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

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...

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Bibliographic Details
Published in:Pediatric surgery international 2017, Vol.33 (1), p.105-108
Main Authors: Lau, C. T., Kan, A., Shek, N., Tam, P., Wong, K. K. Y.
Format: Article
Language:English
Subjects:
China - epidemiology
Cystic Adenomatoid Malformation of Lung, Congenital - diagnosis
Cystic Adenomatoid Malformation of Lung, Congenital - epidemiology
Female
Humans
Incidence
Infant
Infant, Newborn
Lung - abnormalities
Male
Medicine
Medicine & Public Health
Original Article
Pediatric Surgery
Pediatrics
Pregnancy
Prenatal Diagnosis - methods
Prospective Studies
Rare Diseases
Registries
Surgery
Ultrasonography, Prenatal
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Summary: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.
ISSN:0179-0358
1437-9813
DOI:10.1007/s00383-016-3991-1