J.Jpn. Surg. Soc.. 124(6): 472-477, 2023

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DISEASE CONCEPT UPDATE

Congenital cystic lung disease (CCLD) includes several disease entities with different embryological backgrounds. Guidelines on CCLD recently developed in Japan divide CCLD into five major categories based on embryology：1) congenital pulmonary airway malformation (CPAM)； 2) lung bud anomalies； 3) foregut anomalies；4) bronchial atresia； and 5) others. This novel classification aims to avoid the overlapping of categories and clarify the clinical features of each. A certain cohort of CCLD, especially CPAM, is known to result in intrauterine fetal death and respiratory distress immediately after birth. The lung lesion volume index and MRI assessment have been reported to be predictive of perinatal risk, although none of the indices are yet reliable enough to indicate the necessity for fetal surgery. The etiology of CPAM is widely recognized as suspension or delay of lung development during organogenesis. On the other hand, a totally new concept has been proposed as the etiology of CPAM. The concept of bronchopulmonary foregut malformation, a CCLD type with communication between the airway and gastrointestinal tract, remains unestablished. Future studies are required to establish the concept of CCLD.

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