Chilaiditi Syndrome Complicated by a Closed-Loop Small Bowel Obstruction

Chilaiditi syndrome is a rare disease in which intestinal obstruction is caused by hepatodiaphragmatic interposition of the colon or small bowel. Demetrius Chilaiditi described the first cases of this disease in 1910.¹ Most patients with this intestinal anomaly are asymptomatic throughout their lives; however, they can manifest with intermittent abdominal pain, distention, vomiting, anorexia, and constipation that on rare occasions require surgical intervention.²

Case Report

A 55-year-old African-American woman presented to the emergency department with a history of gradually increasing abdominal pain localized to the epigastrium and right upper quadrant that radiated to the right shoulder. Her pain was associated with nausea, constipation, and obstipation, as well as nonbilious and nonbloody emesis on several occasions. The patient reported having vomiting episodes during her childhood. Her surgical history was significant for a Cesarean section, ovarian cystectomy, and appendectomy. On physical examination, she was hemodynamically stable. Her abdomen was distended with decreased bowel sounds, tenderness in the right upper quadrant, and a positive Murphy sign with rebound tenderness. Results of the patient’s complete blood count, liver function tests, and urinalysis were normal. A right upper quadrant ultrasound demonstrated irregular shadowing in the liver, with a morphology similar to that of the small bowel. The patient’s gallbladder had normal dimensions and wall thickness, and there was no evidence of cholelithiasis.

Computed tomography (CT) scans of the abdomen and pelvis performed with intravenous and oral contrast demonstrated an abnormal course and configuration of the small bowel and colon, with portions of the transverse colon traveling through the Morrison pouch; multiple small bowel loops were also seen interposed among the liver, abdominal wall, and diaphragm. The CT scans also demonstrated mild small bowel wall thickening with inflammatory changes, air-fluid levels, and an abrupt transition in bowel caliber that was characteristic of small bowel obstruction (Figure 1).

Figure 1.

Computed tomography scans with contrast showing loops of small bowel between the dome of the liver and diaphragm, with a subsequent, abrupt change in the caliber of the bowel. Figure 1A shows a loop of small bowel located between the liver and the abdominal wall. Figure 1B shows the incarcerated small bowel loop and the hepatic flexure of the colon between the liver and the abdominal wall. Figure 1C shows the transverse colon interposed between the liver and the abdominal wall. The arrow points to an area of fat stranding in the mesentery.

C=colon; L=liver; RK=right kidney; S=stomach; SB=small bowel.

On the basis of the patient’s presentation and imaging results, the patient was taken to the operating room for an exploratory laparotomy. A 6–8-inch segment of the terminal ileum that was approximately 3 feet from the ileocecal valve was involved in a closed-loop obstruction above the liver secondary to adhesions between the liver and the abdominal wall. The bowel initially appeared to be mildly ischemic, but it regained viability after the closed loop was untwisted and warm saline was applied; hence, resection was not necessary. The colon did not have any abnormalities.

Discussion

In 1910, the radiologist Demetrius Chilaiditi described 3 patients who had an interposition of the bowel between the liver and right hemidiaphragm. A Chilaiditi sign is thus used to describe the incidental radiologic finding of a colonic or intestinal hepatodiaphragmatic interposition in an asymptomatic patient. This sign is commonly misinterpreted as pneumoperitoneum. The prevalence of Chilaiditi sign in the general population is 0.025–0.28%, and the sign is more prevalent in male patients than female patients.³ The bowel segments most commonly found interposed between the liver and diaphragm or abdominal wall are the colonic hepatic flexure and transverse colon, although interposition of the small bowel has also been reported.^4,5

Physiologic and conformational patterns of hepatic and colonic embryogenesis and adult anatomy usually prevent the development of colonic interposition. Factors that predispose patients to the development of Chilaiditi sign include reduced liver dimensions, elongation of the ligamentous suspension of the liver, and redundancy of the colon. Congenital diaphragmatic, hepatic, or intestinal anomalies and pathologies associated with the development of this sign include right hepatic lobe segmental agenesis, relaxation or agenesis of the mesentery suspensory ligaments, chronic constipation, redundant and hypermobile transverse mesentery and transverse colon, and significant weight loss. Another important cause of Chilaiditi sign is severe chronic obstructive pulmonary disease and its subsequent elongation of the diameter of the lower thoracic cage, which results in a broader space in which colonic interposition can occur. Elevation of the right hemidiaphragm from congenital hernias and eventration of the diaphragm also predispose patients to development of Chilaiditi sign. Furthermore, the characteristics most frequently associated with Chilaiditi sign—cirrhosis, ascites, and decreased hepatic size—increase the space between the liver and diaphragm; these characteristics occur in up to 5% of patients.⁶

Patients with Chilaiditi syndrome present with symptoms of bowel obstruction, including anorexia, nausea, emesis, abdominal pain, distension, and obstipation.⁷ The patient in this case study presented with these symptoms, as well as right shoulder pain from diaphragmatic irritation.

Chilaiditi syndrome has been associated with a volvulus of the transverse colon. Due to mesenteric attachments, normal anatomy prevents the transverse colon from rotating and developing a volvulus. Factors that predispose patients to developing a volvulus include increased colonic mobility and a site of axial colonic rotation; specific factors that predispose patients to developing a transverse colon volvulus include congenital malrotation of the midgut and associated agenesis of the phrenocolic ligament or mesenteric root axial shortening.

Diagnosis of Chilaiditi syndrome is based upon clinical findings and signs observed on plain radiographs and CT scans. CT scans of the abdomen can enable clinicians to differentiate among subphrenic fluid, true pneumoperitoneum, and air within the bowel lumen. This differentiation is of the utmost importance for establishing the diagnosis of hollow viscus perforation, which can also complicate Chilaiditi syndrome when the involved bowel segment strangulates and eventually perforates.⁸ The radiologic differential diagnosis is established by observing an elevation of the right hemi-diaphragm due to caudal displacement of the liver, haustral markings between the liver and diaphragmatic surface, and the absence of image displacement with changes in the patient’s position. Pneumoperitoneum and subdiaphrag-matic fluid collections are mobile on lateral decubitus radiographs and are accompanied by pulmonary findings such as ipsilateral pleural effusion and basilar atelectasis.

In most cases of Chilaiditi syndrome, management is conservative and consists of bowel decompression, bowel rest, and aggressive fluid rehydration.⁹ Patients who fail conservative therapy should undergo an exploratory lapa-rotomy.¹⁰ Failure of nonsurgical treatment for Chilaiditi syndrome has been associated with colonic volvulus and obstruction.¹¹ To our knowledge, there have not been any previous reports of small bowel obstruction necessitating surgical treatment in patients with Chilaiditi syndrome.

Summary

Chilaiditi syndrome is a rare form of bowel obstruction caused by the interposition of the colon or small bowel into the hepatodiaphragmatic space. This condition usually resolves with conservative management, but in this case report, the patient had a closed-loop small bowel obstruction that required surgical intervention.