Perinatal features of congenital cystic lung diseases: results of a nationwide multicentric study in Japan

Study Group of Pediatric Chest Surgery Japanese Study Group of Pediatric Chest Surgery, Tatsuo Kuroda, Eiji Nishijima, Kosaku Maeda, Yasusih Fuchimoto, Seiichi Hirobe, Yuko Tazuke, Toshihiko Watanabe, Noriaki Usui

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Purpose: The current study aimed to assess the perinatal risk and clinical features of congenital cystic lung diseases (CCLD). Materials and 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.

Original languageEnglish
Pages (from-to)827-831
Number of pages5
JournalPediatric surgery international
Volume32
Issue number9
DOIs
Publication statusPublished - 2016 Sep 1

Keywords

  • Congenital cystic adenomatoid malformation
  • Congenital cystic lung disease
  • Congenital pulmonary airway malformation
  • Lung lesion volume ratio
  • Prenatal diagnosis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery

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  • Cite this

    Japanese Study Group of Pediatric Chest Surgery, S. G. O. P. C. S., Kuroda, T., Nishijima, E., Maeda, K., Fuchimoto, Y., Hirobe, S., Tazuke, Y., Watanabe, T., & Usui, N. (2016). Perinatal features of congenital cystic lung diseases: results of a nationwide multicentric study in Japan. Pediatric surgery international, 32(9), 827-831. https://doi.org/10.1007/s00383-016-3930-1