小腸狭窄症状を呈した全身性進行性硬化症の1例

A Case Report of Progressive Systemic Sclerosis Simulating Chronic Intestinal Obstruction

　最近われわれは，5年前に発症した全身性進行性硬化症（Progressive Systemic Sclerosis，以下PSSと略す）症例で，1年前より多彩な消化器症状を伴い，また腸閉塞症を併発し，腸管切除術ならびに術後経中心静脈高カロリー輸液を10カ月間施行したという興味あるpssの1剖検症例を経験した．かかる症例の報告は内外の文献をみてもまれであり，外科医の立場からみたPSSの胃腸病変について若干の文献的考察を加えて報告する．

　A 40 years old woman was admitted to our hospital in Oct. 1974, complaining chiefly of abdominal distension and diarrhea. Five years ago laparotomy had been done for a uterus carcinoma and after discharge she noted onset of Raynaud's phenomena. Her face was equivocal, masklike but otherwise skin was normal. Roentgen studies showed dilatation of the duodenum and jejunum, and narrowed segment of the sigmoid colon. The diagnosis, progressive systemic sclerosis, was made but we considered that the narrowed segment was the result of roentgen therapy after hysterectomy and the small intestinal changes were caused by recurrence of uterus carcinoma. In Nov. 1974, when she had laparotomy, duodenal and jejunal dilatation without mechanical obstruction was found. Only resection of the sigmoid colon was performed. On the 11th postoperative day failure of the sutures occured. Nausea, vomitting and diarrhea continued. She had intravenous hyperalimentation containing glucose amino-acid and fat by supraclavicular route for ten months. In Oct.1975, she died of increasing dyspnoe and atrial tachycardia. At necropsy atrophy and fibrosis of muscular layer of all the alimentary tract, especially marked in duodenum, were seen, but skin involvement was slight. Review of the literature showed that laparotomy was performed in 27 cases of PSS patients. We found that resection of the intestine affected sclerosis and intravenous highcaloric alimentation have good effects in the treatment of PSS.