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Congenital Anomalies of the Lungs

*Anomaliae congenitales pulmonum*

For medical students2 min readUpdated 2026-10-10

Congenital anomalies of the lungs are a group of developmental malformations originating during embryogenesis. Among these, congenital lung cysts and various forms of bronchopulmonary sequestration hold key clinical and anatomical significance due to their distinct blood supply, anatomical location, and potential complications.

Cyst PathogenesisSeparation of tissue fragments from the primitive gut during embryogenesis
Cyst LiningBronchogenic cysts are lined by bronchial epithelium
VascularizationThe sequestered segment receives arterial blood from the aorta or its branches
ComplicationsSuppuration, pyothorax, pneumothorax, hemorrhage, atelectasis

Pathogenesis and Morphology of Congenital Lung Cysts

The development of congenital lung cysts is rooted in impaired embryonic differentiation, characterized by the separation of tissue fragments from the primitive gut. The resulting pathological cavities become isolated within the thoracic cavity.

The most common variant is the bronchogenic cyst. The key morphological feature of this type is that the inner wall of the cavity is lined with bronchial epithelium.

The clinical course of cystic malformations is frequently complicated by secondary pathological processes. The main clinical and anatomical complications include:

Nature of Bronchopulmonary Sequestration

Bronchopulmonary sequestration is a developmental anomaly characterized by the formation of an isolated lung segment. The defining feature of this malformation is that the affected area has no connection to the normal tracheobronchial tree or the pulmonary arterial system.

Due to complete separation from the pulmonary circulation, the arterial blood supply to the sequestered tissue is anomalous, originating directly from the systemic circulation, namely from the aorta or its major branches.

Classification and Forms of Sequestration

Depending on the location of the sequestered fragment relative to the organ parenchyma, two clinical and anatomical forms are distinguished:

  1. Extralobar Sequestration:
  2. Localization: The pathological lesion is located outside the lung tissue—in the mediastinum;
  3. Age group: Typically diagnosed in neonates;
  4. Features: Frequently associated with other congenital malformations.
  1. Intralobar Sequestration:
  2. Localization: The abnormal segment is located directly within the lung parenchyma;
  3. Age group: Characteristic of adults;
  4. Manifestations: Clinically presents with recurrent infectious complications.

Comparative Characteristics of Sequestration Variants

The following systematic comparison is used to quickly differentiate the two types of bronchopulmonary sequestration:

CriterionExtralobar SequestrationIntralobar Sequestration
LocalizationIn the mediastinumWithin the lung parenchyma
Age at PresentationNeonatesAdults
Associated AnomaliesAssociated with other malformationsRecurrent infectious complications
Blood SupplyFrom the aorta or its branchesFrom the aorta or its branches

Mnemonic

EXTRA-MEDIASTINUM (Extralobar — in the mediastinum, neonates), INTRA-PARENCHYMA (Intralobar — in the parenchyma, adults with infectious complications).

Frequently asked questions

Which structures give rise to congenital lung cysts during embryogenesis?

Congenital lung cysts form as a result of tissue fragments separating from the primitive gut during embryogenesis. Common bronchogenic cysts are internally lined with bronchial epithelium.

Where does the blood supply to the sequestered lung segment originate?

The sequestered segment is not connected to the pulmonary artery. Its blood supply consists of arterial blood delivered directly from the aorta or its branching vessels.

What complications can arise from the destruction of congenital lung cysts?

Complications include purulent inflammation (abscesses), and upon destruction, pyothorax, pneumothorax, pulmonary hemorrhage, as well as hemothorax and atelectasis.

What are the main differences between extralobar and intralobar sequestration?

Extralobar sequestration is located in the mediastinum, diagnosed in neonates, and often associated with other malformations. Intralobar sequestration is located within the lung parenchyma, typically presents in adults, and manifests with recurrent infectious complications.

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