I present a 17-year-old female patient with recurrent diarrhea, daily upper abdominal pain in the epigastric angle as well as in the left flank, pulsating headache, and a strong dependency of the abdominal pain on food intake.

The patient was diagnosed with Wilkie’s syndrome in her home country because of the apparent narrowing of the angle between the superior mesenteric artery and the aorta.  Unfortunately, as I have described, this is a complete misdiagnosis of Wilkie’s syndrome.

Wilkie’s syndrome requires the coexistence of postprandial pain immediately after ingestion of food, accompanied by sudden projectile vomiting or at least synchronous peristaltic cramping pain in the upper abdomen, often accompanied by a bulging of the right upper quadrant and a sensation that food is stuck in this area.

However, the patient’s pain began about 30 minutes after ingestion and increased steadily, and although she had the sensation of gastroesophageal reflux, she never vomited or burped. Furthermore, and this is crucial, the pain was a steadily increasing sensation and was not characterized by the peristaltic synchronous wave-like pain sensations that are very characteristic of Wilkie’s syndrome. To understand this, it is helpful to remember that Wilkie’s syndrome is a mechanical constriction of the duodenum. Therefore, the symptoms increase with each peristaltic wave that is blocked by this constriction. Continuously increasing pain is quite atypical and does not exclude Wilkie’s syndrome.

In addition, a relevant blockage of intestinal transport is always accompanied by significant weight loss, which was also absent in this patient.

This patient did not have Wilkie’s syndrome but a massive compression of the left renal vein as shown in the video below. It is important to note that both tubular structures, the duodenum and the left renal vein, use the same area for transport from one side to the other – the angle between the aorta and the superior mesenteric artery.

Since compression syndromes are generally a consequence of increased lordosis, it is not uncommon for both structures to be compressed. However, since the vein is much softer and cannot push the blood by peristaltic motion, the left renal vein is usually the structure that is compressed first. The duodenum, on the other hand, has a strong longitudinal and circular layer of muscle that pushes the bolus across the midline. Thus, the left renal vein is usually the first to be compressed and only very late, often after the left common iliac vein and celiac trunk have been compressed, does it undergo significant constriction with the typical clinical symptoms mentioned above.

This was the case with this patient.

She had a massively congested left renal vein due to a high degree of compression of the vessel to the right of the aorta by the elevated curved origin of the right renal artery.

PixelFlux measurements are the only technique to show that there is a real effect of food intake on the left renal vein.

The graph shows that while fasting and lying horizontally, there appears to be a sufficient collateral network to redirect the congested blood back to the heart. However, after ingestion of food, and especially in the upright position, a significant reduction in left renal perfusion is the obvious sign that the additional pressure exerted on the left renal vein by the expanding stomach and distending duodenum contributes to a further challenge of the collateral network of the left renal vein. In this case, the transport capacity of this network was already exhausted under resting, horizontal, and fasting conditions. The ingestion of food thus increases the pressure in the vein, reducing the arterial inflow and thus the overall parenchymal blood flow, as clearly demonstrated by the PixelFlux measurements. The implications for the patient are enormous. She can now avoid surgical correction of her GI tract, such as a duodenal jejunostomy, because Wilkie’s syndrome has been safely ruled out. The correct treatment for her is decompression of the left renal vein.

The pelvic pain was the result of an enormous uterine congestion: