Chronic mesenteric ischaemia due to median arcuate ligament syndrome

Clinical History

49-year-old male patient without a specific medical history, presents with a history of severe recurrent abdominal pain for 15 years, sometimes associated with vomiting, resulting in progressive precariousness and social isolation. The patient had many emergency consultations and explorations since 2002: 5 CT, 1 MRI, 1 coloscopy and 1 gastroscopy with biopsies.

Imaging Findings

CT findings:
Coeliac trunk ostial stenosis of 50% with poststenotic dilatation. The superior mesenteric artery is normal. (Fig. 1). Dilated duodeno-pancreatic arcades (Fig. 2).

Arteriography findings:
- Sagittal view (aortography): stenosis of the first 2 cm of the coeliac trunk followed by a dilatation, and the superior mesenteric artery is normal. (Fig. 3)
- Coronal view (arteriography of SMA): dilatation of the duodeno-pancreatic arcades, with a reverse flow towards the common hepatic artery and the splenic artery. (Fig. 4).

Discussion

For 15 years no diagnosis was made on any clinical or imaging findings except for the last CT reporting a 50 % stenosis at the origin of the coeliac trunk due to a median arcuate ligament. This finding was not mentioned in the report conclusion.
This patient became dependent on morphine, consulted several psychiatrists, lost his job and progressively isolated himself from society.
Eventually a general practitioner sent him to our interventional radiology unity to “exclude a median arcuate ligament syndrome” on the base of the CT findings.
The median arcuate ligament is a fibrous arch that unites the diaphragmatic crura on either side of the aortic hiatus. In some people, this ligament inserts low and can cause superior compression of the proximal portion of the coeliac trunk [1].
The angiography shows that with the decrease of pressure in the coeliac trunk due to the stenosis, the duodeno-pancreatic arcades developed and provided perfusion backward to the hepatic and splenic arteries.
With a second look at the CTs, especially at the coronal MIP view, we could see the dilated duodeno-pancreatic arcades. These natural anastomoses between the coeliac trunk and the superior mesenteric artery are usually not or sparsely viewed on an angio-CT. So these findings should have strongly suggested this diagnosis, from the CT in 2009 (8 years ago), when a good quality angio-CT was made with an arterial time that could undergo MIP in a coronal view.
Symptoms are explained by a vascular steal syndrome. Blood of the superior mesenteric artery goes for the coeliac trunk territory (via the duodeno-pancreatic arcades), resulting in a decreased arterial flow in the superior mesenteric territory, causing a chronic mesenteric ischaemia [2].
The treatment of this condition is the surgical section of the median arcuate ligament.
Chronic abdominal pain can be of many origins and surgery is often unnecessary, nevertheless when an arcuate ligament syndrome is present, prognosis factors linked to the resolutions of symptoms after surgery are: age between 40 and 60, post-prandial pain, significant weight loss and a coeliac trunk stenosis due to a arcuate ligament with post-stenotic dilatation and collateral vessels present on imaging studies [1].
An interesting feature of this condition is that the stenosis of the coeliac trunk is predominant in expirium [1], and CT examinations are usually made in inspirium, that could explain the lack of sensitivity of this pathology on CT examination.

Differential Diagnosis List

Final Diagnosis

Chronic mesenteric ischaemia due to median arcuate ligament syndrome.

URL:	https://eurorad.org/case/14599
DOI:	10.1594/EURORAD/CASE.14599
ISSN:	1563-4086