|Year : 2019 | Volume
| Issue : 4 | Page : 140-143
Management of superior mesenteric artery syndrome: Two paths to go by
Nitin Paul Ambrose, Pranay Gaikwad, Srujan Lam Sharma
Department of Surgery Unit – 1, Christian Medical College, Vellore, Tamil Nadu, India
|Date of Submission||18-Oct-2019|
|Date of Decision||04-Nov-2019|
|Date of Acceptance||13-Nov-2019|
|Date of Web Publication||11-Dec-2019|
Dr. Pranay Gaikwad
Department of Surgery Unit – 1, Christian Medical College, Vellore - 632 004, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Superior mesenteric artery (SMA) syndrome is a rare gastrointestinal disorder characterized by vascular compression of the third part of the duodenum in the angle between the aorta and the SMA, causing partial or complete duodenal obstruction. Symptoms include postprandial vomiting, epigastric pain, and weight loss. Conservative management is the initial treatment; however, in case of failure of conservative management, surgical therapy is unavoidable. We report two cases that were diagnosed to have SMA syndrome. Both the patients were managed differently with successful outcomes. This short case series reviews the literature on the various aspects of SMA syndrome.
Keywords: Cast syndrome, duodenal ileus, duodenojejunostomy, superior mesenteric artery syndrome
|How to cite this article:|
Ambrose NP, Gaikwad P, Sharma SL. Management of superior mesenteric artery syndrome: Two paths to go by. Curr Med Issues 2019;17:140-3
| Introduction|| |
Acute angulation of the superior mesenteric artery (SMA) and the aorta causing obstruction of the third part of the duodenum has been described as the SMA syndrome. This compression of the anterior duodenal wall by the SMA was first described by Carl Von Rokitansky in 1842. Wilkie, 1927, described the clinical and pathophysiology of a superior mesenteric syndrome in a series of 75 patients and suggested treatment approaches. The SMA syndrome is known by several names – arteriomesenteric duodenal compression, chronic duodenal ileus, and cast syndrome while the incidence of the syndrome ranges between 0.103% and 0.78%., This unsuspected condition remains undiagnosed for long durations due to vague abdominal complaints. In this brief report, we discuss the important clinical aspects and two different approaches through which two cases of SMA syndrome were successfully managed.
| A Short Case Series|| |
We present a short case series of two patients diagnosed to have SMA syndrome who were managed differently.
A 21-year-old female presented with complaints of loss of appetite and loss of 16 kg body weight over a period of 1 ½ years. In addition, she complained of dull aching abdominal pain in the epigastric and periumbilical regions with postprandial aggravation and bilious vomiting but no radiation. An empirical antitubercular treatment elsewhere failed to improve her symptoms.
Blood investigations revealed hypokalemia and metabolic alkalosis. Electrolyte abnormalities were corrected. Chest and abdominal radiographs were normal. The contrast computed tomography (CT) scan of the abdomen showed markedly dilated stomach and duodenum with abrupt narrowing in the third part of the duodenum as it travels under SMA. The aortomesenteric distance (AMD) measured 4 mm and the aortomesenteric angle (AMA) was 14° [Figure 1]a and [Figure 1]b. She was diagnosed to have SMA syndrome and underwent endoscopic nasojejunal (NJ) tube placement. After 3 months of enteral feeding, she was asymptomatic, and a repeat CT scan of the abdomen showed an improvement in the AMA to 38° and AMD to 11 mm. The NJ tube was removed. At 4 months, the body mass index (BMI) was 17.7. She remains asymptomatic, tolerating normal diet with a stable BMI above 17 at 1-year follow-up.
|Figure 1: Sagittal section contrast-enhanced computed tomography of the abdomen showing narrow aortomesenteric angle – 14° (a) and axial contrast-enhanced computed tomography of the abdomen section image showing decreased aortomesenteric distance – 4 mm (b)|
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An 18-year-old male presented with the complaints of dull aching pain in the periumbilical region and epigastric region with multiple episodes of postprandial fullness and nonbilious vomiting for 2 weeks. There was weight loss despite a normal appetite. On examination, he was emaciated with normal vital parameters. The abdominal examination was unremarkable. Hemogram and biochemical investigations were within normal limits. On imaging, the plain radiograph of the abdomen showed a grossly dilated stomach and duodenum. Barium meal revealed dilated proximal duodenum with an abrupt cutoff at the mid-third (D3) segment of the duodenum with delayed passage of small amounts of contrast distally. The contrast CT scan of the abdomen showed grossly distended stomach and the first and second parts of the duodenum with abrupt narrowing and beaking of the third part at the level of the SMA. The AMD was 6 mm and the angle was about 22°. He was diagnosed to have SMA syndrome.
Endoscopically, an NJ tube was placed for the administration of enteral nutrition for the next 4 months during which period he gained 5 Kg of body weight. A repeat CT scan of the abdomen at 4 months continued to show dilated duodenum up to the middle of its third part. The AMD was 17 mm and the AMA was about 33°. At this juncture, he was initiated on oral feeds which he tolerated for 1 month, but he developed a relapse of the original symptoms. The repeat CT scan of the abdomen showed persistent dilatation of the stomach and proximal duodenum. These findings were confirmed the failure of the conservative management to indicate a surgical option of treatment. He underwent duodenojejunostomy while the NJ tube was retained in its position distal to the anastomotic site.
Postoperatively, the patient continued to have high output through his nasogastric tube associated with 2–3 episodes of bilious vomiting but otherwise stable. On the 10th postoperative day, a barium meal follow-through was done to ensure the integrity of the anastomosis and to exclude gastroparesis resulting in a high nasogastric (NG) aspirates. The patient developed the surgical wound dehiscence that necessitated an emergency laparotomy. Intraoperatively, the duodenojejunostomy was found to be healthy and patent with the naso-jejunal (NJ) tube in place. After the second surgery, his recovery was uneventful. At discharge, the patient was tolerating a normal diet. Two months following his surgery, he was asymptomatic, tolerating a normal diet, and his BMI was 17.54.
The comparative characteristics of both cases are presented in [Table 1].
|Table 1: Summary of clinical features, treatment, and outcomes of each patient|
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| Discussion|| |
Low AMA resulting in vascular compression of the third part of the duodenum causes the SMA syndrome. The normal angle between the SMA and the aorta is between 38° and 56°, attributed to the erect posture of humans, while the AMD is between 10 and 28 mm.,,, Factors that decrease the intervening space around the duodenum result in external compression of the duodenum., In the SMA syndrome, the AMA is <38° and the AMD is <10 mm. Wilkie's syndrome is highly associated with the individuals having the BMI below the 3rd percentile.
The congenital causes of SMA syndrome include the following: a short mesenteric root, duodenal malrotation, a short or a high insertion of the ligament of Treitz at the duodenojejunal flexure that can lead to the elevation of the duodenum cranially into the vascular angle, a congenitally low origin of the SMA, peritoneal adhesions, and compression by Ladd's bands.,,
The acquired causes of SMA syndrome are: (a) severe debilitating illnesses or chronic wasting diseases secondary to cachexia; (b) trauma including polytrauma patients in the intensive care unit, extensive burn injury, and brain and spinal cord injury; (c) dietary disorders: causing severe weight loss leading to the depletion of the fatty cushion around the SMA, malabsorption syndrome, anorexia nervosa; (d) postoperative states such as bariatric surgery, esophagectomy, aortic aneurysm repair, spinal instrumentation, scoliosis with kyphosis causing severe trunk collapse, vertebral fractures, external compression by a body cast (Cast syndrome), proctocolectomy and ileoanal pouch anastomosis due to tension and caudal pull of the small bowel mesentery, and Nissen fundoplication; and (e) other conditions: increased lumbar lordosis as in pregnancy and hyperthyroidism.,,
The presenting complaints include postprandial pain and dyspeptic symptoms, persistent vomiting of large quantities, and hypochloremic hypokalemic metabolic alkalosis. Symptoms are relieved by vomiting, crouching forward. Hayes maneuver – cephalad pressure – applied below the umbilicus in a dorsal direction, helps relieve tension on the small bowel mesentery by elevating it.,
On examination, asthenic habitus is seen in 80% of the patients. Epigastric fullness with tenderness and succussion splash may be noted.,
Due to its insidious presentation, the diagnosis of SMA syndrome can be challenging. Radiographic criteria to diagnose SMA syndrome by Hines include (a) dilatation of the first and second part of the duodenum, with or without gastric dilatation, (b) abrupt vertical and oblique compression of the mucosal folds, (c) antiperistaltic flow of contrast medium (barium) proximal to obstruction, producing to and fro movements, (d) delay of 4–6 h in gastroduodenojejunal transit time, and (e) relief of the obstruction when the patient is placed in a prone, knee-chest, or left lateral positions. Contrast-enhanced CT or magnetic resonance angiography has been very helpful in enabling the visualization of the vascular compression of the duodenum and in precise measurement of the AMD and angle. An endoscopic examination may reveal a pulsatile extrinsic compression, suggesting SMA syndrome.,
Conservative management traditionally consisted of measures such as gastric decompression, parenteral nutrition, and/or postpyloric feeding when possible followed by oral diet as tolerated. There has been no defined time limit for medical management. If conservative management fails, surgery may be considered. Indications for surgical intervention as described by Berner and Sherman 1963 are: (a) failed conservative treatment, (b) long-standing disease with progressive weight loss and duodenal dilatation with stasis, (c) complicated peptic ulcer disease and pancreatitis secondary to biliary stasis and reflux, (d) some other local pathologies requiring laparotomy, and (e) patient preference for the surgical correction rather than prolonged conservative management. Duodenojejunostomy with a success rate of up to 90% is the operation of choice to relieve the obstruction. Strong's procedure, which involves the lysis of the ligament of Treitz with the mobilization of the duodenum, is a less invasive procedure described to relieve obstruction. However, the failure rate of Strong's procedure was 25%. Gastrojejunostomy has been abandoned due to its adverse sequelae., Other surgical procedures of historical interest are anterior transposition of the third part of the duodenum, Billroth II gastrectomy, transposition of the SMA to the infrarenal aorta, and duodenal circular drainage.
The two patients discussed in this short case series were managed differently based on their clinical profile and outcome. Through this article, the authors would like to highlight these.
| Conclusion|| |
SMA syndrome is often underdiagnosed, and a high index of suspicion is crucial for the early recognition of the condition, institution of appropriate conservative measures, and timely selection of a definite surgical method to prevent the development of severe complications.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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