CASE 12372 Published on 28.01.2015

Wilkie syndrome or superior mesenteric artery syndrome


Abdominal imaging

Case Type

Clinical Cases


Lesyk Sonia, Nissen Nicolas, Palau Guillermo

Sanatorio de la Trinidad San Isidro
Diagnostico por Imagenes;
Fondo de la Legua 851
Buenos Aires San Isidro, Argentina;

15 years, female

Area of Interest Abdomen ; Imaging Technique Digital radiography, CT-High Resolution
Clinical History
A 15-year-old female patient of slight build presented with abdominal pain for the past 15 days.
Gynaecological and abdominal ultrasound did not produce any findings. Her Body Mass Index was 18.2.
An Upper GI series with barium was performed, showing no findings of mucosal lesions. However, a persistent vertical filling defect area was seen in the third part of the duodenum.
Imaging Findings
Her physician requested an Upper GI series, which showed a compression at the third part of the duodenum of approximately 10 mm in size that later regained its usual diameter. A light distension of the second part of the duodenum could also be seen.
These findings suggested an aorto-mesenteric compass, and an abdominal CT examination with contrast medium was suggested.
An angio CT was performed, using a GE Light speed 64 channel scan with non-ionic contrast.
Sagittal view shows the origin of the superior mesenteric artery from the aorta with a 15.6° angle. A 3D reconstruction is also shown.
Axial view shows the crossing point of the mesenteric artery and the third part of the duodenum, with a 6 cm distance between them.
Fig. 4 shows an example of a CT examination with a normal angle at the aorta-mesenteric bifurcation (72°).
Duodenum, renal vein and fat tissue are also seen.
In 1842, Rokitansky was the first to describe the physiopathogenesis, and therefore this disorder is also termed Rokitansky syndrome. In 1907 Bloodgood suggested its surgical resolution, which was accomplished by Stavely in 1908 with a duodenojejunal anastomosis. In 1921 Wilkie published 75 cases. [1, 2]
It has a very low incidence, prevailing in young women. The predisposing factors for the decrease of the aorto-mesenteric angle are weight loss, food disorders, trauma, surgeries (correction of scoliosis, etc), congenital anatomic anomalies, local pathology (aneurysms, neoproliferative processes). [3]
The anatomy of the third part of the duodenum must be known. It is surrounded by perimesenteric fat tissue, between the aorta and the superior mesenteric artery, which originates from the aorta at L1-L2 level, with a 20-50° angle in normal patients.
This angle comprises the renal vein, the pancreatic uncinate process, the third portion of the duodenum and the retroperitoneal fat tissue. [4, 5] An angle between 6° and 16° can cause duodenal compression.
The normal distance between the aorta and the superior mesenteric artery at the duodenal crossing point is 13 to 34 cm, and 5 to 11 cm in patients with SMAS.
Differential Diagnosis List
Wilkie syndrome or superior mesenteric artery syndrome
Duodenal obstruction
Wilkie syndrome
Final Diagnosis
Wilkie syndrome or superior mesenteric artery syndrome
Case information
DOI: 10.1594/EURORAD/CASE.12372
ISSN: 1563-4086