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Published in: Pediatric Surgery International 9/2016

01-09-2016 | Original Article

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

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

Published in: Pediatric Surgery International | Issue 9/2016

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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.
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Metadata
Title
Perinatal features of congenital cystic lung diseases: results of a nationwide multicentric study in Japan
Authors
Tatsuo Kuroda
Eiji Nishijima
Kosaku Maeda
Yasusih Fuchimoto
Seiichi Hirobe
Yuko Tazuke
Toshihiko Watanabe
Noriaki Usui
Japanese Study Group of Pediatric Chest Surgery
Publication date
01-09-2016
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Surgery International / Issue 9/2016
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI
https://doi.org/10.1007/s00383-016-3930-1

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