Pemberton's sign

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

Pemberton's sign
Differential diagnosissuperior vena cava syndrome

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 (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]

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References
  1. Pemberton, HS (1946). "Sign of submerged goitre". Lancet. 248 (6423): 509. doi:10.1016/s0140-6736(46)91790-4.
  2. Wallace, C; Siminoski K (1996). "The Pemberton sign". Ann Intern Med. 125 (7): 568–569. doi:10.7326/0003-4819-125-7-199610010-00006. PMID 8815756.
  3. Pemberton, HS (1946). "Sign of submerged goitre". Lancet. 248 (6423): 509. doi:10.1016/s0140-6736(46)91790-4.
  4. Basaria, S; Salvatori R (2004). "Pemberton's sign". New England Journal of Medicine. 350 (13): 1338. doi:10.1056/nejmicm990287. PMID 15044645.
  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 (5): 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 (5): 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.

Further reading
  • Abu-Shama Y and Cuny T. Pemberton’s Sign in a Patient with a Goiter. N Engl J Med. 2018;378:e31. DOI: 10.1056/NEJMicm1712263. A short case illustrating Pemberton's sign, with images and a video.
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