Severe ataxia in a young woman with severe spinal congestion – complete resolution after decompression of the left renal vein
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There are numerous consequences of a spinal cord congestion as a consequence of left renal vein and the left iliac vein compression in patients with midline congestion syndrome.

The possible numerous clinical consequences are outlined on a separate page.

I report here on a young woman developing severe vegetative symptoms, abdominal pain, convulsions and gynaecological problems due to multiple abdominal compression syndromes on the basis of a hypermobile Ehlers-Danlos syndrome.

She became wheelchair bound due to extreme ataxia of the trunk, the head and the extremities. Routine neurological diagnostics an standard  imaging  procedures in her home country and abroad did not reveal any treatable reason for her suffering.

Only functional colour Doppler sonography with quantification of all relevant abdominal and pelvic vessels could demonstrate the very reason of her ordeal – a massive venous congestion of the spinal cord fed by a high flow tronc réno-rachidièn connecting the congested because completely obstructed left renal vein with the epidural plexus within the spinal canal. This produced a neurological deficit the compression and subsequent venous congestion of the spinal cord.

This video shows her miserable condition before the operation im May 2022

 

This video was taken in  March 2023, 7,5 months after the successful decompression of the left renal vein and subsequent complete collapse of the tronc réno-rachidièn

Medical history

January 2016

  • Hyperexcitability, muscle cramps, weight gain due to edema of the legs

January 2017

  • Went to the emergency department because of severe muscle cramps and convulsions
  • Arrhythmia, dizziness, vomiting, numbness in both hands and tremor of the trunk
  • Presents several times to the emergency room of the psychiatric hospital because of general shakiness. There she was diagnosed with a mental disorder: anxiety disorder

February 2017

  • Presenting to the emergency department with pain on the right side of her spine and a pollacisuria
  • Taken to the psychiatric ward because of repeated generalised convulsions
  • Psychiatrist refuses the diagnosis of an anxiety disorder
  • Repeated convulsions

May 2017

  • Was diagnosed with syndrome of intestinal bacterial overgrowth and eosinophilic oesophagitis

May 2021

  • Had seen many doctors within her home country and abroad without getting a proper diagnosis and treatment for her increasing truncal instability and convulsions
  • Is suffering also from abdominal pain localised under the diaphragm radiating towards both sides increasing post-prandially
  • Call allannot breathe properly
  • Difficulties to swallow
  • Chest pain
  • Always agitated
  • Severe brain fog, visual disturbance, migraine, dizziness, feels like being drunk, back pain, cold and bluish hands, weak feet and hands
  • Increasing fatigue
  • Hyperflexibility of joints

 

First presentation in my functional colour Doppler sonographic clinic in June 2021

The following diagnoses had been established during the first examination:

  1. Pelvic congestion syndrome as a consequence of
  2. A May- Thurner syndrome with a venous spur
  3. Severe lumbar lordosis as the main reason for all vascular compression syndromes of the patient
  4. Complete lordogenetic compression of the left renal vein with a bypass via a
  5. Tronc réno-rachidièn feeding the epidural plexus and an
  6. Enlarged left ovarian vein transporting blood down towards the pelvis
  7. Substantial venous pooling in an upright position as a sign of a clinically a relevant connective-tissue disorder – in accordance with certain clinical signs of such a disorder
  8. Severe median arcuate ligament syndrome with typical symptoms, the main symptoms of the patient
  9. Ptosis of both kidneys
  10. Severe strangulation of blood flow towards both kidneys, mainly on the left side due to renal ptosis while being in an upright posture – this is contributing substantially to the pooling of venous blood in the pelvis and in both legs
  11. Heavy reduction of the circulating blood volume inside the aorta while being upright
  12. Double uteri

 

 

Preoperative renal parenchymal perfusion measurement in June 2021

The patient was operated in June 2021 and July performing a decompression of the celiac trunc (which was complex due to heavy scar tissue found  around the coeliac trunc), shielding the  left common iliac vein and the left renal vein by means of a ringenforced PTFE graft.

The operation was performed by Professor Sandmann at the Clinic BelEtage in Düsseldorf (Germany). I’m very grateful for his open-minded cooperation and allownace to publish his intraoperative photos.

Liberation of the coeliac trunk after the section of massive scar tissue compressing it

 

Severe compression of the left common iliac vein by the crossing right common iliac artery-the sonographically diagnosed May Thurner syndrome

Degenerated wall of the left common iliac vein at the crossing site with the right common iliac artery

Liberated left renal vein with the tronc réno-rachidièn – yellow sling (blue sling: left suprarenal vein, red sling: left ovarian vein)

PTFE shield around the left renal vein protecting it from the compression from the aorta

Post-operative renal parenchymal perfusion measurement in November 2022 after additional decompression of the left renal vein by a PTFE-shield in July 2022

In an upright posture the left to right renal perfusion ratio was 12% and is now 31%.

Altogether a much better perfusion of the left kidney was achieved by the operation increasing the flow volume towards the left kidney by 87%

At the same time no blood flow across the tronc réno-rachidièn was detectable anymore whereas preoperatively about 1000 mL/min have been pushed via the tronc réno-rachidièn into the epidural plexus. This caused a substantial pressurization of the cerebral spinal fluid with a subsequent session of the spinal cord.

The reopening of the left renal vein by wrapping it with a PTFE graft made the flow across the tronc réno-rachidièn the clinical result is a complete disappearance of all neurological symptoms of the patient!

We (Prof. Sandmann and me) thank the patient for her permission to publish this report and her videos and especially for her steadfastness in not giving up hope for a cure in the face of a progressive, severe disease that couldn’t be clarified in her home country!

 

 

 

 

 

 

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