Clinical Features of Congenital Cystic Lung Diseases: A Report on a Nationwide Multicenter Study in Japan.
OBJECTIVE
The current study aimed to assess the perinatal risk of congenital cystic lung diseases (CCLD) and to establish a suitable surgical strategy in consideration of postoperative lung growth and problems during adulthood.


METHODS
Among 874 CCLD patients identified in a nationwide survey, 428 patients were born between 1992 and 2012 and treated at 10 high-volume centers were retrospectively reviewed with statistical analysis.


RESULTS
In the 194 patients who were prenatally diagnosed to have CCLD, 16.7% presented with fetal hydrops as observed using ultrasonography. The Apgar score (5 minutes) was lower than 5 in 5.4%. As of postnatal day 30, 14.0% of the neonatal patients required respiratory support, and 3.3% (8/243) had died because of pulmonary hypoplasia. Among those who were asymptomatic immediately after birth, 33.6% of the patients developed the respiratory infectious symptoms during their first year of life, and 22.1% did so between the age of 1 and 2 years. The postoperative percent vital capacity among the prenatally diagnosed patients was significantly higher than that among the postnatally diagnosed patients (98.3 ± 11.9 vs. 81.7 ± 9.7, p < 0.0222). Late complications included thoracic deformity in 30 patients and persistent lung cyst in 4 patients, whereas malignancy was not observed in the present series.


CONCLUSIONS
Approximately 10 to 15% of prenatally diagnosed CCLD patients may carry a high risk of perinatal respiratory distress. Early operation before developing episodes of lung infection, seem to be associated with a better development of the reserved lung during later life. The incidence of carcinogenesis among patients with CCLD may be extremely low.
Department of Pediatric Surgery, School of Medicine, Keio University, Tokyo, Japan.
Tatsuo
T
Kuroda
Department of Pediatric Surgery, Takatsuki Hospital, Takatsuki, Japan.
Eiji
E
Nishijima
Department of Pediatric Surgery, Hyogo Prefectural Kobe Children's Hospital, Kobe, Japan.
Kosaku
K
Maeda
Department of Pediatric Surgery, National Center for Child Health and Development, Tokyo, Japan.
Yasushi
Y
Fuchimoto
Department of Pediatric Surgery, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
Seiichi
S
Hirobe
Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.
Yuko
Y
Tazuke
Department of Radiology, Kanagawa Children's Medical Center, Kanagawa, Japan.
Kumiko
K
Nozawa
Department of Pediatric Surgery, National Center for Child Health and Development, Tokyo, Japan.
Kentaro
K
Matsuoka
Department of Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
Noriaki
N
Usui
D058070:Asymptomatic Diseases; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000150:complications / Q000175:diagnosis* / Q000401:mortality / Q000601:surgery; D042241:Early Diagnosis; D005260:Female; D005500:Follow-Up Studies; D006306:Health Surveys; D006801:Humans; D007231:Infant, Newborn; D007564:Japan; D008297:Male; D011013:Pneumonectomy / Q000379:methods; D011183:Postoperative Complications / Q000453:epidemiology; D011247:Pregnancy; D011296:Prenatal Diagnosis; D012189:Retrospective Studies