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J.Jpn. Surg. Soc.. 124(6): 507-513, 2023

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NATURAL HISTORY OF CONGENITAL CYSTIC LUNG DISEASE: RETROSPECTIVE CLINICAL DATA OBTAINED IN OUR CENTER OVER THE PAST TWO DECADES AND FUTURE PROSPECTS

1) Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, Tokyo, Japan
2) Division of Pulmonology, Department of Medical Specialties, National Center for Child Health and Development, Tokyo, Japan

Yutaka Kanamori1), Goro Koinuma2)

Congenital cystic lung disease (CCLD) is a rare condition that is caused by various types of fetal maldevelopment in the lung. Most forms of the disease are now diagnosed in the fetal stage, and some patients have no respiratory symptoms after birth. Some of those asymptomatic patients undergo surgery, and others are followed conservatively. We analyzed 220 patients with CCLD seen in our center over the past two decades, of whom 136 were diagnosed in the fetal stage. Among them, 33 patients underwent surgery as neonates and 88 underwent elective surgery after 6 months of age. Only 1 patient underwent surgery at the age of 3 months because a left lung cyst that enlarged over a short period. The other 14 patients were followed conservatively according to our treatment strategy, among whom 1 underwent surgery at 4 years of age because of repetitive coughing episodes with exercise. The treatment strategy for asymptomatic CCLD remains controversial, and a prospective large-scale study is needed to establish a definitive treatment strategy for asymptomatic CCLD.

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