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Cullen Sign: Symptoms, Causes, Diagnosis, and Treatment

Bruising around the navel can point to a serious condition, such as pancreatitis

Cullen sign is the superficial swelling and bruising of tissue around the umbilicus (navel) most often seen in people with acute pancreatitis (inflammation of the pancreas). It tends to develop within 24 to 48 hours and typically occurs when the condition is severe. Metal Switch Button

Cullen Sign: Symptoms, Causes, Diagnosis, and Treatment

Cullen sign can also occur in people with ectopic pregnancy, ruptured aneurysm, esophageal cancer, and internal bleeding.

Cullen sign is relatively rare. It is seen in 1% to 3% of pancreatitis cases and 1% of ectopic pregnancies. When it does appear, it is associated with an increased risk of death.

This article describes the symptoms and causes of Cullen sign. It also explains how diseases associated with Cullen sign are diagnosed and treated.

Cullen sign is characterized by ecchymosis (bruising) and edema (tissue swelling) around the navel (belly button). The bruising can vary based on the severity of the underlying condition, ranging from a pale yellow to a dark purple, Some people also feel belly button pain.

Cullen sign tends to develop suddenly and often precedes all other symptoms of acute pancreatitis, including:

Cullen sign is sometimes accompanied by another symptom called Grey Turner sign in which bruising develops around the flank (the sides of the torso below the ribcage).

The co-occurence of the Cullen sign and Grey Turner sign is common with necrotizing pancreatitis, a complication of pancreatitis in which parts of the pancreas start to die.

Cullen sign develops when blood and/or pancreatic enzymes pool in tissues just beneath the surface of the skin. Several conditions can cause this, including:

The pancreas is an organ that produces digestive enzymes and hormones.

Pancreatitis occurs when digestive enzymes are activated before being released into the small intestine. These enzymes can damage the pancreas, causing pain and the formation of pancreatic cysts. If the enzymes leak into the abdomen, they can damage blood vessels and lead to internal bleeding.

The appearance of Cullen sign is associated with an increased risk of death in people with acute pancreatitis. Studies have found that up to 37% of acute pancreatitis cases that involve the Cullen sign end up being fatal.

Traumatic injury to the abdomen can cause internal bleeding that results in Cullen sign. Many of these are caused by blunt-force impact injuries, such as a car accident, leading to a ruptured spleen, liver, or pancreas.

Others result from less traumatic circumstances, such as violent coughing that can cause spontaneous bleeding of the abdominal wall.

An ectopic pregnancy happens when a fertilized egg implants outside the uterus, most commonly in the fallopian tubes. Ectopic pregnancies are not viable, meaning they can’t come to term. If an ectopic pregnancy is left untreated, the developing fetus can rupture the fallopian tube and cause dangerous internal bleeding.

With an ectopic pregnancy, Cullen sign is a clear indication of a medical emergency. Other symptoms include extreme abdominal or pelvic pain, lightheadedness, and fainting.

An aortic aneurysm is the abnormal bulging of the major artery servicing the heart called the aorta . The bulging causes a weakened area of tissue that can easily rupture (burst).

The abdominal aorta is the part of the aorta that moves blood from the heart to the abdomen. The rupture of this vessel can readily cause Cullen sign. Other symptoms include severe abdominal or back pain, dizziness, clammy skin, nausea, vomiting, rapid heartbeat, and fainting,

Ovarian cysts are fluid-filled sacs in the ovaries (the organs where immature eggs are stored and released). In most cases, ovarian cysts do not cause any symptoms.

However, some ovarian cysts are hemorrhagic, meaning that they are filled with blood. These occur when the release of a mature egg causes excessive bleeding and the pooling of blood in the ovary. If the hemorrhagic ovarian cyst grows large enough, it can burst, causing Cullen sign.

Esophageal cancer affects the tube that connects the throat to the stomach, called the esophagus. Symptoms of esophageal cancer are often absent until the disease is advanced. Symptoms include dysphagia (difficulty swallowing), chest pain, nausea, and hematemesis (bloody vomit).

Cullen sign typically appears when the cancer has metastasized (spread throughout the body). The prognosis is generally poor if Cullen sign develops.  

Non-Hodgkin lymphoma (NHL) is a type of cancer that affects the lymphatic system (the organ system responsible for filtering waste and regulating fluid levels in the body).

NHL can cause a wide range of symptoms, including chest or abdominal pain, persistent fatigue, coughing, difficulty breathing, and the swelling of lymph nodes in the throat, armpits, and groin. NHL can also damage the spleen, one of the organs of the lymphatic system, leading to Cullen sign.

A percutaneous liver biopsy is a diagnostic procedure in which a needle is passed through the abdomen to obtain a small sample of liver tissue. The biopsy is typically performed when liver cancer is suspected.

A percutaneous liver biopsy is a minimally invasive procedure. In rare cases, however, the procedure can cause excessive bleeding that can lead to Cullen sign.

Entamoeba histolytica is a type of amoeba that causes amoebiasis . The single-cell parasite targets the liver, causing the buildup of pus at the site of the infection.

E. histolytica-associated amoebiasis is a rare tropical disease seen in areas with poor sanitation and crowded conditions. It can lead to a range of severe symptoms, including explosive diarrhea, stomach pain, and cramping.

Amoebic dysentery is a severe form of the disease associated with bloody diarrhea, high fever. and intestinal bleeding. Cullen sign is a common symptom.

The umbilical cord connects the mother and fetus during pregnancy. The cord is cut after birth and heals up to form the belly button. In around 20% of babies, it can take longer for the umbilical cord to heal, and, in some cases, it may not heal at all.

This can lead to a gap in the muscles surrounding the belly button. allowing the intestine to protrude and become trapped (strangulation). The strangulation causes swelling and bruising characteristic of Cullen sign.

Cullen sign is not a disease, but a symptom of a disease or medical condition. In and of itself, it is not diagnostic of any medical condition. If you develop Cullen sign, you need to seek immediate care to determine if the cause is serious.

Different tests may be ordered based on your medical history, accompanying symptoms, and risk factors for diseases like pancreatitis or ectopic pregnancy. These may include:

There is no treatment for Cullen sign. Rather, the underlying cause is treated. In some cases, emergency medical care is required.

As Cullen sign generally indicates some form of internal bleeding, emergency interventions like a blood transfusion, surgery, and intravenous (IV) fluids may be needed to stem the bleeding and prevent death.

Other causes may require hospitalization. With acute pancreatitis, for example, you may given IV fluids and pain medications, nutritional support, and oxygen until the inflammation has passed. Surgery may be needed in severe situations.

Ectopic pregnancy may be treated with laparoscopic surgery or drugs like methotrexate that stop cells from growing. Cancers may be treated with surgery, chemotherapy, or radiation. 

Cullen sign is bruising and swelling around the navel that is associated with many serious medical conditions. including pancreatitis, ectopic pregnancy, abdominal trauma, ruptured aortic aneurysms, and certain cancers. Diagnosis and treatment is based on the underlying cause.

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By Mark Gurarie Mark Gurarie is a freelance writer, editor, and adjunct lecturer of writing composition at George Washington University.  

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Cullen Sign: Symptoms, Causes, Diagnosis, and Treatment

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