Wilkie syndrome
Wilkie Syndrome Wilkie Syndrome, also known as Superior Mesenteric Artery Syndrome (SMAS), is a rare gastrointestinal disorder characterized by the compression of the third part of the duodenum between the abdominal aorta and the superior mesenteric artery (SMA). This condition leads to partial or complete blockage of the duodenum, causing a variety of gastrointestinal symptoms.
Etiology[edit | edit source]
Wilkie Syndrome is primarily caused by a decrease in the angle between the abdominal aorta and the superior mesenteric artery, which normally ranges from 38 to 56 degrees. When this angle is reduced to 6 to 25 degrees, the duodenum can become compressed. Factors contributing to this narrowing include rapid weight loss, anatomical abnormalities, and certain surgical procedures.
Symptoms[edit | edit source]
The symptoms of Wilkie Syndrome can vary in severity and may include:
- Nausea and vomiting
- Abdominal pain, particularly after meals
- Early satiety
- Weight loss
- Bloating and fullness
Diagnosis[edit | edit source]
Diagnosing Wilkie Syndrome can be challenging due to its rarity and the nonspecific nature of its symptoms. Diagnostic methods include:
- Barium X-ray: Shows the compression of the duodenum.
- CT Scan: Provides detailed images of the SMA and aorta, confirming the reduced angle.
- Upper Gastrointestinal Series: Helps visualize the obstruction.
Treatment[edit | edit source]
Treatment options for Wilkie Syndrome focus on relieving the duodenal compression and may include:
- Nutritional Support: High-calorie diets or enteral feeding to promote weight gain.
- Postural Therapy: Encouraging patients to lie in a prone position to relieve compression.
- Surgical Intervention: In severe cases, procedures such as duodenojejunostomy or gastrojejunostomy may be performed to bypass the obstruction.
Prognosis[edit | edit source]
The prognosis for individuals with Wilkie Syndrome varies. With appropriate treatment, many patients experience significant improvement in symptoms. However, some may require long-term nutritional support or surgical intervention.
See Also[edit | edit source]
External Links[edit | edit source]
NIH genetic and rare disease info[edit source]
Wilkie syndrome is a rare disease.
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Contributors: Prab R. Tumpati, MD
