2．先天性横隔膜ヘルニア

Current Management of Congenital Diaphragmatic Hernia

横隔膜は4つの胚性要素が癒合して発生するが，先天性横隔膜ヘルニア（congenital diaphragmatic hernia：CDH）は，その発生過程の異常により横隔膜に欠損が生じ，腹腔内臓器が胸腔内へ脱出する疾患である．発生部位により，欠損孔が横隔膜の背外側を中心に発生する胸腹裂孔（Bochdalek孔）ヘルニア，胸骨背部の横隔膜胸骨部と肋骨部の境界部から前縦隔に発生する傍胸骨裂孔（右側：Morgagni孔，左側：Larry孔）ヘルニア，食道裂孔ヘルニアの大きく三つに分類される．一般的には，胸腹裂孔ヘルニアをさして先天性横隔膜ヘルニアと呼ぶことが多い．

Bochdalek hernia is the most common type of congenital diaphragmatic hernia （CDH）. Patients with CDH often develop cardiopulmonary dysfunction secondary to pulmonary hypoplasia and pulmonary hypertension, which necessitates emergency surgery. Advanced cardiopulmonary management is essential in such cases；therefore, patients should be treated at a well-facility that is familiar with the treatment of CDH. Respiratory management using gentle ventilation and control of pulmonary hypertension using nitric oxide are necessary for preoperative stabilization of patients. Intraoperatively, the hernia should be cautiously reduced and contents should be returned into the abdominal cavity. Surgery is performed to secure the circumference of the rim and ensure firm closure of the defect. Thoracoscopic surgery may be considered for late-onset cases with a stable general condition.