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.

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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.

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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.

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【目的】待機手術(DA)施行例の炎症消退と治療日数との関係を調べ, 入院期間短縮の可能性を検討する.【対象】DA施行例(22例)を, 初回入院後保存療法を行い約4週間で手術した前期群(9)といったん退院の後3∿4カ月後に再入院し虫垂を切除した後期群(13)に分け比較, 検討した.【結果】保存療法は19例に, ドレナージは3例に行われ, 炎症消退までの日数はドレナージ例で有意に短かった.治療開始後炎症消退までに前・後期共に約10日(抗生剤投与は12日)を要したが.それ以上に無治療下の経過観察は長かった(前期でDA前に約2.5週間, 後期で1.5週間).DA時の残存炎症は後期が前期に比べ軽度で, 合併症も少なかった.【結論】待機手術は二期的に行うべきで, 入院期間の短縮は膿瘍ドレナージ, 無治療経過観察の排除およびday surgeryに準じた予定手術への組み込みなどにより可能と考える.

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8歳時に硬化性腸管膜炎のため回盲部を含む回腸結腸切除術が行われた男児の1症例である.17歳時, 血便・下血の精査にて入院.回腸結腸吻合部潰瘍からの動脈性出血を認めたため, 吻合部切除が行われた.術後3カ月時の大腸内視鏡検査では, 吻合部より口側1 cmに潰瘍の再発をきたしたが, 9カ月後には潰瘍の瘢痕化を認めた.小児期に行った回腸結腸切除術後の吻合部に遅発性潰瘍が発症し, さらに病変部腸管切除術後も早期より再吻合部に潰瘍の再発をきたした稀な症例を経験したので文献的考察を含めて報告する.

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症例は出生前診断で羊水過多, 両側上肢奇形を指摘され, 37週1日, 自然経腟分娩で出生した男児, C型食道閉鎖と診断され生後2日目に胃瘻造設術を行った際にcongenital microgastriaを診断された.生後4カ月時に食道閉鎖根治術を予定したが術中病態悪化のため気管食道瘻(TEF)切離のみで終わった.生後8カ月時急性呼吸不全で死亡した.病理解剖で小胃症の他, ファロー四徴症, 異所性右鎖骨下動脈, 両側肺分葉不全, 右中葉無形成, 食道閉鎖, 口側食道盲端に異所性胃粘膜, 右嚢胞性異形成腎, 両側横隔膜筋性部部分欠損, 多発性肝内異所性膵組織が証明された.microgastriaは極めてまれな先天性異常で多くは致死的先天性奇形を合併する.また肝内異所性膵との合併の報告は検索し得ない.我々の症例は右鎖骨下動脈走行異常が死因に関与したと考えられる.

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