Volume 3 - Issue 8
1Consultant, Emergency Medicine, Kauvery Hospital, Electronic City, Bengaluru, India
2Resident, Emergency Medicine, Kauvery Hospital, Electronic City, Bengaluru, India
Superior mesenteric artery (SMA) syndrome (known as Wilkie's syndrome) is a rare cause of upper gastrointestinal obstruction. It is an acquired disorder in which acute angulation of the SMA causes compression of the third part of the duodenum between the SMA and the aorta. This is commonly due to the loss of fatty tissue as a result of a variety of debilitating conditions. We report a 22-year-old male who presented with intermittent abdominal pain and intractable vomiting following significant weight loss. Symptoms persisted for 5 to 6years and the patient underwent extensive invasive and non-invasive tests, but to no avail. This case emphasizes the challenges in the diagnosis of SMA syndrome and the need for increased awareness of this entity. This shall facilitate early recognition and avoid inappropriate tests and unnecessary treatments.
Keywords: Wilkie's syndrome, Superior mesenteric artery syndrome, Intestinal obstruction, Weight loss.
Superior mesenteric artery (SMA) syndrome, also called Wilkie's syndrome or cast syndrome is a rare disorder in which acute angulation of the SMA causes compression of the third part of the duodenum between the SMA and the aorta, leading to obstruction. SMA syndrome is an atypical cause of proximal intestinal obstruction, most frequently occurring in young patients who have had a significant weight loss. Surgeries for spinal deformities as well as high insertion of the ligament of Treitz are other potential causes for the occurrence of SMA syndrome [1,2]. Loss of retroperitoneal fatty tissue as a result of this variety of conditions is believed to be the etiologic factor causing acute angulation. Symptoms vary from postprandial nausea and bilious vomiting to abdominal pain as well as weight loss and can occur acutely or chronically . The severity of the symptoms largely depends on the degree of the compression as reflected by the aortomesenteric angle. SMA syndrome was first described by the Austrian professor Carl Freiherr von Rokitansky in 1861 as an autopsy finding . Later, Wilkie provided a more detailed clinical and pathophysiologic description of a series of 64 patients and suggested treatment approaches . After that, a controversy regarding the actual existence of this syndrome started, especially because of the lack of specificity of the symptoms and the long list of differential diagnoses. However, advances in imaging, such as computed tomography (CT) and magnetic resonance imaging, have tremendously helped to get a clear visualization of the angle between the aorta and the SMA and thus improved the diagnostic rate . In adults, clinical SMA syndrome manifestations appear if the angle drops below 20Â°, and it is believed that values of this angle may be lower for pediatric patients . Thus, in the appropriate clinical context, detailed history, as well as imaging findings, should raise the clinical suspicion of the diagnosis of SMA syndrome. A delay in this diagnosis can potentially lead to many complications, such as electrolyte imbalance, catabolic wasting, peritonitis, and gastric perforation. Conservative therapy mainly consists of weight gain achieved orally or parenterally, with the aim of restituting the mesenteric fat pad and increasing the aortomesenteric angle . If this non-invasive approach fails, surgical therapy may be the next approach, with duodenojejunostomy being the currently preferred treatment . In this paper, we describe a case of SMA syndrome in a young male with characteristic symptoms of duodenal obstruction, whose diagnosis was delayed for 6 years. That led to multiple office and emergency room visits, along with a poor quality of life until the diagnosis was made. Identification of this underestimated syndrome can be a diagnostic dilemma and is frequently delayed. We present this case to heighten the awareness of this syndrome and the need for early management to prevent delays in diagnosis and serious complications.
A 22-year-old gentleman presented to the ER with complaints of multiple episodes of vomiting immediately after food intake, bilious in nature, not blood-stained associated with a retrosternal burning sensation since the previous night.
He appeared to be a thin-built person weighing 50 kg.
A detailed history revealed similar recurrent episodes for the past 6 years, along with anorexia nervosa of restrictive subtype which contributed to the significant weight loss of around 7 kg over the past three months.
The occurrence of such episodes was found to be provoked by mental stress.
A quick assessment was made. Clinically he had severe epigastric tenderness and bowel sounds were heard. His vitals were stable. Gastric decompression was done with 16Fr. nasogastric tube and managed symptomatically with analgesics, antiemetics, proton pump inhibitors, and IV fluids. ABG revealed increased lactates. Complete blood count, liver function test, serum amylase, serum lipase, serum electrolytes, and serum creatinine were sent. Leukocytosis with left shift was evident and antibiotics started. Ultrasound of abdomen and pelvis revealed no abnormality.
Upper GI endoscopy (Fig. 1): duodeno-gastric bile reflux, scaffolding/dilated D2 and D3; suspected distal small bowel pathology/ SMA syndrome.
On further evaluation: CECT -abdomen (Figs 2-4) revealed:
Fig. 2. Superior mesenteric artery origin-level of mid body of L1.
Aorto-mesenteric angle is narrow measuring 16 degrees.
Aorto-mesenteric distance is short measuring 5mm at level of L2.
The left renal vein appears compressed in the cranial aorto-mesenteric recess.
Duodenum (D3) cross the midline at level of lower end plate of L2 (cranial than usual)
Surgical gastroenterology consult was obtained. The patient got admitted for further management.
He was subjectively feeling better, the nasogastric tube was removed, and started on clear liquids. He was counselled for surgical management. The patient refused surgical management and opted for conservative management.
The patient was discharged in stable condition and advised to take small frequent meals throughout the day, do postural therapy, and to gain weight.
We feel that SMA syndrome is underdiagnosed. A high index of clinical suspicion is of utmost importance, especially in patients with severe weight loss and symptoms of gastric distension. Surgical stress should as well be considered a trigger factor of SMA syndrome. Interdisciplinary teamwork provides the most beneficial diagnostic and therapeutic result in this often-underestimated disease. Lastly, we would like to point out the difficulty of achieving an accurate diagnosis of SMA syndrome in an atypical clinical setting similar to what happened in our case. Heightened awareness is advised for early recognition to avoid unnecessary suffering for the patient