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Diagnostic Radiology · Pediatric Diagnostic Radiology

Pulmonary alveolar proteinosis

Proteinosis alveolaris pulmonum

Pulmonary alveolar proteinosis in infants is a secondary pathological process occurring against the background of pulmonary surfactant dysfunction (Surfactant Dysfunction Disorder). In diagnostic radiology, the disease is characterized by specific lung tissue changes that require careful evaluation on computed tomography, along with mandatory monitoring of concomitant life-support equipment.

CT pattern:“Crazy paving” — ground-glass opacity plus septal thickening.
Arterial catheterOptimal UAC levels: T6–T9 (high) or below L3 (low).
Venous catheterThe UVC tip should be positioned at the junction of the inferior vena cava and the right atrium.
Chest X-rayFirst-line method in pediatrics; lateral projections are performed rarely and strictly according to indications.
4 min readFor medical studentsUpdated Sep 25, 2026
Material prepared by WhiteCoat Pro editorial team based on platform study guidesReviewed by medical editor: Aulov A. A., general practitioner · 09/24/2026

Radiological presentation of alveolar proteinosis

Computed tomography plays a key role in examining infants with surfactant dysfunction. Image evaluation is traditionally performed in axial and coronal projections.

Pulmonary alveolar proteinosis is characterized by a combination of two main radiological signs:

  • Appearance of areas of decreased attenuation in the lungs resembling “ground-glass opacification” (ground-glass opacification).
  • Pronounced interlobular septal thickening (septal thickening).

The simultaneous presence of these phenomena forms the classic “crazy paving” pattern (crazy paving) on tomograms, which is a visual hallmark of this condition.

Control of catheter and tube position

Patients in neonatal and pediatric intensive care units often require the placement of various lines. Assessing their position is a routine yet critical task for the radiologist.

Lower extremity catheters Their positioning is often debated. Two main approaches are used for monitoring:

  1. Ultrasound (US): A safe method for basic navigation.
  2. Radiocontrast imaging: Intravenous administration of a small volume of a water-soluble low-osmolar contrast agent. This allows for precise visualization of the catheter tip, checking the patency of the entire line, and ruling out complications such as thrombin sheaths or physical tube fractures.

Feeding tubes

  • Nasogastric (NG) tube: Its position is always recorded in the report to rule out the risk of misplacement of tube feeds. The ideal position is inside the stomach.
  • Nasojejunal (NJ) tube: Structurally often equipped with a weighted tip. An important nuance: the side outlet port of the tube is located a few centimeters proximal to the tip itself. Correct placement should be assessed precisely by this port — it must reach the level of the duodenojejunal flexure.
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Ideal positioning levels for life-support lines

For proper function and prevention of life-threatening complications, the tips of medical tubes must be visualized within strictly defined anatomical zones:

  • Endotracheal (ET) tube: Above the carina (tracheal bifurcation). When evaluating the image, always adjust for the position of the infant's chin.
  • Umbilical arterial catheter (UAC): Two positions are acceptable. High — at the level of thoracic vertebrae T6–T9 (optimally near T7). Low — below the level of L3. Leaving the tip in the intermediate zone is strictly not recommended.
  • Umbilical venous catheter (UVC): The ideal point is the projection of the junction of the inferior vena cava (IVC) and the right atrium. Note: if the tip is located in the IVC on the image, technically it should be advanced more distally.
  • Temperature probe (Temperature probe): Should project within the thoracic esophagus.

Features of pediatric chest radiography

In older children, chest radiography (Chest radiograph) remains the absolute first-line method and is performed more frequently than other examinations.

The spectrum of detected airway pathologies is extremely wide: from common minor infections and congenital anomalies to complex malignancies and immunodeficiency syndromes.

The difficulty in interpreting images lies in the fact that pediatric pathology can visually mimic adult diseases. To avoid overdiagnosis, the physician must thoroughly understand pediatric normal variants. At the same time, performing frontal (anteroposterior) projections is standard; lateral views are done much less frequently and require strict clinical indications.

Briefly: answers to topical questions

What are the main etiological causes of pulmonary alveolar proteinosis in infants?

The main etiological cause of pulmonary alveolar proteinosis in infants is a secondary process resulting from surfactant dysfunction (Surfactant Dysfunction Disorder). These disorders arise from genetic anomalies:

  • Surfactant protein B mutations (SpB) — autosomal recessive, manifesting with respiratory symptoms immediately after birth.
  • ATP-binding cassette transporter A3 mutations (ABCA3) — autosomal recessive, manifesting with respiratory symptoms immediately after birth.
  • Surfactant protein C mutations (SpC) — often autosomal dominant, can manifest in later infancy.

What specific histological changes in lung tissue are found in alveolar proteinosis?

A specific histological change in lung tissue in alveolar proteinosis is the accumulation of PAS-positive (PAS-positive) lipoproteinaceous material in the alveoli. This process is caused by impaired surfactant clearance from the lungs due to macrophage dysfunction. Depending on the histopathological pattern of injury, computed tomography visualizes the characteristic “crazy paving” symptom (crazy paving).

How to memorize

“Crazy paving” pattern in alveolar proteinosis: GG + S (Ground-glass + Septa).

Class and exam questions

What makes up the CT “crazy paving” pattern?

This pattern is formed by the superposition of two features: areas of decreased attenuation resembling “ground-glass opacification” and pronounced interlobular septal thickening.

At what level should the tip of the umbilical arterial catheter be located?

A high position at the T6–T9 vertebral level or a low position below the L3 level is acceptable. Placing the tip between these zones is strongly discouraged.

Why use contrast when evaluating lower extremity venous lines?

The administration of low-osmolar water-soluble contrast helps verify the position of the tip, assess line patency, and rule out catheter fractures and surrounding thrombin sheaths.

What is the specific feature of assessing nasojejunal tube position?

The nasojejunal tube has a weighted tip, but the feeding outlet port is located proximal to it. It is the level of the port that must be evaluated — it should project at the level of the duodenojejunal flexure.

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What else to study on this topic

  • Pathogenesis of surfactant dysfunction (Surfactant Dysfunction Disorder)
  • Differential diagnosis of the “ground-glass” pattern in infants
  • Pediatric normal variants on frontal chest radiographs
  • Effect of head and chin position on endotracheal tube excursion
  • Topographic anatomy of the inferior vena cava and right atrium for UVC positioning

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