全結腸ヒルシュスプルング病の診断治療

概要

論文の詳細を見る
Nine cases with entire colon aganglionosis were reported, of which 5 cases haved survived. Most reliable findings on X-ray studies were ileal dilatation and delayed passage of barium. Histological investigation of the intestinal biopsied specimen during the preliminary operation is essential to make a difinite diagnosis, bacause the change in caliber of the involved ileum is not so reliable as it is in a case with classical Hirschsprung's disease. According to English and Japanese literatures, a great number of cases with entire colon aganglionosis have died before radical operation due to malnutrition and due to fluid imbalance caused by short gut or enteritis. An aggresive supportive measures including intravenous hyperalimentation, are mandatory in the period waiting for the radical operation. Although Martin's operation may be effective to restore the digestive ability, it does not seem to be always to correct all of unphysiological status observed in entire colon aganglionosis.
日本小児外科学会の論文