title: Perinatal features of congenital cystic lung diseases: results of a nationwide multicentric study in Japan.
abstract: OBJECTIVE
  The current study aimed to assess the perinatal risk and clinical features of congenital cystic lung diseases (CCLD).
  
  
  METHODS
  Of the 874 CCLD patients identified in a nationwide survey, 428 patients born between 1992 and 2012 and treated at 10 high-volume centers, were retrospectively reviewed.
  
  
  RESULTS
  Fetal hydrops was visualized using MRI in 9.2 % of the patients. Prenatal interventions were described for 221 of the 428 patients, including the maternal administration of steroid and pleuro-amniotic shunting. Postnatally, a right-to-left shunt flow through a persistent ductus arteriosus was observed in 7.8 % of the patients. The fetal lung lesion volume ratio (LVR) was significantly higher among these symptomatic patients (2.04 ± 1.71 vs. 0.98 ± 0.50, P < 0.00071), and decreased to a greater degree in non-CCAM patients compared with CCAM patients during the late gestational period (from 1.37 ± 1.28 to 1.14 ± 0.84 in CCAM and from 1.08 ± 0.47 to 0.46 ± 0.64 in non-CCAM).
  
  
  CONCLUSIONS
  An estimated 8-9 % of prenatally diagnosed patients carry the highest risk of perinatal respiratory distress. Fetal LVR remaining at a high level during the late gestational period seems to predict a high risk.
authors.affiliation: Department of Pediatric Surgery, Keio University, School of Medicine, 35 Shinanomachi, Shinjuyku-ku, Tokyo, 160-8582, Japan. kuroda-t@z8.keio.jp.
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Kuroda
authors.affiliation: Department of Pediatric Surgery, Takatsuki Hospital, Osaka, Japan.
authors.forename: Eiji
authors.initials: E
authors.lastname: Nishijima
authors.affiliation: Department of Pediatric Surgery, Jichi Medical College, Shimotsuke, Japan.
authors.forename: Kosaku
authors.initials: K
authors.lastname: Maeda
authors.affiliation: Department of Surgery, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Yasusih
authors.initials: Y
authors.lastname: Fuchimoto
authors.affiliation: Department of Surgery, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
authors.forename: Seiichi
authors.initials: S
authors.lastname: Hirobe
authors.affiliation: Department of Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
authors.forename: Yuko
authors.initials: Y
authors.lastname: Tazuke
authors.affiliation: Department of Surgery, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Watanabe
authors.affiliation: Department of Pediatric Surgery, Osaka University, Osaka, Japan.
authors.forename: Noriaki
authors.initials: N
authors.lastname: Usui
mesh_terms: D000015:Abnormalities, Multiple; D000328:Adult; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000175:diagnosis* / Q000628:therapy; D005260:Female; D006801:Humans; D015160:Hydrops Fetalis / Q000209:etiology; D007564:Japan; D008279:Magnetic Resonance Imaging; D008297:Male; D011247:Pregnancy; D011296:Prenatal Diagnosis; D012189:Retrospective Studies; D016216:Ultrasonography, Prenatal
keywords: Congenital cystic adenomatoid malformation; Congenital cystic lung disease; Congenital pulmonary airway malformation; Lung lesion volume ratio; Prenatal diagnosis