Answer to “What’s your diagnosis?” Chilaiditi syndrome
Abdominal CT images display the Chilaiditi sign, which is the radiographic term used to describe interposition of the colon, usually at the hepatic flexure, with the liver and right diaphragm.1 This is considered an incidental radiographic finding and is generally asymptomatic; however, when one develops clinical symptoms such as abdominal pain, bloating or distension, anorexia, constipation, or nausea it is called Chilaiditi syndrome.
First described by Greek radiologist Demetrius Chilaiditi in 1910, Chilaiditi syndrome is a rare occurrence with an incidence rate of 0.25%-0.28% in the general population.2 The etiology of Chilaiditi syndrome is felt to be congenital or acquired with predisposing congenital abnormalities such as absent suspensory or falciform ligaments, redundant colon, malposition of the colon, dolichocolon, and paralysis of the right diaphragm. Other risk factors for development of Chilaiditi syndrome include chronic constipation, cirrhosis, ascites, and obesity. Men are times times as likely as women to develop Chilaiditi syndrome and it is more common in the elderly, occurring in 1% of the elderly population.3 Chilaiditi sign is diagnosed with radiographic imaging meeting the following criteria: the right hemidiaphragm must be elevated above the liver by the intestine, the bowel must be distended by air to illustrate pseudopneumoperitoneum, and the superior margin of the liver must be depressed below the level of the left hemidiaphragm.1
Chilaiditi syndrome is managed conservatively with close observation. Recurrent symptoms can be treated with colopexy. This syndrome has been known to cause severe complications including volvulus of the cecum, splenic flexure, or transverse colon, cecal perforation, and subdiaphragmatic perforated appendicitis, which all require surgical intervention.3 It is important to recognize Chilaiditi syndrome on presentation to prevent unnecessary diagnostic studies and unwarranted surgical intervention.
References
1. Uygungul, E., Uygungul, D., Ayrik, C., et al. Chilaiditi sign: why are clinical findings more important in ED?. Am J Emerg Med. 2015;33:733.e1-733.e2.
2. Ho, M.P., Cheung, W.K., Tsai, K.C., et al. Chilaiditi syndrome mimicking subdiaphragmatic free air in an elderly adult. J Am Geriatr Soc. 2014;62:2019-21.
3. Kang, D., Pan, A.S., Lopez, M.A., et al. Acute abdominal pain secondary to Chilaiditi syndrome. Case Rep Surg. 2013;2013:756590.