A Case of Wilkie's Syndrome: Consideration of Alternative Diagnosis in the Setting of Chronic Abdominal Pain.
Aventura Hospital and Medical Center
close loop referral, family medicine, graduate medical education, patient safety, primary care, rare disease, rural health, rural medicine, sma syndrome, wilkie's syndrome
Diagnosis | Digestive System Diseases | Family Medicine | Gastroenterology
Superior mesenteric artery syndrome (SMAS), also known as Wilkie's syndrome, is an exceedingly rare condition concerning intestinal obstruction. SMAS occurs when the space between the superior mesenteric artery (SMA) and the abdominal aorta narrows, resulting in compression of the duodenum. Functionally, the SMA supplies the distal duodenum, two-thirds of the transverse colon, and the pancreas. The location of the SMA is at about the level of the first lumbar vertebra branching off the anterior portion of the abdominal aorta. Generally, SMAS is due to rapid, excessive weight loss, resulting in the loss of the duodenal fat pad. The loss of the fat pad consequently changes the angle between the abdominal aorta and the SMA, or aortomesenteric angle, causing intestinal obstruction. Typical symptoms of acute cases of SMAS include postprandial abdominal pain, nausea, and vomiting; however, chronic cases may present with vague gastrointestinal symptoms and further weight loss. Herein, we discuss the case of a woman with chronic abdominal pain and previous substantial weight loss in whom we note features consistent with SMAS. Several factors can contribute to the syndrome, but, most commonly, it is observed after sudden, significant weight loss accompanied by nonspecific symptoms such as postprandial epigastric pain, emesis, and anorexia. Given that there is continued debate whether the syndrome even exists, SMAS is usually a diagnosis of exclusion, if diagnosed at all. First-line treatment involves conservative management, but if symptoms become too severe, several proven surgical methods are available. SMAS is a rare condition and is difficult to diagnose, but it should be suspected if clinical manifestations are present. This case illustrates the need for primary care physicians to receive additional training on the recognition of rare diseases to broaden their differentials. Training of this sort is especially crucial for rural family medicine residency programs focused on producing full-spectrum physicians.
Publisher or Conference
Adame JD, Falconi G, Harberger S. A Case of Wilkie's Syndrome: Consideration of Alternative Diagnosis in the Setting of Chronic Abdominal Pain. Cureus. 2019;11(11):e6074. Published 2019 Nov 5. doi:10.7759/cureus.6074