Proctoring System of Pediatric Laparoscopic Surgery for Choledochal Cyst.

Background: Endoscopic surgery also has been becoming widespread in the field of pediatric surgery. However, most disease treated by pediatric surgery in a single institution are small number of cases. Besides, the variety of operative procedures that need to be performed in this field is quite wide. For these reasons, pediatric surgeons have limited opportunities to perform endoscopic surgery. Therefore, it is difficult to introduce advanced endoscopic surgery at a single local hospital. To educate pediatric surgeons in local hospitals, for widespread advanced pediatric endoscopic surgery safely, and to eliminate the need for patient centralization, we have introduced a proctoring system. We compared the surgical results of our institution, a center hospital, with other local institutions, to investigate the feasibility of our proctoring system. Methods: The experienced pediatric surgeon of our institution visits local hospitals to provide onsite coaching and supervises pediatric surgeons on the learning curve. All patients who underwent laparoscopic cyst excision and hepaticojejunostomy for choledochal cysts, one of the advanced pediatric endoscopic surgeries was retrospectively reviewed. Results: Thirty-four cases were evaluated (14 cases in our institution, 20 cases in 9 other institutions). The procedures of all 34 cases were performed by surgeons with 0-2 cases of experience in the procedure. There were no open conversion cases. There was no significant difference in the operative date. There was 1 case (6.7%) of postoperative complications during hospitalization at our institution and 3 cases (14.3%) at other institutions (P = .47). Two cases of late complications (13.3%) occurred at our institution, whereas 6 cases (28.6%) occurred at other institutions (P = .28). Conclusion: With the proctoring system, the performance and completion of advanced pediatric endoscopic surgery at local institutions was feasible. This has important implications given the ever-growing demand for pediatric endoscopic surgery and the increasing need for competent pediatric endoscopic surgeons.

Malignant Ovarian Steroid Cell Tumor, Not Otherwise Specified, Causes Virilization in a 4-Year-Old Girl: A Case Report and Literature Review.

We report a case of a 4-year-old girl with an ovarian steroid cell tumor, not otherwise specified (SCT-NOS). She was admitted to the hospital with progressing virilization and Cushing's syndrome, which included abnormality of the perineum, hirsutism, hypertrichosis, flushing of face, hoarseness, and weight gain. Blood testing showed a significantly increased testosterone level and slightly increased cortisol level. Computed tomography scan revealed an 8.0 × 5.0 × 5.0 cm tumor of the right ovary. The patient underwent right salpingo-oophorectomy, and pathological examination showed malignant potential. Three courses of bleomycin, etoposide, and cisplatin were administered as postoperative chemotherapy. After tumor resection, her testosterone decreased to undetectable levels. However, during the course of the treatment, the patient suffered from adrenal insufficiency resulting in the need for hydrocortisone replacement therapy. Although SCT-NOS in childhood are typically benign, pathological findings should be carefully observed for potential malignancy. In cases of cortisol-producing SCT-NOS, serum levels should be monitored, and hydrocortisone replacement therapy should be considered before resection.

Using an indwelled test tube as a good "navigator" during enterostomy closure.

Surgeons often have difficulty in identifying a suitable incision line to enter the peritoneal cavity for stoma mobilization during enterostomy closure. A mini-size test tube that is preoperatively placed into the stoma can act as an efficient guide in finding a free area to enter the peritoneal cavity, by supplying efficient counter traction and a palpable marker of the intestinal wall.

High expressions of neuronatin isoforms in favorable neuroblastoma.

Neuroblastoma is a malignant solid tumor of children, which derives from the embryonal sympathoadrenal linage. Clinical cases can vary widely from a favorable to an unfavorable prognosis according to the presence of genetic aberrations, such as MYCN amplification. Our cDNA microarray analysis which compared the gene expressions between favorable and unfavorable neuroblastomas showed a high expression of the neuronatin (Nnat) gene in favorable neuroblastomas. Nnat is highly conserved in mammalian species, and its expression appears in nervous systems from the hindbrain to the peripherals during the prenatal periods. The Nnat mRNA expression, investigated in 63 of neuroblastoma samples by quantitative reverse-transcription polymerase chain reaction, was found to be significantly higher in the favorable prognosis groups than in the unfavorable groups. Nnat is an imprinted gene, and its expression in IMR32 neuroblastoma cell line was up-regulated by treatment with a demethylating agent. High expressions of Nnat isoforms induced in an IMR32 neuroblastoma cell line changed the cell morphology to the extension of the neural processes, which thus indicated the occurrence of cell differentiation. In conclusion, the high expressions of Nnat were found to be associated with good prognoses in neuroblastoma, which might indicate tumor differentiation, and its suppressions in unfavorable tumors are considered to be under epigenetic control.

Superior mesenteric and portal vein thrombosis caused by congenital antithrombin III deficiency: report of a case.

A 50-year-old man presented with a 24-h history of gradually worsening abdominal pain. Enhanced computed tomography showed segmental dilation of the small intestine, wall thickening, and ascites, as well as thrombosis of the superior mesenteric vein (SMV) and portal vein. Thus, an emergency laparotomy was performed, which revealed segmental intestinal infarction caused by the thrombosis in the SMV and portal vein. We resected the necrosed intestine and performed anastomosis. The patient was given intravenous heparin and nafamostat mesilate as anticoagulation therapy. The abdominal pain again recurred 4 days after this operation, necessitating a second laparotomy. Segmental congestion of the intestine was found and another resection was done, after which he recovered rapidly. Blood chemistry subsequently revealed an antithrombin III deficiency, which was confirmed to be inherent, after screening his family. Thus, laboratory testing for these proteins may help define the cause of mesenteric venous thrombosis.

Biological diagnosis for neuroblastoma using the combination of highly sensitive analysis of prognostic factors.

BACKGROUND/PURPOSE: To select the optimal treatment according to the degree of malignancy of neuroblastoma, it is essential to accurately and rapidly identify any genetic abnormalities associated with the prognosis. This study aims to assess the correlation between the combination of prognostic factors and the biologic findings of neuroblastoma using a highly sensitive analysis of prognostic factors. METHODS: In 44 neuroblastoma primary samples, we determined the gene dosages of MYCN and Survivin (as the target of 17q gain) and the expression levels of MYCN, Survivin, and BIN1 using highly sensitive analysis (the quantitative polymerase chain reaction method); furthermore, we assessed the correlation between the combination of their prognostic factors and the biology of neuroblastoma. RESULTS: The gene dosage of MYCN or Survivin was significantly associated with all known prognostic factors. The expression level of MYCN or Survivin was not significantly associated with any prognostic factors, whereas the expression level of BIN1 was significantly associated with 5 of 6 prognostic factors. Regarding the combination of MYCN amplification and 17q gain (the gene dosage of Survivin), and the low expression of BIN1, the rates of advanced stages (stage III or IV) were 100% for the cases with 3 factors, 63% for the cases with 2 factors, 42% for the cases with 1 factor, and 0% for the cases with null factor. Furthermore, the survival rates were 20% for the cases with 3 factors, 50% for the cases with 2 factors, 100% for the cases with 1 factor, and 100% for the cases with null factor. CONCLUSION: The combination of gene dosages of MYCN and Survivin and the expression level of BIN1 using the quantitative polymerase chain reaction method was significantly correlated with the clinical stage and the patients' outcome. This combination of biologic factors may enhance the accuracy to the conventional criteria, but this would have to be shown in a much larger study that is adequately powered to detect such an advantage.