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Use of an amplatzer vascular plug in embolization of a pulmonary artery aneurysm in a case of hughes-stovin syndrome: a case report
© Tzilalis et al; licensee BioMed Central Ltd. 2011
Received: 19 May 2011
Accepted: 1 September 2011
Published: 1 September 2011
Hughes-Stovin syndrome is a rare condition characterized by peripheral deep venous thrombosis accompanied by single or multiple pulmonary arterial aneurysms. The limited number of cases has precluded controlled studies of the management of pulmonary artery aneurysms, which usually cause massive hemoptysis leading to death. This is the first report of a new endovascular treatment of a single large pulmonary arterial aneurysm.
An 18-year-old Caucasian man was referred to our department with recurrent severe hemoptysis. His medical history included Hughes-Stovin syndrome diagnosed during a recent hospital admission. The patient was initially treated with corticosteroids. Because of his recurrent hemoptysis, we decided to embolize a 3.5 cm pulmonary arterial aneurysm using an Amplatzer Vascular Plug. The procedure was not complicated, and the patient's post-intervention course was uneventful. The patient has remained free from any complications of the embolization 36 months after the procedure.
Percutaneous embolization of a single large pulmonary artery aneurysm with an Amplatzer Vascular Plug in a patient with Hughes-Stovin syndrome is a less invasive procedure that represents the best multidisciplinary approach in treating these patients.
Hughes-Stovin syndrome is a rare condition first described in 1959 that is characterized by peripheral deep venous thrombosis (DVT) often involving the vena cava and accompanied by single or multiple pulmonary arterial aneurysms . The syndrome's typical symptoms are recurrent cough, dyspnea, chest pain, fever, and hemoptysis. The natural course of the disease is usually fatal because of fulminant hemoptysis. The patients with this syndrome are often men in the second to fourth decades of life. The pathogenesis of the syndrome remains obscure. In this report, we describe a new endovascular treatment of a single large pulmonary arterial aneurysm in a patient with Hughes-Stovin syndrome which, to our knowledge, is the first of its kind published in the literature.
An 18-year-old Caucasian man with recurrent hemoptysis was referred to our hospital. A diagnosis of Hughes-Stovin syndrome had already been established in a previous hospital admission . He had had some incidents of spontaneous non-productive cough several days prior to his admission.
The patient was initially treated with corticosteroids. His hemoptysis resolved with intravenous methylprednisolone treatment after about 25 days. Upon his second admission, because of his recurrent hemoptysis, we decided to perform percutaneous embolization of the pulmonary artery aneurysm.
The limited number of patients with identified Hughes-Stovin syndrome has precluded the initiation of controlled studies of the management of patients with pulmonary arterial aneurysms . Corticosteroids, alone or in combination with an immunosuppressant, are generally considered first-line therapy . Surgical resection has been the traditional treatment of choice when the risk of lethal hemoptysis necessitates more definitive therapy. Total pneumonectomy or lobectomy has been performed with some successful results .
The high morbidity associated with surgery and the frequent bilaterality and multifocality of the pulmonary artery aneurysms make transcatheter embolization an attractive alternative to surgery in most cases. To our knowledge, few patients with Hughes-Stovin syndrome have been treated by performing embolization of a pulmonary arterial aneurysm with the use of several agents, including steel coils, Ethibloc, and isobutyl cyanoacrylate, an epoxy [4, 6, 7].
This is the first reported successful use of transcatheter embolization with steel coils and a vascular plug of a pulmonary artery aneurysm in a patient with Hughes-Stovin syndrome. The Amplatzer Vascular Plug is a type of nitinol-based, self-expanding device that is used mostly to occlude the internal iliac artery in patients undergoing aortoiliac or common iliac aneurysm endograft repair, or both . Its use has been extended to the treatment of many patients undergoing emergency embolization and has had a high technical success rate .
The less invasive procedure described in this report enables selective treatment of the arterial complications of Hughes-Stovin syndrome, such as a single large pulmonary artery aneurysm, and contributes to the best multidisciplinary approach to treating these patients.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of the journal.
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