Vascular compression syndromes

Wilkie syndrome

Also known as superior mesenteric artery syndrome, it is compression of the third part of the duodenum between the aorta and the superior mesenteric artery. It is uncommon, usually appears after significant weight loss and can coexist with Nutcracker syndrome, because both are caused by the same arterial “pincer”.

Why does it happen?

Normally, a pad of fat keeps the superior mesenteric artery apart from the aorta. When this fat disappears, the angle between the two arteries closes and the duodenum becomes pinched. The most common factors are:

• Significant weight loss (more than 10 kg), due to diets, illness, eating disorders or after bariatric surgery.
• Serious or debilitating illness, burns or trauma.
• Scoliosis correction surgery.
• A short ligament of Treitz, which holds the duodenum in a higher position than normal.

Symptoms

• Pain in the upper abdomen after eating.
• Early fullness and very slow digestion.
• Nausea and vomiting, sometimes of bile.
• Weight loss, which in turn worsens the compression and creates a vicious circle.
• Relief when lying on the left side, face down or in the knee-to-chest position.

Diagnosis

More common digestive causes must be ruled out first. Investigation is based on CT or MR angiography, which shows an abrupt cut-off in the third part of the duodenum with dilatation above it and allows the arterial pincer to be measured: an aortomesenteric angle of less than 25° and a distance of less than 8 mm support the diagnosis. A barium meal helps to confirm it.

As the same pincer can compress the left renal vein, these patients should also be assessed for possible Nutcracker syndrome, especially if there is left flank pain, blood in the urine or pelvic varicose veins.

Treatment

I treat it in coordination with gastroenterology, nutrition and general surgery.

• Conservative treatment: this is the first step. Dehydration and electrolyte imbalances are corrected and weight is regained through nutrition, preferably via the digestive tract, so that the fat pad can re-form.
• Surgery: if conservative treatment fails, the reference technique is duodenojejunostomy, which bypasses the duodenum in front of the compression, nowadays usually by keyhole surgery. Strong’s procedure, which repositions the duodenum, fails in about a quarter of cases.
• If Nutcracker syndrome is also present, it is assessed together which compression explains the symptoms and in what order each should be treated.

Frequently asked questions

Is Wilkie syndrome a vascular disease?

The compression is caused by an artery, but the organ affected is the duodenum. That is why it is managed by several specialists, and the vascular surgeon assesses the arterial pincer and any associated compression of the renal vein.

Can it be cured by regaining weight?

In many cases it improves with weight gain, because the fat separating the two arteries re-forms. When this is not enough, surgery is used.

How is it related to Nutcracker syndrome?

Both are caused by the same pincer between the aorta and the superior mesenteric artery: in Wilkie syndrome the duodenum is compressed and in Nutcracker syndrome the left renal vein. They can occur together.

References

Patient information reviewed by Dr Óscar Merino, vascular surgeon. It does not replace an individual medical assessment.