一次元T.L.C.法によるC.A.代謝産物7種の同時分離定量法を考案し, 今回, 30例の神経芽細胞腫患児の尿を検体とし, とくに5種類の代謝産物について検討を行った.1.神経芽細胞腫における各C.A.代謝産物の異常検出率は, VMA65%, HVA2%, DOPAC76.9%, MHPG30.8%, MHPE30.8%であり, 通常2種類以上が異常高値を示した.診断に際しては, VMAとともに, 特にHVA, DOPACといったD.A.系代謝産物の測定が重要と思われた.2.神経芽細胞腫患児尿中のC.A.代謝産物排泄パターンは極めて多彩であり, 腫瘍内で行われるC.A.産生とカタボリズムが極めて旺盛かつ異常としてとらえられたが, D.A.系, NE系両者の異常程度が総じて著るしかった症例11例中10例は副腎を原発とする神経芽細胞腫であった.3.神経芽細胞腫26例中, VMA低値であった12症例(A群)のC.A.代謝につき, 他の代謝産物の尿中排泄パターンと, 腫瘍内酵素活性の面から, 考えられる腫瘍内C.A.異常代謝パターンについて考察した.

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昭和37年より昭和53年11月までに千葉大学第二外科, 小児外科で経験したWilms腫瘍は27例である.このうち23例を検索対象とし, 腫瘍の正常腎組織への浸潤度を三段階に分類して従来の諸因子と比較検討した結果, 次の結論を得た.1.腫瘍重量600g以下のものでは浸潤度の低いものが多い.2.glomerular型, tubular型の量を分化度の指標とすると, 分化度の高いものは浸潤度が低い傾向にある.3.浸潤度の高い腫瘍は血管侵襲を起しやすく, また肺転移をきたしやすい.4.浸潤度と手術時年齢, 病期分類, 組織型及び被膜侵襲とは相関しないと考えられる.5.この浸潤度分類は予後推定の因子として今後十分活用することが出来る.本論文の要旨は第16回日本癌治療学会総会において発表した.

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The anorectal resting pressure and defecation sensory pressure were measured in 61 infants and children consisting of 18 with Hirschsprung's disease, 6 with suspected Hirschsprung's disease, 19 with imperforate anus, and 22 controls. The results obtained were as follows : 1) Anorectal reflex was observed in all controls and the patients with suspected Hirschsprung's disease. 2) The reflex was not observed in any of the patients with Hirschsprung's disease. 3) The rectal pressure, anal canal pressure, and pressure difference between rectum and anal canal of the patients with Hirschsprung's disease were similar to those of the controls. The anal canal pressure and pressure difference between rectum and anal canal of the patients with suspected Hirschsprung's disease were higher than those of the controls. 4) Anorectal reflex was observed in 38% of the surgically treated Hirschspung's disease patients. There was no close correlation between clinical improvement and occurrence of the reflex. 5) The rectal pressure of the patients with Hirschsprung's disease measured after operation was hardly different from that obtained before operation. The anal canal pressure and pressure difference between rectum and anal canal of the patients with Hirschsprung's disease taken after operation were lower than those measured before operation; and they were lower in patients who showed excellent clinical improvement than in those who did not. 6) After operation for imperforate anus, anorectal reflex was observed in patients with low anomalies, but not in patients with intermediate or high anomalies. Clinical improvement was excellent in the patients who had postoperative reflex. 7) After operation for imperforate anus, the rectal pressure did not differ according to type of anomaly or clinical results. The anal canal pressure and pressure difference between rectum and anal canal were lower in patients with hign or intermediate anomalies and with poor surgical results. 8) The defecation sensory pressure of the patients with Hirschsprung's disease was similar to that of the controls, and was lower than the pressure difference between rectum and anal canal. (normal relation : defecation sensory pressure<pressure difference between rectum and anal canal.) The defecation sensory pressure of the surgically treated imperforate anus patients was similar to that of the controls except for those with high anomalies. In patients with good surgical results, the sensory pressure was lower than the pressure difference (normal relation). In patients with incontinentia, the sensory pressure was higher than the pressure difference (reversed relation).

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(1)幼若乳児期の鼠径ヘルニア手術創感染に起因した後天性臍瘻の2例を経験した.生後6ヵ月以内の幼若乳児期は免疫, 解剖の面から考えても創感染を合併しやすく, 臍瘻へ発展しやすいと考えられた.(2)この時期の手術は容易に行うべきでないが, やむなく手術を行うときは創感染の防止に細心の注意を払うことが大切である.そのためには, 無駄のない手術操作, 術式とともに縫合糸の材質についても検討されてしかるべきである.(3)創感染に対する免疫防御機構の不充分なこの時期の鼠径ヘルニア手術後は, 充分な抗生物質投与を行った方がよいと考える.

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小児に発生する平滑筋肉腫は極めて稀である.現在までの文献的考察の結果では, 性差はなく, 乳児期の発生が比較的多い.原発部位はさまざまであるが, 成人と異なり, 性器発生はない.消化器や表在性軟部組織原発例の予後が比較的良好であるのに比し, 泌尿器や後腹膜原発例のそれは極めて悪い.再発が非常に多く, 治療には, 早期の根治手術を望むことはもちろん, 積極的な後療法の実施が必要である.ADMは本腫瘍に比較的有効と考えられ, 今後, これらによる術後化学療法に対して予後改善のための検討が加えられるべきであろう.

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2か月男児の外鼠径ヘルニヤ根治術及び1歳10ヵ月男児の睾丸固定術の際, 偶然に副々腎皮質結節を発見した.本症は小児では稀有なものではないが, 本邦では報告が少く, 発見率も明らかでない.小児外科的手術の際に本症の存在を知り, 注意深い観察をすれば, より多く発見されるものと思われる.

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In general lymphangioma, if infected, may have a specific feature of swelling. We have experienced two cases of retroperitoneal lymphangioma which showed positive inflammatory findings by laboratory work-up. In one case, a one-year-old girl, lymphangioma was found attached medially to the ascending colon, and in another case, a four-year-old boy, it was found in the retroperitoneal space near the pancreatic tail. By antibiotherapy the tumors both subsided in size, to a great extent in the former case, and to some extent in the latter case. Large retroperitoneal lymphangiomas being reduced in size, the operative maneuvers were rather simple, and complete removal of lymphangiomas could be accomplished.

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A five years old boy was admitted to the Children's Medical Center of Osaka City with abdominal pain lasting for a weak. He had been affected by thrombosis of the superior sagital sinus 10 months prior to this admission. He was explored on an emergency base soon after admission. Operative findings disclosed a presence of superior mesenteric venous thrombosis with no specific cause. The infarcted jejunum was removed and an end to end anastomosis was performed. Inspite of a postoperative anticoagulant therapy, a reccurence of venous thrombosis distal to the initial anastomosis required the second operation 5 days after. Anticoagulant therapy had to be discontinued, because of multiple reccurrences of intra abdominal hematoma which required re-explorations. As the causes of mesenteric venous thrombosis, hypercoagulability due to oral contraceptives, platelet hyperfunction, low Antithrombin III, low fibrinolytic activity in vein itself, have been proposed in the recent reports. In our case, an elevated antifibrinolytic factors (α_2-Macroglobulin and α_1-Antitripsin) were determined as significance, which was considered as one of the potential causative factors of mesenteric venous thrombosis. Since determination of coagulative and fibrinolytic factors has been difficult, these were hardly considered as the cause of mesenteric venous thrombosis. Recent improvement of labo ratory techniques enabled us to detect these abnormalities. Our experience suggested that an elevated antifibrinolytic factors may be a cause of mesenteric venous thrombosis.

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Most of the congenital diaphragmatic hernia can be easily classified into three major categories from the location of the hernia orifice, but in a case with atypical location of the orifice and with non-muscular membranous sac, one may be embarrassed to define it. This is a case report of one-day-old female infant with a left sided diaphragmatic hernia whose initial symptom was hematemesis. She started to vomit caffee ground material from the next day of life. There was neither dyspnea nor cyanosis. A scout film of the abdomen revealed a large gas shadow with an air-fluid level in the upper quadrant of the abdomen (Fig. 1). No other abdominal gas shadow was visible except in the rectum. Gastrografin examination disclosed the dilated esophagus with tapered narrowing in the distal end and the up-sidedown stomach with complete obstruction of the outlet showing a beak (Fig. 3). At laparotomy, the anterior portion of the left diaphragm was thin and membranous but the posterior half was seemed normal. The anterior half was imbricated and sutured to the posterior half of the diaphragm (Fig. 5). Excised portion of the anterior half of the diaphragm had no muscular layer. The postoperative course of the patient was uneventful. There are many arguments about the histological findings and the definition of localized eventration of the diaphragm and diaphragmatic hernia with sac. It seems unable and unnecessary to distinguish these two conditions clinically. It is postulated that acute volvulus of the stomach is a cause of gastric rupture associated with diaphragmatic hernia including eventration.

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During the past nine years, 48 patients were admitted to our department of pediatric surgery under the suspicion of obstructive jaundice. 28 cases were congenital biliary atresia (C. B. A.) and the other 17 cases were neonatal hepatitis (N. H.). Two cases of C. B. A. were investigated too long time for radical operation for follow up. Nine of the 17 cases, N. H. were diagnosed by means of exploratory laparotomy. Two of these nine cases showed no intrahepatic bile duct by operative cholangiography, but were diagnosed N. H. because of absence of fibrosis of the liver and extrahepatic bile ducts. Satisfactory bile excretion was obtained in all cases. Since there was no significant difference between C. B. A. and N. H. by measuring serum bilirubin, Al-p, GOT, GPT and alfa-fetoprotein, immediate exploratory laparotomy should be performed in case of difficult to diagnose. Considering the close similarity of clinical feature and laboratory data between C. B. A. and N. H. in some cases, Landing's theory of obstructive cholangiopathy is agreeable.

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我々は, 肺転移を伴なうウィルムス腫瘍の3例を経験した.症例1は治療に抗して反復出現する片側肺の孤立性転移の為, 外科的転移巣摘出の適応となった.症例2は両側肺への多発性転移であり, 肝転移も同時に疑われたが, 化学療法及び放射線療法でいずれも消失した.症例3は肺門縦隔リンパ節への転移で化学療法のみでよく反応し治癒した.以上3症例いずれも初回術後6年以上経過した現在再発転移の徴候なく健康である.(本論文の要旨は1977年度小児悪性腫瘍研究会 : 於東京で発表した.)

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我々は生後早期のBochdalekヘルニア14例の肺機能について, 呼吸管理を行う立場から検討し, 以下の結果を得た.1)術前PaCO_2の高い群は術後も高値であった.2)術後のM indexは改善し, PaCO_2高値の群でも充分に低下した.3)これらの推移は患側肺の拡張程度と明らかな相関はみられなかった.4)術後PaCO_2高値の原因は, 術前すでに生じた健側肺の障害によるものと推定され, 本症の呼吸管理は健側肺の機能改善を基本とするべきであると考えられる.

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1)気管食道瘻の再開通は瘻の大きさにより症状は異なる.2)根治術後に慢性気管支炎や頻発する肺炎のみられる場合は, 気管食道瘻の再開通を念頭において食道造影を繰り返す.3)非手術例では80%以上の死亡率を示すため積極的に手術を行う.(手術例でも約50%の死亡率を示す).4)診断がつき次第, 胃瘻造設, transpyloric tubeないし, 空腸瘻による経管栄養を行う.5)全身状態, 肺合併症の改善が得られ次第, 早期に手術を行う.6)瘻切断閉鎖部の間に, 胸膜, 筋膜, 肺などを介在させる.7)術後の食道造影でleakageがあり, 肺炎や気管支炎を繰り返し, leakageと異なる瘻が発見されるものは, たとえ気管との交通が食道造影で証明されなくとも試験開胸を考える必要がある.

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昭和46年〜昭和51年の6年間に, 神経芽細胞腫566例, 神経節細胞腫18例の計584例(年平均97例)が登録されている。このうち, 外科側追加記載は登録症例の94%神経芽細胞腫記載は85%である。登録症例の84%は0才〜4才児であり, 0才児に最も多く, 年令の進むに従って発生頻度は減少してくる。性別では男児が54%と女児よりやや多い。

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