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.
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.
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.