Pemberton's sign explained

Differential:	superior vena cava syndrome

The Pemberton's sign is a physical examination tool used to demonstrate the presence of latent pressure in the thoracic inlet.^[1] The sign is named after Hugh Pemberton, who characterized it in 1946.^[2]

The Pemberton maneuver is achieved by having the patient elevate both arms (usually 180 degrees anterior flexion at the shoulder) until the forearms touch the sides of the face. A positive Pemberton's sign is marked by the presence of facial congestion and cyanosis, as well as respiratory distress after approximately one minute.^[3]

Causes

A positive Pemberton's sign is indicative of superior vena cava syndrome (SVC), commonly the result of a mass in the mediastinum. Although the sign is most commonly described in patients with substernal goiters where the goiter "corks off" the thoracic inlet,^[4] the maneuver is potentially useful in any patient with adenopathy, tumor, or fibrosis involving the mediastinum. SVC syndrome has been observed as a result of diffuse mediastinal lymphadenopathy of various pathologies such as cystic fibrosis^[5] and Castleman’s disease.^[6] Park et al. reported enlarged cervical lymph nodes associated with hemophagocytic lymphohistiocytosis as the cause of internal jugular vein compression, which presented clinically similar to SVC syndrome.^[7]

Further reading

• Abu-Shama Y and Cuny T. Pemberton’s Sign in a Patient with a Goiter. N Engl J Med. 2018;378:e31. . A short case illustrating Pemberton's sign, with images and a video.

Notes and References

1. Wallace . C . Siminoski K . 1996 . The Pemberton sign . Ann Intern Med . 125 . 7 . 568–569 . 10.7326/0003-4819-125-7-199610010-00006 . 8815756 . 32532890.
2. Pemberton. HS. Sign of submerged goitre. Lancet. 1946. 248. 6423. 509. 10.1016/s0140-6736(46)91790-4 .
3. Pemberton. HS. Sign of submerged goitre. Lancet. 1946. 248. 6423. 509. 10.1016/s0140-6736(46)91790-4 .
4. Basaria. S. Salvatori R. Pemberton's sign. New England Journal of Medicine. 2004. 350. 13. 1338. 10.1056/nejmicm990287. 15044645.
5. Chow. J . McKim DA . Shennib, H. Superior vena cava obstruction secondary to mediastinal lymphadenopathy in a patient with cystic fibrosis. Chest. 1997. 112. 5 . 1438–1441. 10.1378/chest.112.5.1438. 9367491 . etal.
6. Tekinbas. C. Erol MM . Ozsu S. Giant mass due to Castleman's disease causing superior vena cava syndrome.. Thorac Cardiovasc Surg. 2008. 56. 5. 303–305. 10.1055/s-2008-1038408. 18615381. 21183690. etal.
7. Park. M. Choi JW . Park HJ. Hemophagocytic lymphohistiocytosis can mimic the superior vena cava syndrome. J Pediatr Hematol Oncol. 2012. 34. 4. 152–154. 10.1097/MPH.0b013e3182422a20. 22395213. 22479614. etal.