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Updated: Oct 6, 2025

Protocol and Guidelines for Point-of-Care Lung Ultrasound in Diagnosing Neonatal Pulmonary Diseases Based on International Expert Consensus
Published on: March 6, 2019
[Congenital Malformations of the Lung - an Overview]
Birte Ohm1, Wolfgang Jungraithmayr1
1Klinik für Thoraxchirurgie, Universitätsklinikum Freiburg, Medizinische Fakultät, Albert-Ludwigs-Universität Freiburg, Freiburg, Deutschland.
Insights
Congenital pulmonary malformations are rare lung diseases requiring prompt surgical intervention for respiratory distress. This review covers causes, symptoms, and treatments for common airway and lung malformations.
Area of Science:
- Pediatric Surgery
- Thoracic Surgery
- Developmental Biology
Background:
- Congenital pulmonary malformations (CPMs) are rare developmental disorders affecting the airways and lung parenchyma.
- Common CPMs include tracheal bronchus, bronchial atresia, bronchogenic cyst, pulmonary sequestration, congenital lobar emphysema, and congenital pulmonary airway malformation.
- These conditions often cause significant postnatal respiratory distress due to their space-occupying nature, necessitating surgical management.
Purpose of the Study:
- To provide a comprehensive review of congenital pulmonary malformations.
- To elucidate the causes, clinical presentations, and therapeutic strategies for prominent CPMs.
- To inform clinical decision-making regarding the management of both symptomatic and asymptomatic lesions.
Main Methods:
- Systematic literature review of congenital pulmonary malformations.
- Analysis of etiological factors and pathogenetic mechanisms.
- Synthesis of current therapeutic approaches, including surgical interventions.
Main Results:
- Detailed description of the epidemiology and classification of common CPMs.
- Outlined clinical manifestations, ranging from severe neonatal respiratory distress to asymptomatic incidental findings.
- Discussed the debate surrounding the management of asymptomatic lesions, with a general recommendation for early elective surgery.
Conclusions:
- Congenital pulmonary malformations represent a diverse group of pediatric lung diseases with varied clinical impacts.
- Early diagnosis and appropriate surgical intervention are crucial for optimizing outcomes and preventing long-term complications.
- This review consolidates essential information for clinicians managing these rare conditions.
Abstract:
Congenital pulmonary malformations comprise a heterogenous group of rare developmental diseases. The most common malformations are the tracheal bronchus, bronchial atresia, bronchogenic cyst, pulmonary sequestration, congenital lobar emphysema, and congenital pulmonary airway malformation. Due to their space-consuming effect, patients suffer early postnatal respiratory distress which generally requires immediate surgical resection. The management of asymptomatic lesions remains subject to debate, but early elective surgery is generally recommended to avoid respiratory and infectious complications at a later time point.We here provide a comprehensive review in which we present causes, clinical presentation and therapeutic options for the most prominent congenital malformations of the airways and lung parenchyma.
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