Pylephlebitis

What is Pylephlebitis?

Pylephlebitis is a rare, severe condition where the portal veins (veins that carry blood from the digestive organs to the liver) become infected and form blood clots. This can happen due to any kind of infection within the abdomen or pelvic area. Before antibiotics were commonly used, this condition used to be deadly almost all the time. However, nowadays, medical imaging technology has made it possible to spot and diagnose Pylephlebitis earlier.

When a patient comes in with an abdominal infection that has multiple types of bacteria, it’s essential to consider Pylephlebitis as a potential cause. This is crucial to do quickly because, if not treated, the infection could lead to serious complications. For example, it could block blood flow to the small intestines, form pus-filled pockets in the liver, or even cause infected blood clots to end up in the lungs.

What Causes Pylephlebitis?

Pylephlebitis, a complicated health condition, can be caused by nearly any type of infection in the belly or pelvis that involves the organs and drains through a system of veins that carry blood to the liver. In the early 1900s, appendicitis was a common trigger for these problems, but with the use of antibiotics, this is not common these days.

Nowadays, a condition called diverticulitis – where small pouches in the digestive system get infected or inflamed – is one of the usual culprits. But, there are also many other inflammatory and infectious diseases that can lead to this ailment. These include conditions like inflammatory bowel disease, pancreatitis (a condition that leads to inflammation of the pancreas), gastroenteritis (commonly known as stomach flu), cholangitis (an inflammation of the bile ducts), peptic ulcer disease, liver abscesses (pockets of pus in the liver), amoebiasis (a type of stomach infection), and even some rare cases associated with umbilical vein catheters (a plastic tube placed in the vein of a newborn’s umbilical cord) and migration of an adjustable gastric band (a weight loss device that is placed around the stomach).

Risk Factors and Frequency for Pylephlebitis

It’s hard to find exact numbers for this health condition because it’s quite rare. From the information available, which includes a review of 100 cases between 1971 and 2009, those affected ranged in age from just 20 days to 77 years old. The average age among these people was 42.3 years. Whether one gender is more likely to be affected than the other isn’t known.

Signs and Symptoms of Pylephlebitis

Pylephlebitis is a medical condition that often presents with several symptoms. However, these symptoms tend to be fairly general and not specific to the condition. Common complaints include fatigue, fever, abdominal pain, nausea, vomiting, diarrhea, and weight loss. During a physical examination, doctors might also notice certain signs such as tenderness in the abdomen, enlargement of the spleen (splenomegaly) or liver (hepatomegaly), fluid accumulation in the abdominal cavity (ascites), and jaundice or yellowing of the skin. The last symptom is usually indicative of a more widespread infection affecting the liver.

• Fatigue
• Fever
• Abdominal pain
• Nausea
• Vomiting
• Diarrhea
• Weight loss
• Abdominal tenderness
• Splenomegaly (enlarged spleen)
• Hepatomegaly (enlarged liver)
• Ascites (accumulation of fluid in the abdomen)
• Jaundice (yellowing of the skin)

Testing for Pylephlebitis

In cases of early leukocytosis, which is an increased amount of white blood cells in your body, laboratory tests may show significant results. However, there have been instances where a patient can show a lower than normal or a standard white blood cell count, as found in multiple studies. Abnormal liver function tests, which gauges how well your liver is working, might display increased levels of alkaline phosphatase, AST, ALT, and gamma-glutamyl transferase. These are enzymes that indicate liver disease. However, the level of bilirubin, a waste product in your blood, usually does not increase unless the liver disease has spread widely.

Currently, there are no specific criteria to diagnose pylephlebitis, which is a serious infectious disease that can block blood flow in the liver. Thus, the diagnosis is often made after an image reveals a blood clot in a patient’s portal vein, a large vein that carries blood from the intestines to the liver. This is commonly seen in a patient with sepsis, a serious infection that affects the whole body, and other symptoms consistent with pylephlebitis. However, it’s important to remember that just having a blood clot does not necessarily mean you have pylephlebitis.

Both computed tomography (CT) scans and color flow Doppler ultrasonography (an imaging method that uses sound waves to show blood flowing in vessels) can show a blood clot in the portal vein. However, CT scans may be more useful because they can also detect complications like liver abscesses (pockets of pus in the liver) or intestinal ischemia (reduced blood flow to the intestines).

In one study, CT scans and ultrasonography were the most common ways to take images of patients, but occasionally other techniques like Magnetic Resonance Imaging (MRI) and Positron Emission Tomography/Computed Tomography (PET/CT) were used. Thanks to improved imaging techniques, more patients have been diagnosed earlier for pylephlebitis, compared to the past when many patients were only diagnosed after death during an autopsy.

Treatment Options for Pylephlebitis

The main treatment for an infection that involves a mixture of different types of bacteria (polymicrobial infection), that often includes organisms like gram-negative aerobes, anaerobes, and streptococcus species, is a suitable antibiotic routine. It’s important to remember there isn’t a single “one-size-fits-all” treatment, and the effectiveness of various antibiotic plans hasn’t yet been proven through “gold standard” scientific studies, like randomized controlled trials. Consequently, many research studies suggest that a broad-spectrum antibiotic treatment, which works against a wide range of bacteria, should be used even if the bacteria are not in the bloodstream.

Typically, the treatment starts with a customized selection of antibiotics based on the type and susceptibility of the bacteria causing the infection, determined from the laboratory culture results. Initially, these antibiotics are administered through an injection method, but they can be switched to an oral form based on how sensitive the bacteria are to the antibiotics and the patient’s condition. Ordinarily, the antibiotic treatment lasts for around four to six weeks, but it’s advisable to consult with an infectious disease specialist for personalized guidance.

Currently, there’s no definite consensus on the role of anticoagulation (a treatment that prevents blood clots) in pylephlebitis (a rare condition involving a vein clot in the liver), mainly due to a scarcity of quality research data and randomized control trials. A systematic examination of several cases found a lower death rate in patients who received anticoagulation in addition to antibiotics, compared to those who received antibiotics alone, but this isn’t definitive evidence.

In the absence of conclusive information, anticoagulation may be considered in patients with an underlying cancer, a genetic predisposition to blood clots, or expansion of the clot into veins supplying the intestines. Anticoagulation may also be considered if the bloodstream infection is due to Bacteroides species since these bacteria have surface components that increase clotting and can produce enzymes that break down a specific anticoagulant called heparin. If the clot burden increases, a therapy to dissolve the clot can be considered, but its effectiveness isn’t fully established.

What else can Pylephlebitis be?

When doctors are trying to diagnose pylephlebitis, a condition where a vein in the liver becomes infected, they also consider other conditions that can cause similar symptoms. These include:

• Cancer that may affect the liver or surrounding area
• Decompensated cirrhosis, a very advanced stage of liver disease
• Inherited thrombophilia, a genetic disorder that makes clots more likely
• Connective tissue diseases, a group of disorders that affect the tissues that support, bind, or separate other tissues and organs

What to expect with Pylephlebitis

According to a review of past cases, before 1990, the death rate was 75%. However, this dropped to 25% after 1990. Even though there have been improvements in detecting pylephlebitis (a serious infection in the liver) through advanced imaging technology and newer antibiotics, the diagnosis still has a significant death rate. Various studies report the death rate ranging from 11% to 32%.

Possible Complications When Diagnosed with Pylephlebitis

Pylephlebitis is viewed as a complication resulting from an underlying infection or inflammation. However, more complications can arise from it including chronic thrombosis, a blood clot that expands into your mesenteric vein, limited blood flow to your bowel, liver abscess, liver infarction, and splenic infarction. Unfortunately, it can even lead to death in severe cases. A review of 95 cases indicated that these complications happen in about 20% of people.

List of Possible Complications:

• Chronic Thrombosis
• Blood clot that reaches the mesenteric vein
• Restricted blood flow to the bowels (Bowel Ischemia)
• Liver Abscess
• Liver Infarction
• Splenic Infarction
• Death

Preventing Pylephlebitis

If you have been diagnosed with pylephlebitis, it’s crucially important to follow through with taking your antibiotics as recommended by your doctor. Pylephlebitis is a health condition caused by inflammation in the veins of your liver. Failing to adhere to your prescribed treatment plan can lead to serious complications.

Pylephlebitis may occur due to different reasons. Some people may be more at risk because they have inherited specific blood clotting disorders (thrombophilia) or because they have liver damage (cirrhosis). If you have any such chronic conditions, your doctor will advise on the best way to manage and continually monitor them to reduce your chances of developing pylephlebitis. It’s important to keep in touch with your doctor and follow their recommendations carefully.