Pulmonary veno-occlusive disease

Case contributed by Dr Bruno Di Muzio

Presentation

Chronic dry cough, progressive dyspnoea, and fatigue.

Patient Data

Age: 55 years
Gender: Female
CT

CT Pulmonary Angiogram

There is a patchy mosaic pattern of attenuation associated with interlobular septal thickening throughout both lungs. No pleural effusion. The pulmonary arteries are dilated, with a 3.8 cm pulmonary trunk and suggestive right ventricular hypertrophy. Prominent mediastinal lymph nodes are felt reactive. 

Annotated image

Features of PVOD:

* = pulmonary hypertension

dotted circle = interlobular septal lines (Kerley B lines)

arrows = mosaic pattern of attenuation

Macroscopy: Labelled "Left native lung". Left lung weighing 530 g measuring 218 x 182 x 102 mm. Pleura appears grey/pale tan with a smooth texture. Bronchovascular resection margins are clear with prominent atheroma of vessels. On sectioning, parenchyma appears pale tan with patches of brown scattered throughout both lobes. The vessels are prominent with mildly thickened walls, however, no thrombi are identified. The texture is spongy. No lesions are identified. Hilar lymph nodes appear brown.

Labelled "Right native lung". Right lung weighing 718 g measuring 211 x 194 x 112 mm. Pleura appears grey/pale tan with a smooth texture. Bronchovascular resection margins are clear with prominent atheroma of vessels. On sectioning, parenchyma appears pale tan with patches of brown scattered throughout both lobes. The vessels are prominent with mildly thickened walls, however — no no thrombi are identified. Texture is spongy. No lesions are identified. Hilar lymph nodes appear brown.

Microscopy: Sections of lung, show fibrosis of lung sector with veins and venules within the septa, showing varying degrees of hyalinisation and focal arterialisation. Within the adjacent parenchyma, small arterioles and large arterioles, show myointimal thickening. There are focal areas within the lung within which there is interstitial fibrosis with haemosiderin macrophage. The main pulmonary artery shows severe atherosclerosis. Pulmonary capillary haemangioblastosis is not seen. Pulmonary thromboemboli is not seen.

The patchy interstitial fibrosis seen throughout the lung may related to past radiotherapy.

Diagnostic Opinion: Left and Right native lung showing pulmonary venoocclusive disease, in the setting of patchy interstitial fibrosis.

Case Discussion

This patient was referred to our hospital with a history of idiopathic pulmonary hypertension. He had normal left heart function and normal pulmonary capillary wedge pressure. Imaging features of diffuse mosaic pattern attenuation, interlobular septal thickening, and dilated pulmonary arteries. Consideration for pulmonary veno-occlusive disease was then made 

The patient was offered bilateral lung transplantation and the histopathology of her native lungs confirmed features of PVOD. 

Special thanks to Dr Samanta Ellis, who has been deeply involved in the discussion of this and other similar thoracic imaging cases. 

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Case information

rID: 64473
Published: 2nd Dec 2018
Last edited: 2nd Dec 2018
System: Chest
Inclusion in quiz mode: Included

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