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Endovascular Coil Embolization of Multiple Pulmonary Arteriovenous Malformations in Osler-Weber-Rendu Disease: A Challenging Pursuit
Jerelyn B. Adviento, Gino Rei A. Quizon, Jonas D. del Rosario,
Maria Teresa B. Abola, Wilfred G. Dee and Richard Henry P. Tiongco III
Section of Cardiology, Department of Medicine, College of Medicine and
Philippine General Hospital, University of the Philippines Manila
Pulmonary arterionevous malformation (PAVMs) are a rare disorder with an incidence of 2-3/1,000,000 population. Approximately 70% of cases are associated with Osler-Weber-Rendu Disease. This intrapulmonary malformation causes hypoxemia and dyspnea largely attributed to the right to left shunting.
We present a case of 25-year-old male who was admitted for fever and headaches with chronic history of epistaxis, hemoptysis, cyanosis and clubbing. Central cyanocis and clubbing were evident with hypoxemia of 65%. Heart sounds and peripheral pulses were normal. There was no systolic bruit noted in the lung bases. Multiple telangiectasias were seen in the truncal area and abdomen. Chest x-ray revealed multi-chambered cardimegaly with no opacifications. CBC showed
erythrocytosis with hematocrits of 0.68-0.78. Transthoracic echocardiography showed intact interatrial and interventricular septum, with contrast study suggestive of intrapulmonary shunting. CT angiography revealed PAVMs in bilateral lung fields. Pulmonary angiography demonstrated diffuse PAVMS in the left lung with 3 large PAVMs with multiple feeders > 7mm and smaller PAVMs in the Right lower lobe. The diagnosis was Multiple Pulmonary Arteriovenous Malformation, Osler-Weber-Rendu Disease, Brain Abscess Left Temporoparietal area. Craniotomy with brain abscess evacuation was done. Left pneumonectomy or Right lobectomy was considered but was deemed unacceptable due to high morbidity and mortality. Endovascular coil embolization was done on the 3 large fistulas on the Left lobe. Post procedure, his arterial oxygenation improved to 96%. He has no recurrence of hemoptysis, no headaches and with less episodes of shorness of breath 2 months on follow-up. Diagnosing the complex diffuse intrapulmonary malformations requires a high index of suspicion among patients with chronic cyanosis, chronic history of bleeding, and brain abscess. Treatment of this condision and it's associated complications remain a big challenge and should be highly individualized.
Keywords: endovascular coil embolization, multiple pulmonary arteriovenous malformations, Osler-Weber-Rendu disease, Hereditary Hemorrhagic Telangiectasia