Fetopathology and developmental pathology of the embryo and fetus: Congenital lobar emphysema

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+ Atlas of fetal pathology

+ Congenital malformations

+ Physiological developement of an embryo and fetus, growth and changes of the external shape

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+ Congenital malformations of the respiratory system

+ Congenital cystic adenomatoid malformation (CCAM)

+ Lobar sequestration

+ Congenital lobar emphysema

+ Pulmonary hyperplasia

+ Pulmonary hypoplasia

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+ Congenital malformations of the internal genitalia

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Congenital lobar emphysema

Introduction

Hyperinflation most commonly affecting the upper left lobe.

Etiology

Variable, the most common factors are:

external compression of the bronchus by anomal blood vessels
bronchogenic cysts
mucosal valves
insufficiently formed bronchial cartilage

Clinical signs

quickly developing respiratory distress syndrome after the birth, shift of the mediastinum and compression of the rest of the lungs
other congenital anomalies are often present, namely heart defects

Histology

Broad dilatation of interalveolar ducts and alveoli without any destruction of the pulmonary tissue, sometimes with some fibrosis.