title: Is congenital pulmonary airway malformation really a rare disease? Result of a prospective registry with universal antenatal screening program.
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).
  
  
  CONCLUSIONS
  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.
authors: Department of Surgery, The University of Hong Kong, Queen Mary Hospital, Pokfulam Road, Hong Kong SAR, China. eugenelauct@hotmail.com.
authors: C T
authors: http://orcid.org/0000-0001-6252-9362
authors: CT
authors: Lau
authors: Department of Obstetrics and Gynaecology, The University of Hong Kong, Queen Mary Hospital, Pokfulam Road, Hong Kong SAR, China.
authors: A
authors: A
authors: Kan
authors: Department of Obstetrics and Gynaecology, The University of Hong Kong, Queen Mary Hospital, Pokfulam Road, Hong Kong SAR, China.
authors: N
authors: N
authors: Shek
authors: Department of Surgery, The University of Hong Kong, Queen Mary Hospital, Pokfulam Road, Hong Kong SAR, China.
authors: P
authors: P
authors: Tam
authors: Department of Surgery, The University of Hong Kong, Queen Mary Hospital, Pokfulam Road, Hong Kong SAR, China. kkywong@hku.hk.
authors: K K Y
authors: KK
authors: Wong
mesh_terms: D002681:China / Q000453:epidemiology; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000175:diagnosis* / Q000453:epidemiology; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000002:abnormalities*; D008297:Male; D011247:Pregnancy; D011296:Prenatal Diagnosis / Q000379:methods*; D011446:Prospective Studies; D035583:Rare Diseases; D012042:Registries; D016216:Ultrasonography, Prenatal
keywords: Antenatal; Congenital pulmonary airway malformation; Screening; Ultrasound