An Overview of Cullen Sign

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Characterized by edema (swelling), bruising, and discoloration of the fatty tissue surrounding the umbilicus (navel), Cullen sign can arise due to pancreatitis (inflammation of the pancreas), cancer of the pancreas, ectopic pregnancy, liver problems, thyroid cancer, or other sources of internal bleeding in the abdomen. First identified by Dr. Thomas Stephen Cullen (1868—1953) in 1918, this condition often coexists with Grey Turner’s sign, a bruising of the abdominal walls or flanks.

While occurrence is relatively rare—it’s seen in less than 1% of ectopic pregnancies, and in 1-3% of pancreatitis patients —Cullen sign is clinically viewed as a marker of serious health problems.

Symptoms

Primarily, Cullen sign presents as severe discoloration and bruising around the navel, accompanied by swelling in the region. The color of the bruise varies based on the severity of the condition, ranging from yellow/green in more moderate cases, to purple in severe ones.

In addition, some but not all patients feel pain and discomfort in the area. Notably, in cases of acute pancreatitis, symptoms tend to emerge 24 to 72 hours after onset of the condition.

Causes

Regardless of the specific disease that’s brought on Cullen sign, the underlying cause is the pooling of blood and pancreatic enzymes in tissues just beneath the skin around the navel. This can occur due to a range of cases, including:

Acute Pancreatitis

This is an inflammation of the pancreas due to its digestive enzymes becoming active before being released into the small intestine. These enzymes start to damage the pancreas, itself, leading to pain, internal bleeding, and the formation of cysts. Acute cases can form quickly and even become fatal if untreated.

Ectopic Pregnancy

This is when a fertilized egg attaches itself to a location outside the cavity of the uterus, usually in the fallopian tubes. In these cases, the pregnancy isn’t viable, and, if untreated, the internal bleeding that results can become very dangerous.

Ruptured Abdominal Aorta

The abdominal aorta is a major pathway of blood from the heart to the abdominal region. If this vessel ruptures, the resultant hemorrhage leads to severe pain in the area as well as the low back and can lead to Cullen sign.

Thyroid Cancer

Among the many symptoms of cancer of the thyroid—a butterfly shaped gland in the throat—is Cullen sign. In many cases, however, this cancer can be asymptomatic, and it’s known to be highly curable if caught in a timely fashion.

Ruptured Spleen

The rupture of the spleen—an organ located under the left rib cage—is considered a medical emergency as this leads to blood pooling throughout the abdomen. 

Amoebic Liver Abscess

When a certain type of amoeba, Entamoeba histolytica, infects the tissues of the liver, there’s a build-up of pus. This issue, more common in areas where there is poor sanitation and crowded conditions, leads to a range of health problems and can be expressed as Cullen sign.

Esophageal Cancer

In cases where the esophagus in the throat develops cancer, Cullen sign is known to be a marker of a particularly advanced case. Because it’s a sign that cancer has started to spread throughout the body (metastasize), prognosis is very poor in these cases. 

Non-Hodgkins Lymphoma

Non-Hodgkins lymphoma is a dangerous condition that occurs when cancer starts to develop in white blood cells, causing, among other symptoms, swelling of the lymph nodes in the throat. Among the cascade of effects of this disease is damage to the spleen, which can lead to Cullen sign.

Percutaneous Liver Biopsy

When cancer is suspected, doctors rely on testing of tissue samples to find growth of cancerous cells. One such approach is percutaneous liver biopsy, which involves using a specialized needle to extract a sample from the organ. In rare cases, bleeding can become excessive due to the test, leading to Cullen sign.  

Ovarian Cyst

In women, ovarian cysts—fluid filled sacs in the ovaries—can develop. In most cases this very common condition is asymptomatic, though occasionally these grow so large that they need to be removed. Cullen sign is clinically associated with this development.

Strangulated Umbilical Hernia

After birth, the site of connection between mother and fetus, the umbilical cord, usually heals up to form the belly button of the infant. In 20% of cases, however, this healing takes a while to occur or doesn’t happen at all, requiring treatment. The associated bleeding in the abdominal area can lead to Cullen sign.

Pancreatic/Abdominal Trauma

Trauma or injury to the pancreas or abdominal region due to accident or mishap can also cause internal bleeding. This, in turn, can arise as Cullen sign.

Diagnosis

When it comes to diagnosis of this condition, doctors tend to focus on what the underlying causes are, rather than the issue itself. In this sense, when Cullen sign is seen, the concern is that a more serious condition, such as acute pancreatitis, ectopic pregnancy, or other issue needs to be investigated. Notably, studies have found that up to 37% of pancreatitis cases that display this marker end up being fatal.

Treatment

As with diagnosis, treatment of this condition ultimately means taking on the disease or disorder that is causing it. Pancreatitis, for instance, can be taken on by directed fasting, fluid replacement via IV, medication for pain, or surgery of the pancreas or gallbladder.

Ectopic pregnancy is usually taken on by either chemotherapy, laparoscopic surgery, or other medical procedures.

Cancers of the pancreas, thyroid, esophagus, or as in Hodgkin’s lymphoma require surgery, chemotherapy, or other types of radiation treatment. 

A Word From Verywell

What’s alarming about Cullen sign is the severity of the case it indicates. Many of the underlying issues are severe and require serious medical attention. Certainly, if you’re seeing signs of this condition, talk to your doctor as soon as possible. As with many aspects of human health, the sooner you’re able to isolate a problem and take it on, the better off you’ll be. Most important is that you always feel free to ask for help; getting it in time can be the difference between life and death.

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