April - June 2016, Volume 12, Issue 2

Giulea C, Enciu O, Nadragea M, Badiu C, Miron A

Pemberton’s Sign and Intense Facial Edema in Superior Vena Cava Syndrome due to Retrosternal Goiter

Acta Endo (Buc) 2016, 12 (2): 227-229
doi: 10.4183/aeb.2016.227

Introduction. Retrosternal goitre enlargement can cause compression of several mediastinal structures, especially the trachea and the superior vena cava. Retrosternal goitre as a cause of superior vena cava syndrome is a rare occurrence. We report the case of a middle aged man that underwent surgery for retrosternal goitre with compression of both innominate veins presenting as superior vena cava syndrome. Case Presentation. A 50 year old man presented with a 2 year history of cyanosis of the upper limbs, head and neck, marked facial edema, plethora, dyspnea on exertion and choking sensation. Pemberton’s sign was present. Computer tomography diagnosed retrosternal goitre at the level of the aortic arch, tracheal compression and important collateral circulation. Endocrine evaluation showed normal thyroid function (fT4 15.8 pmol/L) with low-normal TSH (0.5mU/L), normal calcitonin (<2 pg/mL). The patient underwent successful total thyroidectomy with cervical approach and his symptoms dramatically improved. The facial oedema persisted for the next 3 weeks. Discussion. Less than 3% of superior vena cava syndromes are secondary to a variety of benign causes. Superior vena cava syndrome caused by slow growing retrosternal goitres is very rare and can be asymptomatic for a long period due to venous collateral development. Conclusion. Superior vena cava syndrome secondary to retrosternal goitres, a very rare occurrence, is an indication for total thyroidectomy, with low postoperative morbidity and dramatic resolution of symptoms.

Keywords: retrosternal goiter, superior vena cava syndrome, Pemberton’s sign.

Correspondence: Cosmin Giulea, MD, Elias University Emergency Hospital, Dept. of Surgery, 17 Marasti, Bucharest, 011461, Romania, E-mail: gcosmin@yahoo.com