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Is congenital pulmonary airway malformation really a rare disease? Result of a prospective registry with universal antenatal screening program

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Abstract

Background

Congenital pulmonary airway malformation (CPAM) is an increasingly recognized disease with potential mortality. Owing to limited published studies, the true incidence is yet to be determined. We carried out this prospective study with the aim to estimate its true incidence on a population basis.

Methods

An antenatal ultrasonography program was implemented since 2009. Fetuses with suspected intra-thoracic lesions were monitored by regular follow-ups. Antenatal course, postnatal outcomes, and other demographics were compared to those of patients with CPAM in the previous decades (1989–2008). The incidence of CPAM was calculated in different periods.

Results

66 CPAM patients were identified between 2009 and 2014 with 62 patients being detected by antenatal scan. In contrast, 45 patients were identified between 1989 and 2008 with 27 patients being detected antenatally. The incidence rate during the past and recent period was estimated as ~1 in 27,400 and ~1 in 7200 live births, respectively (p = 0.024).

Conclusion

With increasing awareness of clinicians and the universal use of latest ultrasound technology, it is likely that more CPAM cases will be detected in the future. Here, we presented our best estimated incidence rate of CPAM, yet only a larger scale study can reveal its true incidence.

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References

  1. Ch’In KY, Tang MY (1949) Congenital adenomatoid malformation of one lobe of a lung with general anasarca. Arch Pathol 48(3):221–229

    Google Scholar 

  2. Stocker JT, Madewell JE, Drake RM (1977) Congenital cystic adenomatoid malformation of the lung. Classification and morphologic spectrum. Human Pathol 8(2):155–171

    Article  CAS  Google Scholar 

  3. Stocker JT (2009) Cystic lung disease in infants and children. Fetal Pediatr Pathol 28(4):155–184

    Article  PubMed  Google Scholar 

  4. Adzick NS, Harrison MR, Glick PL, Golbus MS, Anderson RL, Mahony BS, Callen PW, Hirsch JH, Luthy DA, Filly RA et al (1985) Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history. J Pediatr Surg 20(5):483–488

    Article  CAS  PubMed  Google Scholar 

  5. Rothenberg SS, Kuenzler KA, Middlesworth W, Kay S, Yoder S, Shipman K, Rodriguez R, Stolar CJ (2011) Thoracoscopic lobectomy in infants less than 10 kg with prenatally diagnosed cystic lung disease. J Laparoendosc Adv Surg Tech Part A 21(2):181–184. doi:10.1089/lap.2010.0138

    Article  Google Scholar 

  6. Laberge JM, Flageole H, Pugash D, Khalife S, Blair G, Filiatrault D, Russo P, Lees G, Wilson RD (2001) Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience. Fetal Diagn Ther 16(3):178–186

    Article  CAS  PubMed  Google Scholar 

  7. Gornall AS, Budd JL, Draper ES, Konje JC, Kurinczuk JJ (2003) Congenital cystic adenomatoid malformation: accuracy of prenatal diagnosis, prevalence and outcome in a general population. Prenat Diagn 23(12):997–1002. doi:10.1002/pd.739

    Article  CAS  PubMed  Google Scholar 

  8. Lau CT, Leung L, Chan IH, Chung PH, Lan LC, Chan KL, Wong KK, Tam PK (2013) Thoracoscopic resection of congenital cystic lung lesions is associated with better post-operative outcomes. Pediatr Surg Int 29(4):341–345. doi:10.1007/s00383-012-3243-y

    Article  CAS  PubMed  Google Scholar 

  9. McCullagh M, MacConnachie I, Garvie D, Dykes E (1994) Accuracy of prenatal diagnosis of congenital cystic adenomatoid malformation. Arch Dis Child 71(2):F111–F113

    Article  CAS  PubMed  Google Scholar 

  10. Laje P, Liechty KW (2008) Postnatal management and outcome of prenatally diagnosed lung lesions. Prenat Diagn 28(7):612–618. doi:10.1002/pd.1966

    Article  PubMed  Google Scholar 

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Acknowledgments

CTL performed data analysis and wrote the manuscript under supervision of KW and PT. AK and NS performed antenatal ultrasound screening. All authors participated in joint antenatal counseling. CTL, KW, and PT performed surgery for patients postnatally.

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Authors and Affiliations

  1. Department of Surgery, The University of Hong Kong, Queen Mary Hospital, Pokfulam Road, Hong Kong SAR, China

    C. T. Lau, P. Tam & K. K. Y. Wong

  2. Department of Obstetrics and Gynaecology, The University of Hong Kong, Queen Mary Hospital, Pokfulam Road, Hong Kong SAR, China

    A. Kan & N. Shek

Authors
  1. C. T. Lau
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  2. A. Kan
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  3. N. Shek
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  4. P. Tam
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  5. K. K. Y. Wong
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Corresponding authors

Correspondence to C. T. Lau or K. K. Y. Wong.

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The authors have no conflicts of interest to declare.

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Lau, C.T., Kan, A., Shek, N. et al. Is congenital pulmonary airway malformation really a rare disease? Result of a prospective registry with universal antenatal screening program. Pediatr Surg Int 33, 105–108 (2017). https://doi.org/10.1007/s00383-016-3991-1

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  • DOI: https://doi.org/10.1007/s00383-016-3991-1

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