Chilaiditi Syndrome

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Diagnosis and reasoning

The acute abdomen is one of the most common presentations encountered in the emergency department; it is also one of the more concerning, potentially heralding serious disease. In these individuals, the location of the pain is the first and most important pointer towards the likely underlying etiology; when considered along with the other characteristics, a targeted differential diagnosis can often be achieved. The lady in this case has presented with pain in the upper quadrant, with radiation to the right shoulder; at first glance, this raises suspicion of biliary colic. However, note several atypical features: first of all, the pain is colicky in nature; despite the name, the pain of biliary colic is constant. Furthermore, biliary colic typically lasts from minutes to upto a couple of hours. A one day duration of pain is distinctly unusual. Another important diagnosis which comes to mind is acute (on chronic) pancreatitis; while this can give rise to constant, worsening pain, note that radiation (if present) is usually towards the back, with reduction of the pain upon leaning forwards often being seen. Peptic ulcer disease is also a possibility, given the prolonged use of NSAIDS for back pain (although the presence of concomitant omeprazole therapy would have a protective action in this respect); however, this too does not entirely fit the the aforementioned clinical picture. Last, but by no means least, the possibility of atypical anginal pain should always be considered in these patients; fortunately, this is made highly unlikely by the absence of a supportive history, and the unremarkable serial ECG tracings. Note that the neutrophil leukocytosis seen in the full blood count (FBC) is a nonspecific finding which can be caused by any of the pathologies listed above. Liver chemistries and imaging of the right upper quadrant are key initial investigations in the workup of right upper quadrant pain; given the possibility of pancreatitis, simultaneous amylase and lipase measurements can also be justified. However, both serum amylase and lipase are within normal ranges, making acute pancreatitis less likely (although it should be appreciated that pancreatic enzyme levels may not significantly rise in patients with coexisting chronic pancreatitis); the unremarkable liver profile is not of diagnostic aid. In addition, the ultrasound scan shows a normal appearing gallbladder with no evidence of gallstones or biliary tract obstruction, arguing against biliary colic. However, sonography also reveals a very interesting finding: the presence of gas echoes of different sizes over the right lobe of the liver. While this initially suggests at pneumoperitoneum, note that the positions of the gas shadows are unaffected by the patient's posture; this is a strong hint against this diagnosis. Follow up imaging via computerized tomography (CT) is a must, as this will accurately delineate the relevant anatomy, and hopefully identify the causative pathology. This reveals the surprising finding of interposition of a loop of colon between the liver and right hemidiaphragm - i.e. Chilaiditi syndrome; note also that there is no evidence of bowel obstruction, a common complication of the disease. Uncomplicated Chilaiditi syndrome should be managed conservatively via nasogastric decompression and enemas. Surgery is only considered if the condition does not resolve, or if there is suspicion of ischemia. IV antibiotics are not indicated in his current management.


Discussion

Chilaiditi's syndrome is a rare condition where transposition of a loop of a large intestine in between the liver and diaphragm results in abdominal pain. The condition is most common in the elderly, with an estimated prevalence of upto 1%; however, it has also been reported in patients as young as 5 months of age. There is a male preponderance, with a 4:1 male to female ratio. The exact pathophysiology of Chilaiditi syndrome is uncertain; however, intestinal, hepatic, and/or diaphragmatic etiologies are believe to contribute to the pathogenesis. In normal individuals, the suspensory ligaments and falciform ligament of the liver, position of the liver, and fixity of the colon impede interposition of the colon between the liver and diaphragm. However, in certain persons, anatomical variations predispose towards pathological interposition of the colon; these include absence, laxity or elongation of the suspensory ligaments or falciform ligament, congenital malpositions, presence of redundant colon or dolichocolon (abnormally long colon), and paralysis of the right diaphragm. Acquired risk factors for Chilaiditi syndrome include chronic constipation (as this may give rise to chronic elongation and redundancy), obesity, multiple pregnancies, ascites, aerophagia, cirrhosis leading to liver atrophy, and acquired causes of paralysis of the right diaphragm. These patients commonly present with abdominal pain, anorexia, nausea, vomiting, flatulence and constipation; their severity can range from mild, intermittent complaints, to an acute abdomen. Other, rare associated symptoms include respiratory distress, angina like chest pain, and a combination of multiorgan symptoms. The complications of Chilaiditi syndrome can be severe, including volvulus of the cecum, splenic flexure or transverse colon, cecal perforation, and rarely, subdiaphragmatic appendicitis. The disease is also considered a rare cause of obstruction of the large and small bowel; it has also been associated with colonic pseudo-obstruction (Ogilvie syndrome). Furthermore, Chilaiditi syndrome has also been associated with a variety of gastrointestinal and pulmonary malignancies. The X-Ray finding of hemidiaphragmatic interposition of the colon is known as the Chilaiditi sign; this may be seen both permanently and sporadically. It is also called a 'pseudo-pneumoperitoneum' as it is easily mistaken for free intra abdominal air. The diagnostic criteria of the Chilaiditi sign are as follows: - The right hemidiaphragm must be displaced superiorly by the intestine. - The bowel must be distended by air so as to illustrate pseudo-pneumoperitoneum. - The superior margin of the liver must be positioned below the level of the left diaphragm. Note that if ultrasonography is performed, alteration of the posture of a patient with the Chilaiditi sign will not change the location of the gas echo, as opposed to an individual with a pneumoperitoneum. In (hemodynamically stable) patients in whom it is impossible to distinguish between intraluminal and free subdiaphragmatic air via radiography or ultrasound, computerized tomography (CT) should be considered; this has the added benefit of excluding diaphragmatic rupture. In most patients, conservative management is all that is required. This should consist of bed rest, nasogastric decompression, fluid supplementation, enema, laxatives and a high fiber diet. If the patient shows no response to conservative management, if repeated imaging shows failure of resolution, or if there is evidence of bowel ischemia, surgical intervention is indicated. Surgical interventions available include segmental colon resection (if the transverse colon is involved), cecopexy, and hepatopexy.


Take home messages

  1. Chilaiditi syndrome is the symptomatic interposition of a large intestinal loop between the liver and diaphragm.
  2. The Chilaiditi sign may be mistaken for pneumoperitoneum by the unwary.
  3. These patients can often by managed conservatively; surgery is only required if complications occur.

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