A case of pulmonary arteriovenous malformation in the setting of Rendu Osler Weber syndrome

Abstract

Rendu Osler Weber syndrome is a rare disorder, in which arteriovenous malformations are a hallmark feature. We describe the case of a 77-year-old female patient who presented with dyspnea, recurrent epistaxis, and signs of right ventricular heart failure, along with hypoxia and severe anemia. Several imaging modalities facilitated diagnostic workup. The computed tomography revealed an area of pulmonary arteriovenous malformation. Visceral involvement, along with clinical criteria and medical history, established the diagnosis of Rendu Osler Weber syndrome. The patient was scheduled for embolization of the PAVM soon after the diagnosis. Proper imaging, guided by clinical suspicion can be extremely helpful in diagnosing and treating this rare entity.

Keywords: Balloon-assisted embolization; Deep femoral artery; Percutaneous direct glue embolization; Pseudoaneurysm; Tunneled femoral vein hemodialysis catheter.

Fig. 1

Chest radiograph showing an enlarged cardiothoracic ratio, right pleural effusion with adjacent compressive atelectasis.

Fig. 2

Coronal reformatted image of the chest CT in lung parenchyma window showing the dilated vascular structures (grey arrowheads) in the right costophrenic angle.

Fig. 3

Axial (a) and coronal (b, c) reformats showing the pulmonary AVM, optimally opacified, located in the right lower lobe. The feeding and draining vessel are adequately visualized (white arrowheads).