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A large substernal goiter as a cause of a pulmonary perfusion defect.

A pulmonary perfusion defect was caused by an extremely large anterior mediastinal mass subsequently proved by I-131 imaging to be an intrathoracic extension of a cervical goiter. Substernal goiter is not known to be reported previously as one of the causes of a perfusion defect in lung scanning.

Aged

Substernal goiter.

The literature on substernal goiter from the seventeenth century to the present is reviewed. Substernal goiter may be defined as any thyroid enlargement that has its greater mass inferior to the thoracic inlet. Truly ectopic mediastinal goiters are rare, and most substernal goiters arise from and maintain some attachment to the cervical thyroid gland. Patients are generally in the fifth decade of life, and women predominate. Most patients experience dyspnea, stridor, or dysphagia, but 15 to 50% are asymptomatic; symptoms are often positional, and acute stridor may occur. Ten to twenty percent have no cervical mass or tracheal deviation on examination, and virtually all patients are euthyroid. Standard chest roentgenograms are often diagnostic, but computed tomographic or radioactive iodine scans may be helpful. The presence of a substernal goiter in all but the highest-risk patients is an indication for resection, usually through a cervical collar incision; an occasional patient will require sternotomy or thoracotomy. Death or major complications should be rare postoperatively. Substernal goiters are adenomatous and benign, but carcinoma occurs in 2 to 3% and may be occult. Patients should be followed closely, as these goiters may recur.

Adolescent

Substernal goiter with superior vena caval obstruction.

Substernal goiter with vena caval obstruction remains an interesting diagnostic and therapeutic problem. Since 85 to 90 percent of superior vena caval obstructions are due to a malignant disease, an intrathoracic goiter represents one of the benign, curable causes of this syndrome. This report describes the clinical course of a 44-year-old woman who presented with this entity and was successfully corrected by surgical intervention. A discussion of diagnostic, operative, and prognostic considerations is presented.

Constriction

Operative treatment of substernal goiters.

This report describes the operative experience in 72 patients with substernal goiters treated over the past 5.5 years. Even though the incidence of multinodular goiter has decreased in the United States due to the routine use of iodized salt, we continue to see a large number of patients with massive goiters, predominantly from Caribbean Islands. The diagnosis of substernal goiter was made on clinical examination augmented by such radiologic studies, as chest x-ray, barium esophagograms, airway films, and CT scans. Computed tomographic (CT) scanning was particularly helpful in evaluating the extent of substernal extension. Confirmation of the extent of disease was made at the time of operation. Ninety percent of the patients had tracheal deviation and 85% were symptomatic from airway compression. Esophageal compression was noted in 60% of the patients. All patients had a long history of goiter with recent onset of pressure symptoms. Flow-volume-loop studies were performed in 44% of the patients and were useful in the evaluation of pressure symptoms. However, the decision for operation was made primarily based on clinical evaluation of signs and symptoms. Sixteen patients in this group were admitted with acute airway distress requiring airway intubation or semi-emergency decompression. Only 1 patient required mediastinal splitting, while all others were operated by the cervical approach. The decision as to the extent of thyroidectomy was made at the time of operation. Drains were routinely used because of the large dead space. One patient developed a hematoma in the recovery room and required re-exploration.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult

Substernal goiter. Analysis of 80 patients from Massachusetts General Hospital.

Eighty patients at the Massachusetts General Hospital underwent resection of substernal goiter in the years 1976 to 1982. Mean age of the 50 women and 30 men was 56 years, and 10 (19 percent) had undergone prior thyroid surgery. The most common symptoms were cervical mass (69 percent), dysphagia (33 percent), and dyspnea (28 percent); 13 percent were asymptomatic. On examination, cervical mass was present in most (90 percent) but not all patients, 51 percent were obese, and more than one third had tracheal deviation. Fifty-one of 52 patients tested were euthyroid and one was mildly hypothyroid. Chest radiographs showed tracheal deviation in 79 percent and soft tissue mass in 56 percent. Seventy-eight patients underwent resection through a cervical collar incision only; one had cervical incision plus upper partial sternotomy; and one required cervical incision plus full median sternotomy. Pathologic examination revealed multinodular goiter in 41 (51 percent), follicular adenoma in 35 (44 percent), and Hashimoto's thyroiditis in 4 (5 percent). Mean goiter weight was 104 g, and the mean greatest dimension was 9 cm. Occult papillary carcinoma was found in two patients. There were no deaths or major complications. Analysis of our data indicate the following: (1) Substernal goiter may exist in the absence of symptoms or signs. (2) Extensive radiologic evaluation and thyroid function testing are rarely required. (3) With rare exceptions, substernal goiter represents an extension of a cervical growth through the thoracic inlet and can be approached through a cervical collar incision. (4) Histologically, these are multinodular goiters or follicular adenomas, although Hashimoto's thyroiditis may occur. (5) Given the small but present risks of acute stridor or occult malignancy and the negligible surgical risk, operation should be recommended. (6) Patients should be followed since, with or without levothyroxine, goiters may recur.

Adult

Surgical treatment of substernal goiters.

Fifty-one patients (4.6%) underwent resection of a substernal goiter in a fifteen-year period during the course of 1103 thyroidectomies. Forty-eight (94.2%) goiters were benign and three (5.8%) malignant. Mean age was 55 years. Female:male ratio was 2:1. Four patients (7.8%) had undergone prior thyroid surgery. Most had long-standing goiters (mean duration: 15 years). The most common symptoms included airway compression (56.8%), hoarseness (13.7%), dysphagia (11.7%), superior vena cava syndrome (9.8%). Twelve patients (23.5%) were asymptomatic. Chest X-rays showed a tracheal deviation and/or a mediastinal mass in 43 patients (84.3%). Goiter extended into the right mediastinum in 28 patients (54.9%), into the left in 19 (37.2%), and bilaterally in three (5.8%). A cervical collar incision provided adequate exposure in 42 cases (82.3%). Five patients (9.8%) required a cervical incision plus partial median sternotomy and one (1.9%) a cervical incision plus a right postero-lateral thoracotomy. In three asymptomatic patients (5.8%) thoracotomy was followed by cervical incision due to a preoperative incorrect diagnosis. Major postoperative complications included two cervico-mediastinal hematoma with one subsequent death and four (7.8%) recurrent laryngeal nerve palsy. This series showed that: (1) Standard chest roetgenogram with esophagogram is still the most useful investigation, although CAT scan can help in planning the operation. (2) Cervical collar incision provides adequate exposure in nearly all cases. (3) When goiter enucleation is difficult or at risk, a complementary median sternotomy is indicated in right retrovascular goiters. (4) Operation should be recommended in all but the highest-risk patients. (5) Tracheal intubation with small caliber tubes is nearly always possible in patients with acute tracheal compression.

Adult

[Substernal goiters. 218 operated cases].

The authors are presenting a retrospective study on 218 retrosternal goiters operated between 1968 and 1991. 33% of the goiters were incidentally discovered on a plain X-ray of the chest. Symptoms of compression were present in 50.5% of patients and hyperthyroidism in 16.5%. Respiratory manifestations were more frequent and more severe in patients aged 70 and more. Moreover 90% of those old patients were symptomatic. Goiters migrated anteriorly in 57.7% of cases, posteriorly in 33.9%, both anteriorly and posteriorly in 5.5%. Type of migration was unknown in 2.5%. 3.7% were malignant. 27 patients with respiratory symptoms received corticosteroids to reduce the airway compression. Among 52 patients with hyperthyroidism, 36 were given antithyroid drugs. Among them, some received corticosteroid drugs in order to reduce risks of mediastinal compression. A simple cervicotomy was adequate in 92.7% of case and was completed by sternotomy in 7.3%. The operation was iterative for relapsing or forgotten thoracic goiters in 24 cases. Morbidity was slight even for sternotomized patients. Mortality was noted in 2 cases (0.9%) one of them operated on for poorly-differentiated and suffocating carcinoma of the thyroid. We advise a straightforward surgical attitude as a result of the slight morbidity and mortality, even in sternotomized patients in order to avoid severe compressive complications of the airways.

Adult

[Substernal goiter].

In a series of 363 patients operated for goiter, 60 were retrosternal (16.5%). The female incidence although less than in cervical goiters remains predominant (2.5:1). Most patients are in older age group, 68% being above 40 years. The majority of goiters are multinodular and of long duration. Bilateral (37%) and left sided location (43%) were common. The incidence of cancer was 7%, thyrotoxicosis was noted in 5 patients (8%). Most patients were symptomatic (97%). Diagnosis is easily done by physical examination, chest X-ray and thyroid scintigraphy. Computed tomography might be helpful. Retrosternal goiter is an indication to surgery except in high risk patients. The cervical approach has been used in 98% of cases. There were no post-operative death and no major complications (compressive hematoma, laryngeal nerve paralysis, hypocalcemia).

Adult

[Substernal goiters].

In a series of 363 patients operated for goiter, 60 were retrosternal (16.5%). The female incidence although less than in cervical goiters remains predominant (2,5:1). Most patients are in older age group, 68% being above 40 years. The majority of goiters are multinodular and of long duration. Bilateral (37%) and left sided location (43%) were common. The incidence of cancer was 7%, thyrotoxicosis was noted in 5 patients (8%). Most patients were symptomatic (97%). Diagnosis is easily done by physical examination, chest X-Ray and thyroid scintigraphy. Computed tomography might be helpful. Retrosternal goiter is an indication to surgery except in high risk patients. The cervical approach has been used in 98% of cases. There were no post-operative death and no major complications (compressive hematoma, laryngeal nerve paralysis, hypocalcemia).

Adult