Median arcuate ligament syndrome

The trunk - celiac compression syndrome, referred arcuatum as Harjola - Marable syndrome, Dunbar syndrome or ligament syndrome, manifested by abdominal pain and is by clamping the celiac artery (syn: celiac trunk ) and possibly the celiac ganglion caused by the diaphragm. The abdominal pain may be associated with food intake and be accompanied by a weight loss. On auscultation of the abdomen can be heard more often typical Stenosegeräusche.

The diagnosis of the celiac - celiac compression syndrome is a diagnosis of exclusion because many people have this form of obstruction, without, however, to develop symptoms. Therefore, the diagnosis can be made only after exclusion of other causes. For screening is used, duplex ultrasound, confirming the diagnosis by means of computed tomography ( CT) or magnetic resonance imaging ( MRI).

The treatment is surgical. The ligament is severed, thus expanding the aortic hiatus. Under certain circumstances, the ganglion is removed celiac addition. The majority of patients benefited from surgery. The success rate is lower in younger patients, patients with psychiatric disorders, patients with increased alcohol consumption, patients without weight loss and in patients who have no pain associated with meals.

Anatomy and pathogenesis

The medial arcuate ligament originates at the base of the diaphragm, where the right and left diaphragmatic crura ( crus dextrum et sinistrum ) come together at about the level of the 12th thoracic vertebra. This fabric bow is the front of the aortic hiatus, through which draw the aorta to the aortic plexus and the thoracic duct. Normally, the ligament is located above the departure of the celiac trunk, at about 25 % of people, however, the ligament crosses at the level of the disposal and thereby narrowed the artery and adjacent structures such as the celiac ganglion one. For some, the narrowing is so severe that the symptoms arise.

Various theories try the pain due to the compression explain. A suspected as the cause of the pain, the reduced blood flow (ischemia) of the supplied abdominal organs, another comes from the compression of the celiac ganglion from.

History

A compression of the celiac was first observed by Benjamin Lipshutz 1917. The trunk - celiac compression syndrome was described in 1963 by Pekka Tapani - Harjola and two years later by J. David Dunbar and Samuel Marable.

Epidemiology

Only about 1% of people in whom the ligament crosses at the level of the celiac, suffer from a trunk - celiac compression syndrome. The complaints mainly relate to patients between 20-40 years of age, mostly women, preferably slimmer physique.

Clinical picture

The persons concerned complain nausea and burning, cramping pain, which are often located in the epigastrium and not infrequently occur in connection with meals. The pain can lead to weight loss to anorexia. Occasionally, one can Stenosegeräusch epigastric auscultation. Complications arise from the compression of the artery, such as a stomach paralysis or aneurysmal enlargement of the superior pancreaticoduodenal artery, the pancreaticoduodenal inferior because of their connection through the artery to the superior mesenteric artery is used as collateral.

Diagnostics

The diagnosis of exclusion includes, but is Esophagogastroduodenoscopy duodenoscopy and colonoscopy. It also biliousness and a reflux must be ruled out. The diagnosis is based ultimately from the combination of the complaint image with the radiological diagnosis. The classic triad of abdominal pain, weight loss Stenosegeräusch is found only in a few patients. The radiological diagnosis is divided into:

• Screening: duplex ultrasound to measure blood flow in the celiac trunk. A blood flow velocity > 200 cm / s is considered suspicious
• Diagnosis: earlier was performed to confirm the diagnosis, angiography, which has now been replaced by CT angiography or MRI angiography, the CT scan is preferred due to the better representation of the adjacent abdominal organs.

The findings of a short segment narrowing of the celiac trunk at its disposal, with subsequent extension ( post- stenotic dilatation), a notch in the upper aspect of the trunk, and a hook-shaped curve of the trunk to support the diagnosis of celiac - celiac compression syndrome. This image criteria are emphasized in expiration and can be found in some cases even in asymptomatic patients do not suffer from the syndrome.

Also have other possible differential diagnoses in a disposal -related narrowing with post- stenotic dilatation, such as arteriosclerotic changes are taken into account. Here, the hook-shaped course of the celiac trunk can be helpful for the distinction, although this criterion is also not pathognomonic for celiac - celiac compression syndrome. The frequency of this anatomy normal asymptomatic individuals is indicated by 10 to 24 %.

Therapy

The decompression of the celiac is the treatment of choice .. Mostly this is done through a laparotomy with the aim to replace the ligament of the artery at the same time the ganglion is removed and celiac controls the blood flow of the liberated artery by duplex sonography. For further existing reduced blood flow revascularization through a bypass or other vascular surgical intervention may be required.

The decompression can also be done via a laparoscopic access in required Revaskulation of the celiac but must be changed to the open approach.

Endoscopic procedures such as percutaneous transluminal angioplasty (PTA) have been used in patients in which the open or laparoscopic access has not been possible, with the PTA was not successfully alone without decompression of the artery through the ligament.

Forecast

There are few studies on long-term results of treatment of patients with celiac - celiac compression syndrome. In the work of Duncan is reported on a study of 51 patients who were operated on an open access. 44 of these patients were followed up for a period of nine years. 75% of patients in which both decompression, as well as a Revaskulisation was remained symptom-free. Predictors for a good success were:

• Age between 40-60 years
• No psychiatric disorders and alcohol abstinence
• Suffered weight loss > 9 kg

Also, a recent study of 2009 is the success rate of surgical treatment of approximately 70-75 %.