Eur J Cardiothorac Surg 2007;31:561-562. doi:10.1016/j.ejcts.2006.12.005
Copyright © 2007, European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved
Rare case of multiple pulmonary artery aneurysms with caval thrombosis — Hughes-Stovin syndrome
Jung Tae Kim,
Tae Yun Oh*,
Woon Ha Chang
Department of Cardiovascular and Thoracic Surgery, Kangbuk Samsung Hospital School of Medicine, Sungkyunkwan University, 108 Pyoung Dong Jongno-gu, Seoul, Republic of Korea
Received 15 September 2006;
received in revised form 28 November 2006;
accepted 4 December 2006.
* Corresponding author. Tel.: +82 2 2001 2150; fax: +82 2 2001 2148. (Email: csmed7{at}hanmail.net).
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Abstract
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Hughes and Stovin first reported a syndrome consisting of multiple pulmonary artery aneurysms and venous thrombosis in 1959. We encountered a 45-year-old man who had massive hemoptysis and leg swelling. The CT and angiography showed huge bilateral pulmonary artery aneurysms and inferior vena caval thrombosis. The bilateral lower lobe aneurysms were successfully treated with staged operations. The patient tolerated the procedures relatively well and was followed up by steroid therapy with a good response.
Key Words: Aneurysm • Venous disease • Lobectomy
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1. Case report
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A 45-year-old man was first admitted to the pulmonary department with massive hemoptysis, fever, shortness of breath and weight loss of 13 kg over a 4-month-period. There was no prior medico-surgical illness history. Initially, the physical examinations were unremarkable. A chest X ray showed several round opacities in both lower lung fields. Computed tomography showed multiple pulmonary artery aneurysms and thrombi in both lower lobes. (Fig. 1A) Pulmonary artery angiography showed multiple pulmonary artery aneurysms and a large filling defect in the right lower lobe field, which was indicated a pseudoaneurysm. (Fig. 1B) Preoperative bronchoscopy or needle biopsy was not performed for the fear of a possible rupture. Upon admission, meticulous physical examination was done being focused on the oral cavity and external genitalia due to the growing suspicion of Behçet's disease, which was indicated by the clinical signs such as pulmonary aneurysms and vasculitis. However, no ulcer or other abnormal findings were found in his oral cavity or genital region. There were no aberrations in the laboratory finding, except for leukocytosis.
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Fig. 1. (A) Chest CT shows a large pulmonary artery aneurysm and mural thrombus with the pooling and stasis of contrast media. (B) Angiography shows filling defect in the right lower lobe, suggestive pseudoaneurysm. (Arrow).
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Embolization therapy of both lower lungs was performed. The post-embolization angiogram showed no pulmonary arterial aneurysmal filling with contrast material. However, the hemoptysis was continued. Therefore, the patient underwent a right lower lobe lobectomy of the lung. At the postoperative 10th day, the patient was discharged without special problems. Three weeks later, the patient was again admitted to the thoracic department with recurrent hemoptysis and marked swelling in both legs. Computed tomography showed a larger left lower pulmonary artery aneurysm than that of the previous study and an inferior vena caval thrombosis that was absent at the previous study. (Fig. 2A and B) He underwent a left lower lobectomy. At the postoperative 12th day, the patient was discharged with steroid therapy. There was no hemoptysis or other complications during a 10-month follow up after surgery.
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Fig. 2. (A and B) Follow up chest CT shows other pulmonary artery aneurysms with a mural thrombus in the left lower lobe and inferior vena. (Arrow).
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2. Discussion
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Hughes–Stovin syndrome is a rare disease that was first described in 1959 [1]. The typical clinical presentation consists of three phases: a first stage involving symptoms of thrombophlebitis, a second stage consisting of the formation and enlargement of pulmonary aneurysms, and a third stage of an aneurysmal rupture that triggers massive hemoptysis and death [2].
The pathogenesis of Hughes–Stovin syndrome is unknown. In their initial report [1], Hughes and Stovin described the histological evidence of a congenital qualitative defect in the bronchial artery walls, which leads to degenerative changes in the vessel walls, causing inadequate nutrition of the pulmonary arteries. In the setting of pulmonary embolic disease, inflammation and vessel wall destruction occurred and aneurysms were formed. Histologically, these aneurysms may resemble mycotic aneurysms but no infectious agent has been identified. It has been suggested that Hughes–Stovin syndrome may be a variant of Behçet's disease [3,4]. Both diseases are characterized by the destruction of the wall of the pulmonary arteries and perivascular infiltration [3]. However, the typical symptoms of Behçet's disease such as oral or genital ulceration, skin lesions, iritis or arthralgia are missing in most patients with Hughes–Stovin syndrome.
The nonspecific nature of the clinical manifestations of pulmonary artery aneurysms makes them difficult to be diagnosed. Conventional angiography or helical computed tomography remains the best tool for a better evaluation of the aneurysms in Hughes–Stovin syndrome.
Hughes–Stovin syndrome is often fatal as a result of massive hemoptysis but there is no standard treatment yet. Although there have been no controlled trials, the therapeutic options for the management of pulmonary artery aneurysms in Behçet's disease include medical treatment consisting of immunosuppressive agents alone or a combination with steroids. This approach has been found to be the most beneficial when administered at the early stages of the disease before the irreversible damage to the arterial wall develops. Tunaci et al. reported the complete disappearance or regression of a pulmonary artery aneurysm during a 3–42 (mean 21) month follow up period of 13 Behçet's patients receiving immunosuppressant treatment [5]. Steroids may be tapered according to the clinical response but immunosuppressants are often continued for at least 1 year after complete remission [6]. However, despite these treatments, serious hemoptysis occasionally recurs. If pulmonary arterial aneurysms are limited to a single segment or one lung, a lobectomy or pneumonectomy is principally indicated to remove the affected lung. In addition to surgery, the treatment of recurrent hemoptysis due to lesions of the bronchial arteries by embolization is well established [7,8]. Moreover, the use of anticoagulants for treating this condition might impose a therapeutic dilemma if a pulmonary artery embolism and deep vein thrombosis are present. Anticoagulation and platelet inhibitors to prevent further thromboembolic events should not be used due to the increased risk of fatal hemoptysis [9].
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References
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- Hughes JP, Stovin PGJ. Segmental pulmonary artery aneurysm with peripheral venous thrombosis. Br J Dis Chest 1959;53:19-27.[CrossRef][Medline]
- Reimond WV, Emmrich J, Hamjanz D, Kochsiek K. Multiple aneurysms of the pulmonary artery following recurrent septic pulmonary embolism (Hughes–Stovin syndrome): report of one case. Arch Klin Med 1968;215:1-18[in German].[Medline]
- Durieus P, Bletry O, Huchon G, Wechsler B, Chretien J, Godeau P. Multiple pulmonary arterial aneurysms in Behçet's disease and Hughes–Stovin syndrome. Am J Med 1981;71:736-741.[CrossRef][Medline]
- Francois MF. Is Hughes–Stovin syndrome a particular expression of Behçet's disease?. Chest 1983;83:288.[Medline]
- Tunaci M, Ozkorkmaz B, Tunaci A, Gul A, Engin G, Acunas B. CT findings of pulmonary artery aneurysms during treatment for Behçet's disease. AJR Am J Roentgenol 1999;172:729-733.[Abstract/Free Full Text]
- Erkan F, Kiyan E, Tunaci A. Pulmonary complications of Behçet's disease. Clin Chest Med 2002;23:493-503.[CrossRef][Medline]
- Remy J, Arnaud A, Fardou H. Treatment of hemoptysis by embolization of bronchial arteries. Radiology 1977;122:33-37.[Abstract]
- Uflaker R, Kaemmerer A, Picon PD, Rizzon CFC, Neves CMC, Oliveira ESB, Azevedo SNB, Ossanai R. Bronchial artery embolization in the management of hemoptysis: technical aspects and long-term results. Radiology 1985;157:637-644.[Abstract/Free Full Text]
- Bowman S, Honey M. Pulmonary arterial occlusions and aneurysms: a forme Fruste of Behçet's or Hughes–Stovin syndrome. Br Heart J 1990;63:66-68.[Abstract/Free Full Text]
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Abstract
1. Case report
