The pathology of congenital lung lesions

https://doi.org/10.1053/j.sempedsurg.2015.02.002Get rights and content

Abstract

The spectrum of complications associated with congenital lung malformation is wide. They can range from fetal hydrops in utero to postnatal problems of ventilation, obstruction and infection; presentation may occur from the neonatal period to adulthood. Many lesions will remain asymptomatic while at the other end of the complication spectrum, there is a small risk of neoplasia associated with some forms of cystic lung. A better understanding of the pathology has shown that bronchial atresia/obstruction is the likely hidden pathology underlying many congenital lung lesions leading to downstream cystic maldevelopment. Earlier diagnosis has led to increasing difficulties in ascribing malformations to conventional categories that were originally described in postnatal lungs. It is probably more important to be aware of the potential combination of vascular and airway connections and complications than to try and prescribe a classification of pulmonary lesions associated with rigid definitions.

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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