Vanishing bile duct syndrome is a rare liver condition that affects your bile ducts.
Bile ducts are tubes that connect your liver and small intestine. They’re responsible for carrying bile, the fluid secreted by the liver to digest fats.
Vanishing bile duct syndrome (VBDS) happens when the bile ducts are injured and eventually die off, or “vanish,” one by one.
VBDS can lead to serious, often life threatening complications.
VBDS is very rare. It doesn’t usually occur by itself. Rather, it can be a complication of conditions that cause liver injury, such as hepatitis.
Keep reading to learn more about VBDS, including its symptoms, causes, diagnosis, treatment, and prevention.
Symptoms of VBDS depend on the underlying condition. They may include:
Conditions that can cause VBDS include:
- hepatitis, both viral and non-viral types
- other infections caused by bacteria, viruses, and parasites
- ischemic conditions affecting the biliary tree
- autoimmune conditions
In addition, substances that can injure your liver may cause VBDS, such as:
- certain medications
- alcohol (long-term, heavy use)
In rare cases, VBDS can be congenital, which means it’s present at birth.
Antibiotics can cause VBDS, such as:
- antibiotics of the penicillin family, like Augmentin
- macrolides, like clarithromycin
- fluoroquinolones, like Cipro
- sulfonamide antibiotics, like trimethoprim/sulfamethoxazole
Other drugs that can cause VBDS include:
- antifungal medications
- nonsteroidal anti-inflammatory drugs (NSAIDs)
- tricyclic antidepressants
- certain seizure medications
Read more about the medications that can damage your liver.
The main risk factors for VBDS are long-term heavy alcohol use and exposure to viruses that cause hepatitis.
In addition, people with organ transplants can be at risk of graft-versus-host disease, which can cause VBDS.
VBDS can cause serious complications, including ductopenia and cholestasis.
Ductopenia happens when the number of ducts decreases. Cholestasis is decreased or blocked bile flow.
Ultimately, VBDS complications can lead to liver failure, which can be life threatening.
Once a bile duct has died, your body cannot grow it back. Therefore, treatment of VBDS mainly focuses on preventing further liver injury and managing its effects on your body.
The most effective way to prevent further liver injury is by treating the underlying condition. For example, if cancer is causing your VBDS, doctors will likely use therapies that shrink the tumor.
However, treating the underlying condition isn’t always possible or may not be enough. In these cases, doctors will use medications to alleviate liver injury, such as corticosteroids and ursodiol.
Doctors will also manage the effects that VBDS has on your body, such as itching and hyperlipidemia (high levels of fats in your blood).
Get immediate medical help if you experience any symptoms of liver injury, such as:
- loss of appetite
- weight loss
- bruising or bleeding easily
- edema, or fluid buildup in the legs
- ascites, or fluid buildup in the abdomen
If your doctor diagnoses VBDS, you will likely be hospitalized.
Get immediate medical help if your symptoms return after you’ve been discharged from the hospital.
A doctor called a hepatologist will perform a physical exam to look for signs of liver injury. They will also ask questions to determine whether the injury could be caused by exposure to medications, toxins, or alcohol.
To diagnose VBDS, a hepatologist will order the following lab tests:
- blood tests that check the health of your liver, called liver function tests
- imaging tests that look at your abdomen, such as MRIs, CT scans, or ultrasounds
- liver biopsy
Liver biopsy is the only way to confirm a VBDS diagnosis.
You can’t prevent all the health conditions that cause VBDS. But you can decrease your risk by doing the following:
- getting vaccinated against hepatitis A and hepatitis B (there are no vaccines yet for other viral forms of hepatitis)
- limiting or avoiding alcohol
- avoiding exposure to toxic chemicals
VBDS can be mild if it affects a small number of your bile ducts. It can be severe if the number of injured ducts is large.
People with mild VBDS may recover gradually over a few months to possibly years. People with severe VBDS may have lasting effects on their health.
Even with appropriate treatment,
VBDS is a rare but serious medical condition that affects the bile ducts in your liver. Certain medical conditions, medications, and heavy alcohol use typically cause it. VBDS causes the typical symptoms of liver failure, including jaundice and itching.
The definitive diagnosis of VBDS requires a biopsy. The most effective treatment for VBDS targets the underlying condition. However, even with treatment, some people with severe forms of VBDS may need a liver transplant to survive.