The pathology of congenital lung lesions
Introduction
This review will focus on those congenital lung lesions that usually come to the attention of the Paediatric Surgeon. It is not a comprehensive account of congenital lung pathology: some conditions tend to be incidental and others are confined to medical management. While this focuses on congenital and therefore largely developmental anomalies, acquired lesions will also be mentioned briefly where they form an important part of the differential diagnosis especially in the neonatal period.
Section snippets
Abnormal bronchial origin and branching
Abnormal bronchial origin and branching describes a wide range of anomalies. Most are minor and asymptomatic, so the true incidence is unknown. The majority affects the right upper lobe bronchus; a tracheal bronchus has been described in 2% of children requiring bronchoscopy.
The cause of bronchial branching anomalies is not clear although, as the major branches of the tracheobronchial tree are formed by 16 weeks gestation, the aetiology must have its origins before this time. Branching
Bronchial atresia
The simple definition of bronchial atresia (BA) is airway obstruction. Bronchial atresia (BA) used to be considered a rare abnormality but it has been increasingly recognized as a fundamental component of many adenomatoid and other pulmonary malformations.2 In a North American series, bronchial atresia was associated with all types of developmental lung malformations, cystic adenomatoid malformations, bronchopulmonary sequestrations, congenital lobar emphysemas and lesions of mixed pathology.3
Cystic lung disease
This section concentrates on the congenital pulmonary malformations that commonly lead to a clinical or radiological diagnosis of cystic lung disease in the fetus or young (Table). While the emphasis here is on congenital lesions, cystic lung presenting in the neonatal period also include acquired lesions such as acute and persistent pulmonary interstitial emphysema, postinfectious pulmonary cysts and the cystic changes in tumours.
Cystic lung lesions are described here largely using classic
Summary
In this review we have described many developmental lung malformations including the sometimes controversial subject of cystic lung disease. All developmental atresias (eg, pulmonary atresia, bowel atresia and bronchial atresia) lead to upstream and downstream consequences because of obstruction. Bronchial atresia has been described as a hidden pathology because it is often underdiagnosed.22 In practice, formal diagnosis may be impossible as the atresia is likely to be close to or at resection
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2022, Journal of Radiology NursingCitation Excerpt :When formed later in development, cysts may be located within the lung parenchyma. Cysts are typically lined with respiratory epithelium and cartilage (Fowler & Gould, 2015). Clinically, cysts may present with secondary infection or mass effect with resulting dyspnea.
Chest Wall Deformities and Congenital Lung Lesions: What the General/Thoracic Surgeon Should Know
2022, Surgical Clinics of North AmericaCitation Excerpt :Congenital lung lesions are rare entities. The most common are congenital pulmonary airway malformations (CPAM), pulmonary sequestrations (PS), congenital lobar emphysema (CLE), and bronchogenic cysts (BC).80,81 Formation of the lung begins in the first weeks of in utero development and does not stop until at least 8 years of age.