Video-Assisted Thoracic Surgery for the Management of Pleural and Pericardial Effusion in Behçet's Syndrome

Abstract

A 28-year-old man, who presented with pleural and pericardial chylous effusion secondary to superior vena cava syndrome, was diagnosed with Behçet's syndrome. A pericardial window was created by video-assisted thoracic surgery to relieve cardiac tamponade, and this technique also enabled lung biopsy, pleural abrasion, and drainage of the pleural effusion. We report this case because of its rarity. To the best of our knowledge, the literature contains no other report of the use of video-assisted thoracic surgery for creating a pericardial window for the treatment of cardiac tamponade in a case of Behçet's syndrome.

Behçet's syndrome is a chronic multisystem vasculitis of unknown origin, characterized by uveitis and by oral and genital ulceration. It is a systemic disorder that may involve various organs. Histologic studies show multifocal vasculitis involving the veins, capillaries, and arteries. ^1,2 The prevalence of pulmonary involvement in Behçet's is 5% to 10%. ^1,2 Video-assisted thoracic surgery (VATS) provides an excellent view of the pleural cavity and pericardium, and the creation of a pericardial window for videothoracoscopic management of drainage is less invasive than are the traditional alternatives. ³

Case Report

A 28-year-old white man was admitted to our clinic with a 2-month history of dyspnea, chest pain, and swelling in the face and neck. He also complained of oral and genitourinary ulcerative lesions of the mucous membranes. Physical examination revealed findings indicative of superior vena cava syndrome (SVCS), together with an absence of breath sounds at the left side of the chest and hepatomegaly. Dermatologic findings and the pathergic test were positive for Behçet's. Chest radiography showed pleural and pericardial effusion, and mediastinal widening (Fig. 1). Venography of the upper extremities showed occlusion of the superior vena cava and of the innominate and bilateral subclavian veins (Fig. 2). Computed tomography showed pericardial effusion, left pleural effusion, and thrombosis of the superior vena cava. Echocardiography showed compression of the right ventricle. Diagnostic thoracentesis yielded a milky white fluid, with a triglyceride content of 362 mg/dL⁻¹ and a cholesterol level of 72.4/mg dL⁻¹. Cytologic examination of the fluid revealed a predominance of lymphocytes. The sedimentation rate was 57 mm/h, the white-blood-cell count was 9,980/mm³, and hypoproteinemia was observed. Other laboratory examinations and pulmonary function tests were normal.

Fig. 1 Chest radiograph showing pericardial and left pleural effusion.

Fig. 2 Digital subtraction angiogram showing obstruction of the superior vena cava, innominate, and subclavian veins in the right upper extremity.

For diagnosis and treatment of the pleural and pericardial effusion, we performed VATS under general anesthesia, aspirating chylous fluid in the amounts of 900 cc (pleural) and 450 cc (pericardial). Thickening of the pleura and pericardium was minimal. Histologic examination of pericardial and pleural frozen sections showed them to be benign, but there was evidence of vasculitis both in these biopsy materials and in tissue samples gathered from the lung and pleura. Mechanical pleural abrasion and pericardial window were performed (Fig. 3).

Fig. 3 Videothoracoscopic view of the pericardial window.

D = diaphragm; L = lung; Pr = pericardium; Pw = pericardial window

In the postoperative period, the patient's signs and symptoms, both physical and radiographic, regressed. The patient was given cyclophosphamide 2 mg/kg per day for 30 days and methylprednisolone 1 mg/kg per day for 30 days. Pleural drainage continued for 5 days after the operation. The patient was discharged on the 8th postoperative day and has remained asymptomatic during 10 months of follow-up.

Discussion

Behçet's syndrome, 1st described in 1937 by a Turkish dermatologist named Halushi Behçet, ^1,2 is difficult to diagnose because its clinical manifestations are so various. Antiaggregants, steroids, and both immunosuppressive and immunostimulant agents may be used in the treatment of Behçet's. ¹ Vascular complications of the disorder include thromboembolism and arterial aneurysms and occlusions. Caval thrombosis has been observed, but is relatively uncommon. ⁴ Cases such as ours have been reported, in which SVCS was found in association with pleural or pericardial effusion, or both. ^4,5 Indeed, SVCS is usually the cause of chylous pleural and pericardial effusion in Behçet's. ⁵ In our patient, milky pleural effusion was observed at thoracentesis and VATS.

Pericardiocentesis for placement of a subxiphoid tube drain or thoracotomy for drainage of pericardial fluid can be performed for relief of tamponade. ^3,6 However, we performed VATS for diagnosis and treatment of the right ventricular compression observed at echocardiography, because the creation of a pericardial window by means of VATS is easier for the surgeon and safer and more comfortable for the patient than are the older and more traditional alternatives. ^3,6

To the best of our knowledge, the literature contains no other report of the use of VATS for creating a pericardial window for the treatment of cardiac tamponade in a case of Behçet's syndrome. Çöplü's group ⁵ reported a case of chylous pericardial and pleural effusion in Behçet's, but they performed conventional thoracoscopy (direct thoracoscopy by thoracoscope) for pleural effusion and subxiphoid tube drainage for pericardial effusion. Their pleural biopsy had a non-specific result and their pericardial biopsy revealed fibrosis. ⁵ In our patient, pericardial and pleural biopsies indicated vasculitis.

In summary, we report a case of Behçet's syndrome in which the patient had SVCS and pericardial and pleural chylous effusion, which we treated with VATS and medical therapy.