心内奇型, 横隔膜, 心膜, 胸骨下部欠損を合併する臍上部腹壁形成不全の手術経験

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  • A Syndrome of Congenital Defects Involving the Supraumbilical Abdominal Wall, Sternum, Diaphragm, Pericardium, and Heart : An operative case report of 14 months female infant
  • ココロナイ キケイ オウカクマク シンマク キョウコツ カブ ケッソン オ ガ

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A case of combined congenital defects of the supraumbilical abdominal wall, sterum, diaphragm, pericardiam, and heart is reported. Wide diastasis recti with lateral insertion of the rectus muscles into the lower costal cartilages, combined with an absence of the xyphoid process and the bified lower sternum. The chest roentogenogram showed dextroversion of the heart, and by chest ausculation, III° grade systolic murmur could be audible. Echocardiographically, VSD was suspected. At the age of 14 months, the chest roentogenogram showed the intestinal gas shadow in the mediastinum, so that an operative repair of defects was performed. A large oval defect in the anterior diaphragm with a corresponding defect in the diaphragmatic pericardium was identified. Loops of the transverse colon herniated into the pericardial cavity. A membranous connection between the apex and the right adjasent costal margine, and the abnormal blood supply to the apex from the terminal branch of the right internal mammary artery was present. The transverse colon was redused from the pericardial cavity, and the diaphragmatic pericardial defect was closed, attaching the diaphragmatic membrane to the undersurface of the anterior chest wall. The diastasis recti was repaired by bringing rectus muscles together without the use of relaxing incisions. The postoperative course was uneventful. The clinical features and treatment of the syndrome was discussed.

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