| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
J Thorac Cardiovasc Surg 1996;111:486-487
© 1996 Mosby, Inc.
BRIEF COMMUNICATIONS |
Rome, Italy
From the Department of Thoracic Surgery, University of Tor Vergata, Rome, Italy.
Accepted for publication August 9, 1995.
Jaretzki and Wolff
1 in 1988 recommended the maximal thymectomy procedure in the treatment of myasthenia gravis with or without thymoma and of thymoma with or without myasthenia gravis. The wide mediastinal and cervical exposure ensures complete removal of all thymic tissue and eventually of ectopic thymus. Recently, this operation allowed us to find a small cervical thymoma and to perform a curative resection in a patient with multiple autoimmune disorders.
The patient was a 32-year-old woman who successfully underwent splenectomy, followed by administration of immune serum globulin, in 1988 for the treatment of idiopathic thrombocytopenic purpura unresponsive to corticosteroids. In December 1994, the patient was admitted to our hospital with petechiae, mucous membrane bleeding, bilateral ptosis, and nasal speech. She also had oral lichen planus. Results of laboratory examinations were remarkable for a platelet count of 11,000 cells/ml. A direct Dixon test had positive results for the presence of antiplatelet autoantibodies. A bone marrow biopsy specimen showed an increased number of megakaryocytes and hypoproduction of platelets. No signs of myelodisplastic syndrome were observed, and a normal karyotype was revealed by chromosomal analysis. Her serum antibody against acetylcholine receptor was high (19.4 pmol/ml), and electromyography confirmed the diagnosis of myasthenia gravis. Chest radiography and computed tomography failed to reveal any intrathoracic and extrathoracic abnormalities. Treatment was started, but the patient's condition responded poorly to pyridostigmine bromide (60 mg orally every 6 hours) and prednisone (50 mg/day). When seen by us in February 1995, the patient had petechiae, severe shortness of breath, ptosis, myopathic facies, nasal speech, dysarthria, dysphagia of both liquids and solids with nasal regurgitation, and generalized weakness. The patient reported recent memory loss and had gustatory and olfactory hallucinations. She also became dysphoric. Pulmonary function tests revealed forced vital capacity of 2.2 L (90% predicted), and forced expiratory volume in 1 second of 1.7 L (42% predicted). Arterial blood gases with the patient breathing room air revealed an arterial oxygen tension of 68 mm Hg, an arterial carbon dioxide tension of 29 mm Hg, and a pH of 7.50. Plasmaphereses were performed in five sessions, and 4500 ml of plasma was exchanged. After this treatment, the patient's respiratory status, myasthenic symptoms, and psychiatric symptoms improved, and pyridostigmine bromide was successfully readministered (60 mg orally every 8 hours).
Two units of platelets concentrate were transfused prophylactically, and the patient underwent a total median sternotomy with extensive removal of all thymic tissue, mediastinal fat, and both mediastinal pleural sheets. The dissection was continued through a transverse low cervical incision and a 1 x 3 cm flat, well-encapsulated, solid mass was found under the sternocleidomastoid muscle, connected to the left superior pole of the thymus gland and adherent to the left lobe of the thyroid. The mass, which was easily and completely excised, was histologically classified as an epithelial, medullary type, Masaoka stage I thymoma. Microscopic examination of the removed mediastinal tissue demonstrated involution of normal thymic tissue without any specific findings. The patient made an uneventful recovery and was discharged 11 days after the operation on a regimen of prednisone (50 mg/day) and pyridostigmine (60 mg orally every 8 hours). Two months later, platelet count had increased to 210,000 cells/ml and results of direct Dixon test were negative. At the same time, oral lesions completely disappeared and she was able to stop taking steroids. Four months after operation, the patient was well and did not have further psychiatric symptoms. Pyridostigmine bromide was discontinued, and computed tomographic scan did not reveal any signs of recurrent tumor.
It has been estimated that ectopic thymic tissue in the neck has an incidence as high as 21.0%, 2 but thymoma arising from undescended cervical thymus is exceedingly rare. To the best of our knowledge, fewer than 10 cases of true cervical thymoma have been previously reported. The diagnosis of aberrant cervical thymus or thymoma is rarely made before operation. Awareness of this entity and the need for as complete as possible removal of thymic tissue compel the surgeon to perform a meticulous neck dissection in all myasthenic patients, with or without known thymoma.
The association of myasthenia gravis and other autoimmune disorders with thymoma has been usually described in the form of isolated reports. Although in most of these reports the extent of the procedure has not been clearly described, thymectomy has been reported as effective for control of myasthenia gravis but failing to improve the clinical course of the other associated diseases. 3-5 We believe, however, that even in immune-mediated disorders other than myasthenia gravis extended and meticulous surgical resection could result in good remission rates.
Footnotes
J THORAC CARDIOVASC SURG 1996;111:486-7 
References
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| ANN THORAC SURG | ASIAN CARDIOVASC THORAC ANN | EUR J CARDIOTHORAC SURG |
| J THORAC CARDIOVASC SURG | ICVTS | ALL CTSNet JOURNALS |
