Cover Image: Disappearance of huge pulmonary aneurysms in Hughes-Stovin syndrome
Kenichi Shimizu, Ichizo Tsujino, and Masaharu Nishimura
First Department of Medicine, Hokkaido University School of Medicine, Kita-ku, Sapporo, Japan
Hughes-Stovin syndrome is a rare clinical entity characterized by multiple pulmonary aneurysms and recurrent deep vein thrombosis (DVT). We here report a case of Hughes-Stovin syndrome where dramatic disappearance of massive pulmonary aneurysms was delineated by a series of CT scans, including three-dimensional (3D) CT. A 17-year-old woman was diagnosed as having multiple thromboses in dural sinuses in August 2001, and 1 month later, DVT in her left leg appeared. At this time, systemic CT revealed complete occlusion of her superior vena cava and severely narrowed inferior vena cava. In April 2002, she experienced sudden chest pain and a diagnosis of recurrent pulmonary thrombo-embolism was made. In September 2002, she developed another episode of sudden chest pain and her chest CT scan revealed massive aneurismal changes with mural thrombus in her bilateral pulmonary artery (Panel 1A). We constructed 3D CT images that clearly demonstrated pulmonary aneurysms in segment 6 and in the proximal portion of the left lower lobe artery in her left lung (Panel 1B, 1C).We were not able to perform pulmonary angiography due to total occlusion of both caval veins. Given this unusual clinical history and the CT findings, we made a diagnosis of Hughes-Stovin syndrome. We immediately administered adrenocorticosteroids and cyclosporin and observed marked improvement in the aneurismal changes one month later (Panel 2). In February 2003, CT scans showed the disappearance of the aneurysms and mural thrombosis (Panel 3). This is the first case in which a series of CT scans, including 3D CT, clearly demonstrated reversible pulmonary artery aneurysms associated with this syndrome.
Figure legend:
Panel 1: (A) Enhanced chest CT scan obtained in September
2002. Partially thrombosed aneurysms of right and left pulmonary
arteries are shown (arrows). The external diameters of the two
aneurysms in her left lung reached 3.8 x 3.7 (PA in segment 6) and 2.7
x 2.2 cm (proximal portion of the left lower lobe artery), which
exceeded the size of the thoracic descending aorta. Ao: thoracic
aorta, PA: pulmonary artery. (B, C) Three dimensional CT images of the
aneurysms in her left pulmonary artery (B: left horizontal view, C:
left posterior superior view). The most characteristic feature is the
dilatation of the pulmonary artery in segment 6 (arrow heads) and in
the proximal portion of the left lower lobe artery (arrows) in her
left lung.
Panel 2: Enhanced chest CT scan obtained 1 month after introduction of
corticosteroid and immunosuppressive therapy shows decreased luminal
diameter and increased amount of thrombus in the left pulmonary
artery. The aneurysms in her right lung decreased in number.
Panel 3: CT scan taken in February 2003 shows disappearance of the
pulmonary arterial aneurysms in both lungs. High density spots in the
mediastinal soft tissue and near the left pulmonary artery are dilated
collateral vessels caused by occlusion of both vena cava (arrows).
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