CASE 18607 Published on 09.07.2024

Pulmonary vein thrombosis with pulmonary infarction in child with APLA syndrome

Section

Chest imaging

Case Type

Clinical Case

Authors

Priti Bolke, Avani Natu, Swaksh Nemani

Department of Radiodiagnosis, Seth Gordhandas Sunderdas Medical College and King Edward Memorial Hospital, Mumbai, Maharashtra, India

Patient

8 years, male

Categories
Area of Interest Cardiovascular system, Lung ; Imaging Technique CT-High Resolution
Clinical History

An 8-year-old boy presented with high-grade fever, non-productive cough and exertional breathlessness for seven days. On examination, the child was tachypnoeic with absent air entry on the left. Initial sputum studies revealed no pathogenic organism. He had TLC of 14,000 cells/cubic millimetre, prompting repeat-induced sputum analysis, which was positive for cotrimoxazole-sensitive Acinetobacter species. Anti-phospholipid and anti-cardiolipin IgM returned positive.

Imaging Findings

Frontal chest radiograph showed near-complete opacification of the left hemithorax. The pleural line was visible with peripheral absence of broncho-vascular markings suggestive of pleural effusion. There was no mediastinal shift, suggesting concomitant underlying lung collapse (Figure 1).

Multiplanar HRCT chest with contrast, done three days later, showed wedge-shaped non-enhancing parenchyma of the left upper lobe without air bronchograms suggestive of pulmonary infarct. There was consolidation in the lingular segment of the left upper lobe with subsegmental collapse of the left lower lobe (Figures 2 and 3). There was moderate pleural effusion on the left with smooth enhancement of the pleura. There was thrombosis of the left superior pulmonary vein, with the thrombus extending into the tributaries and into the left atrium (Figures 4, 5, 6, 8 and 9). Pulmonary arteries were normal.

Discussion

Background

Pulmonary vein thrombosis (PVT) is a rare disease entity with limited aetiologies, most commonly open or VATS-assisted pulmonary surgery, lung malignancy and RFA for atrial fibrillation. Other aetiologies include congenital narrowing of pulmonary veins, sclerosing mediastinitis or left atrial clots [1]. Few case reports suggested the possibility of procoagulant states like polycythaemia vera [2]. In post-surgical cases, it is likely secondary to direct injury. In malignancy, it can be caused by direct extension, hypercoagulable state, or extrinsic compression [3].

Clinical Perspective

Clinical presentation can be divided into acute and chronic spectrum. Acute onset pathology presents with features of pulmonary infarction like cough, pleuritic chest pain and haemoptysis. Chronic disease process presents with repeated bouts of pulmonary infections, progressing to lung fibrosis or cardiac failure [4].

Imaging Perspective

Imaging is the mainstay of diagnosis as the clinical history and examination findings can be non-specific. The key imaging modalities are CT pulmonary angiography (CTPA) and trans-oesophageal echocardiography (TEE). CTPA allows direct visualisation of the pulmonary venous clot and evaluation of aetiological factors and complications in the pulmonary parenchyma. Drawbacks of CTPA are the possibility of overlooking a potential clot due to slow flowing contrast and cardiac motion artefacts [6]. TEE, on the other hand, provides indirect evidence of thrombosis from the flow velocities in the pulmonary veins and extent into the left atrium. Drawbacks of TEE are that it is limited to proximal PVT and it is an invasive modality [5]. Primary evaluation of pulmonary veins and detection of thrombus is challenging unless there is a high degree of suspicion. However, in patients with atrial clots and pulmonary infarction, PVT must be evaluated and appears as filling defects within the pulmonary veins juxtaposed to the left atrium. In our case, the HRCT suggestive of pulmonary infarct with likely secondary infection causing necrotising pneumonia raised suspicion for a vascular thrombo-embolic cause.

Outcome

All patients with PVT are to be treated with anticoagulation in the form of parenteral LMWH with or without oral warfarin [5]. Additional therapeutic options include mechanical thrombectomy in post-surgical patients, antibiotic prophylaxis and pulmonary resection in cases of large infarcted or gangrenous lungs.

Teaching Points

PVT is a rare disease entity and is usually missed on imaging due to a primary focus on the pulmonary arteries in cases of lung infarction. Imaging-centric diagnosis is essential to guide the line of clinical work-up. Timely diagnosis can prevent devastating complications secondary to embolism or infarction.

Written informed patient consent for publication has been obtained.

Differential Diagnosis List
Pulmonary vein thrombosis with pulmonary infarct
Segmental lobar collapse
Pulmonary thrombo-embolism
Typical lobar pneumonia
Final Diagnosis
Pulmonary vein thrombosis with pulmonary infarct
Case information
URL: https://eurorad.org/case/18607
DOI: 10.35100/eurorad/case.18607
ISSN: 1563-4086
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