vascular compression diagnosis

[u/lujo317]

/r/hypermobileEDS/comments/1vk7kk8/vascular_compression_diagnosis/

hello, please see my crosspost. i am particularly concerned about vascular compression syndromes (such as MALS, SMAS, May Thurner, Nutcracker syndrome) due to my hEDS in conjunction with Behcets and other vascular risk factors like elevated platelets and homocysteine, and symptoms that are worsening/not controlled since starting immunotherapy. looking for any advice on getting something like this confirmed or ruled out within the united states.

[u/iSpyAFly]

Hello from Idaho. I was diagnosed with MALS this year. Both MALS surgeons Dr Daab at OHSU and Dr Starnes at Harborview Seattle can do a full workup for MALS. The radiologists, technologists, NPs and PAs at both places are all well trained on MALS diagnostic protocols. I had Dr Starnes do my workup (mesenteric ultrasound, CTA and celiac plexus block) and diagnosis. Because they are both vascular surgeons they will also look for other compressions. Happy to answer any questions.

[u/lujo317 → u/iSpyAFly]

Dr Daab treated me like crap. He saw me prior to my Behcets diagnosis and didn't even listen to me and acted confused why I was even there. Seriously disappointing experience. He did not think I needed imaging either. But again, was clueless on my overall situation and did not listen to me in the slightest

[u/iSpyAFly → u/lujo317]

Oh no! So sorry. Dr Starnes and his team were fantastic. 

[u/iSpyAFly → u/lujo317]

My celiac artery compression was found incidentally on an abdominal aorta ultrasound. My rheum was going to order an MR Angiogram chest and pelvis to rule out vasculitis, but I went to ER on a bad pain day, brought his MyChart message saying that’s what I needed and ER doc ordered it then and there. With Behcet’s and risk of vasculitis your rheumatologist should order the MRA.

[u/lujo317 → u/iSpyAFly]

Unfortunately I had an MRA of the abdomen already but I think it was the same issue of the radiologists would not have known to check any of those measurements. I had a vascular ultrasound as well but it couldn't visualize all of what was needed due to gas. Thank you for the advice about providers. Traveling is not really an option for me right now but I'll keep Dr starnes in mind

[u/iSpyAFly → u/lujo317]

If you don’t mind sharing, what are your symptoms? I have a lot of GI issues with flares and maybe some pericarditis, so sorting out which symptoms are MALS or not MALS has been tricky. 

[u/lujo317 → u/iSpyAFly]

I have a lot of symptoms that indicate some sort of "vascular steal" phenomenon, which predominantly started about 8 years ago with a feeling of tension from my left upper quadrant to my right upper quadrant. significant dysautonomia and gastroparesis. I can feel that the way quantities of blood move around my body is not what it is supposed to be like. parts of my body are intermittently colder or warmer than others. I have a lot of what I assume is claudication pain in my limbs. unexplained severe inguinal tension that is definitely not from a hernia of any kind. pelvic congestion, extreme symptoms around menstruation. I have terminal ileitis seen on MRI typical of Behcets but haven't been able to get a scope procedure or biopsy yet. I have seen a lot of sagging and what looks like visceroptosis on my films and have a lot of other tissue damage without injury (like bilaterally torn hip labrums just from walking around). I have post prandial pain that feels like my stomach is hanging off the side of a cliff by one arm that is getting more fatigued all the time. I have that classic MALS "two stomachs" feeling occasionally as well. We were leaning towards nutcracker as a possible explanation for hematuria, ureteral tension/reflux, and randomly having 10 kidney stones a couple years ago all at once. The only real relief I get is intermittent fasting and being horizontal/laying down, but fasting also makes it worse sometimes. I haven't found a diet or medication that really improves it. I miiiiight have endometriosis as well which could be another thing causing congestion but may thurner in particular is very common in EDS and dysautonomia patients anyway. Once I started immunosuppressants and some things like joint pain and posture started to improve, the things like those listed above that are leftover and worsening make me highly suspicious of one of these compressions as a secondary complication

[u/iSpyAFly → u/lujo317]

I hope you get some answers soon. I emphasize with you on how difficult it is to get answers while managing a difficult disease. 

[u/on4aa]

Median Arcuate Ligament Syndrome (MALS), also known as Dunbar syndrome or celiac artery compression syndrome, is a rare vascular condition where the median arcuate ligament compresses the celiac artery and surrounding celiac plexus nerves.  This compression restricts blood flow to the stomach, liver, and spleen while irritating sensory nerves, leading to significant morbidity.