What is the management of Wilkie's syndrome (superior mesenteric artery syndrome)?

Management of Wilkie's Syndrome (Superior Mesenteric Artery Syndrome)

The initial management of Wilkie's syndrome should be conservative with nutritional support, followed by surgical intervention only if conservative measures fail. 1, 2

Understanding Wilkie's Syndrome

• Wilkie's syndrome (superior mesenteric artery syndrome) is a rare condition characterized by compression of the third portion of the duodenum between the superior mesenteric artery (SMA) and the aorta 1
• It predominantly affects females with a 3:2 ratio over males, with a median age of 23 years (range 0-91 years) 1
• Common symptoms include postprandial abdominal pain, nausea, vomiting, early satiety, anorexia, and weight loss 1, 2
• The condition can mimic anorexia nervosa or functional dyspepsia, making diagnosis challenging 1

Diagnostic Approach

• Computed tomography angiography (CTA) is the first-line diagnostic tool with approximately 94% sensitivity and 95% specificity 3
• Diagnostic findings include:
  • Decreased SMA angle (normally 38-65°)
  • Shortened aortic-mesenteric distance
  • Narrowing of the third portion of the duodenum 4
• Ultrasonography offers advantages in safety and capability for real-time assessment of SMA mobility and duodenal passage 1

Management Algorithm

First-Line: Conservative Management (70-80% success rate) 1

1. Nutritional Support:

   • Small, frequent meals in the left lateral decubitus or prone position 2
   • Nasojejunal tube feeding beyond the obstruction 1
   • Jejunostomy tube feeding for severe cases to regain weight 5
   • Total parenteral nutrition may be necessary in severe cases 2

2. Supportive Measures:

   • Gastroduodenal decompression to relieve symptoms 1
   • Postural changes to relieve duodenal compression 1
   • Fluid resuscitation to enhance visceral perfusion 3

Second-Line: Surgical Intervention (80-100% success rate) 1

When conservative management fails after 4-6 weeks, surgical options include:

1. Laparoscopic duodenojejunostomy - preferred surgical approach 1
2. Roux-en-Y duodenojejunostomy - alternative reconstruction 6
3. Strong's procedure - division of the ligament of Treitz with mobilization of the duodenum 2
4. Infrarenal transposition of the SMA - novel approach for selected cases 6

Special Considerations

• Rapid weight loss is a major risk factor for developing Wilkie's syndrome 2
• The condition is also associated with surgical correction of spinal deformities 2
• Early diagnosis is crucial as recurrent vomiting can lead to aspiration pneumonia or respiratory depression via metabolic alkalosis 1
• Patients may present with both Wilkie's syndrome and nutcracker syndrome (compression of the left renal vein) 5

Pitfalls to Avoid

• Misdiagnosis as anorexia nervosa or functional dyspepsia can delay appropriate treatment 1
• Underdiagnosis may put patients at risk for serious complications 4
• Failure to address the underlying cause (usually weight loss) may lead to persistent symptoms 2
• Surgical intervention should not be the first-line approach unless there are signs of peritonitis or bowel infarction 3