Ehlers Danlos-syndrome the flashing redlight of vascular compressions
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Many connective tissue disorders cause a loosening of structures containing larger amounts of connective fibers. Such organs are the lung, the intestines as well as blood vessels, joints and ligaments.
Veins contain a lot of connective fibres, so they are affected by connective tissue disorders as are arteries. Because of the blood pressure differences in veins and arteries both types of vessels react differently, as soon as the pressure rises.
Arteries tend to develop aneurysms whereas veins tend to dilate more generally.
The greater distensibility of veins is the reason for their easier compression if they are set under pressure from the outside. The venous segment which lies before the compression site reacts with a widening (dilatation), later on the vessel starts to elongate and begins to meander like a river which has not enough force to overcome a long stretching obstacle. If the driving force and the obstructing force are nearly equal, as in a river in a flat plane, it starts meandering. If the venous pressure is nearly equal to the compressive force, the vein starts first to widen, as a river flooding it’s banks and later becomes varicose, as a meandering river.

If there is no connective tissue disorder the vein would be less distensible and the pressure inside the vein would increase faster than in a soft and lax vein of a patient with the connective tissue disorder. Thus the transport of blood would remain intact.
If the pressure increases further, the blood is bypassed through connecting veins which serve as collaterals. These collaterals guide the blood to areas with lower pressure. Over time these collaterals also lose their normal shape, they dilate and form varicoses. Such varicose veins are well-known from the lower limbs but also exist within the abdomen. Their wall is excessively stretched and thus becomes damaged. Microfractures of the connective tissue attract white blood cells which are specialised on repairing damaged tissues. These white blood cells secrete interleukins as mediators of an inflammatory reaction. The hallmarks of inflammation are well-known: Pain, swelling, redness and loss of function can be experienced by the patient.
But not only the vessels are affected in connective tissue disorders. With respect to vascular compressions the loosening of the ligaments of the spine is especially devastating. Over time the spine develops large curves under the gravitational forces. In the beginning a lumbar lordosis and a thoracic kyphosis are the most eye-catching changes of the spine. In the course of the disease severe scoliosis may develop. Scoliosis is a consequence of lordosis and kyphosis. Its development can be illustrated by a crank drill. The crank is a lever to turn the drill. The bending of the spine produces a crank-like deformation which also functions as a lever to drag the spinal curvature from a sagittal towards a frontal orientation. That means in the beginning the bending of the spine is directed forward and backward. The forward curvature develops in the abdominal area and the backward curvature within the thorax. At the apex of this curvature which works as a crank muscles drag the spine towards the side. Such a sideward deformation of the spine is called scoliosis. This is a general mechanims, not only but more gravely in patients with connective tissue disorders.

The strong lordotic bending of the spine produces a pressure against structures which lie in front of the spine. Very frequently, only some millimetres remain between the front of the spine and the inner lining of the abdominal wall. This is quite often invisible for the naked eye, since the spine is hidden by the surrounding muscles and subcutaneous fatty tissue. The very extent of the scoliosis and its effect on the shaping of the abdominal cavity can best be seen with ultrasound. It is often surprising, how narrow the space is which is left by the spine. The abdominal cavity then resembles a dumbbell and all the structures which have to pass from the one side of the abdomen towards the other side has to pass the narrow grip of the dumbbell.

More explanations on the significance of lordosis for all the vasular compression syndromes can be found in the chapters on lordosis and lordogenetic midline congestion syndrome of my website.

In general, EDS is expected to occur in 1 of 5000 individuals. In our personal experience with patients with compression syndromes EDS is much more frequent. From 28.02.2017 – 31.12.2019 we (Sandmann + Scholbach) saw in 30% of 116 patients with compression syndromes phenotypical signs of EDS. This underscores the overwhelming impact of loose connective tissue to the compressibility of vessels and intestines in vascular compression syndromes.

From the patient’s perspective, it is important to understand this causal relationship. The treatment of vascular compression in EDS patients could help them to treat frequently observed complaints successfully .

Patients often suffer from complaints that can be identified on close examination as a result of abdominal vascular compressions . These include:

      • Vegetative dysfunctions (bloating, belching, anxiety, nausea, vomiting, dizziness, shortness of breath, gastric emptying disorders, postprandial pain, inappetence, weight loss, cachexia, anorexia and bulimia
      • headache, migraine,
      • abdominal pain, flank pain (left),
      • colic,
      • diarrhea,
      • constipation,
      • hyperemesis, swelling of the legs,
      • paraparesis and sensory deficits

Without knowledge of the connection to vascular compression, these frequent and very general symptoms are all too often dismissed as psychogenic. However, since they have a specific treatable organic cause, they can also be treated causally by alleviating or eliminating the underlying vascular compression.

In EDS all abdominal organs sink down while standing (ptosis). This stretches the renal vessels and can reduce kidney perfusion dramatically. The blood , not taken up by the kidneys,  then runs into the pelvis and the legs. The patients can barely tolerate the ensueing congestion of their genitals, bowels, urinary bladder and legs. Bluish veins pop out on the feet and thighs.

When the ptosis of the kidneys significantly drives the complaints or when venous compressions further impedes the blood return to the heart, a surgical fixation of the kidneys (nephropexy) and a venous decompression can help.

Over the years we developed a special technique to tailor functional color Doppler ultrasound to the unique needs of patients with connective tissue disorders.

I assess the degree of their lumbar lordosis  by transabdominal sonography in supine position.
Special attention is but paid to the actual effect of their hyperlordosis onto the individual compression site of vessels and parts of the intestinal tract and the effect of changing body posture on the location and degree of the compression.
The effect of the compression is thus measured sixfold:
1. As diameter reduction and its changes
2. As flow acceleration and its changes
3. As changed and changing flow directions in feeding vessels
4. As changed and changing flow volumes in the compressed vessel and its feeders
5. As changed and changing flow volumes in three consecutive parenchymal layers in both kidneys by the PixelFlux technique
6. As changed and changing flow volume ratio of left to right kidney

Here changed refers to the effect of the compression itself and changing refers to the different effects of various body postures.

Since patients with connective tissue disorders do not only suffer from a higher compressibility of tubular organs but also substantially from their increased distensibility I measure the effects of gravity on their circulating arterial blood volumes.
So I compare the sonographically determined aortic flow volume, peak velocity and heart rate immediately cranially to the diaphragm in a supine and standing position. The drop of volume while standing is used to gauge the pooling venous volume which was trapped in the legs and pelvic vessels. Moreover, I measure pelvic and femoral vessel’s flow volumes, directions and diameters plus the changing transsectional area of the vena cava inferior at its compression site by manual tracing of its contour.
All these parameters are used to compare the separate effects of the pure genetic component of the connective tissue disorder and the additional effect of the compressions in order to estimate the chances of symptom relief by a decompression operation.
If a compression of the duodenum is seen in the fasting patient a possible relevance of this finding is then confirmed by observing the duodenal peristalsis and concomitant symptoms after ingestion of solid food and liquids. Moreover, a compression and upstream dilation of the stomach is looked for. Then attention is paid to the additional compressive effect of the enlarging stomach onto the precompressed left renal vein and subsequent flow changes as described under 1 to 6 above. This us to discriminate the postprandial symptoms of MALS, Wilkie-syndrome and lordogenetic left renal vein compression. This is crucial for prioritizing the extent and sequence and synchronicity of the various decompression operations.
In many cases the compressions are not found exactly in the midline. The hyperlordosis regularly produces a scoliosis and a concomitant shift of the aorta to the left side of the spine, the concavity of its lumbar scoliosis. This produces a clockwise twist of the aorta from a causal perspective with a subsequent anterior course of the right renal artery. Its curvature is the hypomochlion,  the point of compression.

Our clinical,surgical and sonographic approach to patients with connective tissue disorders is published here.

Our contributions to the EDS-Echo Summit 2020


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