(Cast syndrome, Wilkie syndrome)

Rare disease (prevalence 0,01 to 0,3 %) that may be encountered in the multi-handicapped child or adolescent, but also in case of anorexia nervosa or following a scoliosis treatment (brace or surgery). A few cases have been described in newborns: the clinical presentation is then similar to congenital duodenal stenosis.

It results from the extrinsic compression of the third part of the duodenum between the proximal superior mesenteric artery (or one of its main branches) and the abdominal aorta (and spine), this produces signs of intermittent high intestinal obstruction.

The clinical picture is chronic high intestinal occlusion with loss of appetite, nausea, bilious vomiting, abdominal distention and pain. The contributing factors are weight loss, rapid growth, spastic quadriparesis, a major scoliosis or an exaggerated lumbar lordosis and immobilization in a spinal brace. The symptoms are improved if the patient is placed in the left lateral decubitus, the prone or the genupectoral position after the meals. The diagnosis is confirmed by ultrasound or CTScan (which allows measuring the angle formed by the origin of the superior mesenteric artery and the aorta (or one of its branches): normally this angle varies between 38° and 56° at adult age; it is pathologic if < 25°) and opacification shows dilation of the first two parts of the duodenum.

Conservative medical treatment: gastric aspiration with enteral feeding by a jejunal  catheter pushed beyond the obstacle; a parenteral nutrition to correct malnutrition is often associated with  left lateral decubitus positioning. If all fails, a laparotomy or laparoscopy is performed to perform either a duodenal derotation by freeing the Treitz's ligament or a gastro- or a duodeno-jejunostomy.

Occasionally: presence of gas in the portal vein or even portal thrombosis if the pressure upstream of the  site of compression is very important (in case, for example, of fundoplication that prevents vomiting).

Anesthetic implications:

full stomach situation, malnutrition, polyhandicapped patient

References : 

• Delgadillo X, Belpaire-Dethiou MC, Chantrain C, Clapuyt P, Veyckemans F, de Ville de Goyet J, Otte JB, Reding R. 
  Arteriomesenteric syndrome as a cause of duodenal obstruction in children with cerebral palsy. 
  J Pediatr Surg 1997; 32:1721-3.
• Rod J, Sarnacki S, Petit T, Ravasse P. 
  Portal venous gas and thrombosis complicating superior mesenteric artery syndrome (Wilkie’s syndrome) in a child. 
  J Pediatr Surg 2010; 45: 826-9
• Mathenge N, Osiro S, Rodriguez II, Salib C, Tubbs RS, Loukas M.
  Superior mesenteric artery syndrome and its associated gastrointestinal implications.
  Clin Anat 2014 ; 27 : 1244-52.

• Rioualen S, Lavenant P, Delpeut J, Tissot V et al.
  Syndrome de l’artère mésentérique supérieure ou syndrome de Wilkie.
  Arch Pédiatr 2016 ; 23 :769-71 
• Kawawakia T, Sakaia S, Kubotab Y, Tania M.
  Superior mesenteric artery syndrome in a neonate: a case report.
  J Pediatr Surgery Case Reports 2024 ; 104 : 102806, doi.org/10.1016/j.epsc.2024.102806

Updated: June 2024