Pemberton's sign

Pemberton's sign was named after Dr. Hugh Pemberton, who characterized it in 1946.^[1]

The Pemberton maneuver is a physical examination tool used to demonstrate the presence of latent pressure in the thoracic inlet.^[2] The maneuver is achieved by having the patient elevate both arms until they 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 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] More recently, Tipton et al. described a patient with diffuse mediastinal lymphadenopathy due to amyloidosis. The unique configuration of enlarged lymph nodes resulted in a positive Pemberton's Sign.^[8] Not surprisingly, apical lung cancers may cause a positive Pemberton's sign and a high index of suspicion should be maintained in patients with symptoms of dyspnea and facial plethora with an extensive smoking history.

References

1. Pemberton, HS (1946). "Sign of submerged goitre". Lancet 248: 509. doi:10.1016/s0140-6736(46)91790-4.
2. Wallace, C; Siminoski K (1996). "The Pemberton sign". Ann Intern Med 125: 568–569. doi:10.7326/0003-4819-125-7-199610010-00006.
3. Pemberton, HS (1946). "Sign of submerged goitre". Lancet 248: 509. doi:10.1016/s0140-6736(46)91790-4.
4. Basaria, S; Salvatori R (2004). "Pemberton's sign". New England Journal of Medicine 350: 1338. doi:10.1056/nejmicm990287.
5. Chow, J; McKim DA; Shennib, H; et al. (1997). "Superior vena cava obstruction secondary to mediastinal lymphadenopathy in a patient with cystic fibrosis". Chest 112: 1438–1441. doi:10.1378/chest.112.5.1438.
6. Tekinbas, C; Erol MM; Ozsu S; et al. (2008). "Giant mass due to Castleman's disease causing superior vena cava syndrome.". Thorac Cardiovasc Surg 56: 303–305. doi:10.1055/s-2008-1038408.
7. Park, M; Choi JW; Park HJ; et al. (2012). "Hemophagocytic lymphohistiocytosis can mimic the superior vena cava syndrome". J Pediatr Hematol Oncol 34: 152–154.
8. Tipton, Philip; Blanchard JJ; Guider WP; et al. (April 30, 2014). "Revising the exclusivity of Pemberton's sign: A case report". American Journal of Internal Medicine 2 (3): 41–43. doi:10.11648/j.ajim.20140203.11.