Wilkie Syndrome and Pseudo-Nutcracker Syndrome a Rare Combination: Description of a Case

Abstract

Wilkie's syndrome is a very rare vascular alteration caused by congenital or acquired reduction of space between the superior mesenteric artery (SMA) and aorta. In acquired form, it is caused by perivascular adipose tissue reduction due to rapid weight loss and, if symptomatic, causes postprandial vomiting and weight loss. The left renal vein (LRV) when it has a retro-aortic course can be compressed in aorto-vertebral space (AVS); if the stenosis is severe it can cause venous congestion symptoms with left flank pain, microhematuria, and thrombosis, this vascular alteration is known as a pseudo-nutcracker syndrome. The combination of Wilkie's and pseudo-nutcracker's syndrome (PNCS) is very rare and has not yet been described in the literature. We describe a case of a 62-year-old woman who presented symptoms and alterations typical of two syndromes.

Keywords: cardiovascular abnormalities; color-doppler ultrasound; ct (computed tomography) imaging; nutckracker syndrome; wilkie syndrome.

Figure 1. CT shows retroaortic LRV compression, duodenum compression, and left pelvic varicosities

(a) A single 60-second post-contrast axial view shows LRV compression (black arrow) in aorto-vertebral space: LRV (with arrow). (b) A single 60-second post-contrast axial view shows duodenum (arrow) compression in aorto-mesenteric space. (c) In a 45-second post-contrast axial view aorto-mesenteric distance (arrow) appears reduced (6 mm). (d) A single 60-second post-contrast coronal view shows dilation of the left gonadal vein (long arrows) and ipsilateral gonadal plexus (short arrow).

Figure 2. CT shows sagittal plane reconstructions

(a) In sagittal view, it is possible to measure aorto-vertebral distance (arrow) which appears considerably reduced (3.8 mm). (b) In sagittal view, the aorto-mesenteric angle can be measured, which appears reduced (18 degrees), and superior mesenteric artery originating from aorta can be seen with an acute angle "beak sign."

Figure 3. Images showing ultrasound examination

(a) Longitudinal scan of abdominal aorta and MSA with aorto-mesenteric angle (a) measurement (18 degrees). (b) Aorto-mesenteric distance (a) measurement (6 mm). (c) Color Doppler US of left kidney shows regular Resistive Index (0.69).

Figure 4. Scheme describing main anatomical structures involved in WS and PNCS

(a) WS. Diagram according to the coronal plane, showing relationships between MSA, abdominal aorta, and duodenum. b: PNCS. Diagram according to the sagittal plane, showing relationships between the aorta, left renal vein, and spine.