先天性肺形成異常 (無形成), 心奇形を伴った食道閉鎖症の2例

概要

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Case one : A 3-day-old girl with birth weight of 2.3kg was admitted for esophageal atresia and ano-rectal malformation. Chest X-ray showed complete opacification of the right lung field. Gastrostomy and cut-buck operation were performed. Inspite of vigorous treatment, she died of respiratory failure associated with septic shoch on 9th day. Autopsy revealed esophageal atresia and right lung agenesis with ectoplastic lung tissue arising from lower esophagus. Case two : A 1-day-old boy with birte weight of 1.9kg was admitted for esophageal atresia and RDS. Gastrostomy and lower esophageal banding were performed immediately. After recovering from RDS, primary anastomosis of the esophagus with division of TEF and removal of the banding tape were succeeded on the 5th day of life. Post operative course was complicated with both respiratory and cardiac failure, but he recovered by intensive treatement including "High Frequency Jet Ventilation". The right pulmonary agenesis was confirmed by lung scintiscanning and CT. He also had ASD, PDA and still have severe GER at 7 months of age.
日本小児外科学会の論文
1989-04-20