|Year : 2012 | Volume
| Issue : 1 | Page : 49-51
Wilkie's syndrome: A case report with review of literature
B Ananda Rama Rao
Department of Surgery, SVS Medical College, Mahabubnagar, Andhra Pradesh, India
|Date of Web Publication||21-Mar-2012|
B Ananda Rama Rao
Professor of Surgery, Department of Surgery, SVS Medical College, Mahabubnagar, Andhra Pradesh
Wilkie's syndrome, well known as superior mesenteric artery syndrome, is a rare but well-recognized clinical entity causing upper abdominal symptoms. A 26-year-old woman presented with recurrent upper abdominal pain, vomiting, loss of weight, early satiety, and fullness of upper abdomen for 2 years. She was subjected to open appendicectomy and laparoscopic adhesionolysis within 20 months without relief. Clinical suspicion and thorough investigation revealed compression at transverse portion (third part) of duodenum. Though ideally a duodenojejunostomy is preferred, due to technical reasons an anterior gastrojejunostomy was performed, which resulted in relief of obstructive symptoms.
Keywords: Superior mesenteric artery syndrome, duodenal obstruction, duodenojejunostomy, gastrojejunostomy
|How to cite this article:|
Rama Rao B A. Wilkie's syndrome: A case report with review of literature. J Dr NTR Univ Health Sci 2012;1:49-51
| Introduction|| |
Wilkie's syndrome, well known as superior mesenteric artery (SMA) syndrome, was first described in 1861 by Carl Freiherr von Rokitansky in victims at autopsy. Wilkie published the first comprehensive series of 75 patients in 1927.  Only 0.013-0.3% of upper-gastrointestinal (GI) tract barium studies support a diagnosis. Only 500 cases are reported in world literature.
| Case Report|| |
A 26-year-old woman presented with recurrent upper abdominal pain, vomiting, loss of weight, early satiety, and fullness of upper abdomen for 2 years. The abdominal fullness and pain gets relieved after vomiting. She underwent repeated upper GI endoscopy examinations. Mild antral gastritis was noted. Conservative management with antacids and PPIs gave no relief. She was subjected to open appendicectomy in 2007 and later for laparoscopic adhesionolysis in 2009 without any relief to her suffering. She used to skip meals for fear of abdominal discomfort and pain, resulting in loss of weight.
On clinical examination, she was underweight. Fullness in the upper abdomen with visible gastric peristalsis from left to right resulted in suspicion of gastric outlet obstruction. Hematological and other biochemical investigations were within normal limits. Plain X-ray abdomen showed large fundal gas shadow but no other signs of duodenal obstruction. An ultrasonogram of the abdomen showed reduced aorto-mesenteric angle [Figure 1].
Computerized tomography with intravenous and oral contrast (CECT) of the abdomen showed dilated stomach and proximal duodenum [Figure 2]. CT angiogram with 3D reconstruction showed reduced aorto-mesenteric angle at about 10° versus normal angle of 30°-60° [Figure 3].
|Figure 3: CT angio 3D reconstruction showing proximal duodenum reduced aorto-mesenteric angle|
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With the diagnosis of SMA syndrome, duodenojejunostomy was planned. Intraoperatively, in view of narrow space and technical difficulty, we opted for anterior gastrojejunostomy and vagotomy as against recommended procedure of duodenojejunostomy [Figure 4]. Postoperative period was uneventful. In 2 years' follow-up period, the patient is asymptomatic and has gained normal weight.
| Discussion|| |
SMA syndrome is a rare, life-threatening gastrovascular disorder, characterized by a compression of the third portion of the duodenum by the abdominal aorta (AA) and the overlying SMA, typically caused by an angle of 6-25° between AA and SMA, (normal 38-56°), and aorto-mesenteric distance decreased to 2-8 mm (normal 10-20 mm) due to lack of retroperitoneal and visceral fat.  Symptoms include early satiety, nausea, bilious vomiting of large quantities of undigested food, "stabbing" postprandial abdominal pain (due to duodenal compression and compensatory reversed peristalsis), abdominal distention/distortion, eructation, tender abdomen, and severe malnutrition.  This, in turn, increases the duodenal compression, spurring a vicious cycle. "Food fear" is common with the chronic form of SMA syndrome. Symptoms are partially relieved when in the left lateral decubitus or knee-to-chest position. A Hayes maneuver (pressure applied below the umbilicus in cephalad and dorsal direction) elevates the root of the SMA, also slightly easing the constriction. Symptoms are often aggravated when leaning to the right or taking a supine position. Patients with the chronic, congenital form of SMA syndrome predominantly have a lengthy history of abdominal complaints with intermittent exacerbations depending on the degree of duodenal compression. Acute form of SMA syndrome develops rapidly after traumatic incidents that forcibly hyperextend the SMA across the duodenum, inducing the obstruction. Common co-morbid conditions include peptic ulcer disease (25-45%), pancreatitis, and scoliosis. Female preponderance is double, and common between 10 and 30 years.
Diagnosis is usually by exclusion. SMA syndrome is considered only after patients have undergone extensive evaluation of their GI tract, including upper endoscopy, colonoscopy, and evaluation for malabsorption, ulcerative, and inflammatory intestinal conditions. X-ray examination reveals duodenal dilation followed by abrupt constriction proximal to the overlying SMA and delay in transit of 4-6 h through the gastroduodenal region. Standard diagnostic tests are CECT abdomen and pelvis, upper gastrointestinal series (UGI), and, for equivocal cases, hypotonic duodenography. Vascular imaging by ultrasound and CT angiography may be used to indicate increased blood flow velocity through the SMA, to assess risk of sudden, fatal circulatory collapse. Symptoms do not always correlate well with radiologic findings, and may not always improve following surgical correction due to persistent reverse peristalsis.
Acute cases usually respond to medical management, while chronic cases require surgical intervention. In acute or mild cases, conservative treatment should be attempted.  If conservative treatment fails, or if the case is severe or chronic, surgical intervention is required. Duodenojejunostomy is the preferred surgical procedure, bypassing the compression caused by the AA and the SMA.  Other procedures are Roux-en-Y duodenojejunostomy, gastrojejunostomy, anterior transposition of the third portion of the duodenum, intestinal derotation, and division of the ligament of Treitz. Lysis of the duodenal suspensory muscle has the advantage that it does not involve the creation of an intestinal anastomosis. The world's first robotically-assisted intestinal bypass for SMA syndrome was performed on July 30, 2008, at the London Health Sciences Centre in Ontario, Canada.  Persistence of symptoms after surgical bypass is due to reversed peristalsis. Reversed peristalsis has been shown to respond to duodenal circular drainage-a complex and invasive open surgical procedure originally implemented and performed in China.
| References|| |
|1.||Welsch T, Büchler MW, Kienle P. Recalling superior mesenteric artery syndrome. Dig Surg 2007;24:149-56. |
|2.||Shetty A. Superior Mesenteric Artery Syndrome. eMedicine. WebMD. http://www.emedicine. com/ped/topic2175. htm. Retrieved 2008-04-09. |
|3.||Baltazar U, Dunn J, Floresguerra C, Schmidt L, Browder W. Superior mesenteric artery syndrome: An uncommon cause of intestinal obstruction. South Med J 2000;93:606-8. |
|4.||Yang WL, Zhang XC. Assessment of duodenal circular drainage in treatment of superior mesenteric artery syndrome. World J Gastroenterol 2008;14:303-6. |
|5.||Bloodgood JC. Acute dilatation of the stomach: Gastromesenteric ileus. Ann Surg 1907;46:736. |
|6.||World's first robotically assisted intestinal bypass surgery for SMA Syndrome, http://www.lhsc.on.ca/About_Us/CSTAR/News/SMA.htm, October 22, 2008. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]