Abstract
Although substernal enlargement of a goiter can cause compression of several mediastinal structures, superior vena cava syndrome (SVCS) is an uncommon complication of retrosternal goiter, particularly in the 21st century (1). We present a patient who was seen recently; she developed SVCS secondary to substernal goiter (2).
A 79-year-old woman was referred to our department for treatment of an enlarged thyroid. She had partial thyroidectomy 35 years ago, but the histopathological diagnosis and the details of her thyroid history were unclear. Physical examination revealed a marked thyroid swelling, with an impressive enlargement of jugular and superior limbs' veins (Fig. 1). There was no evidence of dyspnea, dysphagia, gastrointestinal bleeding, recurrent, or phrenic nerve palsies. Ultrasonography and computed tomography of the neck and thorax revealed a huge, multinodular goiter that was mostly intrathoracic. The goiter extended from the thyroid lamina to the tracheal bifurcation. There was upper airway and brachiocephalic vessel compression (Fig. 2). Thyroid function was normal (thyrotropin, 1.4 mg/mL; free thyroxine, 5.6 mg/mL), and the dominant nodule was benign on fine-needle aspiration.
Enlargement of jugular and superior limbs' veins.
Computed tomography image of the goiter.
Due to the large size of the goiter and the compression of mediastinal structures, a total thyroidectomy was proposed and performed without complications. The weight of the surgical specimen was 334 g. Histological examination showed nodular thyroid hyperplasia (Fig. 3). One month after surgery, there was complete remission of SVCS.
Histopathology: nodular thyroid hyperplasia.
SVCS usually presents with edema and lividity of the face with enlargement of jugular and superior limbs' veins (3). Malignancy is the most common cause (97%) of SVCS (4), with lymphoma and lung cancer being the most frequent diagnosis (5). There are also reported cases of SVCS associated with Grave's disease and thyroid carcinomas. Computed tomography scan is the best imaging technique, and surgery (thyroidectomy) remains the most appropriate therapy for patients with compressive substernal nontoxic multinodular goiters. However, radioactive iodine might be an attractive alternative, especially in elderly patients in whom surgery is contraindicated (6). High-dose radioactive 131I treatment has been effectively used to reduce the size of nodular goiters with intrathoracic extension, with improvements in obstructive symptoms (7). Pretreatment with recombinant human thyrotropin might allow treatment with lower doses of radioactive iodine for thyroid volume reduction (8,9). Nevertheless, we have to keep in mind that radiation thyroiditis might result in worsening of airway obstruction. Also, if there is a need to rule out the diagnosis of carcinoma, the surgical approach is necessary (10).