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A review of congenital lung malformations with a simplified classification system for clinical and research use

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Abstract

Purpose

Congenital lung abnormalities are rare malformations increasingly detected early by prenatal ultrasound. Whether management of these frequently asymptomatic lesions should be surgical or conservative is an unresolved issue. The necessary prospective studies are limited by the absence of a widely accepted practical classification system. Our aim was to develop a simple, clinically relevant system for classifying and studying congenital lung abnormalities.

Materials and methods

We based our proposed grou** on a detailed analysis of clinical, radiological, and histological data from well-documented cases, plus an extensive review of the literature.

Results

The existence of hybrid lesions and common histological findings suggested a unified embryological mechanism—possibly obstruction of develo** airways with distal dysplasia. Malformations could be classified by their anatomical and pathological findings; however, a system based on the prenatal ultrasound plus initial chest X-ray findings had greater clinical relevance: Group 1—Congenital solid/cystic lung malformation, Group 2—Congenital hyperlucent lobe, Group 3—Congenital small lung.

Conclusions

Pathological classification is academically important but is unnecessarily complex for clinical and research use. Our simple radiological-based system allows unambiguous comparison between the results of different studies and also guides the choice of necessary investigations specific to each group.

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Acknowledgements

We would like to thank Dr Geoffrey Blair for his help with the development of this manuscript.

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Correspondence to Michael Seear.

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Conflict of interest

None of the seven authors has personal or financial conflicts of interest with any aspect of the research study.

Ethical approval

The manuscript is based on the analysis of data from eight clinical cases and a review of relevant studies published in medical journals, all of which followed conventional rules of patient confidentiality. No individuals were identifiable, no human subjects were directly involved and no treatments were administered. Given these study conditions, our Institutional and University research ethics committees waived the need for ethical approval.

Funding source

No research funding was used. Costs were covered by the Divisional operating budget.

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Seear, M., Townsend, J., Hoepker, A. et al. A review of congenital lung malformations with a simplified classification system for clinical and research use. Pediatr Surg Int 33, 657–664 (2017). https://doi.org/10.1007/s00383-017-4062-y

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  • DOI: https://doi.org/10.1007/s00383-017-4062-y

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