Superior mesenteric artery syndrome is a gastro-vascular disorder in which the third and final portion of the duodenum is compressed between the stomach aorta (AA) and the artery superior mesenteric on it. This potentially life-threatening rare syndrome is usually caused by an angle of 6 Â ° -25 Â ° between AA and SMA, compared to the normal range of 38 Â ° -56 Â °, due to lack of retroperitoneal and visceral fat (mesenteric fat). In addition, the aortomesenteric distance is 2-8 millimeters, compared with a typical 10-20. However, a narrow SMA angle alone is not enough to make a diagnosis, since patients with low BMI, especially children, have been known to have a narrow SMA angle with no symptoms of SMA syndrome.
The first high school syndrome was described in 1861 by Carl Freiherr von Rokitansky on autopsy victims, but remains pathologically undefined until 1927 when Wilkie published the first comprehensive series of 75 patients. According to a 1956 study, only 0.3% of patients referred for barium study of the upper gastrointestinal tract fit this diagnosis, making it one of the rarest gastrointestinal disorders known by medical science. The recognition of SMA syndrome as a distinct controversial clinical entity, partly because of possible confusion with a number of other conditions, although it is now widely recognized.
High school syndrome is also known as Wilkie syndrome, cast syndrome, mesenteric root syndrome, chronic duodenal duodenum and intermittent arterio-mesenteric occlusion. This is different from the Nutcracker syndrome, which is the left renal vein trap between AA and SMA, although it is possible to be diagnosed with both conditions.
Video Superior mesenteric artery syndrome
Signs and symptoms
Signs and symptoms include full satiety, nausea, vomiting, extreme postprandial abdominal pain "piercing" (due to reversed duodenal and inverting peristalsis), abdominal distension/distortion, burping (eructation), external hypersensitivity or pain in the abdominal area. , reflux, and heartburn. In infants, difficulty eating and poor weight gain are also symptoms that often arise.
In some cases of high school syndrome, severe malnutrition accompanying it can occur spontaneously. This, in turn, increases duodenal compression, which worsens the underlying cause, creating a cycle of worsening symptoms.
"Fear of food" is a common development among patients with chronic form SMA syndrome. For many people, symptoms are relief in the left lateral decubitus or knee position to the chest, or in a vulnerable position (face down). Hayes maneuver (the pressure applied under the umbilicus in the cephalad and dorsal directions) elevates the roots of the SMA, also slightly lessening the constriction. Symptoms can be worsened when leaning to the right or taking a supine position (facing up).
Maps Superior mesenteric artery syndrome
Cause
Retroperitoneal fat and lymphatic tissue usually serves as a cushion for the duodenum, protecting it from compression by SMA. SMA syndrome is thus triggered by any condition that involves a less strong bearing and narrow mesenteric angle. SMA syndrome can be present in two forms: chronic/congenital or acute/induced.
Patients with severe and severe forms of chronic high school syndrome have a history of long or even long abdominal complaints with intermittent exacerbations depending on the level of duodenal compression. Risk factors include anatomical characteristics such as: aesthenic (very thin or "thin") builds up the body, very high insertion of the duodenum on Treitz ligaments, very low origin of SMA, or intestinal malrotation around the axis formed by SMA. Predisposition is easily aggravated by one of the following: poor motility of the gastrointestinal tract, retroperitional tumor, loss of appetite, malabsorption, cachexia, excessive lumbar lordosis, visceroptosis, abdominal wall flexibility, peritoneal adhesion, abdominal trauma, rapid adolescent growth acceleration, body, starvation, catabolic status (such as cancer and burns), and a history of neurological injury.
The acute form of SMA syndrome develops rapidly after traumatic incidents that forcibly extend high high school in the duodenum, induce obstruction, or sudden weight loss for any reason. Causes include prolonged sleep on your back, scoliosis surgery, left nephrectomy, ileo-anal pouch surgery.
It is important to note, however, that while SMA syndrome can mimic eating disorders, distinguishing these two conditions is very important, since misdiagnosis in this situation can be dangerous.
Diagnosis
Diagnosis is very difficult, and usually one of the exceptions. High school syndrome is considered only after the patient undergo extensive evaluation of the gastrointestinal tract including upper endoscopy, and evaluation for various conditions of malabsorptive, ulcerative and inflammatory indications with higher diagnostic frequency. The diagnosis may follow an x-ray examination revealing a duodenal dilatation followed by a sudden constriction proximal to its upper SMA, as well as a delay in transits of four to six hours through the gastroduodenal region. Standard diagnostic examinations include CT scan of abdominal and pelvic scans with oral contrast and IV, upper gastrointestinal series (UGI), and, for vague cases, hypotonic duodenography. In addition, vascular imaging such as ultrasound and angiographic contrast can be used to show an increase in blood flow velocity through SMA or narrower SMA angles.
Despite some case reports, there is controversy surrounding the diagnosis and even the existence of SMA syndrome because symptoms do not always correlate well with radiological findings, and may not always improve after surgical correction. However, the reason for the persistence of gastrointestinal symptoms even after surgical correction in some cases has been traced to the residual protruding peristaltic opposite to direct peristalsis.
Because women between the ages of 10 and 30 are most commonly affected, it is not uncommon for doctors to initially and mistakenly assume that laxness is the patient's choice rather than the consequences of high school syndrome. Patients in the early stages of SMA syndrome often remain unaware that they are sick until major damage to their health is done, as they may try to adapt to the condition by gradually reducing their food intake or being naturally attracted towards a lighter and more easily digested food.
Treatment
High school syndrome may appear in acute form, acquired (eg suddenly appearing in hospitalization after scoliosis surgery) as well as chronic forms (ie developing throughout the course of a lifetime and progressing due to environmental triggers, life changes, or other ailments). According to some recent sources, at least 70% of cases can usually be treated with medical care, while the rest require surgical treatment.
Medical treatment was first tried in many cases. In some cases, emergency surgery is required at the time of presentation. A six-week medical treatment trial is recommended in pediatric cases. The goal of medical care for high school syndrome is the resolution of basic conditions and weight. Medical treatment may involve placement of nasogastric tubes for duodenal and gastric decompression, mobilization into the lateral or left decubitus position, inversion or removal of precipitating factors with proper nutrition and fluid and electrolyte replacement, either by surgery inserted into the jejunal tube, nasogastric intubation , or peripherally inserted central catheter (PICC line) that regulates total parenteral nutrition (TPN). Pro-motility agents such as metoclopramide may also be helpful. Symptoms may improve after weight recovery, unless the reversed peristalsis persists, or if the recovered fat does not accumulate in a mesenteric angle. Most patients seem to benefit from nutritional support with hyperalimentation regardless of the history of the disease.
If medical treatment fails, or is not feasible due to severe disease, surgical intervention is required. The most common surgery for SMA syndrome, duodenojejunostomy, was first proposed in 1907 by Bloodgood. Performed as open surgery or laparoscopy, duodenojejunostomy involves the formation of anastomosis between the duodenum and jejunum, bypassing compression caused by AA and SMA. Less common surgical treatments for high school syndrome include Roux-en-Y duodenojejunostomy, gastrojejunostomy, anterior transposition of the third part of the duodenum, intestinal derotation, division of Treitz ligaments (Strong surgery), and transposition of SMA. The transposition of SMA and lysis of the duodenal suspensory muscle has the advantage that they do not involve the formation of bowel anastomosis.
The possibility of persistence of symptoms after bypass surgery can be traced to the remaining peristaltic protruding opposite to direct peristaltic, although sedimentation factor (duodenal compression) has been bypassed or relieved. Reverse peristalsis has been shown to respond to duodenal circular drainage - a complex and invasive open surgical procedure originally practiced and performed in China.
In some cases, high school syndrome can occur in conjunction with serious life-threatening conditions such as cancer or AIDS. Even in these cases, the treatment of SMA syndrome can lead to decreased symptoms and improved quality of life.
Prognosis
The delay in the diagnosis of SMA syndrome can lead to fatal catabolism (late malnutrition), dehydration, electrolyte abnormalities, hypokalaemia, acute gastric rupture or intestinal perforation (due to prolonged mesenteric ischaemia), gastric distension, spontaneous upper gastrointestinal bleeding, hypovolemic shock, and aspiration. pneumonia.
The 1-in-3 mortality rate for Superior Artery Mesenteric syndrome has been cited by a small number of sources. However, after extensive research, the original data establishing this mortality rate has not been found, suggesting that the number may not be reliable. Although studies that establish official mortality may not be present, two recent studies of high school syndrome patients, published in 2006 with 22 cases and one in 2012 with 80 cases, showed a mortality rate of 0% and 6 respectively , 3%. According to doctors in one of these studies, the expected outcomes for the treatment of SMA syndrome are generally considered very good.
Epidemiology
High school syndrome is very rare, proving only 0.3% of the upper barium gastrointestinal tract study. However, unfamiliarity with this condition in the medical community coupled with intermittent and nonspecific symptomatology may lead to underdiagnosis.
Because this syndrome involves a deficiency of essential fats, more than half of them are diagnosed with weight loss, sometimes to the point of pain and laxity. Women are more often affected than men, and while syndrome can occur at any age, it is most commonly diagnosed early in adulthood. The most common co-morbid conditions include mental and behavioral disorders including eating disorders and depression, infectious diseases including tuberculosis and acute gastroenteritis, and nervous system diseases including muscular dystrophy, Parkinson's disease, and cerebral palsy.
Society and culture
American actor, director, producer and author Christopher Reeve has an acute form of high school syndrome as a result of spinal cord injuries.
Nonprofit
In 2017, the only non-profit US began distributing grants to those suffering from SMAS. They are listed on the IRS under the name Superior Mesenteric Artery Syndrome Research, Awareness and Support, but also work under DBA SMAS Patient Assistance. Their website is www.smasyndrome.org This is their goal to help those who are not insured and under-guaranteed in the US to receive the medical help they need to treat their SMAS.
See also
References
External links
Source of the article : Wikipedia
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