Superior mesenteric artery syndrome

Superior mesenteric artery (SMA) syndrome, also known as Wilkie syndrome, is a rare acquired vascular compression disorder in which acute angulation of superior mesenteric artery (SMA) results in compression of the third part of the duodenum leading to obstruction.

It should not be confused with nutcracker syndrome, also a superior mesenteric artery compression disorder, where the SMA compresses the left renal vein, although some authors use the terms interchangeably.

It is an uncommon but well-recognised clinical entity. About 400 cases have been described in the English literature. Is seen more commonly in females than in males, and usually occurs in older children and adolescents.

Patients with SMA syndrome may present acutely, with chronic insidious symptomatology, or with an acute exacerbation of chronic symptoms:

  • acute presentation is usually characterised by signs and symptoms of duodenal obstruction
  • chronic cases may present with long-standing vague abdominal symptoms, early satiety and anorexia, or recurrent episodes of abdominal pain, associated with vomiting

Fat and lymphatic tissues around the SMA provide protection to the duodenum against compression. Under conditions of severe weight loss, this cushion around the SMA is diminished, causing angulation and reduction in the distance between the aorta and the superior mesenteric artery. This is usually associated with conditions causing significant weight loss such as:

  • anorexia nervosa
  • malabsorption
  • hypercatabolic states (burns, major surgery, malignancy)
  • severe congestive heart failure causing cachexia

Other conditions may also precipitate this syndrome:

  • increased spinal lordosis
  • application of a body cast
  • short ligament of Treitz
  • unusually low origin of SMA

The diagnosis of SMA syndrome is based on clinical symptoms and radiologic evidence of obstruction.

Plain radiograph
  • dilated, fluid and gas-filled stomach and proximal duodenum
Fluoroscopy

Upper GI fluoroscopy can demonstrate dilatation of the first and second part of the duodenum, extrinsic compression of the third part, and a collapsed small bowel distal to the crossing of the SMA.

CT/MRI

CT and magnetic resonance angiography (CTA/MRA) enable visualisation of vascular compression of the duodenum and measurement of aortomesenteric distance:

  • normally, the aortomesenteric angle and aortomesenteric distance are 25-60° and 10-28 mm, respectively
  • in SMA syndrome, both parameters are reduced, with values of 6° to 15° and 2 to 8 mm

The SMA syndrome was first described by Carl von Rokitansky in 1861. Later, Wilkie provided a more detailed clinical and pathophysiologic description in a series of 64 patients and suggested treatment approaches.

Traditionally, treatment has consisted of conservative measures such as:

  • nasogastric decompression and hyperalimentation followed by oral feeding with small frequent meals
  • posturing manoeuvres during meals and motility agents may be helpful in some patients

Surgery may be considered if conservative treatment fails:

  • duodenojejunostomy is effective in the majority of patients
  • laparoscopic duodenojejunostomy offers a new minimally invasive therapeutic approach to SMA syndrome
  • laparoscopic surgery involving lysis of the ligament of Treitz with the mobilisation of the duodenum is another minimally invasive approach
Share Article

Article Information

rID: 11142
Section: Syndromes
Tags: refs, syndrome
Synonyms or Alternate Spellings:
  • SMA syndrome
  • Wilkie's phenomenon
  • Wilkie syndrome
  • Wilkie's Syndrome

Support Radiopaedia and see fewer ads

  • Drag
    Distended stomach...
    Case 1
    Drag here to reorder.
  • Drag
    Compression of th...
    Case 2
    Drag here to reorder.
  • Drag
    Case 3
    Drag here to reorder.
  • Drag
    Case 4
    Drag here to reorder.
  • Drag
     Case 5
    Drag here to reorder.
  • Drag
    Case 6
    Drag here to reorder.
  • Drag
    Case 7
    Drag here to reorder.
  • Drag
    Case 8
    Drag here to reorder.
  • Drag
    Case 9
    Drag here to reorder.
  • Updating… Please wait.
    Loadinganimation

    Alert accept

    Error Unable to process the form. Check for errors and try again.

    Alert accept Thank you for updating your details.