Ann Thorac Surg 1998;66:946-948
© 1998 The Society of Thoracic Surgeons
Case Reports
Surgical management of superior vena caval syndrome in sarcoidosis
Deepak Narayan, MDa,
Louis Brown, MDa,
John O. Thayer, MDa
a Department of Surgery, St. Francis Hospital, Hartford, Connecticut, USA
Accepted for publication April 2, 1998.
Address reprint requests to Dr Thayer, 1000 Asylum Ave, Suite 3212, Hartford, CT 06105
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Abstract
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Sarcoidosis causing the superior vena caval syndrome has been only rarely documented in the literature. We report surgical management of this problem with a spiral vein graft.
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Introduction
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Sarcoidosis is a disease of unknown etiology, which is more prevalent in blacks than whites and commonly affects the pulmonary system [1]. Cases of sarcoidosis producing a superior vena caval (SVC) syndrome reported in the literature generally have been treated medically. Here we report a patient requiring surgical intervention.
A 52-year-old black man presented with complaints of a gradually increasing swelling of his head and both arms associated with shortness of breath over a period of 1 year. He was thought to have an allergic reaction and was treated with steroids for approximately 2 months before his eventual surgical intervention. His past medical history was significant for hypercholesterolemia and asthma. His medications included theophylline, simvastatin, and albuterol. Physical examination was remarkable for a striking swelling of his head and upper arms as well as the presence of dilated veins over his anterior chest wall. His laboratory values were within normal limits. A chest radiograph showed the presence of a widened mediastinum with some calcification in the mediastinum. A superior vena cavogram performed via injection of both arms revealed an obstruction of the right subclavian vein just before its junction with the right internal jugular vein as well as a complete obstruction of the left innominate vein (Fig 1). A computed tomographic scan demonstrated calcified lymph nodes in the mediastinum with obstruction of the SVC without any evidence of a pulmonary mass. He had an episode of acute shortness of breath, which necessitated a visit to the emergency room, where this was thought to be the result of upper airway obstruction brought on by edema secondary to his SVC syndrome. Surgical intervention was indicated for the severity of the patient’s symptoms and failure of medical therapy.
Exploration via a median sternotomy revealed a fibrotic and thickened pericardium and multiple dilated veins in the mediastinum. The left saphenous vein was harvested and a spiral vein graft of a suitable length was created using a 40F chest tube as a stent, giving it a diameter of approximately 13 mm. An end-to-side anastomosis to the left innominate vein was performed followed by an end-to-end anastomosis to the right atrial appendage, after excision of part of the wall and trabeculae. Pathologic examination of the excised tissue showed noncaseating granulomata consistent with sarcoidosis. Cultures for fungi, bacteria, and acid-fast bacilli were negative. He was placed on a maintenance dose of steroids after an uneventful recovery. A follow-up venogram done 4 months later revealed a patent graft with excellent emptying (Fig 2). The patient was completely asymptomatic at this time.
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Comment
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Superior vena caval obstruction secondary to sarcoidosis is a rarely reported event. A review of the literature revealed 6 reported cases, none of which were treated surgically. On the other hand, surgical series dealing with benign causes of SVC obstruction such as the one by Doty and associates [2] do not mention sarcoidosis as a causative entity. Of the 6 cases of sarcoidosis producing a SVC syndrome recorded in the literature, 3 of the patients described died in a time frame ranging from 1 day to 13 years. The first of these patients was reported by Morgans and colleagues [3], who described a 59-year-old woman with mitral stenosis who presented with SVC obstruction. A biopsy of her enlarged supraclavicular and mediastinal nodes confirmed the diagnosis of sarcoidosis. After a minimal response to steroids, digitalis, and diuresis she succumbed to a profound bone marrow depression resulting from sulfonamide therapy administered after a closed mitral valvotomy. The second patient, a 22-year-old black man reported by Fincher and Sherman [4], presented in extremis with cardiogenic shock. On autopsy he was found to have massive enlargement of the paratracheal nodes, which showed the classic noncaseating granulomata of sarcoidosis. The last patient was a 64-year-old black woman in whom SVC syndrome, breast granulomata, and tracheobronchial stenosis developed secondary to sarcoidosis, which was confirmed by a scalene node biopsy [1]. Despite a good response to steroids she died of massive hemoptysis 13 years later, presumably because of granulomatous erosion into a major pulmonary vessel. Of the remaining 3 cases 1 apparently recovered without any therapeutic intervention either medical or surgical [1]. The case reported by Gordonson and associates [5] involved a venographically documented SVC obstruction in an asymptomatic patient without a tissue diagnosis. This patient was treated with steroids, isoniazid, and pyridoxine (until cultures were negative) with a complete resolution of the mediastinal mass on a chest radiograph. Brandsetter and colleagues’ patient [6] had clinical evidence of an SVC syndrome with a transbronchial biopsy positive for sarcoidosis and responded well to steroids. Although most of the cases of SVC obstruction are secondary to malignancy, the incidence of benign iatrogenic venous obstruction is thought to be rising because of the increased use of central venous catheters.
The role of mediastinoscopy in the SVC syndrome has not been clearly defined. The risks of bleeding in the presence of venous hypertension and the poor prognosis have discouraged the use of mediastinoscopy in this condition. Jahangiri and Goldstraw [7] state that mediastinoscopy is a safe and effective technique for establishing histologic diagnosis in SVC obstruction when less invasive methods have been unsuccessful. A vast majority of their cases (33 of 34), however, carried a diagnosis of a malignancy, and they go on to state that mediastinoscopy for a benign process is a far more extensive procedure.
Isolated case reports such as the one by Rantis and Littooy [8] have demonstrated the successful treatment of benign SVC obstruction by thrombolytic therapy. However the long-term results of such therapy in contrast to surgical intervention are unknown. It is possible that with increasing experience it may play an important role in the management of this disorder. Although the long-term patency of grafts in the treatment of SVC obstruction caused by malignancy is rarely an issue because of the grim prognosis, it is much more important in the treatment of benign obstruction. The spiral vein graft appears to be the treatment of choice for benign disease processes requiring surgical reconstruction of the SVC [2].
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References
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- McPherson J.G., Yeoh C.B. Rare manifestations of sarcoidosis. J Natl Med Assoc 1993;85:869-872.[Medline]
- Doty D.B., Doty J.R., Jones K.W. Bypass of superior vena cava. J Thorac Cardiovasc Surg 1990;99:889-896.[Abstract]
- Morgans W.E., Al-Jihawi A.N., Mbatha P.B. Superior vena cava obstruction caused by sarcoidosis. Thorax 1980;35:397-398.[Free Full Text]
- Fincher R.M.E., Sherman E.B. Superior vena caval obstruction due to sarcoidosis. South Med J 1986;79:1306-1307.[Medline]
- Gordonson J., Trachtenberg S., Sargent E.S. Superior vena cava obstruction due to sarcoidosis. Chest 1973;63:292-293.[Abstract/Free Full Text]
- Brandsetter R.D., Hansen D.E., Jarowski C.I., King T. Superior vena cava syndrome as the initial clinical manifestation of sarcoidosis. Heart Lung 1981;10:101-104.[Medline]
- Jahangari M., Goldstraw P. The role of mediastinoscopy in superior vena caval obstruction. Ann Thorac Surg 1995;59:453-455.[Abstract/Free Full Text]
- Rantis P.C., Littooy F.N. Successful treatment of prolonged superior vena cava syndrome with thrombolytic therapy. J Vasc Surg 1994;20:108-113.[Medline]
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Abstract
Introduction
