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An elderly man with chest pain, shortness of breath, and constipation

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Q1: What do the chest radiographs show?

The upright posteroanterior and lateral films (see p 180) show a marked elevation of the right hemidiphragm with distended loops of bowel interposed between the liver and right abdominal wall. Haustration identifies the large bowel, distinguishing colonic hepatodiaphragmatic interposition from subphrenic pneumoperitoneum or abscess. The lung fields and pleural spaces are clear. Note the normal heart size and median sternotomy wires. Interestingly, the hepatodiaphragmatic interposition of the right colon had not been seen on films taken five and seven years previously, but persisted after complete resolution of the symptoms and was still present on follow up two weeks later. The computed tomogram confirmed the hepatodiaphragmatic interposition of the colon and did not show any signs of pulmonary embolism.

Q2: What important physical sign may have been missed?

Absent liver dullness may be a useful diagnostic clue pointing to hepatodiaphragmatic interposition.

Q3: What is the differential diagnosis?

The differential diagnosis must include a number of cardiac and non-cardiac causes of chest pain and breathlessness. The initial approach would be to rule out life threatening causes such as myocardial ischaemia and pulmonary embolism as well as acute pneumonia. In this patient the diagnostic difficulty was compounded by the history of coronary artery disease and high risk of pulmonary embolism (postoperative state and malignancy). The pain occurred on walking (characteristic for angina), was intensified by inspiration (mimicking pleuritic pain typical for pulmonary infarction), and was associated with shortness of breath and hypoxia. However, the pain lasted from 30 minutes to two hours, was not associated with new ECG changes nor elevation of cardiac enzymes, it responded to bed rest (but not to nitrates), and a computed tomographic pulmonary angiogram revealed no embolism. Absence of fever, productive cough, sputum, and the chest radiograph findings tend to exclude pneumonia. The patient became asymptomatic when constipation was successfully treated with enemas, stool softeners, and laxatives. However, to verify that colonic hepatodiaphragmatic interposition is the only cause of angina-like pain, other investigations may be needed to exclude coronary ischaemia.

Q4: What were the predisposing factors to this condition?

Colonic elongation due to longstanding constipation and probably adhesions (after prior stomach surgery) in an elderly patient (after hip fracture) with limited mobility taking a narcotic analgesic.

Q5: What is the management of this condition?

Most patients are treated conservatively with bed rest, increased fluid intake, fibre supplementation, laxatives, and enemas. In rare complicated cases (volvulus, internal hernias, intestinal obstruction, subdiaphragmatic appendicitis) appropriate surgical intervention is required. In our patient, who was treated conservatively, the symptoms completely disappeared after he opened his bowels with no recurrence or complications on follow up (for two months) since treatment of constipation started.

Discussion

A temporary or permanent hepatodiaphragmatic interposition of the colon, small intestine (rare), or stomach (exceptionally rare) is an uncommon and usually asymptomatic process. However, it can cause serious complications and be a potential source of misdiagnosis for a variety of intrathoracic and intra-abdominal disorders.

The condition was named after Viennese radiologist Demetrius Chilaiditi who in 1911 reported three asymptomatic cases with temporary hepatodiaphragmatic interposition of the colon and described their anatomoradiographic aspects. The condition was first described by Cantini in 1865, and Beclere in 1899 presented the necropsy and roentgenological findings in a patient thought to have a subdiaphragmatic abscess.1,2 It has been proposed to use the term “Chilaiditi’s sign” in asymptomatic patients and the term “Chilaiditi’s syndrome” in symptomatic patients.3,4

In Western countries the condition is uncommon and is found in 0.02% to 0.2% of routine chest radiographs with a male to female ratio of 4:1.1,5,6 In some reports the incidence is even lower with only 0.002% (one of 50 000 adults7) or 0.000003% (three of 11 378 0008). Importantly, an increase in prevalence of hepatodiaphragmatic interposition has been recorded in patients above 65 years of age: from 0.02% to 0.2% in men and from 0.006% to 0.02% in women.5 In one study the prevalence in the geriatric population was found to be 1%.9 In 135 persons with learning disabilities in New York, the incidence was 8.8%, or 63 times that in general population.1 A high incidence of hepatodiaphragmatic interposition was observed in Iran: 0.22% in the normal population, 2% in women near term pregnancy, 2.7% in patients with chronic lung disease, and 22% in patients with postnecrotic cirrhosis.10

Box 2: Learning points

  • Intestinal hepatodiaphragmatic interposition is a rare condition recognisable by chest radiography and is most often asymptomatic (Chiliaditi’s sign).

  • When symptomatic (Chilaiditi’s syndrome) the interposition may present with a variety of clinical symptoms and signs, mainly gastrointestinal, but also with chest pain and dyspnoea.

  • Certain groups are predisposed to this condition (elderly persons with constipation, the intellectually disabled, patients with chronic lung disease, emphysema, cirrhosis, and pregnant women).

  • In elderly patients the differential diagnosis of chest pain and respiratory distress should include Chilaiditi’s syndrome among other gastrointestinal disorders.

  • The treatment is usually conservative (bed rest, increased fluid and fibre intake, laxatives, enemas), although rarely surgical intervention is needed (volvulus, obstruction).

The normal anatomy and physiology of the intestine, liver, and diaphragm usually prevent hepatodiaphragmatic interposition. The pathophysiology is thought to be multifactorial and includes an enlarged subphrenic space, congenital and/or acquired elongation, malrotation or malfixation of the intestine with increased mobility, laxity of the hepatic suspensory ligaments, reduction of liver volume, and weakness (hyper-relaxation) of the diaphragm (due to defective innervation, either centrally or peripherally mediated).1 Chilaiditi emphasised hepatic mobility as the primary pathophysiological cause of hepatodiaphragmatic interposition, while others postulated that a redundant colon with increased mobility is a prerequisite for development of this condition.1 Chronic constipation, meteorism, aerophagia, adhesions, and mechanical obstructions are considered as important factors.3,4,8 It is worth noting that chronic constipation is the most common cause of colonic elongation and redundancy, leading to increased colonic mobility.3 Predisposing factors to intestinal hepatodiaphragmatic interposition are shown in box 1.

Box 1: Factors predisposing to intestinal hepatodiaphragmatic interposition

  1. Anatomical

    • Congenital elongation, malrotation, or malfixation of the bowel.

    • Redundant bowel with a long mesentery.

    • Congenital or acquired laxity of hepatic suspensory ligaments.

    • Reduction of liver volume (lobar agenesis, atrophic cirrhosis).

    • Lower thoracic outlet enlargement with high abdominothoracic pressure gradient (pregnancy, obstructive airway disease, emphysema, scoliosis, ascites).

    • Adhesions and mechanical obstruction.

    • Obesity.

  2. Functional

    • Increased intestinal mobility.

    • Longstanding constipation (due to immobilisation, diet, medications, etc).

    • Gaseous distention of the intestine (meteorism).

    • Diaphragm paralysis (centrally mediated or due to phrenic nerve injury).

    • Aerophagia.

There are different anatomic types of intestinal hepatodiaphragmatic interposition. Most frequently it occurs under the diaphragm anterior and superior to the right lobe of the liver (as in the described case). Hepatodiaphragmatic interposition in the posterior subphrenic space is much rarer. A case of combined anterior and posterior types of colon displacement has been reported.11

Only a minority of patients with intestinal hepatodiaphragmatic interposition have symptoms. These range from non-specific gastrointestinal symptoms such as nausea, anorexia, vomiting, flatulence, and constipation to signs of pseudo-obstruction and rarely to life threatening complications like volvulus or intestinal obstruction. The condition may rarely present with central chest pain, cardiac arrhythmias, or respiratory distress.7 Symptoms probably occur only when the intestine is distended or obstructed. Because the accumulated gas naturally ascends the patients usually become symptomatic in a sitting position or standing upright while bed rest diminishes the symptoms.1 Our case is unusual in that the patient developed uncommon symptoms of chest pain and breathlessness (without gastrointestinal symptoms apart from constipation). After complete resolution of clinical symptoms, in many cases the radiological picture of intestinal hepatodiaphragmatic interposition remained unchanged (as in our patient), indicating the importance of colonic distention in the pathophysiology of the syndrome.

A wide range of coexisting disorders has been reported including hiatus hernia, skeletal abnormalities (spinal scoliosis), multiple congenital anomalies, obesity, pneumatosis cystoides intestinalis, melanosis coli, and lung cancer. Severe complications requiring surgery such as sigmoid volvulus, incarceration of colon, and suprahepatic appendicitis have been reported.5–7,12,13

On physical examination a marked decrease or even absent liver dullness and/or a “mass” in the right upper quadrant or mid-abdomen (displaced liver) may be diagnostically useful.

Radiogically three signs are characteristic: (1) colon or small bowel interposed between the liver and the diaphragm (in symptomatic patients usually markedly distended), (2) elevated right hemidiaphragm, and (3) caudal and medial displacement of liver. However, when the bowel gas is lateral and posterior it may not get above the liver or displace it; this is termed incomplete hepatodiaphragmatic interposition.14

The differential diagnosis of radiographic findings include subdiaphragmatic abscess, pneumoperitoneum, cysts in pneumatosis intestinalis, hepatomegaly, posterior hepatic lesions, and retroperitoneal masses. In the first two of these conditions, which are associated with elevation of the right hemidiaphragm and subdiaphragmatic air collection, the haustral markings (usually best seen on lateral films) are absent. With pneumoperitoneum the free air is shifting (usually obvious in the lateral decubitus view) and may be bilateral. In subdiaphragmatic abscess, the air-fluid level is smaller and often associated with basal atelectasis and pleural effusions. Hepatodiaphragmatic interposition of the intestine may also be diagnosed with abdominal ultrasound.15 If doubt remains, contrast enema, thoracoabdominal computed tomography or nuclear scintigraphy are recommended.

Chilaiditi’s syndrome may present with a wide range of symptoms and signs which could be misleading to the attending clinician. The entity may mimic a number of cardiac, respiratory, and other non-cardiac disorders. The clinical differential diagnosis may be particularly difficult in elderly persons because of the frequent coexistence of two or more conditions contributing to the clinical picture (as in the described case). Although in patients with chest pain, the differential diagnosis initially must include myocardial ischaemia, pulmonary embolism, aortic dissection or pericarditis, other types of non-cardiac chest pain should also be considered. Constipation and colonic distention as a cause of praecordial pain,16–18 hypoxia, and respiratory distress19,20 has been described but infrequently diagnosed. Constipation which is common in elderly people affecting a third of elderly women and a quarter of elderly men,21 is a significant predisposing factor for intestinal hepatodiaphragmatic interposition.

This case emphasises the importance of considering Chilaiditi’s syndrome in differential diagnosis of chest pain and dyspnoea, especially in elderly people, as well as in patients with intellectual disability, chronic lung disease, or cirrhosis.

Final diagnosis

Colonic hepatodiaphragmatic interposition (Chilaiditi’s syndrome).

References

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