A 52-year-old male was referred to the endocrine clinic due to fatigue, thyromegaly, and neck discomfort for 2 months. He was seen by his primary doctor for fatigue and neck discomfort and treated with antibiotics without improvement.
- What are the important points in history that you would ask when dealing with a case of Goiter ?
- Onset , duration and rapidity of thyroid enlargement
Rapidly enlarging goiter or thyroid nodule gives a suspicion of malignancy
- Signs of Obstruction
- Hoarseness of voice
- Pain over the swelling
- History of drug intake
- Antithyroid drugs
- Symptoms of hypothyroidism
- Symptoms of hyperthyroidism
- Day time sleepiness or snoring- suggestive Obstructive sleep apnea
- Pendred syndrome
- Severe hypothyroidism
- Family history of thyroid swelling
- Familial thyroid cancer syndrome
- History of neck irradiation
- History of iodised salt intake or intake of Goitrogens
Q Which substances are Goitrogens ?
Q Which is the most common symptom of obstruction due to goiter ?
Q What is the symptom of obstruction due to substernal or restrosternal goiter ?
Cough, stridor and dyspnea on
- Lying down
- Raising hand above the hand
- Bending forward
Q What are complications due to obstructing goiter ?
- Dyspnea – tracheal compression
- Dysphagia- esophageal compression- less common
- Hoarseness of voice- recurrent laryngeal nerve involvement
- Orthopnea and dyspnea- phrenic nerve palsy
- Horner’s syndrome
- Vascular involvement
- Jugular vein compression/ thrombosis
- Cerebral steal syndrome
- Superior vena cava obstruction
He underwent an ear, nose, and throat (ENT) evaluation and was subsequently referred to endocrinology after a diagnosis of primary hypothyroidism was made (thyroid-stimulating hormone [TSH] 65 μIU/mL, free thyroxine [T4] 0.27 ng/dL), and he was started on levothyroxine.
Because of suspicion for upper airway obstruction, a contrast-enhanced computed tomography (CT) scan was obtained and revealed a large goiter with mild tracheal compression without substernal extension (Fig. 1). The thyroid gland extended behind the esophagus and larynx with left lobe measuring 8 × 3 × 5 cm and right lobe 10 × 4 × 6 cm (Fig. 2).
A diagnosis of Hashimoto thyroiditis was eventually made based on thyroid peroxidase antibodies of 498 IU/mL (0–9). He had no history of head or neck radiation. A physical exam was remarkable for thyromegaly and mild plethora of his face and upper neck, which significantly worsened upon raising his arms above his head. Additionally, he developed engorged neck veins and stridor with significant distress. (Click here)
Q What are the different methods of Palpating a Goiter ?
- Pizzilo’s method- examiner stands behind the patient and uses both the hands with thumbs are the occiput
- Lahey’s method- Examiner sits in front and pushes the gland towards the opposite side and palpates with the other fingers
- Crile’s method- examiner uses the thumb to palpate the nodules
Q What is the maneuver described above ?
It is the Pemberton’s Maneuver
Q What is the clinical importance of the maneuver ?
It suggests a substernal goiter or a large goiter obstructing the thoracic inlet
Q How is the Pemberton’s sign carried out ?
Patient is asked to raise the hand above the head and the arms are touching the ears when raised. After about 60 seconds the patient develops facial plethora, cyanosis and discomfort. This is because of venous obstruction.
Q What the principle behind the Pemberton’s ?
Because of the substernal goiter the thorasic inlet is already narrowed. By raising the hands above the head, the inlet is narrowed further leading the patient experience symptoms of inlet obstruction .
Q What is ‘Thyroid cork’ phenomenon ?
On raising the hand and impact the thoracic inlet moves up leading to the goiter obstructing the inlet. Some say it is because of the cervical goiter moving down into the thyroid inlet. This is called ‘Thyroid cork’ phenomenon. Since there is no substernal involvement in this case, the positive Pemberton’s sign may be because of this phenomenon in this case. Flexion of the neck may also produce similar phenomenon.
Q What is the recent theory explaining the Pemberton’s sign ?
Recent theory is proposed by De Fillips et al . They propose that the acromian end of the clavicles move medially and inferiorly on movement of the hand above the head . The veins get trapped between a large relatively fixed thyroid gland and the clavicles- like a nut getting trapped between a ‘nutcracker’ .
Thyroid ultrasound showed a diffusely enlarged heterogeneous thyroid with increased vascularity and without focal lesions; the lower poles of thyroid lobes were completely visualized.
The patient elected initial medical treatment. However, he continued to worsen clinically with progressive neck discomfort, dysphagia, a choking sensation, and engorged neck veins when combing his hair or raising his hands for other activities.
He underwent an uneventful total thyroidectomy. The specimen weighed 240 g.
Q What is the normal weight of the thyroid gland ?
Grossly, the thyroid was composed of tan-red, fleshy, vaguely lobulated cut surfaces with no well-defined nodules. Microscopic evaluation revealed chronic lymphocytic thyroiditis. Size of thyroid gland was 10.5 × 9.5 × 5.0 cm. He no longer complained of neck discomfort, and the Pemberton sign was no longer present. He maintained normal TSH on levothyroxine 275 mcg/day.
Q Is Pemberton sign common in patients with hypothyroidism ?
Strangely no ! Pemberton’s sign tends to be more common in patients with euthyroid or hyperthyroid cases. Hypothyroidism often gets diagnosed early so the that the thyroid gland is never that large or substernal to cause the Pemberton’s sign.
Q Summarize the various theories for Pemberton’s sign ?
- Narrowing of an already narrow inlet due to substernal goiter
- Thyroid cork phenomenon
- Ascent of the thorasic inlet
- Descent of the cervical goiter
- Nutcraker phenomenon – De Fillipis theory
LEARNING POINTS FROM THIS CASE
- Pemberton’s sign is generally rare in Hypothyroidism due to autoimmune thyroiditis.
- Pemberton’s sign can be explained by a new theory –‘Nutcracker theory’ which says that the phenomenon is because of entrapment of the external jugular vein between the large goiter and the medial end of the clavicle on movement of the hand above the head.