This 20 year old female patient suffered from the following complaints:

• Nausea since November 2023
• Vomiting since November 2023 now controlled with medication.
• Feeling of fullness after eating very small amounts
• Bloating
• Pain above navel below ribs only when eating 9/10; With morphine 6/10 constant (right kidney area), 7/10 constant (left kidney area); Without morphine 9/10.
• Urinary retention since April 2024 Selfcatheterization currently 34 times a day
• No sensation of bowel movement since April 2024, enema every 4 days.
• Migraines: about one every two weeks
• Pressure headaches constant 2/10
• POTS : migraine, dehydration, increase in heart rate (controlled by medication), fainting, clammy. Started in 2020, developing into nonepileptic seizures with concomitant headache 20202024. Last seizure was in hospital when pain relief was removed September 2024.
• has a PICC (peripherally inserted central line) right arm – tip is in the SVC and has 1 litre of saline per day.

The patient has been refused parenteral nutrition by her treating physicians, claiming that all her suffering was in her head, despite a weight loss of 13 kg from 65 kg in November 2023 to 52 kg now.

Clinical findings:

The patient uses a wheelchair and cannot stand without support. She wears a collar around her neck to stabilize her head. She cannot lift her head without support from her hands or by being held while lying on her back. She cannot turn from a supine position to a prone position without support. If she stands with support, she almost faints. When lying on her back without a pillow to support her head, she develops a severe headache that requires an extra dose of morphine.

At present, the patient has pain in the upper abdomen in the midline above the navel. Palpation of the abdomen reveals maximum pain in the left hypochondrium and to a lesser extent in both lower quadrants suprainguinally. No pathological lump is palpable. There are no striae or distended subcutaneous vessels. Her joints are hyperdistensible with a Beighton score of 7/9. A formal diagnosis of hypermobile EDS has already been made.

Auscultation reveals a pulsatile murmur in the epigastric angle. Peristalsis sounds normal.

Sonographic findings:

This is an intra-operative image of a strong tronc réno-rachidièn which, by pressurising the epidural plexus, increases the spinal and cerebral congestion to the same level as the severely compressed left renal vein, since the tronc réno-rachidièn connects the left renal vein to the epidural plexus.

The tronc réno-rachidièn is fed by a pressurised left renal vein. This massive increase in pressure is best illustrated by the enormous acceleration of flow as shown in the images below.

This video shows the severe pelvic congestion with a focus on the urethra and the right wall of the rectum. The cuff-like surrounding periurethral veins increase the pressure on the urethra to such an extent that the patient is unable to void and has to catheterise herself.

This image shows the reverse flow in the left internal iliac vein and the corresponding flow volume. The abnormal flow direction can be easily deciphered by comparing the colour with the adjacent artery. Under normal circumstances, the vein should have a different colour to the arteries as the flow should be in the opposite direction to the artery.

This image shows a much wider right internal iliac vein with a stronger flow volume pointing to the transfer of blood from the left towards the right pelvic hemisphere across the pelvic midline organs.

This picture shows an important cause of massive pelvic congestion – May-Thurner syndrome with compression of the left common iliac vein. The striking difference in size between the enlarged part on the right and the compressed part on the left is the reason for the acceleration of the flow, which is a sign of pressurisation. This may contribute to spinal congestion via collaterals.

This image shows the sonographic signs of median arcuate ligament syndrome, which is responsible for the patient’s nausea and epigastric murmur, which was new to the patient as she had never had an abdominal auscultation before.

These images show the acceleration of the flow as a sign of the pressurisation of the left internal jugular vein with the head in a neutral position, without the support of a pillow. This pressurisation contributes to an increase in intracranial pressure, which is even greater when the inflow via the epidural plexus, due to the transfer of volume from the left renal vein, mediated by the tronc réno-rachidièn, pours more volume into the intracranial compartment.

The patient’s cerebral symptoms consisted of migraine, constant pressure headache and seizures. Nausea and vomiting may be due to increased intracranial pressure, but are also clearly related to the compression of the celiac artery – the median arcuate ligament syndrome.

The patient was treated with a decompression of the coeliac trunk, the left renal vein and the left common iliac vein by wrapping them into a PTFE sheath.