Introduction 

Chilaiditi syndrome is a rare condition, seen in less than 0.5% of the entire population,¹ with no known cause. It is more common in older adults but can also occur at any age. It is where a segment of the intestine, usually the colon, is abnormally located between the liver and the diaphragm.³ The diaphragm is the main muscle for breathing, which also separates the chest cavity from the abdomen. This results in various symptoms which can vary from one person to another, such as abdominal pain, nausea, vomiting and possible small bowel obstruction.² However, Chilaiditi syndrome is usually asymptomatic, meaning that no symptoms are displayed and the syndrome is usually found incidentally. When Chilaiditi syndrome displays with no symptoms, it is known as Chilaiditi’s sign. 

In this article, we will be focusing on what is known as ‘acquired Chilaiditi’s’, which is Chilaiditi syndrome, which develops due to unknown reasons after birth. Whilst there is no known cause, it has been found that the condition is more common in those with chronic lung disease, liver scarring (known as cirrhosis) and when there is a buildup of fluid in the abdomen (called ascites). Other predisposing factors have also been found, such as the elongation of the colon.⁴ Chilaiditi syndrome is characterised by three key features: liver atrophy, a redundant colon and diaphragmatic elevation. We will further discuss these features in the article and how this is the body’s way of accommodating and adapting to the displaced colon. 

The triad explained 

In Chilaiditi syndrome, three factors (referred to as ‘the triad’) contribute to the unusual positioning of the colon: liver atrophy, a redundant colon and diaphragmatic elevation. 

Firstly, we will break down the different terminology used in the following sections:

• Diaphragm: a muscle located at the bottom of the lungs, it separates the chest cavity from the abdominal cavity. The muscle is dome-shaped and its main function is to aid breathing⁶
• Liver: an organ which is found below the diaphragm and takes up the majority of the upper right-hand portion of the liver. However, part of the liver also extends into the upper left abdomen area⁷
• Colon: the colon is the main part of the large intestine, located in the abdomen and extending from right to left. This forms a large U-shape around the small intestine⁸

Liver atrophy 

Liver atrophy is a phrase used to describe the shrinkage of the size of the liver. This can result from a multitude of factors, including alcohol abuse, disease and general ageing. However, in Chilaiditi syndrome, it is due to the pressure of the displaced organs from the abnormal placement of the colon.⁹ The liver is usually located in the right upper side of the abdomen, but because the liver has shrunk, there is extra space. This empty space causes other organs, such as the intestine, to become displaced.¹⁰ 

Redundant colon

A redundant colon refers to a colon/large intestine (the colon is the main part of the large intestine, so colon and intestine may be used interchangeably) that is longer than average, so it has extra loops or folds. As the colon is longer, there is an increased movement of segments. The increased length and flexibility can enable the colon to shift into places that it is not usually found, such as between the liver and diaphragm - this may result in symptoms such as constipation. 

Diaphragmatic elevation 

Diaphragmatic elevation is a phrase used to describe the diaphragm being pushed upwards. The diaphragm is a dome-shaped muscle that is located under the lungs and aids breathing. However, in some people, the diaphragm sits higher than usual, which is referred to as diaphragmatic elevation. This elevation can occur due to several reasons, for example, diaphragm muscle weakness, neurologic disease, traumatic injury and decreased lung volume.¹¹ When the diaphragm is elevated to a greater extent than usual, it causes the upper abdomen to be pushed into the diaphragm, creating less space for the liver, resulting in an altered anatomy. This results in a gap, making it easier for parts of the intestine, making it easier for the intestine to move into the space created between the liver and diaphragm. In Chilaiditi syndrome, the right diaphragm is found to be elevated above the liver, next to the intestine.³

To summarise, these three anatomical changes play a vital part in creating room for the colon to move into an unusual position, located between the liver and diaphragm. Liver atrophy reduces the usual size of the liver, creating extra space. A redundant colon results in greater intestinal flexibility due to the extra length, increasing the likelihood of it shifting and diaphragmatic elevation results in the diaphragm raising higher than normal. This unusual elevation results in a large gap in the upper abdomen. These all encourage an intestinal slip, which may lead to Chilaiditi syndrome.   

Symptoms of chilaiditi syndrome 

The syndrome usually presents as asymptomatic and is often found incidentally on radiological findings, such as X-rays - this is referred to as Chilaiditi's sign.³ However, the syndrome can manifest into symptoms such as abdominal cramping and pain, constipation and vomiting. It is important to understand the signs and symptoms of Chilaiditi syndrome as it can sometimes become life-threatening. The misplaced intestines may become twisted and stuck between the liver and the diaphragm, forming an intestinal blockage which is potentially life-threatening if not treated.¹

Diagnosis

Chilaiditi syndrome is diagnosed by using imaging techniques to confirm the abnormal positioning of the colon and the presence of certain symptoms associated with the syndrome. Examples of imaging techniques include chest and abdominal X-rays, ultrasounds and CT scanning.²

Additionally, there is a certain criterion that must be met to diagnose Chilaiditi based on radiological findings:³

• The right hemidiaphragm needs to be elevated higher than the liver by the intestine 
• The bowel must be distended by air 
• The liver must be below the left hemidiaphragm 

Treatment and management

Regarding Chilaiditi’s sign, where the patient appears asymptomatic, intervention is often not necessary, and any treatment will adopt a conservative approach as long as the possibility of more serious conditions has been ruled out. In symptomatic cases, treatment may include: 

• Intravenous fluids: these help keep the patient hydrated, promoting healthy bowel function and also rehydrating the patient if they have been vomiting 
• Nasogastric bowel decompression: This procedure involves a thin tube being placed through the nose into the stomach. This relieves pressure due to the trapped air, reducing discomfort. 
• General bed rest 
• Stool softeners and enemas: to prevent constipation 
• Repeat radiographic monitoring to observe the air present below the diaphragm 

In some cases, surgical intervention is needed. Surgery is often advised when a patient does not respond to the previous conservative approach to treatment, meaning the obstruction does not resolve or there is evidence of a lack of blood supply to the intestines, which is referred to as intestinal ischemia. 

Living with chilaiditi syndrome

For the majority of those diagnosed with Chilaiditi syndrome, it is relatively manageable. Many often experience mild symptoms, which can be managed via conservative measures such as keeping hydrated, diet changes (increasing fibre intake) and the administration of stool softeners. It is also important that any changes in abdominal discomfort are monitored and followed up using radiographic techniques, ensuring there are no dangerous complications. Chilaiditi is a condition that, once correctly diagnosed, can easily be managed safely and effectively, allowing individuals to maintain a normal and healthy lifestyle. 

Conclusion

While Chilaiditi syndrome is rare, it is difficult to fully understand the syndrome due to the potential misdiagnoses that can occur. The condition is defined by the presence of a misplaced colon, located between the liver and the diaphragm. This occurrence may be made possible due to the presence of three anatomical features: liver atrophy, a redundant colon and diaphragmatic elevation - each of these features resulting in features that encourage colon misplacement. Recognising this condition is important due to its tendency to mimic different diseases and conditions, some of which are life-threatening and require immediate treatment. For example, bowel obstruction or an intestinal twist. Misdiagnosis could possibly lead to unnecessary procedures that may cause more harm than good, highlighting the importance of understanding both the anatomical features and clinical presentation of Chilaiditi.