Unbearable pain only while standing extending from the right lower abdomen towards the right lower thoracic aperture
The patient is a 33-year-old mother of 4 children. She was diagnosed with hypermobile Ehlers-Danlos syndrome. She suffered from severe left sided pelvic pain already for years. This was referred to a May Thurner syndrome which subsequently was unsuccessfully treated with a stent insertion into the left iliac vein. The stent caused her unbearable pain immediately post insertion and thus was removed. The May Thurner syndrome was then treated with an elongation of the right common iliac artery to decompress the left common iliac vein with little success.
Later, a severe postural tachycardia developed which caused frequent fainting. To prevent fainting the patient had to sit or lie down which rapidly improved her situation.
Then the diagnosis of a left renal vein compression was established. The left renal vein compression and the persistent left iliac vein compression had then been successfully operated with a PTFE shield wrapped around the compression sites. The symptoms of a concomitant median arcuate ligament syndrome disappeared nearly completely after excision of the median arcuate ligament.
The patient was nearly symptom-free for about 6 months but during this time observed an increasing lumbar lordosis which is one of the skeletal consequences of Ehlers-Danlos syndrome.
Now more than half a year after the successful decompression of left iliac and renal vein an unbearable pain solely while standing emerged that extends from the right lower abdomen towards the right lower thoracic aperture.
Functional sonography revealed an orthostatic compression of the vena cava inferior causing a rapidly increasing congestion of the uterus, mainly at its right side. Here tortuous vein were visible which were nevertheless painless at the beginning of the examination.
To find the reason for her inability to stand longer than 30 min she assumed this posture and expectedly developed an unbearable aching pain which extended from the right side of the uterus towards her right lower thoracic aperture.
During this development of pain, the vena cava underwent impressive changes of its size and shape.
The inferior vena cava, normally sized at the beginning of teh examination while lying supine, impressively dilated immediately after standing up. But after 30 min of standing a long compression at the cranial slope of the lumbar lordotic spine extending over 7,5 cm had developed .
In such a situation it is not easy to decide if such a morphologic finding is related to the pain reported by the patient.
With the PixelFlux technique this becomes feasible by measuring the changing degree of an organ’s perfusion.
In this patient it was possible to compare the uterine congestion – i.e., blood vessel filling – with standardized serial PixelFlux measurements.
These correlated precisely with the severity of pain.
Since under sonographic observation the painful structures can be palpated, and the patient can define whether this increases the pain – ultrasound is a favorable technique to identify painful structures.
By one-finger-palpation the pain could be referred to the right sided parauterine varicoses from where a painful enlarged right internal iliac vein transmitted the pressure and thus the pain towards the compressed vena cava inferior up to the right subcostal region where the long caval compression prevented a recirculation of the pelvic blood. This led to an ever-growing blood pool in the pelvis and a subsequent diminution of the still circulating blood volume. Its consequence is a sharply dropping blood pressure. To compensate for the heavy reduction of the circulating volume a postural tachycardia developed to maintain a basic perfusion of the peripheral organs. Since this was not sufficient as the acute obliteration of the vena cava overstrained the collaterals, fainting was the only possible consequence.
Even with the suspicion of such a pathophysiological mechanism it is barely impossible to correlate the growing congestion to the pain in an individual patient. With the naked eye the exact degree of the uterine congestion during the development of the pain attacks cannot be defined.
With PixelFlux however this becomes feasible and thus a diagnosis is achieved which opens therapeutic options.
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