[Crossed renal ectopia. Report of two cases and literature review]. / Ectopia renale crociata. Descrizione di due casi e revisione della letteratura.

Renal ectopia is a malformative uropathy classified as simple and crossed. The authors report two patients with renal ectopia (one case of ERC without fusion and one case of ERC with fusion). The authors review the literature about crossed renal ectopia and discuss the importance of early diagnosis and the advantage of thorough investigation in order to exclude associated anomalies.

Interstitial cells of Cajal network in primary obstructive megaureter.

OBJECTIVES: Recent reports showed an altered density of interstitial cells of Cajal (ICCs) and of peripherin immunoreactive nerve fibres in obstructed uretero-pelvic junction. Our aim was to investigate ICCs immunoexpression and ureteral innervation in primary obstructive megaureter (POM). METHODS: 8 specimens of POM were obtained during tailoring. Restricted segments of ureters were divided from the dilated ones. C-kit and peripherin immunohistochemistry were performed. RESULTS: A normal distribution of ICCs was documented in both circular and longitudinal muscular layers of dilated segments. Marked muscular hypoplasia and a sparse or absent ICCs occurred in longitudinal muscular layer of restricted ureteral segments. A normal distribution of peripherin positive fibres was present in both dilated and restricted segments. CONCLUSIONS: Our data confirmed a defective muscolarization in restricted, aperistaltic POM. The lacking of ICCs in the longitudinal muscular layer is, probably, due to the absence of c-kit positive muscular embryological precursors. No alteration of peripherin immunoreactive nerve fibres network was observed in both dilated and restricted ureteral segments.

Sarcoglycan immunoreactivity is lacking in infantile hypertrophic pyloric stenosis. A confocal laser scanning microscopic study.

OBJECTIVES: The Dystrophin-Glycoprotein Complex (DGC) is a large multisubunit complex that plays a crucial role in maintaining the structural integrity and physiology of muscle fibers. Dystrophin has been reported to be absent in the pyloric muscle of infantile hypertrophic pyloric stenosis (IHPS) patients. The present study was designed to investigate the other two patterns of DGC (dystroglycan and sarcoglycan complexes) in normal pyloric muscle and their possible modifications in IHPS patients. METHODS: Ten pyloric muscle biopsies were obtained from babies operated for IHPS and five control pylorus biopsy taken at autopsy from cases without gastrointestinal disease. The DGC sub-complexes (beta-dystroglican and beta, delta- sarcoglycans) were localized immunohistochemically using specific monoclonal antibodies. The results were evaluated using a confocal laser scanning microscope. RESULTS: Positive immunolocalization of the two DGC sub complexes was demonstrated in the smooth muscle cells (SMCs) of the pyloric region of control patients. Similarly, a positive immune expression of beta-dystroglican was observed in the pyloric SMCs of IHPS patients. On the other hand a negative immunoreaction for sarcoglycans was recorded within the full thickness of the pyloric SMCs of these patients. CONCLUSIONS: The absence of sarcoglycans within the hypertrophied pyloric muscle may be a predisposing factor in the pathogenesis of IHPS since it could alter the normal physiology of SMCs through the modifications of structural integrity of sarcolemma and signaling between the extracellular and intracellular compartment.

[Conservative treatment of pediatric spleen trauma. Twenty years of experience]. / Trattamento conservativo dei traumi splenici in età pediatrica. Venti anni di esperienza.

OBJECTIVE: The spleen is the most frequently involved organ in abdominal blunt traumas. In the last decade the treatment has changed from an exclusive surgical approach to a conservative approach. We report our experience in the last twenty years. METHODS: Between 1983-2003, 54 patients with blunt abdominal traumas and isolated injury have been treated. The age at the time of diagnosis was 3 to 16 years (mean, 6.5). The diagnosis was made clinically and with imaging techniques (radiographic assessment, scintigraphy, US, CT). In all cases, a conservative treatment was started with continuous vital parameters monitoring, blood red cells count, hemoglobin, hematocrit; volume infusion and when necessary hemo-transfusion. Clinical follow-up and US monitoring were performed for six months after the trauma. RESULTS: In the period 1983-1993 a conservative treatment was tried in 23 patients; in 15 cases it was successfully, in 8 cases a laparotomy with splenectomy was necessary due to hemodinamic instability. In the second decade we observed 31 patients, all had been treated conservatively. The mean hospital stay was of 12 days with no deaths. CONCLUSIONS: Conservative treatment for splenic injuries in patients with hemodinamic stability is nowadays the treatment choice. Constantly monitoring of clinical, laboratory parameters and use of modern imaging techniques allows to follow the evolution of the lesion and to avoid, in most cases, a surgical treatment.

C-kit positive interstitial cells of Cajal network in primary obstructive megaureter.

AIM: Recent reports have shown an altered density of interstitial cells of Cajal (ICCs) and peripherin immunoreactive nerve fibres in uretopelvic junction obstruction. The aim was to investigate ICCs immunoexpression and ureteral innervation in primary obstructive megaureter (POM). METHODS: Eight specimens of POM were obtained during tailoring. Restricted segments of ureters were divided from dilated segments. C-kit and peripherin immunohistochemistry were performed. RESULTS: A normal distribution of ICCs was observed in both the circular and longitudinal muscle layers of the dilated segments. Marked muscle hypoplasia and sparse or no ICCs occurred in the longitudinal muscle layer of the restricted ureteral segments. A normal distribution of peripherin positive fibres was present in both the dilated and restricted segments. CONCLUSIONS: Our data confirm defective muscularization in restricted aperistaltic POM. The lack of ICCs in the longitudinal muscular layer is probably due to the absence of c-kit positive muscle embryological precursors. No alteration in the peripherin immunoreactive nerve fibres network was observed in either the dilated or the restricted ureteral segments.

[Acute abdomen caused by Salmonella typhi acute appendicitis]. / Addome acuto da appendicite acuta secondaria a Salmonella typhi.

Salmonella infections can manifest themselves as acute abdominal problems and lead to emergency surgery. Some examples are: salmonella-related intestinal perforations, gallbladder involments, salpingitis, and peritonitis. Mesenteric lymphadenitis associated with salmonella mimics acute appendicitis and it is often difficult to establish a timely and tempestive diagnosis in children with right lower abdominal pain. Because of the difficult diagnostic process, a significant number of patients with salmonella infections present acute abdomen and undergo needless operations. Instead, in our case of salmonella-related acute abdomen, laparotomy was the right therapeutic choice. The conclusion is drawn that, even if there is not a precise diagnosis, in salmonella-related acute abdomen the surgical approach is the right choice, considering the high morbidity and mortality associated with untreated appendicitis and intestinal perforations.

Immunoexpression of aquaporin-1 in adolescent varicocele testes: possible significance for fluid reabsorption.

OBJECTIVES: To investigate the immunoexpression of aquaporin-1 (AQP-1), a major transmembrane water channel, in high-grade varicocele testes, which are known to imply an unbalanced transmembrane water flow, in both tubular and extratubular compartments. METHODS: Light microscopy and AQP-1 immunohistologic examination were carried out on incisional testicular biopsies from 20 adolescent boys, aged 13 to 18 years, with grade 2 or 3 idiopathic varicocele. Testicular tissue from 4 autopsied subjects of matched age were also investigated as parallel controls. RESULTS: At microscopic examination, the tubular lumina and extracellular matrix were expanded and venular profiles dilated. Cytoplasmic vacuoles were frequent in the Sertoli cells, spermatogonia, and spermatocytes, together with premature sloughing of germ cells and residual cytoplasmic droplets inside the spermatozoa, if present. Diffuse AQP-1 positivity occurred at venular endothelial cell membranes and, unexpectedly, at the cell membranes of the Sertoli cells, diploid germ cells, and haploid cells. In the control testes, focal AQP-1 immunolabeling was confined to the microvessel endothelial cells, without any positive reaction in the tubular or extratubular compartments. CONCLUSIONS: This is the first report showing AQP-1 cell expression in adolescent varicocele testes in both tubular and extratubular compartments. It was associated with the main histologic features of unbalanced water flow in the tubular and interstitial compartments of the testis. Immunocytochemical patterns revealed AQP-1 as a possible critical reabsorption factor, acting to reduce abnormal fluid retention in endotubular cells and the extracellular matrix and, to a lesser extent, in Leydig cells.

Surgical correction of penoscrotal transposition associated with hypospadias and bifid scrotum: our experience of two-stage repair.

OBJECTIVE: To assess the results of surgical correction of incomplete penoscrotal transposition and bifid scrotum using the Glenn-Anderson technique, and its impact on subsequent definitive urethroplasties and final outcome. PATIENTS AND METHODS: We retrospectively reviewed 31 children that underwent two-stage repair for incomplete penoscrotal transposition with severe hypospadias and bifid scrotum. Patient age at stage 1 ranged from 12 to 24 months (average 16 months). The operative principle was based on achieving a normal anatomical position of the penis and scrotum using the Glenn-Anderson technique. In cases with associated scrotal or perineal hypospadias this was transformed into a penoscrotal hypospadias. Final stage urethroplasty was performed after a period of 6 months with a modified Thiersch-Duplay technique. RESULTS: Cosmetic and functional results of the Glenn-Anderson operation were excellent. No major complications were observed. Of 31 patients, 12 (38%) had complications secondary to urethral repair. CONCLUSION: The Glenn-Anderson technique for reconstruction of penoscrotal transposition and bifid scrotum is a simple technique, free of major complications. The purpose of this intervention is to improve the cosmetic appearance and function of the penis. A minimum period of 6 months between consecutive urethroplasties is important. The final stage guarantees good functional and cosmetic results preserving the prepuce.

A case of bilateral prenatal testicular torsion: Ultrasonographic features, histopathological findings and management.

OBJECTIVES: The aim of this study was to demonstrate the ultrasonographic features of prenatal bilateral torsion of the testis, and its histological correlation and management. PATIENT: A newborn presented at delivery with both testes enlarged, swollen and tender. Prenatal ultrasound (US) showed enlarged, hyperechoic testes. Colour Doppler US examination was performed. RESULTS: US revealed both testes to be heterogeneous. Colour Doppler US did not reveal any flow signal. On inguinal exploration both testes appeared necrotic. Histology showed recognizable seminiferous tubules and Leydig cells. CONCLUSION: We believe that both testes should be left in situ after bilateral detorsion even if their macroscopic appearance is necrotic.

Melatonin reduces oxidative stress in surgical neonates.

BACKGROUND/PURPOSE: Cytokines are inflammatory mediators found in the circulation after surgery. Newborns have less protection against oxidation and are very susceptible to free radical oxidative damage. Melatonin has been reported recently to reduce oxidative stress in neonates with sepsis, asphyxia, and respiratory distress. The aim of this study has been to determine if melatonin would lower interleukin (IL)-6, IL-8, tumor necrosis factor alpha (TNF-alpha) and nitrite/nitrate (NOx) levels and modify serum inflammation parameters, improving the clinical course of surgical neonates. METHODS: Ten newborns (group 1), 5 with surgical malformations and respiratory distress (group 1a) and 5 with isolated abdominal surgical malformations (group 1b) received a total of 10 doses of melatonin (10 mg/kg) at defined times interval for 72 hours. The treatment was started within 3 hours after the end of surgery. Ten surgical neonates (group 2), did not receive melatonin. Twenty healthy neonates (group 3) served as control. Blood samples were collected at the end of operation; before treatment with the antioxidant; and 24 hours 72 hours, and 7 days after start of treatment with melatonin or placebo, respectively. RESULTS: Postoperative value of cytokines and NOx levels of groups 1 and 2 were significantly higher than group 3. Compared with group 1b, group 2 displayed significantly higher cytokines and NOx levels at 24 hours, 72 hours, and at 7 days. In group 1a the immediate postoperative values of cytokines were significantly higher than group 1b and group 2, but a significant improvement was observed after administration of melatonin with significantly lower levels of IL-6 and IL-8 with respect to group 2. An improvement of clinical outcome was observed by progressive reduction of clinical parameters of inflammation. CONCLUSIONS: Melatonin reduces cytokines and NOx levels showing potent antioxidant properties with improvement in clinical outcome. Further studies are warranted to define, on larger numbers, the role of melatonin in surgical patients.

Disorders of the esophageal motor activity in atresia of the esophagus.

Esophageal dysfunction has been reported after successful repair of esophageal atresia but its nature has not been clearly defined. We studied esophageal motility in 20 newborns with esophageal atresia by recording intraluminal pressure of both proximal and distal segments. The investigation was made by pressure monitoring of the upper pouch via the mouth and of the distal segment via the gastrostomy. In all cases we found motility disorders. Two patients (12.5%) showed incomplete relaxation of the upper esophageal sphincter. The resting pressure of the esophageal body in both segments was constantly positive in all cases. Lower esophageal sphincter (LES) function was normal in all but two patients (16.7%) in whom the LES pressure was reduced and one case (8.4%) with incomplete relaxation of the LES. These studies suggest that motility disorders are also present in esophageal atresia before surgery.