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Q1: What is the diagnosis?
The diagnosis is coeliac band with coeliac artery stenosis causing chronic intestinal ischaemia. This is such a rare clinical condition that even institutions with an enormous experience in peripheral vascular surgery have accumulated some experience over several years. It is only by reflex consideration of this condition after excluding other common diagnosis that the alert surgeon will reach this diagnosis.
Q2: What are the differential diagnoses in the patient?
The main differential diagnoses include mesenteric angina that may be secondary to atherosclerosis, Buerger's disease, fibromuscular dysplasia, and arteritis such as Takayasu's disease. Other possibilities include renal and biliary colic.
Q3: How would you establish the diagnosis?
This patient's presentation was atypical of common abdominal conditions and the routine investigations were normal. With a history such as this, atherosclerotic disease of the coeliac/mesenteric arteries causing chronic mesenteric ischaemia was a possibility. Measuring the erythrocyte sedimentation rate would help to rule out an inflammatory cause. A duplex scan would be performed as the first step of vascular imaging as this is non-invasive.1 However because of their anatomical location, assessment of visceral arterial lesions by duplex scan is not as reliable as it is in other situations. Contrast computed tomography (fig 1) or magnetic resonance angiography of the coeliac/mesenteric arteries, provide better information including detection of any coexisting extrinsic pathology. Finally the definitive investigation is an angiogram, which in this case clearly demonstrated pathology at the origin of the coeliac artery. This was left until last due to its invasive nature and the risks, although rare, associated with it.
Q4: What do the investigations illustrate?
The angiogram demonstrates a tight stenosis (lateral film) (fig 2) at the origin of the coeliac artery. This may be due to extrinsic or intrinsic factors or a combination of both; the superior mesenteric artery (SMA) is of normal calibre. The inferior mesenteric artery (IMA) is not seen.
Q5: What are the treatment options available?
Treatment options should be based on risk versus benefit for each procedure. In this patient median arcuate ligament (coeliac band) syndrome had to be ruled out as an extrinsic cause of compression. The risk of releasing the band is much less than arterial reconstruction. At operation he was found to have a coeliac band. This was divided, however a thrill over the coeliac artery persisted. This indicated the presence of dual pathology, with stenosis of the coeliac artery being also present. In this case the stenosis was localised to the proximal portion of the coeliac artery; arterial reconstruction between the aorta and hepatic artery was seen as the best treatment option. The SMA was of normal calibre. The IMA was found to be small and could possibly have contributed to the patient's symptoms as the collateral circulation had failed to develop. A reversed long saphenous vein graft was anastomosed between the infra renal aorta and the hepatic artery and the graft was tunnelled behind the pancreas.
Aortocoeliac grafting is an option, however due to the short course of the coeliac artery, the procedure is often difficult. Another option is to reimplant the coeliac artery into the aorta at another site; this is particularly suitable if there is a tight proximal stenosis with an aortic component.2 Angioplasty with or without stenting of the mesenteric vessels is a further option, the risk being distal embolisation and dissection with acute thrombosis causing ischaemia or infarction.
When reviewed in the clinic at three months and six months the patient was completely free from symptoms and has returned back to his normal exercise programme. Contrast computed tomography performed at three months confirmed the patency of the aortohepatic graft.
It has been reported in the literature that up to 33% of the population have an anatomical variation in the structure of the coeliac axis.3 Such anomaly seldom gives rise to symptoms of chronic intestinal ischaemia. The coeliac axis, SMA, and IMA along with the iliacs provide the main blood supply to the gastrointestinal tract. Due to the presence of a good collateral circulation, stenosis usually has to be of a severe degree before symptoms occur. This can be graded by measuring the pressure gradient across the stenotic lesion. Stenoses likely to compromise the collateral circulation may become clinically manifested during the postprandial phase or during muscular exertion. It has been also demonstrated that mesenteric vascular resistance increases during exercise and produces a marked decrease in arterial inflow.4
Chronic gastrointestinal ischaemia is classically described as presenting with postprandial abdominal pain (termed visceral angina), weight loss, and an epigastric bruit (quoted in over 80% of cases). Lord et al, in their series of 12 patients with coeliac axis compression, noted that 75% (9) of their patients had atypical abdominal pain, 58% (7) of the patients had slight weight loss, and only 42% (5) had history of diarrhoea.5 The presentation in this patient was unusual in that he had no postprandial symptoms, but pain was induced by heavy exercise perhaps due to redistribution of blood flow. In the presence of coeliac artery stenosis and a small IMA the collateral blood flow to the gut through the internal iliac arteries was possibly diverted to the lower limbs during exercise. Since the SMA was patent, the patient had very little symptoms of weight loss, diarrhoea, or postprandial symptoms.
Coeliac band and coeliac artery stenosis.
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