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The Pemberton's sign is a physical examination tool used to demonstrate the presence of latent pressure in the thoracic inlet. The sign is named after Hugh Pemberton, who characterized it in 1946. 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 Cyanosis is the change of Tissue (biology), tissue color to a bluish-purple hue, as a result of decrease in the amount of oxygen bound to the hemoglobin in the red blood cells of the capillary bed. Cyanosis is apparent usually in the Tissue (bi ...
, as well as respiratory distress after approximately one minute.


Causes

A positive Pemberton's sign is indicative of
superior vena cava syndrome Superior vena cava syndrome (SVCS), is a group of symptoms caused by obstruction of the superior vena cava ("SVC"), a short, wide vessel carrying circulating blood into the heart. The majority of cases are caused by malignant tumors within the me ...
(SVC), commonly the result of a mass in the
mediastinum The mediastinum (from ;: mediastina) is the central compartment of the thoracic cavity. Surrounded by loose connective tissue, it is a region that contains vital organs and structures within the thorax, mainly the heart and its vessels, the eso ...
. Although the sign is most commonly described in patients with substernal goiters where the goiter "corks off" the thoracic inlet, the maneuver is potentially useful in any patient with
adenopathy Lymphadenopathy or adenopathy is a disease of the lymph nodes, in which they are abnormal in size or consistency. Lymphadenopathy of an inflammation, inflammatory type (the most common type) is lymphadenitis, producing swelling (medical), swolle ...
, tumor, or
fibrosis Fibrosis, also known as fibrotic scarring, is the development of fibrous connective tissue in response to an injury. Fibrosis can be a normal connective tissue deposition or excessive tissue deposition caused by a disease. Repeated injuries, ch ...
involving the mediastinum. SVC syndrome has been observed as a result of diffuse mediastinal lymphadenopathy of various pathologies such as
cystic fibrosis Cystic fibrosis (CF) is a genetic disorder inherited in an autosomal recessive manner that impairs the normal clearance of Sputum, mucus from the lungs, which facilitates the colonization and infection of the lungs by bacteria, notably ''Staphy ...
and Castleman’s disease. Park ''et al.'' reported enlarged
cervical lymph nodes Cervical lymph nodes are lymph nodes found in the neck. Of the 800 lymph nodes in the human body, 300 are in the neck. Cervical lymph nodes are subject to a number of different pathological conditions including tumours, infection and inflamma ...
associated with
hemophagocytic lymphohistiocytosis In hematology, hemophagocytic lymphohistiocytosis (HLH), also known as haemophagocytic lymphohistiocytosis ( British spelling), and hemophagocytic or haemophagocytic syndrome, is an uncommon hematologic disorder seen more often in children than ...
as the cause of
internal jugular vein The internal jugular vein is a paired jugular vein that collects blood from the brain and the superficial parts of the face and neck. This vein runs in the carotid sheath with the common carotid artery and vagus nerve. It begins in the posteri ...
compression, which presented clinically similar to SVC syndrome.


References


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. {{Cardiovascular system symptoms and signs Medical signs