The Pemberton Sign

Case Report

A 62-year-old man had been aware of an anterior neck mass for approximately 25 years. He denied having any problems breathing or swallowing. Examination showed mild plethora of the face and upper neck and partial dilatation of several neck veins. The cervical portion of the thyroid was enlarged to approximately 120 g, but the lower poles descended behind the clavicles and were not palpable, even with swallowing and the Valsalva maneuver. When the patient did the Pemberton maneuver, striking facial suffusion developed within seconds; a sharp line of demarcation was seen at the base of the neck (Figure 1). Cervical veins dilated further, and the conjunctivae became markedly injected. As the patient continued to keep his arms elevated, redness of the face changed to cyanosis; however, no dizziness, headache, or respiratory symptoms developed. No stridor was seen, and wheeze was not noted on auscultation. Internal and external rotation of the shoulders did not affect the degree of plethora.

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Figure 1. Head and neck with arms down ( ) and arms elevated ( ). The Pemberton sign.leftright

The patient had a suppressed thyroid-stimulating hormone level (0.08 mU/L; normal range, 0.13 to 6.10 mU/L) and normal thyroxine and triiodothyronine levels. Chest radiography, computed tomography, and technetium scanning showed a large, retrosternal, multinodular goiter. Tracheal compression to a minimum diameter of 7 mm at the thoracic inlet was noted. A computed tomographic scan obtained while the patient's arms were elevated above his head showed contrast enhancement of veins in the cervical paraspinal muscles, indicating obstructed venous outflow. When the patient's arms were not elevated, the pulmonary flow-volume loop was normal; when the arms were raised, peak expiratory flow decreased to 73% of the baseline value.

Discussion

A substernal goiter may have few physical signs. Inability to palpate the lower pole of the thyroid can be a substantial clue; however, in some patients with substernal goiter, the thyroid is not present in the neck [3]. In such persons, the gland may become visible or palpable with swallowing, extension of the neck, or actions that increase intrathoracic pressure, such as coughing or the Valsalva maneuver. Evidence of venous obstruction is not uncommon in substernal goiters; distended veins over the neck and thorax have been reported in 8% to 18% of patients [2, 4, 5]. Although rare, full-blown superior vena caval syndrome has been described in patients with benign goiter [6].

The Pemberton sign is a potentially useful addition to physical examination of substernal goiter. H.S. Pemberton described the sign that bears his name in a brief letter to the Lancet in 1946, and the value of his technique was recognized soon after [2]. Pemberton was first quoted by Hamilton Bailey in the 1949 edition of Demonstration of Physical Signs in Clinical Surgery, and the maneuver is now included in many textbooks of clinical examination skills [7]. The presence of the Pemberton sign appears to indicate more serious manifestations of increased pressure in the thoracic inlet. In several reported cases [8], the sign has been associated with thrombosis of the right subclavian and axillary veins. It has also been correlated with tracheal compromise; this was seen in our patient, who had reduced peak expiratory flow when his arms were elevated [9].

The Pemberton sign is an important indicator of increased pressure in the thoracic inlet. It denotes impaired venous outflow from the head and neck and may be associated with vascular or airway compromise. We propose that the Pemberton maneuver be done in patients with a goiter and positional symptoms of the head and neck, a large cervical goiter, or evidence of substernal extension of the thyroid gland.