Abstract
Purpose
For the approximately three quarters of patients with a prenatal diagnosis of congenital pulmonary airway malformation (CPAM) who are asymptomatic at birth, the chance of eventually developing symptoms is unknown. We sought to explore the natural history of asymptomatic CPAM.
Methods
We searched EMBASE, MEDLINE, and the first 50 results from Google Scholar. Studies describing the natural history of prenatally diagnosed, initially asymptomatic CPAM were included. For asymptomatic patients initially managed non-surgically, we tabulated the proportion who went on to develop symptoms as well as the median age at symptom development.
Results
We included data from 19 retrospective studies on 353 patients. Of the 128 patients managed expectantly, 31 (24.2%) developed symptoms requiring surgical intervention. The median age at symptom development was 7.5 months (range 15 days–5 years).
Conclusion
The risk for developing respiratory symptoms exists with originally asymptomatic CPAM patients, but the exact risk is difficult to predict. Parents may be given the value of approximately 1 in 4 as an estimate of the proportion of asymptomatic CPAM patients who go on to develop symptoms, which will help them make an informed decision regarding the option of elective surgery.
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Acknowledgements
We thank Margaret Sampson, MLIS, PhD, AHIP (Children’s Hospital of Eastern Ontario) for developing the electronic search strategies.
Funding
University of Ottawa Faculty of Medicine Studentship.
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Kantor, N., Wayne, C. & Nasr, A. Symptom development in originally asymptomatic CPAM diagnosed prenatally: a systematic review. Pediatr Surg Int 34, 613–620 (2018). https://doi.org/10.1007/s00383-018-4264-y
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DOI: https://doi.org/10.1007/s00383-018-4264-y