What is Chilaiditi Syndrome?
Chilaiditi sign is a term used to describe a specific finding on an X-ray or other imaging scan. It refers to a lump of the large bowel or small intestine that has shifted its position and is now located between the liver and the diaphragm (the muscular sheet separating the chest and abdomen). When this shift leads to digestive problems, such as pain, bloating, or difficulty eating, it is labeled as Chilaiditi syndrome.
What Causes Chilaiditi Syndrome?
Normally, there are several structures in your body that hold the colon in place and prevent it from moving between the liver and diaphragm. However, if these structures are different in any way – for example, if certain ligaments are absent, too loose, or too long – this can lead to a condition called Chilaiditi syndrome, where the colon ends up in an unusual position.
Other factors that can raise your risk of developing Chilaiditi syndrome include being born with your organs in unusual positions, chronic constipation due to an unusually long colon and redundancy, a swelling of the colon due to gas, a small liver caused by cirrhosis or liver surgery, increased pressure inside your stomach due to fluid build-up, significant weight loss in people who were previously obese, and a diaphragm that is unusually high or paralyzed. The last can occur with conditions such as diaphragmatic muscle degeneration or injury to the phrenic nerve, a chronic lung disease causing enlargement of the lower chest cavity, or multiple pregnancies.
Intellectual disability and schizophrenia are also associated with changes in body shape that can lead to Chilaiditi syndrome. There have even been a few cases where medical procedures such as weight loss surgery, the insertion of feeding tubes, or colonoscopies have unintentionally caused Chilaiditi syndrome.
Risk Factors and Frequency for Chilaiditi Syndrome
Chilaiditi syndrome, named after Greek radiologist Demetrius Chilaiditi, was first described in 1910. It involves the movement of a loop of bowel in between the liver and the right diaphragm, and it usually shows up as free air in the abdomen on imaging studies. It’s quite rare, affecting about 0.025% to 0.28% of people globally, with a higher prevalence in men than women at a ratio of 4:1. Chilaiditi syndrome is most observed in the elderly, with a 1% incidence, but can occur at any age from 5 months to 81 years.
- Chilaiditi syndrome was first described in 1910 by Demetrius Chilaiditi, a Greek radiologist.
- The condition shows up as free air in the abdomen on imaging studies.
- Global incidence is between 0.025% to 0.28%.
- It affects men four times more than women.
- It is mainly seen in the elderly, with a 1% incidence rate.
- The age range of patients can be as young as 5 months to as old as 81 years.
- It is also found in 8.8% of mentally ill patients.
Signs and Symptoms of Chilaiditi Syndrome
Chilaiditi syndrome is a medical condition with a range of potential symptoms. The most common signs that patients display include abdominal pain, loss of appetite, feeling sick, vomiting, gas, constipation, and changes in bowel habits. In some cases, patients also experience respiratory distress, like difficulty breathing. Less often, people may encounter heart-related symptoms such as chest pain similar to angina and abnormal heart rhythms. These symptoms can worsen when the patient is lying down at night. Sometimes, patients might present with a combination of these symptoms. Gastrointestinal symptoms might vary from mild to severe and may even result in an acute abdomen, a condition that needs urgent medical attention
- Abdominal pain
- Loss of appetite
- Feeling sick
- Vomiting
- Gas
- Constipation
- Changes in bowel habits
- Respiratory distress
- Chest pain (similar to angina)
- Abnormal heart rhythms
- Worsening of symptoms at night
- Potential combination of these symptoms
- Variable gastrointestinal symptoms (mild to severe)
Testing for Chilaiditi Syndrome
The Chilaiditi sign is a condition that doctors often find unexpectedly while examining chest X-rays or abdominal computer tomography (CT) scans – these are different types of pictures taken of the inside of your body. It’s not very common; it shows up in roughly 1.18% to 2.40% of abdominal CT scans and between 0.025% and 0.280% of general chest and belly X-rays. These signs can show up just for a short time, or they can be permanent.
To say for sure whether a person has the Chilaiditi sign, doctors look for certain things in the radiological images. Firstly, X-ray or CT images must show the right side of the diaphragm (a muscle that helps you breathe) lifted above the liver by the intestine. Secondly, the intestine must have a bit of air trapped within, giving the appearance of air in the belly cavity. Finally, the top edge of the liver must sit lower than the left side of the diaphragm. How the bowel is positioned around the liver can make this sign appear in two ways – either from the front (anterior) or the back (posterior).
The portion of the intestine that most often gets into this position is usually the hepatic flexure, ascending colon, or transverse colon, but in some cases, the small intestine can do it too. If the X-ray shows free air in the abdomen, doctors would ask for an abdominal CT scan to be sure. Free air could mean that surgery is needed, while the presence of the Chilaiditi sign typically means that you would just need to follow some simple treatments.
Treatment Options for Chilaiditi Syndrome
If you are found to have a condition called Chilaiditi sign and you’re not experiencing any symptoms, you typically won’t need any special treatment. First, the doctors will make sure you don’t have any serious issues that need immediate attention. If you’re having symptoms related to this condition, known as Chilaiditi syndrome, you’ll typically have a very relaxed, easy-going treatment plan. You might receive fluids through a drip (intravenous fluids), they might use a tube to relieve gas or contents from your stomach (nasogastric bowel decompression), and they might ask you to rest in bed, use stool softeners, or have an enema. An enema involves injecting liquid or gas into the rectum to clear out the bowels. Afterwards, an x-ray or other imaging will be likely be done again to check if the air below your diaphragm has gone away, which would suggest that you have Chilaiditi syndrome.
If this relaxed treatment plan doesn’t work, if the food in your digestive system (your bowel) doesn’t continue to move as it should, or if there is a concern that your bowel isn’t getting enough blood (a condition called bowel ischemia), you might need surgery. However, there’s no specific agreement amongst doctors about which surgery will best fix the issue when your bowel is in the wrong place. One option is called a “ceceopexy,” which is done to stop the same issue from happening again if you have a specific problem, known as “cecal volvulus,” and surgery is not needed to remove any bowel. If you have a “volvulus” in another part of your bowel called the transverse colon, you might need that section of your bowel removed. A volvulus is a twisting of the intestine that can cause an obstruction.
Colonoscopy, a procedure used to look at the inside of the colon, is usually not recommended because it could potentially lead to gangrene, tissue death resulting from an inadequate blood supply. If a colonoscopy is done, it needs to be done very carefully. This is because there’s a risk that the colonoscopy could lead to excessive air getting trapped in the looped and dislocated bowel and that could cause a perforation (a hole in the bowel). Using carbon dioxide instead of air during the colonoscopy can help decrease this risk.
What else can Chilaiditi Syndrome be?
Chilaiditi syndrome is a medical condition that often needs to be distinguished from others such as:
- Trapped air within the abdomen (Pneumoperitoneum)
- Hernia in the diaphragm (Diaphragmatic hernia)
- Collection of pus beneath the diaphragm (Subdiaphragmatic abscesses)
- Ruptured intestine (Bowel perforation)
- Blocked intestines (Bowel obstruction)
- Ogilvie syndrome (a type of bowel obstruction)
- Intussusception (a type of bowel obstruction)
- Volvulus (twisting of the intestines)
- Reduced blood supply to intestines (Ischemic bowel)
- Appendicitis
- Diverticulitis (inflamed pouches in the digestive system)
Each condition has its distinctive signs that can help with the diagnosis. For example, if you can see the normal ‘folds’ or markings of the colon beneath the diaphragm, you can usually rule out a subphrenic abscess. And if you perform an ultrasound scan and the positions of the internal structures don’t change when the patient moves, it suggests that Chilaiditi syndrome could be the right diagnosis, rather than pneumoperitoneum.
It’s crucial to reach the correct diagnosis since mistaking Chilaiditi syndrome for a more serious condition may lead to unnecessary surgery. Moreover, it’s vital to recognize this syndrome when performing a liver biopsy because the intestine’s abnormal position increases the risk of perforation, especially during certain procedures.
What to expect with Chilaiditi Syndrome
Chilaiditi syndrome is typically a non-harmful condition. However, it can sometimes lead to dangerous complications that can be life-threatening.
Possible Complications When Diagnosed with Chilaiditi Syndrome
If misdiagnosed as presence of air or gas in the abdominal cavity, this condition could lead to unnecessary surgery. Other problems include twisting of the cecum, the part of the colon near the appendix, the bend of the colon near the spleen, or the middle part of the colon. There have been reported cases of cecal rupture, decreased blood supply to the tissue that connects the intestines to the abdominal wall, inflammation of the peritoneum, and appendix infection under the diaphragm. All these situations require emergency surgery.
Possible Complications:
- Unnecessary surgery if misdiagnosed
- Twisting of the cecum, splenic part of the colon, or middle part of the colon
- Cecal rupture
- Decreased blood supply to the intestine-connecting tissue
- Inflammation of the peritoneum
- Appendix infection under the diaphragm
- Emergency surgery requirements
Preventing Chilaiditi Syndrome
Chilaiditi syndrome is generally harmless. However, when Chilaiditi sign (an abnormal positioning of the colon) shows up on medical images, it’s crucial to rule out other serious conditions that might need surgery. The primary treatment for Chilaiditi syndrome involves non-invasive tactics, but if the symptoms don’t get better, more aggressive treatments may be needed.