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Developmental lung anomalies in adults: A pictorial review
Sujith V Cherian1, Anupam Kumar2, Daniel Ocazionez3
1Divisions of Pulmonary, Critical Care and Sleep Medicine, USA; McGovern Medical School, The University of Texas Health Science Center at Houston, TX, 77030, USA.
Insights
Developmental lung anomalies, often missed in childhood, present diagnostic challenges in adults. This review covers their types, causes, and clinical presentation in adults.
Area of Science:
- Pulmonology
- Pediatric Radiology
- Thoracic Surgery
Background:
- Developmental lung anomalies are diverse abnormalities of lung parenchyma and vasculature, typically presenting in early life.
- A significant number of cases remain undiagnosed in childhood, manifesting in adulthood with incidental findings or recurrent respiratory infections.
- Defects in tracheobronchial tree and pulmonary vasculature development are implicated in their pathogenesis.
Purpose of the Study:
- To discuss the spectrum of developmental lung anomalies in adults.
- To review their etiopathogenesis, clinical, and radiographic presentations.
- To briefly outline management strategies for these rare conditions.
Main Methods:
- Literature review of developmental lung anomalies.
- Classification of anomalies into bronchopulmonary, vascular, and combined categories.
- Discussion of clinical and imaging findings in adult patients.
Main Results:
- Developmental lung anomalies are categorized into bronchopulmonary (e.g., congenital bronchial atresia, bronchogenic cysts), vascular (e.g., pulmonary artery sling, anomalous pulmonary venous return), and combined types (e.g., scimitar syndrome, bronchopulmonary sequestration).
- Adult presentation often poses diagnostic challenges, frequently being mistaken for other pathologies.
- Rarity and varied presentations contribute to diagnostic difficulties in the adult population.
Conclusions:
- Developmental lung anomalies encompass a wide range of conditions affecting lung development.
- Recognition and accurate diagnosis in adults are crucial to avoid misdiagnosis and inappropriate interventions.
- Understanding the spectrum, presentation, and management is essential for clinicians encountering these rare conditions.
Abstract:
Developmental lung anomalies represent a heterogeneous group of diverse, yet related abnormalities that involve the lung parenchyma, pulmonary vasculature or a combination of both-which usually present prenatally and in early childhood. However, a substantial number of cases go unnoticed during childhood and present either incidentally or with recurrent respiratory infections progressing into adulthood. Defective development of the tracheobronchial tree and the pulmonary vasculature are proposed to cause these developmental anomalies. Encountering these lung anomalies in adults is a diagnostic challenge given their rarity and they are often mistaken as other serious pathological conditions, resulting in unnecessary diagnostic tests and procedures. The developmental lung anomalies in adults can be broadly divided into three categories: bronchopulmonary anomalies encompassing congenital bronchial atresia, bronchogenic cysts, congenital lobar emphysema and congenital pulmonary airway malformations. Vascular anomalies include pulmonary agenesis-aplasia- hypoplasia complex, unilateral absence of the pulmonary artery, pulmonary artery sling, partial anomalous pulmonary venous return, pulmonary venous varix and pulmonary arteriovenous malformations. Finally combined lung parenchymal-vascular anomalies of the lung involve hypogenetic lung (scimitar) syndrome and bronchopulmonary sequestration. This article discusses the spectrum of these developmental anomalies of the lung, their etiopathogenesis, clinical and radiographic presentations in adults and management in brief.
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