old female. The diagnosis of this rare renal pathologic lesion is essentially based on its pathologic features. The treatment consisted of ...nephrectomy followed by a brief course of chemotherapy. The literature is reviewed with regard to the pathologic features and treatment of this lesion, which has a favorable prognosis.
Neoplastic invasion of the inferior vena cava due to renal tumors (especially Wilms' tumor) is uncommon in children. The tumor thrombus, according to the aggressiveness of the original neoplasm, can ...extend in diverse ways, obliterate the vascular lumen completely, and even reach the right atrium. The luminal thrombus might be accompanied by the involvement of the caval wall, which requires wide vascular resection. The purpose of this paper is to present our experience with 7 children, aged 18 months and 6 years, affected by caval invasion due to Wilms' tumor. Furthermore, the diagnostic techniques and the surgical treatment in simple caval thrombosis and in associated invasion of the caval wall are described.
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