Literature DB >> 27770196

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

C T Lau1, A Kan2, N Shek2, P Tam3, K K Y Wong4.   

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.

Entities:  

Keywords:  Antenatal; Congenital pulmonary airway malformation; Screening; Ultrasound

Mesh:

Year:  2016        PMID: 27770196     DOI: 10.1007/s00383-016-3991-1

Source DB:  PubMed          Journal:  Pediatr Surg Int        ISSN: 0179-0358            Impact factor:   1.827


  10 in total

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5.  Thoracoscopic lobectomy in infants less than 10 kg with prenatally diagnosed cystic lung disease.

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7.  Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history.

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8.  Accuracy of prenatal diagnosis of congenital cystic adenomatoid malformation.

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10.  Congenital cystic adenomatoid malformation of the lung. Classification and morphologic spectrum.

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  10 in total
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Review 7.  Developmental Pathways Underlying Lung Development and Congenital Lung Disorders.

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Review 10.  Narrative review of congenital lung lesions.

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  10 in total

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