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Superior mesenteric artery syndrome (SMAS), also known as Wilkie's syndrome, is a rare cause of acute abdominal pain. The diagnosis of SMAS is often delayed and misdiagnosed. To report the early diagnosis of a SMAS in emergency department(ED) and review the diagnostic strategies of this rare disease.
A 19-year-old thin female patient was admitted to ED with postprandial epigastric pain accompanied by intractable vomiting lasting for 3 days. Tracing back her history, the patient denied any significant systemic disease. A general physical examination revealed epigastric tenderness without peritoneal irritation signs, other systems were normal. Her white blood cell count was 11,520/mm3, granulocyte proportion was 84.9%, K 3.4 mmol/L, other blood tests were normal. Abdominal erect and supine radiograph, abdominal ultrasound, upper gastrointestinal endoscopy also were normal. The abdominal computed tomographic angiography(CTA) was initially reported as normal. Antibiotic therapy, esomeprazole and anisodamine were administered intravenously, her abdominal pain and vomiting were recurrent after eating. On second review of the images of CTA with an experienced emergency clinicians, The angle of the SMA in relation to the abdominal aorta was narrower compared with normal. After clinical suspicion and radiological findings, diagnosis of SMAS was achieved after upper gastroenterography, which was demonstrated dilation of the proximal duodenum with abrupt cut-off at its third part.
Electrolyte imbalance was corrected and nutritional support was provided for 5 days. Her symptoms were gradually improved and discharged. The patient was followed up after 9 months, She improved with no further admission with the same complaints and was pregnancy now.
SMAS is a very rare cause of the upper gastrointestinal system obstruction but worth to remember disease. The pathophysiology entails the loss of the fat pad between the superior mesenteric artery and the abdominal aorta., which leads to decrease in Aortomesenteric angle and the third part of the duodenum is compressed. The symptoms of SMAS are usually nonspecific and that easy to make an original misdiagnosis and mismanaged. Both CTA and upper gastroenterography have been used to diagnose SMAS. The early accurate diagnosis is based on experience with increased awareness of SMAS. In this patient, We do not accept radiology reports at face value due to high degree of suspicion of SMAS, As a second opinion from a more experienced clinician make early diagnosis. Once diagnosed, conservative treatment with nutritional support and positioning should be tried first, surgery may be considered when conservative treatment fails as well for patients with recurrent symptoms. In conclusion, We suggest that CTA and upper gastroenterography should be early done leading to diagnose SMAS in thin patients with recurrent postprandial epigastric pain incorporating intractable vomiting.