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Pediatr Surg Int. 2018 Jun;34(6):613-620. doi: 10.1007/s00383-018-4264-y. Epub 2018 Apr 9.

Symptom development in originally asymptomatic CPAM diagnosed prenatally: a systematic review.

Author information

1
Department of Surgery, Children's Hospital of Eastern Ontario, 401 Smyth Rd, Ottawa, ON, K1H 8L1, Canada.
2
Faculty of Medicine, University of Ottawa, Ottawa, Canada.
3
Department of Surgery, Children's Hospital of Eastern Ontario, 401 Smyth Rd, Ottawa, ON, K1H 8L1, Canada. anasr@cheo.on.ca.
4
Faculty of Medicine, University of Ottawa, Ottawa, Canada. anasr@cheo.on.ca.

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.

KEYWORDS:

CPAM; Congenital lung lesion; Pulmonology; Surgery; Systematic review

PMID:
29632964
DOI:
10.1007/s00383-018-4264-y
[Indexed for MEDLINE]

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