Congenital lung anomalies

Congenital lung anomalies

Congenital lung anomalies

On this page

Embryology & Overview - Lung Budding Gone Wild

  • Origin: Respiratory diverticulum (lung bud) arises from the ventral foregut endoderm around week 4.
  • Separation: The tracheoesophageal septum partitions the foregut into the dorsal esophagus and ventral trachea.
  • Errors: Faulty partitioning leads to tracheoesophageal fistulas (TEF); abnormal budding causes bronchogenic cysts, sequestration, or agenesis.

Most common TEF: Esophageal atresia with a distal tracheoesophageal fistula (~85% of cases). This presents with polyhydramnios in utero, and choking/aspiration upon first feeding.

Lung Development & Congenital Anomalies

Pulmonary Sequestration - Rogue Lung Tissue

  • Non-functional lung tissue with no connection to the bronchial tree.
  • Receives arterial blood from systemic circulation (e.g., aorta), not pulmonary arteries.
  • Intralobar (ILS):

    • More common (~85%).
    • Located within a normal lung lobe; shares visceral pleura.
    • Presents in adults with recurrent, localized pneumonia.
    • Venous drainage: Pulmonary veins.
  • Extralobar (ELS):

    • Less common (~15%).
    • Located outside normal lung; has its own pleura.
    • Presents in infants, often with other anomalies (e.g., diaphragmatic hernia).
    • Venous drainage: Systemic veins (e.g., azygous).

⭐ The hallmark is an anomalous systemic artery supplying the sequestered segment, most commonly arising from the thoracic or abdominal aorta.

Congenital Lung Anomalies: Types and Characteristics

CPAM & Bronchogenic Cysts - Airway Malformation Mayhem

  • Congenital Pulmonary Airway Malformation (CPAM):
    • Hamartomatous mass of cystic lung tissue; communicates with airway.
    • Presents as neonatal respiratory distress or recurrent infections.
    • CXR/CT shows a multicystic, air-filled lesion.
  • Bronchogenic Cyst:
    • Arises from abnormal ventral foregut budding; does NOT communicate with airway.
    • Typically a fluid-filled, unilocular cyst in the mediastinum.
    • Can cause compressive symptoms (dyspnea, dysphagia).

⭐ While most CPAMs are benign, they carry a risk of malignant transformation to pleuropulmonary blastoma, especially Type 4.

Congenital Lung Anomalies Diagram

Congenital Lobar Overinflation - One-Way Air Trap

  • Pathophysiology: Ball-valve bronchial obstruction leads to progressive lobar air-trapping and hyperinflation. Often from defective bronchial cartilage (~50% of cases) or extrinsic compression.
  • Presentation: Neonatal respiratory distress, wheezing, cyanosis.
  • Common Lobes: LUL > RML > RUL.
  • Diagnosis:
    • CXR: Hyperlucent lobe, mediastinal shift away from the affected side, atelectasis of adjacent lobes.
    • CT confirms diagnosis.
  • Management: Surgical lobectomy is the definitive treatment.

⭐ Unlike a simple pneumothorax, CLO causes severe mediastinal compression, risking cardiovascular collapse; it's a surgical emergency.

Congenital Lobar Overinflation: CXR & CT

High‑Yield Points - ⚡ Biggest Takeaways

  • Pulmonary sequestration is non-functional lung tissue lacking connection to the airway and pulmonary artery, receiving systemic arterial supply.
  • Bronchogenic cysts are mediastinal masses from abnormal foregut budding that can become secondarily infected.
  • CPAM is a hamartomatous lesion presenting with respiratory distress and a risk of malignant transformation.
  • Pulmonary hypoplasia is frequently secondary to congenital diaphragmatic hernia or severe oligohydramnios.
  • Intralobar sequestration drains to pulmonary veins; extralobar sequestration drains into the systemic venous system.

Practice Questions: Congenital lung anomalies

Test your understanding with these related questions

A 3-year-old girl is brought to the emergency department by her parents with sudden onset shortness of breath. They tell the emergency physician that their daughter was lying on the bed watching television when she suddenly began gasping for air. They observed a bowl of peanuts lying next to her when they grabbed her up and brought her to the emergency department. Her respirations are 25/min, the pulse is 100/min and the blood pressure is 90/65 mm Hg. The physical findings as of now are apparently normal. She is started on oxygen and is sent in for a chest X-ray. Based on her history and physical exam findings, the cause of her current symptoms would be seen on the X-ray at which of the following sites?

1 of 5

Flashcards: Congenital lung anomalies

1/10

_____ is fibrosis of the lung interstitium with unknown cause

TAP TO REVEAL ANSWER

_____ is fibrosis of the lung interstitium with unknown cause

Idiopathic pulmonary fibrosis

browseSpaceflip

Enjoying this lesson?

Get full access to all lessons, practice questions, and more.

Start Your Free Trial