Skip to main content

Advertisement

SpringerLink
Log in

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

  • Original Article
  • Published:
Pediatric Surgery International Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Kuroda T, Nishijima E, Maeda K et al (2016) Clinical features of congenital cystic lung diseases; a report on a nationwide multicenter study in Japan. Eur J Pediatr Surg 26:91–95

    PubMed  Google Scholar 

  2. Crombleholme TM, Coleman B, Hedrick H et al (2002) Cystic adenomatoid malformation volume ratio predicts outcome in prenatally diagnosed cystic adenomatoid malformation of the lung. J Pediatr Surg 37:331–338

    Article  PubMed  Google Scholar 

  3. Kuroda T, Morikawa N, Kitano Y, Sago H, Hayashi S, Honna T, Saeki M (2006) Clinicopathological assessment of prenatally diagnosed lung diseases. J Pediatr Surg 41:2028–2031

    Article  PubMed  Google Scholar 

  4. Stocker JT, Madewell JE, Drake RM (1977) Congenital cystic adenomatoid malformation of the lung. Hum Pathol 8:155–171

    Article  CAS  PubMed  Google Scholar 

  5. Stocker JT (1944) Congenital and developmental diseases. In: Dail DH, Hammar SP (eds) Pulmonary pathology, 2nd edn. Springer-Verlag, New York, pp 174–180

    Google Scholar 

  6. Pryce DM (1946) Lower accessory pulmonary artery with intralobar sequestration of lung; a report of seven cases. J Pathol Bact 58:457–467

    Article  CAS  Google Scholar 

  7. Morikawa N, Kuroda T, Honna T et al (2005) Congenital bronchial atresia in infants and children. J Pediatr Surg 40:1822–1826

    Article  PubMed  Google Scholar 

  8. Adzick NS, Harrison MR, Crombleholme TM et al (1998) Fetal lung lesions: management and outcome. Am J Obstet Gynecol 179:884–889

    Article  CAS  PubMed  Google Scholar 

  9. Davenport M, Warne SA, Cacciaguerra S et al (2004) Current outcome of antenatally diagnosed cystic lung disease. J Pediatr Surg 39:549–556

    Article  PubMed  Google Scholar 

  10. Laberge JM, Flageole H, Pugash D et al (2001) Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience. Fetal Diagn Ther 16:178–186

    Article  CAS  PubMed  Google Scholar 

  11. Winters WD, Effmann EL, Ngheim HV et al (1997) Disappearing fetal lung masses: importance of postnatal imaging studies. Pediatr Radiol 27:535–539

    Article  CAS  PubMed  Google Scholar 

  12. Barret J, Chitayat D, Sermer M et al (1995) The prognostic factors in the prenatal diagnosis of the echogenic fetal lung. Prenat Diagn 15:849–853

    Article  CAS  PubMed  Google Scholar 

  13. Van Leeuman K, Teitelbaum DH, Hirschl RB et al (1999) Prenatal diagnosis of congenital cystic adenomatoid malformation and its postnatal presentation, surgical indications, and natural history. J Pediatr Surg 34:794–799

    Article  Google Scholar 

Download references

Acknowledgments

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).

Author information

Authors and Affiliations

  1. Department of Pediatric Surgery, Keio University, School of Medicine, 35 Shinanomachi, Shinjuyku-ku, Tokyo, 160-8582, Japan

    Tatsuo Kuroda

  2. Department of Pediatric Surgery, Takatsuki Hospital, Osaka, Japan

    Eiji Nishijima

  3. Department of Pediatric Surgery, Jichi Medical College, Shimotsuke, Japan

    Kosaku Maeda

  4. Department of Surgery, National Center for Child Health and Development, Tokyo, Japan

    Yasusih Fuchimoto & Toshihiko Watanabe

  5. Department of Surgery, Tokyo Metropolitan Children’s Medical Center, Tokyo, Japan

    Seiichi Hirobe

  6. Department of Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan

    Yuko Tazuke

  7. Department of Pediatric Surgery, Osaka University, Osaka, Japan

    Noriaki Usui

Authors
  1. Tatsuo Kuroda
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Eiji Nishijima
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Kosaku Maeda
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Yasusih Fuchimoto
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Seiichi Hirobe
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Yuko Tazuke
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Toshihiko Watanabe
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Noriaki Usui
    View author publications

    You can also search for this author in PubMed Google Scholar

Consortia

Japanese Study Group of Pediatric Chest Surgery

Corresponding author

Correspondence to Tatsuo Kuroda.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Japanese Study Group of Pediatric Chest Surgery., Kuroda, T., Nishijima, E. et al. Perinatal features of congenital cystic lung diseases: results of a nationwide multicentric study in Japan. Pediatr Surg Int 32, 827–831 (2016). https://doi.org/10.1007/s00383-016-3930-1

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00383-016-3930-1

Keywords

Search

Navigation