Superior Mesenteric Artery Syndrome, Rare Yet Overlooked Diagnosis

Introduction: Superior mesenteric artery syndrome or Wilkie’s syndrome, an upper gastrointestinal disorder due to compression of the duodenum is rare yet a difficult diagnostic challenge. The pathophysiology entails the loss of the fat pad between the superior mesenteric artery and abdominal aorta, as a result of a variety of debilitating conditions. Case Description/Methods: 39-year-old white white female with a past medical history significant for an ischemic stroke and surgical history of hysterectomy presents with a chief complaint of localized, acute abdominal pain in the epigastric area, associated with nausea and vomiting, poor oral intake, leading to 10 lbs of weight loss, over two days before the current presentation. At admission, laboratory data were unremarkable. A right upper quadrant ultrasound ruled out biliary obstruction. However, a Computed Tomography(CT) of the abdomen and pelvis showed distended stomach and proximal duodenum concerning gastric outlet obstruction. A follow-up Upper GI series showed delayed transit at the level of the third portion of the duodenum, with a focal narrowing corresponding to findings on CT imaging which may represent SMA syndrome. An upper endoscopy was unrevealing with mild antral erythema, biopsies were unremarkable. Further imaging Magnetic Resonance angiogram, confirmed SMA syndrome described as decreased aortomesenteric angle and aortomesenteric distance between the aorta and SMA, resulting in compression of the third part of the duodenum. No surgical consultation requested, and conservative management with dietary modification and education on nutrition, patient symptoms improved gradually throughout the hospital stay. Discussion: SMA syndrome was first described by von Rokitanski in 1861. The incidence of this condition varies from 0.013-0.3% of the barium series of the upper GI tract. Normal imaging anatomy with aorto-mesenteric angle and aorto-mesenteric distance measures as 25°- 60° and 10 to 28 mm respectively. Both parameters are reduced in SMA, with values of 6° to 15° and 2 to 8 mm respectively. The diagnosis of SMAS is based mostly on clinical symptoms and radiologic evidence of obstruction by Barium studies, CT scan, and MR angiogram. Treatment options include dietary modification with an intent to gain lost weight. Medical management may be successful in some patients with a shorter duration of symptoms, however many patients need surgical correction.