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A3162 - Bronchial Dieulafoy’s Disease and Mediastinal Arteriovenous Malformation
Author Block: A. Z. Foo1, A. Hsu2; 1Faculty of Medicine, Nursing and Health Sciences, Monash University, Clayton, Australia, 2Department of Respiratory and Critical Care Medicine, Singapore General Hospital, Singapore, Singapore.
Introduction:
Bronchial Dieulafoy’s disease is an anomaly characterized by a tortuous, abnormal submucosal artery in the bronchus. Mediastinal arteriovenous malformation (AVM) consists of numerous direct communications between arteries and veins in the mediastinum. Each of these conditions is very rare.
This is the first report of mediastinal AVM causing Dieulafoy’s disease complicated by life-threatening hemoptysis.
Case:
A middle-aged, never-smoker male was hospitalized for acute myeloid leukemia. On Day 16 of admission, he coughed out 200mL of fresh blood which was followed by transient hypotension with blood pressure dropping from 140/80 to 90/50mmHg. He was afebrile and his blood results were: hemoglobin 10.0 decreasing to 8.5g/dL within 1 day of hemoptysis, total white counts 23x103/μL, platelet counts 30 x103/μL, normal renal panel and coagulation profile. Thoracic computed-tomography scan (CT) showed numerous tortuous mediastinal vessels of undetermined etiology. During bronchoscopy under platelet cover, a white-capped sessile nodule in the right upper lobe bronchus (RUL) was identified. Active bleeding occurred when the bronchoscope tip grazed the nodule. Bronchial irrigation with ice-cold saline secured hemostasis. A diagnosis of Dieulafoy’s disease associated with mediastinal AVM was made. CT angiogram delineated the nature and locality of the vascular aberration for embolization. No gross contrast extravasation or pooling was detected. The major vessels feeding into RUL were identified and successfully embolized with glue. No further hemoptysis occurred with the subsequent completion of chemotherapy.
Discussion:
It is rare for Dieulafoy’s disease to occur in the bronchus whereas it is relatively more common in the gastrointestinal tract. Similarly, it is unusual for AVM to develop in the mediastinum. This case uniquely presents the possibility of mediastinal AVM causing thoracic Dieulafoy’s disease, leading to life-threatening hemoptysis. The patient unfortunately declined another bronchoscopy to evaluate the ablated sessile lesion.
Though rare, Dieulafoy’s disease should be considered as a differential diagnosis for hemoptysis, particularly given the potential of fatal bleeding associated. Some cases of active hemorrhage following bronchoscopic procedures, including saline wash of the arterial lesion, have been reported. Hence, there should be early recognition before bronchoscopy so that the procedure is done with more caution. In this case report, the nature of bleed and CT findings aroused suspicion of Dieulafoy’s disease associated with mediastinal AVM.
While there is no common consensus of treatment of Dieulafoy’s disease, the first-line therapeutic option for both conditions is often arterial embolization.