vascular compressions: one step closer to surgery

Monday, April 22, 2024, I had my call with Dr. Warren. We have long anticipated this call to pull together all the work I've done over the last months. My first appointment with her was December 19, 2023. In the world of rare, 5 months isn't so bad I suppose. But it's felt like a long time for me. But that's also because I've been working on figuring out my health for several years.

We started off the call with her asking about my venogram and how I'm feeling post-embolization. I explained the type of pain I've been experiencing. She asked if I had seen the x-rays from the venogram and I had not. The doctor had just told me that he didn't see any compression with my renal vein or iliac vein.

Below are pieces of what Dr. Warren said to me during the call if you'd like to read more in depth. I'll try my best to summarize very briefly here and you can continue reading if you wish :)

She is concerned about compression of my left renal vein, despite Dr. Venogram saying it's fine. My body has created more veins along my spine which indicates that something is blocked or damaged so it finds new avenues to flow. This can cause more back pain, which I have been experiencing. We discussed potentially doing another venogram now that I've been coiled to see if the flow has changed at all. She went over what surgery would be like for MALS. You can read more about that below if you'd like. She performs it as an open abdominal surgery. Some doctors do laparoscopic or robotic. Due to my history, I prefer that she is able to take a really good look while she's in there. That way if something else is amiss she can fix it. 

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"You definitely did have the varicose veins in your pelvis, so I could definitely see those. I'm not in 100% agreement with you not having Nutcracker based on my view of the films, but it's difficult because I know he read that as completely normal. But you got quite a bit of collateralization along your back."

(Collateral circulation is alternate or “backup” blood vessels in your body that can take over when another artery or vein becomes blocked or damaged.)

"Now, you definitely have varicose veins in your pelvis, but I was a little concerned when Kody and I both saw that he coiled you. And I'm not saying it's wrong because when you have Gonadal Vein Reflux, that's the right thing to do. That can propagate an emergency, though, with your renal vein if you have bad compression. Now, it may not be a bad idea to do another venogram if you're still having flank pain or if you start having blood in the urine, etc.  Just to kind of see, sometimes it changes the paradigm after you coil those gonadals because then the renal vein really has nowhere to get out except to the spinals."

"I had another young girl. I'm not saying it's going to happen to you because hers was horrible, severe. But she actually ended up in a wheelchair after hers were coiled because the congestion started going into her spine and nobody knew why she was hurting so bad in her back. So I definitely want to keep that in mind, OK?"

"And this is definitely something that, you know, it has to be a thought process that you all think about and you get an opinion over. I feel that you have significant MALS and there's different types of MALS, there's different types of constriction. Sometimes there's more nerve involved than there is constriction, but you actually have a physical kinking. The ligament is actually like a little rubber band. And that's why you saw it straight like this and that when you breathe out it goes like that. (she's demonstrating with her hands the 180 degree kink that happens) Now that may not be taking your blood flow away because it actually doesn't constrict it, but it kinks it to the point to where I think it's causing you pain."

"My thought is I'm leaning towards if you all want to try to get better and you've literally tried everything, we do have strong reason to recommend the median arcuate ligament release. Because it is rubber band, it is in the wrong spot. You've had a lifetime of trouble and I will look at your renal vein a little closer in there and I can move it, if it's safe to move. And that's behind your aorta, it's not easy to move, OK. It's dangerous. This one's behind there, but we do a lot of this. So it's an anomaly. But also being behind the aorta is what's the problem. It's not supposed to be back there. It's supposed to be in front."

"So that's the reason it's probably getting smooshed.  So it may not even be your SMA type of thing as far as that fat pad, it's just that you were born with it running behind the aorta. Now that's a known anomaly, right. But we have a couple other patients like you too."

"How do you move it? The renal vein. So if it's retroaortic, what do you do then?"

"So we do the same thing (as if it were in front).  I'll tell people if it flat out is dangerous and more harm than good. If I get in there and make a judgement call and it just should not be moved, I will not touch it. I'll leave it alone. I'll go in there and look around. If it looks like we physically can't move it safely then we won't do it, OK. That's number one, I always ask before. I discuss all this stuff up front. These surgeries are extremely delicate. They're extremely tedious. Every time I do one, I look at Grant and I say 'tell me why I'm doing this?' Again, it takes a ton of effort and that's why we do open. I always tell people there is a judgement and an art to these."

"The surgical release is a release of your arteries. So we basically get rid of all that possibility to form a kink. Your artery may never completely be normal 'cause you were born like that. But I basically relieve that tension and the ligaments from actually pressing on it every time you breathe out. OK, that is an open surgical release. I actually physically cut the diaphragm muscle in that area. It's not really as dangerous as it sounds, it sounds like, oh, I'm cutting your muscle... but you want it. And sometimes people even say ‘I breathe better’ afterwards. We hear that actually all the time."

(I have issues being able to fully exhale. If I try to do slow deep breathes, the exhale gets cut short. Which then induces more of a panicked breathing trying to catch my breath again. It's a strange feeling and hard to fully explain.)

"Patients like you were born with the wrap too low. So the celiac artery cannot handle that pressure like the aorta can. So basically the wrap is supposed to be up here when your chest and your abdomen come together, but with patients with MALS it's too low. So when it wraps to open for the belly cavity from the chest cavity, the diaphragm where it comes together, makes literally like a little tendinous band, OK. And that's what's actually pressing on you every single time. So we cut all that off. We get all of that nerve tissue, the celiac plexus that was blocked for you. I actually basically cut and remove all that and get rid of that."

"We may want to at least do another CT venogram for you or it's not wrong to get Doctor Parsons involved here in this situation and have another venogram."

"I'm just being honest with you because I'm not in agreement that you don't have spinal collateralizations and evidence of compression. You should not have these collaterals in your spine, OK. It causes back pain and significance flank pain. It could even be leaning over towards your right pain on the right side because it's actually draining into your spine. Now how would we know that? We see people like that. It's actually not really written about much. It's not talked about. And I actually just end up seeing a few patients like this. And we've noticed when you have the spinal collaterals, it actually causes back pain instead of draining into your gonadols, if that makes any sense. Because they can't get out, it has to go somewhere. So they're either going to your spine or to your pelvis and if you take away the pelvic aspect, which that sucks, right, it does cause pain. But if you coil that, it'll start bringing it into your spinal. So sometimes people have more back pain after they're coiled, OK. In our experience."

"A lot of this, again, none of this is really written about heavily enough. We always say that we'll write our own book here in about two or 20 years, but until that, there's just not a little literature about it."

"So I'm always honest with patients. You definitely have enough to offer surgery to. Will you have a phenomenal result? I think you probably will, but I can't guarantee and I never do. Especially the patients where it's been 38 years, you know, dealing with this. But we really don't have another answer. That's the other thing. And this is so crucial with MALS too. We literally rule out everything. And if you don't have another answer, it's definitely reasonable to say OK, if you're miserable and you just flat out can't function, I will help you. I'm offering to help you. But you don't have to have it done. If you want to go on about your life and not have an open surgery and try to deal with this medically increase your fat in your diet, you know, and you and your hubby definitely talk about this. I mean, it's definitely a big surgery. It's not foolproof."

"I have really good results. But I always tell patients, at your age you may not get 100%."

"I do this the same way every time. And then I pray about it and give it to the good Lord because I just feel like I'm here to help people and that's my personal feelings."

"You mentioned that this can come back after surgery?"

"I don't mean that so to speak. We have had patients that may have other things going on and other reasons to hurt. Now once the median arcuate ligament is released, it does not grow back. It is impossible if it's done correctly and thoroughly, which I do every single time. I can tell you that I know that 'cause I do them open. So I know exactly all of this is being released. There is a such thing as scar tissue getting around there. I've heard of patients having recurrent symptoms. Most of the patients that have recurrent symptoms, I think cause they probably didn't have a reasonable enough release. OK. Also some patients have other things going on even in my own practice, like I knew they had some other things going on. Maybe they need their bowel resected or something like that, for an example. OK. I don't really think you have much else going on except this renal vein thing."

"But I always tell people, is it heard of to grow back with scar tissue and still have symptoms after surgery? With my population, it's not common. I can name one to three patients that still were not, like, magically cured after this, thank God. But it was because they had other things going on."

"I know you're mentally OK. I say that reasonably. But a lot of patients with this diagnosis have been told they're crazy for like, their whole life and it takes mental and emotional healing. Sometimes it's hard to be well, and sometimes the parents for these young girls have to have some psychotherapy too, 'cause they don't know that their child can be well, OK, so you are different."

"We'll call you back ASAP as soon as we get all the stuff loaded up for Dr. Parsons and then we'll try to you know, see what else is going on. But I want him to take a look at this. That way I can tell you, hey, he's thinking you need another picture or he agrees with me."

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We then discussed surgery timeline. The request has to go through insurance first. If the surgery is just for MALS the scheduling should be quick once we get approval. She wasn't able to call back herself. Her nurse called two days later to let me know that Dr. Parsons said he would have coiled the same way. And if the other Dr said the renal vein was okay during the venogram, he trusts that. He said the pain from post-embolization is common and it may take a bit for my body to figure things out again. But to call back if it's still really bad in a week or so. Kody then asked if I wanted to proceed with sending the MALS surgery to insurance. I said, yes!

I thought about everything for a few days and looked through all my doctors notes once again. There was still a nagging feeling about it all. So I emailed and asked a few more questions. I got a consult set up with Dr Parsons for 5/15/24. I would like to be able to talk to him personally as sometimes it's hard hearing small summarized fragments from someone else. I will feel more comfortable talking through it with him. Especially since my CTE did highlight nutcracker phenomenon and my pain is inhibiting my quality of life even more than before the embolization.

I am also going to do an Upper GI small bowel follow-thru. This was the test I was supposed to do a few months ago but got switched to a CTE. Since my SMA angle and distance is right on the edge of normal. So that will be on 5/17/24. Trust me, I'm over doing all these tests. But measure twice, cut once.

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Thank you for all your continued support and love through this journey with me!

Ash

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