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Endgames Picture Quiz

A man with dilated veins on his upper chest

BMJ 2011; 343 doi: https://doi.org/10.1136/bmj.d4674 (Published 05 August 2011) Cite this as: BMJ 2011;343:d4674
  1. Sebastian P Wallis, core trainee 21,
  2. Dipan Mistry, specialist registrar1,
  3. Andrew P Coatesworth, ear, nose, and throat consultant2,
  4. Michael Porte, consultant radiologist2
  1. 1Ear, Nose, and Throat Department, York Hospital, York YO31 8HE, UK
  2. 2Radiology Department, York Hospital
  1. Correspondence to: S P Wallis sebwallis{at}hotmail.com

A 77 year old man was referred by his general practitioner because of skin changes over his anterior chest wall (fig 1). These skin changes had been present for several years and were not worsening. He had no history of dysphagia, voice change, shortness of breath, neck pain, neck swelling, or weight loss. His medical history included a goitre treated by radioiodine eight years earlier. He had no other medical history of note, he was not taking any drugs, and he did not smoke.

On examination he appeared well with no stridor. He was haemodynamically normal. Fullness was seen in the lower neck but no discrete lump was palpated. Numerous dilated veins were seen over the upper anterior chest wall on both sides, although they were more pronounced on the right side. He had no cervical lymphadenopathy or cachexia.

Blood tests showed normal thyroid function and thyroid antibodies were not raised. Computed tomography was performed (fig 2). Fine needle aspiration was not performed.

Questions

  • 1 What changes are seen in the photograph?

  • 2 What does the computed tomogram show?

  • 3 What else should you evaluate on the computed tomogram?

  • 4 What are the other potential causes of this condition?

  • 5 What are the management options for this man?

Answers

1 What changes are seen in the photograph?

Short answer

Chronic venous obstruction by a large goitre has led to numerous chest wall collaterals. The right external jugular vein is also dilated.

Long answer

A large goitre is compressing both internal jugular veins at the thoracic inlet. Collateral circulation pathways have formed over time. Blood is diverted via dilated cutaneous vessels to the intercostals veins and then via the internal thoracic vein or azygous vein back to the superior vena cava. The right external jugular may also be acting as a collateral by diverting blood back via the subclavian vein.

2 What does the computed tomogram show?

Short answer

The scan shows a large goitre, which is guided inferiorly by a covering of pretracheal fascia (fig 3).

Figure3

Fig 3 Computed tomogram showing a goitre (red arrow), which is compressing the right and left internal jugular veins (white arrows); the star indicates the trachea

Long answer

The computed tomography scan shows a large goitre extending inferiorly through the thoracic inlet. The goitre is compressing the internal jugular veins, which are dilated as a result. The thyroid gland is covered anteriorly by pretracheal fascia which binds it to the trachea. As the goitre enlarges, it is therefore directed inferiorly into a space between the brachiocephalic veins, the brachiocephalic trunk, and the trachea.

3 What else should you evaluate on the computed tomogram?

Short answer

Tracheal compression, the inferior extent of the goitre, and any features of malignancy.

Long answer

It is important to look for airway compromise in patients with a large goitre because some degree of tracheal compression may be present even in asymptomatic patients. Exertional dyspnoea may be seen when the diameter of the trachea narrows to less than 8 mm, and stridor or wheezing may occur at a diameter of less than 5 mm,1 although respiratory symptoms may develop before these measurements are reached. In our case, the trachea narrowed to 9 mm at the level of T3.

The inferior extent of the goitre should be delineated because this may influence the surgical approach to thyroidectomy. Patients with goitres that extend deep into the mediastinum are more likely to need a sternotomy for safe removal of the goitre. Of note, the neck position at the time of scanning may raise or even push the thyroid gland into the thorax by up to 1.5 cm. In our case the goitre extended down to the level of the brachiocephalic veins. It is important to look for evidence of malignancy such as a large dominant nodule, calcification, or necrosis. This is hard to do with standard ultrasound techniques because of the overlying sternum. The computed tomogram showed no features of malignancy and was consistent with a benign multinodular goitre. The disadvantage of computed tomography is that radiocontrast agents may induce iodine organification in subclinical hyperthyroid multinodular goitre.2

Flow volume loop curves are another simple non-invasive way of looking for upper airway obstruction. These assess airflow dynamics and may pick up subtle changes in asymptomatic patients.3 Our patient did not undergo flow volume loop assessment.

4 What are the other potential causes of this condition?

Short answer

Malignant causes include bronchogenic carcinoma, lymphoma, and metastases; benign causes include mediastinitis, mediastinal tumours, vascular and cardiac diseases, and trauma.

Long answer

The signs of dilated superficial veins over the chest and neck are compatible with superior vena cava syndrome. Patients with this syndrome may also have swelling and cyanosis of the face and upper extremities, cough, headache, dysphagia, and somnolence.4 Obstruction of venous return in the mediastinum and neck is caused by cancer in 80-90% of cases.5 6 7 Malignant causes of superior vena cava syndrome include bronchogenic carcinoma (75-80%), lymphoma (12-15%), and metastatic cancer (6-7%).6 Benign causes are less common but have a more favourable prognosis. Reported causes include mediastinitis, mediastinal tumours such as retrosternal goitre, vascular causes such as aortic aneurysm and superior vena cava thrombosis, cardiac causes such as atrial myxoma, and traumatic mediastinal haematoma.4 7

Retrosternal goitre has been reported in 3-20% of patients undergoing thyroidectomy.8 In two separate series superior vena cava obstruction was a feature in three of 35 and one of 16 patients with retrosternal goitre undergoing thyroidectomy.8 9

5 What are the management options for this man?

Short answer

A conservative (watch and wait) approach, radioiodine, or surgical excision.

Long answer

A conservative approach to patients with asymptomatic goitre with no suspicion of malignancy is considered standard care by some.10 In patients who are not fit for surgery, treatment with radioiodine is an option, with a reported volume reduction of 30-60% if the thyroid tissue is functioning on radionuclide imaging.11 Recombinant human thyroid stimulating hormone has been shown to increase the efficacy of radioiodine treatment for multinodular goitre.12 However, radioiodine is thought to be less effective in reducing the size of large goitres; it may also induce acute swelling, thereby causing potential airway obstruction.13 Although the risk of malignant transformation in retrosternal goitre is small,14 thyroidectomy has been recommended because of the risk of developing other obstructive symptoms.15 Some authors recommend thyroidectomy for asymptomatic goitres that extend below the level of the brachiocephalic veins in patients who are fit for surgery.16 Sternotomy is needed in 2-29% of thyroidectomies for retrosternal goitre.14 17 The risk of complications—including haemorrhage, recurrent laryngeal nerve injury, postoperative hypoparathyroidism, and death—is higher for retrosternal goitre surgery than for cervical thyroidectomy.18 19 Postoperative tracheaomalacia can also occur.16

Patient outcome

Our patient was referred to a specialist thyroid surgeon. He was offered surgery but declined.

Notes

Cite this as: BMJ 2011;343:d4674

Footnotes

  • Competing interests: All authors have completed the ICMJE uniform disclosure form at www.icmje.org/coi_disclosure.pdf (available on request from the corresponding author) and declare: no support from any organisation for the submitted work; no financial relationships with any organisations that might have an interest in the submitted work in the previous three years; no other relationships or activities that could appear to have influenced the submitted work.

  • Provenance and peer review: Not commissioned; externally peer reviewed.

  • Patient consent obtained.

References

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