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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 cystic lung disease: contemporary antenatal and postnatal management
Richard G Azizkhan1, Timothy M Crombleholme
1Fetal Care Center of Cincinnati, Division of Thoracic and General Pediatric Surgery, Cincinnati Children's Hospital Medical Center, University of Cincinnati School of Medicine, 3333 Burnet Ave, Cincinnati, OH 45229, USA. Richard.Azizkhan@cchmc.org
Insights
Congenital cystic lung disease, including congenital pulmonary adenomatoid malformation (CPAM) and bronchopulmonary sequestration (BPS), requires accurate prenatal counseling and management. Elective excision is increasingly recommended for asymptomatic lesions due to long-term risks.
Area of Science:
- Pediatric Surgery
- Developmental Biology
- Medical Genetics
Background:
- Congenital cystic lung disease encompasses rare but significant developmental abnormalities.
- Prenatal diagnosis is increasing, necessitating improved maternal counseling and perinatal care.
- Long-term risks include infection and malignancy, driving a consensus for surgical intervention in some cases.
Purpose of the Study:
- To review current diagnostic and management strategies for congenital pulmonary adenomatoid malformation (CPAM) and bronchopulmonary sequestration (BPS).
- To highlight controversial or unclear aspects in the literature regarding these congenital lung lesions.
- To emphasize the need for a unified classification system for improved research and clinical practice.
Main Methods:
- Literature review focusing on recent advancements in diagnosis and management of CPAM and BPS.
- Analysis of current clinical practices and identification of areas of controversy.
- Synthesis of information to propose a need for a unified classification system.
Main Results:
- Congenital lung lesions are increasingly detected prenatally.
- Growing consensus supports elective excision for asymptomatic CPAM and BPS due to potential complications.
- Significant need exists for a unified classification system to standardize care and research.
Conclusions:
- Accurate prenatal counseling and perinatal management are crucial for congenital cystic lung disease.
- Elective surgical excision of CPAM and BPS is a growing recommendation for asymptomatic cases.
- A unified classification system is essential for advancing the understanding and treatment of congenital lung abnormalities.
Abstract:
Congenital cystic lung disease comprises a broad spectrum of rare but clinically significant developmental abnormalities, including congenital pulmonary adenomatoid malformations, bronchopulmonary sequestrations, bronchogenic cysts, and congenital lobar emphysema that result from perturbations in lung and airway embryogenesis. As congenital lung lesions are now more commonly recognized antenatally, mothers require accurate prenatal counseling and appropriate perinatal management. In light of long-term complications of infection and malignancy, there is growing consensus that infants with asymptomatic lesions should undergo elective excision of congenital pulmonary adenomatoid malformation (CPAM) or bronchopulmonary sequestration (BPS). This review will focus on advancements and current practice in the diagnosis and management of CPAM and BPS, identifying aspects of the literature that are confusing or controversial. Although our knowledge and pre- and postnatal management of lung lesions will continue to evolve and improve, there is a compelling need for a unified clinical and pathological classification system that creates a common platform for discussion, clinical management, and research.
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