Chilaiditi Syndrome - Causes, Clinical Features, Diagnosis, and Management

Introduction:

Suspensory ligaments and colon fixation patterns usually avoid interposing of the intestine. The abnormally placed colon (a part of the small intestine) between the diaphragm and liver occurs in rare cases and may be caused by anatomic variations in the colon, which can lead to gastrointestinal manifestations, which is clinically termed Chilaiditi syndrome. Chilaiditi syndrome is characterized by the Chilaiditi sign, which occurs due to the air being trapped below the right diaphragm. This syndrome was first discovered by a Greek radiologist, Demetrius Chilaiditi, in 1910. He found that the air was trapped due to the abnormal placement of the colon between the right hemidiaphragm and liver in three patients.

What Is Chilaiditi Syndrome?

Chilaiditi syndrome is a rare benign radiological feature that occurs due to the interposition of a segment of the small intestine between the diaphragm and the liver. Most of the cases are asymptomatic. Symptomatic cases present as gastrointestinal manifestations, which include abdominal pain, nausea, vomiting, and constipation, and usually affect the elderly.

What Are the Causes of Chilaiditi Syndrome?

• The anatomic variations include abnormalities in the morphology of the suspensory ligament of the colon and dolichocolons (an abnormally enlarged colon with altered shape) or congenital malpositions.

• Functional disturbances cause anatomic variations, which include:

1. Chronic constipation due to colonic elongation.

2. Aerophagia (excess swallowing of air) occurs due to gaseous distension of the colon.

3. Liver cirrhosis causes atrophy of the liver.

4. Paralysis of the diaphragm.

5. Chronic lung disease leads to an enlarged lower thoracic cavity.

6. Obesity.

7. Multiple pregnancies.

8. Ascites (raised intra-abdominal pressure).

9. Mental retardation and schizophrenia are also linked with the Chilaiditi sign due to anatomic abnormalities caused.

What Are the Clinical Features of Chilaiditi Syndrome?

• Abdominal pain.

• Nausea.

• Vomiting.

• Constipation.

• Respiratory distress.

• Chest pain mimics Angina.

• Indigestion.

• Twisting of the intestine.

• Dysphagia (difficulty in eating).

• Abdominal distension.

• Tenderness in the epigastric region.

• Flatulence (intestinal wind passing out through the anus).

• Arrhythmias (irregular heartbeat).

How Is Chilaiditi Syndrome Diagnosed?

Chilaiditi syndrome is a radiological finding and can be best appreciated by a radiograph. It is characterized by the classic Chilaiditi sign, which is appreciated by air below the right diaphragm on a radiograph. The following criteria should be fulfilled to diagnose Chilaiditi sign in a radiograph.

1. The intestine should allow the elevation of the right hemidiaphragm.

2. Pseudo pneumoperitoneum will be appreciated by the air, causing bowel distension.

3. Chilaiditi sign is best viewed in the superior margin of the liver and is depressed below the level of the left hemidiaphragm.

4. Changing the position of a patient during an ultrasound will not cause changes in the location of the gas echo as in cases of pneumoperitoneum. This can help in differentiating Chilaiditi syndrome from other findings.

5. CT (computed tomography) scan can be employed in cases when the radiographic and ultrasound imaging cannot determine whether subdiaphragmatic air being trapped is either free or intraluminal.

6. CT scan is an important diagnostic tool in identifying the Chilaiditi syndrome, aiding in providing an accurate diagnosis.

What Are the Complications of Chilaiditi Syndrome?

Complications of Chilaiditi syndrome can include:

1. Volvulus of the cecum (twisting of the cecum, part of the large intestine).

2. Transverse colon (a part of the large intestine which goes across the stomach).

3. Cecal perforation (perforation of cecum, part of large intestine).

4. Mesenteric ischemia (impaired blood flow to the small or large intestine).

5. Peritonitis (inflammation of the layer called peritoneum surrounding the abdominal cavity).

How Is Chilaiditi Syndrome Treated?

• Chilaiditi syndrome is initially managed by bed rest, intravenous fluid therapy, bowel decompression, enemas, and laxatives.

• The absence of air below the diaphragm and repositioning of the elongated intestine back to the normal position below the liver in the radiograph after bowel decompression shows regression of the condition.

• Hence, a follow-up radiograph after bowel decompression can clearly point out the condition, and the success of the therapy can also be determined.

• Surgical intervention is planned if the initial conservative treatment fails or because of the failed resolvent of the obstruction or if the condition progresses to bowel ischemia.

• Recent years have witnessed an increase in surgical management, especially in cases of chronic and intermittent abdominal pain.

• Surgical intervention is dictated by the condition of the interposed colonic segment.

• Unless there is a complication like gangrene or perforation, which demands surgical resection, cecopexy can be attempted in case of cecal volvulus.

• Colonic resection remains the mainstay of the treatment for managing transverse colon volvulus, as the chances of gangrene formation are higher in such cases with colonoscopic reduction.

• Asymptomatic cases usually do not require clinical management and can be monitored by regular follow-up checkups.

• More complicated conditions like pneumoperitoneum should be ruled out first when bowel obstruction cases are suspected.

• Only careful examination and radiological findings can reveal the Chilaiditi sign, which can prevent misinterpretations and unwanted surgical interventions.

• When performing liver biopsy and percutaneous transhepatic procedures in cirrhotic patients, Chilaiditi sign recognition is important to prevent impending complications.

• Colonoscopy should be carried out carefully as an interposed segment of the bowel can pose various challenges of air entrapment in an acutely angulated, interposed bowel, which may tend to cause perforations.

• The risk can be minimized by administering carbon dioxide as the insufflating agent during the colonoscopy procedure.

Conclusion:

Most of the cases are asymptomatic and require no treatment. Chilaiditi syndrome is a benign condition that seldom leads to fatal complications. As Chilaiditi syndrome is a benign condition, the conservative mode of approach serves the purpose in most cases, and an invasive treatment approach is considered if the condition progresses to complications. However, differential diagnoses should be made when the Chilaiditi sign is detected in the radiograph, as it can rule out other potential complications that require surgical interventions and, at the same time, can avoid unnecessary surgical interventions in mild cases.