Celiac artery stenosis

Definition

Celiac artery stenosis is a pathological condition in which blood flow through the celiac trunk, one of the main vessels supplying blood to the abdominal organs, is impaired. The most common cause of this condition is either atherosclerotic damage to the vessel from the inside or external compression of the trunk from the outside, known as Dunbar’s syndrome.

Dunbar syndrome is a complex of symptoms that arise from the compression of the celiac trunk and circulatory disorders in the organs of the upper abdominal cavity. Most cases of pathology are caused by an abnormal location of the arc-shaped ligament that overstresses the vessel in the place of its departure from the aorta. The syndrome is manifested by abdominal pain after eating, dyspeptic disorders, and progressive weight loss. For diagnosis, an ultrasound of the visceral arteries of the abdominal aorta, CT, and angiography are performed. Treatment is surgical: decompression of the celiactrunk, supplemented by reconstructive surgeries on the affected vessel and stenting of the celiac artery.

General information

Dunbar syndrome is named after the American surgeon J. D. Dunbar, who, in 1965, associated the symptoms of mesenteric ischemia with characteristic anatomical changes in the diaphragm. Anomalies that can cause impaired blood flow in the vessel occur in one in five people, with only 1% of patients having clinical manifestations. Dunbar syndrome is more prone to young women of asthenic build who have a narrow chest and low diaphragm position, which contributes to extravasal compression.

Causes

Most cases are caused by congenital anomalies. The arch-shaped ligament of the diaphragm is located below the place where the vessel leaves the aorta, which is why connective tissue fibers compress the artery and reduce blood flow to the abdominal organs. The syndrome may be combined with a hernia of the esophageal opening of the diaphragm. More rarely, the cause of compression is an abnormal location of diaphragmatic legs or the presence of neurofibromatous tissue in the area of the glomerular plexus.

In some patients, Dunbar syndrome develops as an acquired disease. In this case, arterial compression is caused by neoplasms and enlarged lymph nodes located near the aorta. Pathology occurs with lymphoproliferative diseases and solid tumors (pancreatic cancer). Vascular compression is possible with sclerosis of periarterial tissue caused by autoimmune pathologies or chronic inflammation.

Classification

There is no universally accepted systematization of Dunbar syndrome in vascular surgery due to its complex pathogenesis, nonspecificity, and diverse clinical course. In practice, classifications that consider the degree of blood supply disruption are most often used, as it is important for choosing treatment tactics. According to this classification, there are four stages of the disease:

• Compensation stage. It includes an asymptomatic course of the disease (Ia) without significant hemodynamic disturbances and a period of minimal symptoms (Ib), which occur only with functional overload of the gastrointestinal tract.
• Subcompensation stage. At this stage, patients suffer from signs of abdominal ischemia with overeating and abuse of high-carbohydrate and fatty foods.
• Decompensation stage. In the third stage, hemodynamic disorders persist even in the functional rest of the digestive organs; pain becomes constant and severe.
• Stage of ulcerative-necrotic changes. A complicated form of Dunbar syndrome is characterized by multiple lesions of the digestive tract’s mucous membranes due to insufficient blood circulation.

Symptoms

Dunbar syndrome is characterized by abdominal ischemia. In 80% of cases, symptomatic forms of celiac trunk compression are manifested by abdominal pain 20-25 minutes after a meal. The severity of the pain syndrome varies from insignificant, which does not really disturb the patient’s life, to intolerable, reminiscent of acute surgical pathology. Clinical manifestations vary frequently and bother a person for a long time.

The second characteristic sign is weight loss, which 48% of patients experience. Weight loss is associated with chronic dyspeptic disorders. Expecting pain, nausea, and discomfort in the abdomen, patients consciously limit their eating, refuse many products, and sharply reduce portions. Malabsorption and maldigestion syndromes, which develop against the background of poor blood circulation, also negatively contribute.

Complications

Dunbar syndrome causes 50-80% of vascular aneurysms, the rupture of which is fatal in every third patient. Prolonged ischemia reduces the level of protective factors of the gastric and duodenal mucosa and contributes to the formation of erosions. In the presence of other risk factors, such as helicobacter infection, abuse of NSAIDs, and frequent stress, peptic ulcer disease occurs.

Chronic malabsorption syndrome is a serious problem for the patient, as it can cause vitamin deficiency, electrolyte balance disorders, and iron deficiency anemia. Nutritional disorders negatively affect the condition of the skin, hair, and nails and the work of all internal organs. In women, protein-energy deficiency is fraught with secondary amenorrhea and infertility.

Diagnosis

Since nonspecific symptoms of digestive disorders manifest Dunbar syndrome, patients are first consulted by a general practitioner or gastroenterologist. After excluding typical causes of GI lesions, the patient is referred to a vascular surgeon. Compression of the celiac trunk requires a set of instrumental studies:

• Abdominal ultrasound is a screening method used to detect lesions of the liver, spleen, or other organs in the upper abdomen. In Dunbar syndrome, there are no signs of pathology, so standard echo-sonography is supplemented with ultrasound of the aorta and its visceral branches to assess blood flow parameters and identify areas of stenosis.However, an atherosclerotic lesion of the celiac artery can be found during this test.
• CT scan of the abdominal aorta. The final diagnosis of compression is made when the vessel is narrowed by 50% or more in combination with a typical clinical picture. To clarify hemodynamic features, CT is accompanied by functional respiratory tests. If indicated, the diagnosis is supplemented with an MRI of celiac primal trunk.
• Angiography. An X-ray contrast study of the abdominal aortic branches gives doctors valuable information about the exact localization and nature of the narrowing of the celiac trunk, the presence of deformation of the vascular walls, and the activity of collateral circulation. Based on the examination results, the technique of surgical intervention on the vessels is chosen.

Differential diagnosis

Nonspecific complaints require careful differentiation of Dunbar syndrome with upper digestive tract diseases: inflammatory processes (gastritis, duodenitis, pancreatitis), peptic ulcer disease, and functional dyspepsia. The prolonged existence of symptoms and significant weight loss exclude pyloric stenosis and gastric cancer. Intense pain syndrome is differentiated by ulcer perforation, biliary stones, and acute pancreatitis.

Treatment for Dunbar syndrome

Decompression and revascularization of the celiac trunk are the only ways to relieve the symptoms and complications of the disease. Surgical intervention involves the dissection of diaphragmatic ligaments and is often supplemented by ganglionectomy to remove the source of constant irritation of the solar plexus and prevent vasoconstriction in the future. The surgery is performed laparoscopically or openly.

Patients with a long history of the disease and degenerative changes in the vascular wall are indicated for reconstructive surgery. This procedure involves replacing the affected arterial sections or bypassing the affected vessel. Such surgery improves the long-term treatment results, reduces the recurrence rate, and normalizes the parameters of blood flow in the visceral vessels of the abdominal cavity.

In the presence of atherosclerotic lesions of the celiac trunk, stenting of the affected area is possible.

All these treatment options are available in more than 420 hospitals worldwide (https://doctor.global/results/diseases/celiac-artery-stenosis). For example, Angioplasty and stenting of visceral arteriescan be performed in 19 clinics across Turkey for an approximate price of $6.5 K(https://doctor.global/results/asia/turkey/all-cities/all-specializations/procedures/angioplasty-and-stenting-of-visceral-arteries-celiac-trunk-superior-mesenteric-artery). 

Prognosis and prophylactics

Dunbar syndrome can be successfully treated surgically, but it often takes several years from the development of the first symptoms to diagnosis, so the risk of complications is extremely high. The long-term prognosis depends on the cause of vessel compression and the volume of surgical intervention. There are no primary preventive measures. In the postoperative period, the patient is under the surgeon’s dispensary supervision and undergoes standard rehabilitation.