Clinical Challenges

April 2018 What's your diagnosis?


By Jordan Orr, MD, and Charles O. Elson III, MD. Published previously in Gastroenterology (2016;151[2]:241-2).

AGA Institute

Figure A

A 67-year-old man presented to the emergency department with complaints of subacute, right-sided flank pain with migratory pain to his right lower quadrant and suprapubic area of increasing intensity for 1 week. He described his pain as cramping in nature and of fluctuating intensity, acutely worse on the day of presentation. However, within 15 minutes of waiting in the emergency department his pain subsided completely. He further denied any associated nausea, vomiting, diarrhea, melena, hematochezia, dysuria, or hematuria. Vital signs and abdominal physical examination were normal. Further, laboratory testing was unremarkable including a normal urinalysis. A bedside ultrasound was negative for gallbladder pathology or nephrolithiasis; however, it revealed an abnormal appearing liver. As further diagnostic work up, an abdominopelvic computed tomography scan revealed the following images (Figures A, B). The patient was discharged from the emergency department with scheduled follow-up in the gastroenterology clinic.

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Figure B

What is your diagnosis and treatment?


 

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.

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