The outcome of transposition surgery has been revolutionized during the past two decades with the arterial switch operation. Important contributions to this strategy have come from Europe, the USA, Japan, South America, and Australia. This paper will serve as an update on some issues relating to the arterial switch operation, including perioperative support, postoperative management, and surgical strategies for various anatomic subgroups. In this review we analyse indications, techniques, and outcome for various TGA subsets, including patients with intact ventricular septum beyond 21 days of age, intramural coronary arteries, aortic arch obstruction, the Taussig Bing anomaly, discordant (corrected) transposition, TGA with LVOTO, and univentricular hearts with TGA and SAS.

View full abstract

Wilms' tumor, the most common renal tumor of childhood, is a triphasic embryonal malignancy. The first report of what is now known as Wilms' tumor is a bilateral tumor described by Hunter in 1793. A number of reports of what appear to be Wilms' tumors were described over the next century under a variety of names. A comprehensive review by Birch-Hirschfeld and Doberlein in 1894 collected the previously reported cases including the first 47 attempted nephrectomies for suspected renal malignancy in childhood to that date. They were the first to recognize that the previously reported terms described the same entity and for a while these tumors were called Birch-Hirschfeld tumors. Five years later, in 1899, Carl Max Wilhelm Wilms published the monograph Die Misch-geschwulste der Niere, in which he recognized that all tissues present in the tumor developed from the same germ cell. He added seven new cases and reviewed the literature with credit to Birch-Hirschfeld and Doberlein. His unifying theory of the origin of nephroblastoma has persisted and recently been confirmed at the molecular genetics level. Since the time of his monograph the treatment of Wilms' tumor is a tribute to multidisciplinary treatment and study. At the turn of the century, survival was approximately 5% due to the rare survivor of nephrectomy. By 1920 the advent of radiotherapy had increased survival to 15%. Between 1920 and 1960 survival rates increased to nearly 70% due to improvements in surgical, anesthetic, and radiotherapeutic techniques. Since 1960 survival has increased to over 90% due to advances in chemotherapy and refinements of therapy resulting primarily from the collaboration of multi-institutional, multi-disciplinary groups in North America (National Wilms' Tumor Study Group, NWTSG) and Europe (Societe Internationale d'Oncologie Pediatrique, SIOP). In North America the fifth NWTSG study is currently in progress. The first four studies identified histological categories of favorable and unfavorable variants, demonstrated the ability of chemotherapy to eradicate undetected residual disease in patients with Stage I and II favorable histology and those with Stage I unfavorable disease due to anaplasia without the addition of abdominal irradiation. The efficacy of doxorubicin added to vincristine and dactinomycin was established in Stage III and IV tumors with favorable histology. NWTS-4 demonstrated that a single dose regimen of doxorubicin and dactinomycin was equivalent to a divided-dose regimen and that 6 months of chemotherapy was as effective as 15 months. The 2 year survival in NWTS-4 was 98% for those with favorable histology and 95% for those with unfavorable histology. The SIOP studies have evaluated the value of throughout the world.

View full abstract

Children with SBS have a high morbidity and mortality rate despite improvements in both enteral and parenteral nutrition. Improved medical treatment must sometimes be combined with surgical procedures including intestinal lengthening and transplantation, thereby improving the status of these children.

View full abstract

Purpose : Prolonged hospitalization is an important problem in delayed appendectomy (DA). This study was undertaken to research the possibility of decreasing the length of hospital stay for DA. Methods : Twenty-two patients were evaluated with respect to duration between the start of treatment and normalization on CRP value and WBC counts. The patients were divided into two groups : one (FG) underwent DA during initial admission and other (LG) upon second admission. Results : Conservative treatment was performed on 19 patients and abscess drainage on 3. The time required to normalize CRP value and WBC counts in the patients treated using drainage was shorter than that with conservative treatment. The interval between the start of treatment and the normalization of these parameters was about 10 days for both groups, and the administration of antibiotics was discontinued at 2 weeks after the start of treatment. Hospital stay to the date of DA after the normalization of parameters was 2 to 3 weeks for AG and 1 to 2 weeks for LG. After DA, 10 days were spent on course observation without treatment for AG and 6 for LG. Conclusion : Our data demonstrates that it is important to adopt prompt abscess drainage and eliminate course observation without treatment in order to decrease the length of hospital stay.

View full abstract

We report a case with anastomotic ulcer diagnosed nine years after the first ileocolic resection. The bowel, from the distal ileum to the transeverse colon, was resected for sclerosing mesenteritis when the patient was eight years old. Nine years later, he first noticed rectal bleeding and was admitted to our hospital. A colonoscopic evaluation revealed bleeding from an ulcer at the ileocolic anastomotic site and endoscopic hemostasis was performed. As the ulcer had progressed and bleeding had persisted over a couple of days, segmental resection of the affected bowel and an end-to-end ileo-colostomy were performed again. Three months later, a recurrent ulcer was found and anti-inflammatory drugs administered afterwords healed the ulcer. An anastomotic ulcer after ileocolic resection during childhood is an uncommon complication and 19 cases have been reported in literature.

View full abstract

[in Japanese]

View full abstract