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Ann Thorac Surg 1996;61:733-735
© 1996 The Society of Thoracic Surgeons

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Case Report

Surgical Therapy of Pulmonary Arterial Aneurysms in Behçet's Syndrome

Hasan Tüzün, MD, Vedat Hamuryudan, MD, Sava Yildirim, MD, Kazim Beirli, MD, Yener Yörük, MD, Sabahattin Yurdakul, MD, Hasan Yazici, MD

Departments of Thoracic and Cardiovascular Surgery and Romatology, Istanbul University, Cerrahpaa Medical Faculty, Istanbul, Turkey.

Accepted for publication August 18, 1995.

	Abstract

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Two patients with Behçet's syndrome were operated for exsanguinating pulmonary artery aneurysm into a bronchus. Lower lobectomy was performed in the first patient. He died of hypoxia and sepsis on the 8th postoperative day. Pneumonectomy was undertaken in the second patient, who is well 30 months after the operation.

	Introduction

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Behçet's syndrome is a multisystemic vasculitis of unknown origin affecting all sizes of arteries and veins [1]. Clinical features include recurrent oral and genital ulcerations, eye lesions (anterior/posterior uveitis), skin lesions (erythema nodosum, pseudofolliculitis or papulopustular lesions), superficial or deep vein thrombophlebitis, epididymitis, arterial occlusions or aneurysms, central nervous system involvement, arthritis, gastrointestinal involvement, and a positive pathergy test [2].

Pulmonary artery is the second most affected vessel after aorta [1]. The prognosis in these patients is poor due to the risk of rupture with fatal hemoptysis [3]. Although surgical intervention with adjunct immunotherapy is considered the best therapy for systemic aneurysms, there is still an ongoing discussion about the role of surgical therapy in the treatment of pulmonary aneurysms [4–7].

Herein we report 2 patients with Behçet's syndrome who were operated on for exsanguinating pulmonary artery aneurysms into a bronchus.

	Case Reports

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Patient 1
A 35-year-old man had symptoms of Behçet's syndrome for 6 years, consisting of orogenital ulcerations, lower extremity thrombophlebitis, arthritis, erythema nodosum, and folliculitis. Initially azathioprine was given but later he discontinued the therapy. Skin reactivity to ordinary needle prick was positive. In March 1990, he was admitted to a pneumology clinic with the symptoms of fatigue, blood-tinged sputum, cough, night sweats, and weight loss. Diagnosis of tuberculosis was made and therapy with streptomycin, isoniazid, and rifampicin was started. In May 1991, he was readmitted with massive hemoptysis, which ceased spontaneously and required transfusion of one unit of blood. The chest roentgenogram and the thoracic computed tomographic examinations at that time revealed prominence of the paracardial pulmonary artery branch. He was transferred to the Rheumatology Department at the Faculty of Cerrahpaa Medical School. On his admission cyclophosphamide was given with an initial bolus of 1 g. The pneumonic infiltration of the right lower lobe showed resolution disclosing a prominent right pulmonary artery.

On the fifth clinical day emergency thoracotomy was undertaken for abundant hemoptysis. Preoperative bronchoscopy revealed organized coagulum in the intermediate bronchus with fresh blood. Lower lobectomy was performed for lower lobe pulmonary artery aneurysm. On the first postoperative day he was reintubated for developing hypoxia. On the fourth day tracheostomy was done to improve aspirations. On the 8th day he died of hypoxia and sepsis.

Macroscopic examination of the lobectomy specimen revealed a ruptured aneurysm of the lower lobe pulmonary artery of 4 x 3 cm into the dorsal segment bronchus (Fig 1). Microscopic examination showed intimal thickening and destruction of the elastic fibers in the media of the pulmonary artery. The aneurysm wall was infiltrated by polymorphonuclear and mononuclear cells. The postmortem study showed a dilated left main pulmonary artery with an adherent thrombus and two thrombosed saccular aneurysms of 1.5 cm before the branching of the left pulmonary artery. In addition, both upper lobes contained fibrocaseous areas with tubercle bacilli. The left lung also showed four small cavitary lesions with a large infarction area. Diffuse pneumonic infiltration was noticed in the remaining right lung. Additional findings were cicatricial ulcers of the terminal ileum and subendocardial scarring of the myocardium.

View larger version (40K): [in this window] [in a new window]   Fig 1. . Lower lobe pulmonary aneurysm penetrating into dorsal segment bronchus.

Nine months later he was readmitted to the Rheumatology Department with recurring hemoptysis. On admission his chest roentgenogram showed two round opacities side by side in the middle zone of the right lung. Pulmonary angiography through the cephalic vein revealed decreased perfusion of the right lung with an abrupt cut-off of the pulmonary artery after the division of the upper lobe branches. The lower lobe branches could not be visualized. A hardly filling sac of 1 x 2 cm was seen next to the main trunk. Operation was not considered in the presence of a thrombosed pulmonary aneurysm. However, 1 week later emergency thoracotomy had to be undertaken for hypovolemia causing massive hemoptysis. Operation revealed severe adhesions and a destroyed middle lobe with intraparenchymal bleeding. Because of dense fibrosis of the major fissure dissection was impossible. Pneumonectomy was done with an uneventful recovery. Since then he has only dyspnea on exercise. He is kept on an immunosuppressive regimen. The pneumonectomy specimen demonstrated an enlarged intermediary pulmonary artery with dense scarring. Medial to this segment there was a thrombosed aneurysm of 1.5 cm from which middle lobe arteries branched. The fistulous communication was between the enlarged intermediary pulmonary artery and the middle lobe bronchus (Fig 2).

View larger version (39K): [in this window] [in a new window]   Fig 2. . A fistulous communication between the enlarged intermediary pulmonary artery and middle lobe brochus.

	Comment

Top Footnotes Abstract Introduction Case Reports Comment References

The most common symptom is hemoptysis with pulmonary opacities on chest radiographs [10]. Intravenous digital subtraction angiography is the procedure of choice in the documentation of pulmonary arterial involvement. Magnetic resonance imaging is most helpful in the presumptive diagnosis of occluded aneurysms [10].

Various therapies with different results have been described. Severity of the hemoptysis and the number of aneurysms are key factors in choosing therapy. Immunosuppressive drugs alone or in combination with steroids are most beneficial when given in the early stages before irreversible damage to the arterial wall develops [6, 7]. If hemoptysis continues transcatheter embolization of a pulmonary artery segment should be tried, especially in cases with bilateral and multiple aneurysms [11]. If the embolization procedure fails or the fistulous communication is through a central pulmonary artery with or without an aneurysm, surgical therapy must be the procedure of choice. In the presence of this potentially fatal symptom the source of bleeding has to be determined without delay [9, 12]. Bronchoscopy could be a useful tool in showing bronchial communication with an organized coagulum in the involved bronchi [9, 13]. Once a fistula develops it would be too optimistic to expect healing of the irreversibly damaged arterial wall by medical therapy. Although the risks of operation in patients with Behçet's syndrome are high, it could be a life-saving procedure.

	Footnotes

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Address reprint requests to Dr Tüzün, Bahçelievler, Basin Sitesi, Kader sk, Kezban apt, 4/9, 34590, Istanbul, Turkey.

	References

Top Footnotes Abstract Introduction Case Reports Comment References

 

1. Lie JT. Vascular involvement in Behçet's disease: arterial and venous vessels of all size. J Rheumatol 1992;19:341–3.[Medline]
2. International Study Group for Behçet's Disease. Criteria for diagnosis of Behçet's disease. Lancet 1990;335:1078–80.[Medline]
3. Kohno S, Fujikawa M, Kanda T, Asai S, Hirota M, Sameshima Y. A case of Behçet's syndrome with rupture of a pulmonary aneurysm: autopsy findings and a literature review. Jpn J Med 1986;25:293–300.[Medline]
4. Hamuryudan V, Yurdakul S, Moral F, et al. Pulmonary arterial aneurysms in Behçet's syndrome (a report of 24 cases). Br J Rheumatol 1994;33:48–51.[Abstract/Free Full Text]
5. Bartlett ST, McCarthy WJ III, Palmer S, et al. Multiple aneurysms in Behçet's disease. Arch Surg 1988;123:1004–8.[Abstract/Free Full Text]
6. Raz I, Okon E, Chajek-Shaul T. Pulmonary manifestations in Behçet's syndrome. Chest 1988;95:585–9.
7. Stricker H, Malinverni R. Multiple large aneurysms of pulmonary arteries in Behçet's disease. Arch Intern Med 1989;149:925–7.[Abstract/Free Full Text]
8. Slavin RE, De Groot WJ. Pathology of the lung in Behçet's disease: case report and review of the literature. Am J Surg Pathol 1981;5:779–88.[Medline]
9. Barberis M, Casadio C, Borghini U. Massive haemoptysis in Behçet's syndrome: case report. Respiration 1987;52:303–7.[Medline]
10. Numan F, Ilak C, Berkmen T, Tüzün H, Çokyüksel O. Behçet's disease: pulmonary arterial involvement in 15 cases. Radiology 1994;192:465–8.[Abstract/Free Full Text]
11. Lacombe P, Frija G, Parlier H, et al. Transcatheter embolization of multiple pulmonary artery aneurysms in Behçet's syndrome. Acta Radiol Diagn 1985;26:251–3.[Medline]
12. Salomon F, Weinberger A, Nili M, Avidor I, et al. Massive hemoptysis complicating Behçet's syndrome: the importance of early pulmonary angiography and operation. Ann Thorac Surg 1988;45:566–7.[Abstract]
13. Yamada H, Masuyama Y, Maesaki S, et al. Observation of bleeding sites by fiberoptic bronchoscopy in a case of vasculo-Behçet's disease with pulmonary involvement. Nippon Kyobu Shikkan Gakkai Zasshi 1991;29:622–6.

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Kazim Besirli Yener Yörük Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Tüzün, H. Articles by Yazici, H. Search for Related Content PubMed PubMed Citation Articles by Tüzün, H. Articles by Yazici, H.