Neonatal surgery is the most specialized and sophisticated field of pediatric surgery. I herein review esophageal atresia, abdominal wall defects, gastrointestinal perforation, and congenital diaphragmatic hernia (CDH) as representative types of neonatal surgery. The clinical results of esophageal atresia have been considered to reflect the level of medicine of an individual country. Owing to an early diagnosis, improved operative techniques, and better perioperative management, the mortality rate has now become almost 0%. In addition, a minimally invasive thoracotomy is considered to improve long-term quality of life. The overall mortality rate of neonatal surgical disease has markedly decreased and is now less than 10%. However, abdominal wall defects, gastrointestinal perforation, and CDH still show a high mortality rate. A high incidence of chromosomal anomalies results in a poor outcome for abdominal wall defect. A gastrointestinal perforation in an infant complicated with an extremely how birth weight shows a high mortality rate. In CDH, pulmonary hypoplasia as well as pulmonary hypertension often causes an acute respiratory and circulatory deterioration after birth. Neither intensive care for pulmonary hypertension, including ECMO, nor fetal intervention has yet achieved a satisfactory outcome. Permissive hypercapnea and a delayed operation aiming at circulatory stabilization have resulted in a good survival rate. However, CDH survivors may be at risk for long-term morbidities. The method to induce pulmonary development is considered to be mandatory to achieve a good quality of life for severe CDH.
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