Median arcuate ligament syndrome | |
---|---|
Other names | Celiac artery compression syndrome Celiac axis syndrome Celiac trunk compression syndrome Dunbar syndrome |
Median arcuate ligament syndrome results from compression of the celiac artery by the median arcuate ligament. The median arcuate ligament is a fibrous arch formed by the left and right diaphragmatic crura, visible here on the underside of the diaphragm. | |
Specialty | Gastroenterology, Vascular Surgery |
Symptoms | Epigastric pain, anorexia, Weight loss |
Complications | Gastroparesis Aneurysm of the superior and inferior pancreaticoduodenal arteries |
Usual onset | 20 to 40 years of age |
Causes | Compression of the celiac artery from the median arcuate ligament |
Risk factors | Female gender |
Treatment | Surgery |
In medicine, the median arcuate ligament syndrome (MALS, also known as celiac artery compression syndrome, celiac axis syndrome, celiac trunk compression syndrome or Dunbar syndrome) is a rare [1] condition characterized by abdominal pain attributed to compression of the celiac artery and the celiac ganglia by the median arcuate ligament. [2] The abdominal pain may be related to meals, may be accompanied by weight loss, and may be associated with an abdominal bruit heard by a clinician.
The diagnosis of MALS is one of exclusion, as many healthy patients demonstrate some degree of celiac artery compression in the absence of symptoms. Consequently, a diagnosis of MALS is typically only entertained after more common conditions have been ruled out. Once suspected, screening for MALS can be done with ultrasonography and confirmed with computed tomography (CT) or magnetic resonance (MR) angiography.
Treatment is generally surgical, the mainstay being open or laparoscopic division, or separation, of the median arcuate ligament combined with removal of the celiac ganglia. The majority of patients benefit from surgical intervention. Poorer responses to treatment tend to occur in patients of older age, those with a psychiatric condition or who use alcohol, have abdominal pain unrelated to meals, or who have not experienced weight loss.
Patients with MALS reportedly experience abdominal pain, particularly in the epigastrium, which may be associated with eating and which may result in anorexia and weight loss. The pain can be in the left or right side, but usually where the ribs meet. [2] Other signs are persistent nausea, lassitude (especially after a heavy meal) and exercise intolerance. Diarrhea is a common symptom, some experience constipation. While some experience vomiting, not everyone does. Exercise or certain postures can aggravate the symptoms. Occasionally, physical examination reveals an abdominal bruit in the mid-epigastrium. [2]
Complications of MALS result from chronic compression of the celiac artery. They include gastroparesis [3] and aneurysm of the superior and inferior pancreaticoduodenal arteries. [4]
The median arcuate ligament is a ligament formed at the base of the diaphragm where the left and right diaphragmatic crura join near the 12th thoracic vertebra. This fibrous arch forms the anterior aspect of the aortic hiatus, through which the aorta, thoracic duct, and azygos vein pass. The median arcuate ligament usually comes into contact with the aorta above the branch point of the celiac artery. However, in up to one quarter of normal individuals, the median arcuate ligament passes in front of the celiac artery, compressing the celiac artery and nearby structures such as the celiac ganglia. [2] In some of these individuals, this compression is pathologic and leads to the median arcuate ligament syndrome. [2]
Several theories attempt to explain the origin of pain caused by compression of the celiac artery. [5] One proposes that compression of the celiac artery causes ischemia, or decreased blood flow, to abdominal organs, leading to pain. Another hypothesizes that there is compression not only of the celiac artery but also of the celiac ganglia, and that pain results from compression of the latter.[ citation needed ]
Median arcuate ligament syndrome is a diagnosis of exclusion. [2] [5] That is, the diagnosis of MALS is generally considered only after patients have undergone an extensive evaluation of their gastrointestinal tract including upper endoscopy, colonoscopy, and evaluation for gallbladder disease and gastroesophageal reflux disease (GERD). [5]
The diagnosis of MALS relies on a combination of clinical features and findings on medical imaging. [2] Clinical features include those signs and symptoms mentioned above; classically, MALS involves a triad of abdominal pain after eating, weight loss, and an abdominal bruit, although the classic triad is found in only a minority of individuals that carry a MALS diagnosis. [5]
Diagnostic imaging for MALS is divided into screening and confirmatory tests. [5] A reasonable screening test for patients with suspected MALS is duplex ultrasonography to measure blood flow through the celiac artery. [5] [6] Peak systolic velocities greater than 200 cm/s are suggestive of celiac artery stenosis associated with MALS. [5]
|
Further evaluation and confirmation can be obtained via angiography to investigate the anatomy of the celiac artery. [5] Historically, conventional angiography was used, although this has been largely replaced by less invasive techniques such as computed tomography (CT) and magnetic resonance (MR) angiography. [2] [5] Because it provides better visualization of intra-abdominal structures, CT angiography is preferred to MR angiography in this setting. [5] The findings of focal narrowing of the proximal celiac artery with poststenotic dilatation, indentation on the superior aspect of the celiac artery, and a hook-shaped contour of the celiac artery support a diagnosis of MALS. [2] These imaging features are exaggerated on expiration, even in normal asymptomatic individuals without the syndrome. [2]
Proximal celiac artery stenosis with poststenotic dilatation can be seen in other conditions affecting the celiac artery. [2] The hook-shaped contour of the celiac artery is characteristic of the anatomy in MALS and helps distinguish it from other causes of celiac artery stenosis such as atherosclerosis. [2] This hooked contour is not entirely specific for MALS however, given that 10–24% of normal asymptomatic individuals have this anatomy. [2]
Decompression of the celiac artery is the general approach to treatment of MALS. [5] The mainstay of treatment involves open or laparoscopic surgery approaches to divide, or separate, the median arcuate ligament to relieve the compression of the celiac artery. [5] This is combined with removal of the celiac ganglia and evaluation of blood flow through the celiac artery, for example by intraoperative duplex ultrasound. If blood flow is poor, celiac artery revascularization is usually attempted; methods of revascularization include aortoceliac bypass, patch angioplasty, and others. [5]
In recent, a laparoscopic approach used to achieve celiac artery decompression; [7] however, should the celiac artery require revascularization, the procedure would require conversion to an open approach. [5]
Endovascular methods such as percutaneous transluminal angioplasty (PTA) have been used in patients who have failed open and/or laparoscopic intervention. [5] PTA alone, without decompression of the celiac artery, may not be of benefit. [5] [8]
There are few studies of the long-term outcomes of patients treated for MALS. [5] According to Duncan, [5] the largest and more relevant late outcomes data come from a study of 51 patients who underwent open surgical treatment for MALS, 44 of whom were available for long-term follow-up at an average of nine years following therapy. [9] The investigators reported that among patients who underwent celiac artery decompression and revascularization, 75% remained asymptomatic at follow-up. In this study, predictors of favorable outcome included:[ citation needed ]
It is estimated that in 10–24% of normal, asymptomatic individuals the median arcuate ligament crosses in front of (anterior to) the celiac artery, causing some degree of compression. [2] [10] Approximately 1% of these individuals exhibit severe compression associated with symptoms of MALS. [2] The syndrome most commonly affects individuals between 20 and 40 years old, and is more common in women, particularly thin women. [2]
Celiac artery compression was first observed by Benjamin Lipshutz in 1917. [11] MALS was first described by Pekka-Tapani Harjola in 1963 [12] and subsequently by J. David Dunbar and Samuel Marable in 1965. [13] It has also been called Harjola-Marable syndrome and Marable syndrome. [11]
Angioplasty, also known as balloon angioplasty and percutaneous transluminal angioplasty (PTA), is a minimally invasive endovascular procedure used to widen narrowed or obstructed arteries or veins, typically to treat arterial atherosclerosis.
A hiatal hernia or hiatus hernia is a type of hernia in which abdominal organs slip through the diaphragm into the middle compartment of the chest. This may result in gastroesophageal reflux disease (GERD) or laryngopharyngeal reflux (LPR) with symptoms such as a taste of acid in the back of the mouth or heartburn. Other symptoms may include trouble swallowing and chest pains. Complications may include iron deficiency anemia, volvulus, or bowel obstruction.
Interventional radiology (IR) is a medical specialty that performs various minimally-invasive procedures using medical imaging guidance, such as x-ray fluoroscopy, computed tomography, magnetic resonance imaging, or ultrasound. IR performs both diagnostic and therapeutic procedures through very small incisions or body orifices. Diagnostic IR procedures are those intended to help make a diagnosis or guide further medical treatment, and include image-guided biopsy of a tumor or injection of an imaging contrast agent into a hollow structure, such as a blood vessel or a duct. By contrast, therapeutic IR procedures provide direct treatment—they include catheter-based medicine delivery, medical device placement, and angioplasty of narrowed structures.
Vascular surgery is a surgical subspecialty in which vascular diseases involving the arteries, veins, or lymphatic vessels, are managed by medical therapy, minimally-invasive catheter procedures and surgical reconstruction. The specialty evolved from general and cardiovascular surgery where it refined the management of just the vessels, no longer treating the heart or other organs. Modern vascular surgery includes open surgery techniques, endovascular techniques and medical management of vascular diseases - unlike the parent specialities. The vascular surgeon is trained in the diagnosis and management of diseases affecting all parts of the vascular system excluding the coronaries and intracranial vasculature. Vascular surgeons also are called to assist other physicians to carry out surgery near vessels, or to salvage vascular injuries that include hemorrhage control, dissection, occlusion or simply for safe exposure of vascular structures.
In medicine, aortoiliac occlusive disease is a form of central artery disease involving the blockage of the abdominal aorta as it transitions into the common iliac arteries.
The celiacartery, also known as the celiac trunk or truncus coeliacus, is the first major branch of the abdominal aorta. It is about 1.25 cm in length. Branching from the aorta at thoracic vertebra 12 (T12) in humans, it is one of three anterior/ midline branches of the abdominal aorta.
Bruit, also called vascular murmur, is the abnormal sound generated by turbulent flow of blood in an artery due to either an area of partial obstruction or a localized high rate of blood flow through an unobstructed artery.
Meralgia paresthetica or meralgia paraesthetica is pain or abnormal sensations in the outer thigh not caused by injury to the thigh, but by injury to a nerve which provides sensation to the lateral thigh.
Fibromuscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory disease of the blood vessels that causes abnormal growth within the wall of an artery. FMD has been found in nearly every arterial bed in the body, although the most commonly affected are the renal and carotid arteries.
The nutcracker syndrome (NCS) results most commonly from the compression of the left renal vein (LRV) between the abdominal aorta (AA) and superior mesenteric artery (SMA), although other variants exist. The name derives from the fact that, in the sagittal plane and/or transverse plane, the SMA and AA appear to be a nutcracker crushing a nut.
The median arcuate ligament is a ligament under the diaphragm that connects the right and left crura of diaphragm.
The popliteal artery entrapment syndrome (PAES) is an uncommon pathology that occurs when the popliteal artery is compressed by the surrounding popliteal fossa myofascial structures. This results in claudication and chronic leg ischemia. This condition mainly occurs more in young athletes than in the elderlies. Elderlies, who present with similar symptoms, are more likely to be diagnosed with peripheral artery disease with associated atherosclerosis. Patients with PAES mainly present with intermittent feet and calf pain associated with exercises and relieved with rest. PAES can be diagnosed with a combination of medical history, physical examination, and advanced imaging modalities such as duplex ultrasound, computer tomography, or magnetic resonance angiography. Management can range from non-intervention to open surgical decompression with a generally good prognosis. Complications of untreated PAES can include stenotic artery degeneration, complete popliteal artery occlusion, distal arterial thromboembolism, or even formation of an aneurysm.
Eagle syndrome is an uncommon condition commonly characterized but not limited to sudden, sharp nerve-like pain in the jaw bone and joint, back of the throat, and base of the tongue, triggered by swallowing, moving the jaw, or turning the neck. First described by American otorhinolaryngologist Watt Weems Eagle in 1937, the condition is caused by an elongated or misshapen styloid process and/or calcification of the stylohyoid ligament, either of which interferes with the functioning of neighboring regions in the body, such as the glossopharyngeal nerve.
Intestinal ischemia is a medical condition in which injury to the large or small intestine occurs due to not enough blood supply. It can come on suddenly, known as acute intestinal ischemia, or gradually, known as chronic intestinal ischemia. The acute form of the disease often presents with sudden severe abdominal pain and is associated with a high risk of death. The chronic form typically presents more gradually with abdominal pain after eating, unintentional weight loss, vomiting, and fear of eating.
Superior mesenteric artery (SMA) syndrome is a gastro-vascular disorder in which the third and final portion of the duodenum is compressed between the abdominal aorta (AA) and the overlying superior mesenteric artery. This rare, potentially life-threatening syndrome is typically caused by an angle of 6–25° between the AA and the SMA, in comparison to the normal range of 38–56°, due to a lack of retroperitoneal and visceral fat. In addition, the aortomesenteric distance is 2–8 millimeters, as opposed to the typical 10–20. However, a narrow SMA angle alone is not enough to make a diagnosis, because patients with a low BMI, most notably children, have been known to have a narrow SMA angle with no symptoms of SMA syndrome.
Ovarian vein syndrome is a rare condition in which a dilated ovarian vein compresses the ureter. This causes chronic or colicky abdominal pain, back pain and/or pelvic pain. The pain can worsen on lying down or between ovulation and menstruation. There can also be an increased tendency towards urinary tract infection or pyelonephritis. The right ovarian vein is most commonly involved, although the disease can be left-sided or affect both sides. It is currently classified as a form of pelvic congestion syndrome.
Anterior interosseous syndrome is a medical condition in which damage to the anterior interosseous nerve (AIN), a distal motor and sensory branch of the median nerve, classically with severe weakness of the pincer movement of the thumb and index finger, and can cause transient pain in the wrist.
Nerve compression syndrome, or compression neuropathy, or nerve entrapment syndrome, is a medical condition caused by chronic, direct pressure on a peripheral nerve. It is known colloquially as a trapped nerve, though this may also refer to nerve root compression. Its symptoms include pain, tingling, numbness and muscle weakness. The symptoms affect just one particular part of the body, depending on which nerve is affected. The diagnosis is largely clinical and can be confirmed with diagnostic nerve blocks. Occasionally imaging and electrophysiology studies aid in the diagnosis. Timely diagnosis is important as untreated chronic nerve compression may cause permanent damage. A surgical nerve decompression can relieve pressure on the nerve but cannot always reverse the physiological changes that occurred before treatment. Nerve injury by a single episode of physical trauma is in one sense an acute compression neuropathy but is not usually included under this heading, as chronic compression takes a unique pathophysiological course.
Fraley syndrome is a condition where the superior infundibulum of the upper calyx of the kidney is obstructed by the crossing renal artery branch, causing distension and dilatation of the calyx and presenting clinically as haematuria and nephralgia. Furthermore, when the renal artery obstructs the proximal collecting system, filling defects can occur anywhere in the calyces, pelvis, or ureter.
Isolated superior mesenteric artery dissection (ISMAD) is a rare but potentially life-threatening condition that causes acute abdominal pain. It refers to a dissection that occurs solely in the superior mesenteric artery (SMA), typically spontaneously, and does not involve the aorta. Although aortic dissection can frequently extend into its peripheral territories, it is rare for these branches to have dissection without main aortic trunk involvement. The SMA is the most common site of dissection among visceral arteries compared to other gastrointestinal arteries.