Case Presentation: 54 yo White Male with PMH of hereditary hemorrhagic telangiectasia (HHT) presented with right-sided focal neurological deficits, word finding difficulties, confusion and headache.  He was treated for a brain abscess with surgical resection and antibiotics X 1 month at an OSH attributed to recently sustained head trauma.  Follow up MRI 1 month later, showed abscess of increasing size.  He continued to have HA and presented for further care.  Imaging performed on admission showed a 3.5 X 1.8 cm brain abscess.  Transthoracic contrast echo (TTCE) was positive for bubble study.  CT angio of the chest/abdomen/pelvis showed previously coiled right lower lobe AVM 1.5 X 3.0cm with patency of the lumen along with other AVMs found in the jejunum and liver.   Neurosurgery was consulted and patient was treated with surgical resection of brain abscess and antibiotics.  He also underwent coil embolization of the pulmonary AVM with interventional radiology to prevent reoccurrence of brain abscess.  Patient had complete resolution of neurological deficits prior to discharge.

Discussion:  HHT is an autosomal dominant vascular disorder that occurs in 1:5000-8000 people and is commonly associated with epistaxis and GI bleeding with subsequent iron deficiency anemia.  Along with telangiectasia, pulmonary, hepatic and cerebral AVMs are commonly seen.  About 50% of patients with HHT will have pulmonary AVMs predisposing them to TIAs, paradoxical embolic strokes and/or brain abscess.   While pAVMs may cause symptoms of dyspnea, hemoptysis, or platypnea, they are often times asymptomatic.  Initial work up of patients suspected of having pAVMs is a TTCE or “bubble study” usually followed by CT confirmation.  Treatment of pulmonary AVMs should always include proper embolization to prevent neurological sequelae—which in unscreened patients may be the first presenting symptom.  While most pAVMs remain stable in size, 25% will enlarge slowly and therefore require surveillance CT every 3-5 years as previously treated AVMs may become patent again and/or new AVMs may develop.                  

Conclusions: It is recommend to screen all adult patients with known HHT for pulmonary AVMs.  Early diagnosis and intervention with embolization can significantly decrease morbidity and mortality from stroke and brain abscess.   Pulmonary AVMs should always be considered in the differential in patients with known HHT and new onset neurological deficits.