Separate diaphragmatic compression of the hepatic veins and the inferior vena cava causes severe right-sided abdominal pain and interferes with the absorption of intestinal blood by the liver.

This 18-year-old female patient with hypermobile Ehlers-Danlos syndrome presented with increasing upper abdominal pain, now mainly localised to the right upper quadrant, worsening after a meal, accompanied by severe vomiting preventing oral intake, severe nausea and significant weight loss of 24 kg over the previous 7 months.

She suffered from a variety of vascular compressions, all related to the reduced intra-abdominal space resulting from a flat thorax and exaggerated lumbar lordosis. The combination of May-Thurner syndrome and severe left renal vein compression resulted in pelvic congestion syndrome. Nausea and epigastric pain could be explained by median arcuate ligament syndrome and weak peristalsis of the dilated descending duodenum. However, the persistent right-sided abdominal pain had an unusual explanation: diaphragmatic compression of the hepatic veins and the vena cava, which passed separately over the diaphragm. While the compression of the hepatic vein was most severe during inspiration and almost normalised during expiration, the flow volume of the portal vein decreased by 25% during inspiration compared to expiration.

The impressive sonographic findings are explained in this video.