TY - JOUR
T1 - Perinatal features of congenital cystic lung diseases
T2 - results of a nationwide multicentric study in Japan
AU - Japanese Study Group of Pediatric Chest Surgery, Study Group of Pediatric Chest Surgery
AU - Kuroda, Tatsuo
AU - Nishijima, Eiji
AU - Maeda, Kosaku
AU - Fuchimoto, Yasusih
AU - Hirobe, Seiichi
AU - Tazuke, Yuko
AU - Watanabe, Toshihiko
AU - Usui, Noriaki
N1 - Funding Information:
The authors are deeply grateful to Ms. Yuko Yamahara of the JCRAC data center for the data management and statistical analysis. This study received a grant from the Rare Diseases Study Project, Ministry of Health and Labor, Japan (No. H24-Rare diseases-general-034).
Publisher Copyright:
© 2016, Springer-Verlag Berlin Heidelberg.
PY - 2016/9/1
Y1 - 2016/9/1
N2 - 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.
AB - 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.
KW - Congenital cystic adenomatoid malformation
KW - Congenital cystic lung disease
KW - Congenital pulmonary airway malformation
KW - Lung lesion volume ratio
KW - Prenatal diagnosis
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U2 - 10.1007/s00383-016-3930-1
DO - 10.1007/s00383-016-3930-1
M3 - Article
C2 - 27461429
AN - SCOPUS:84979697147
VL - 32
SP - 827
EP - 831
JO - Pediatric Surgery International
JF - Pediatric Surgery International
SN - 0179-0358
IS - 9
ER -