Gut compression syndromes; Nutcracker syndrome; Abdominal Vascular Compression Syndrome

[SNT Gatchaman]

This and a number of subsequent posts have been split from another thread

I'm actually a bit worried about the recent proliferation of vascular compression surgeries (Nutcracker, May-Thurner etc) where patients are travelling to Germany to see one of a very small number of surgeons; it reminds me very much of what happened with CCI. I looked but couldn't find many case reports so difficult to get a handle on the kind of radiological evidence that's being used to justify surgery.

(Possibly heading a bit off topic, so may need re-threading.) I've certainly diagnosed abdominal vascular compression syndromes on imaging though they're pretty rare. In fact rare enough that we even published a case report back in the day (SMAS though not EDS-related as far as I recall). I won't link that paper but the imaging was incontestable and the surgery was uncomplicated and definitive/permanently curative.

Anecdotally some of these more recent (h)EDS surgical cases do report doing well, though who knows how many are not improved or have complications? Eg this positive media report —

In November 2022, Jemima was diagnosed with EDS and given three years to live by a private specialist.

Jemima explained she was reliant on artificial nutrition and her central line kept getting infected while Mckenna said the prognosis was based on her “rapid deterioration”.

The specialist suspected AVCS and gave her some information about the condition and surgery in Germany.

In April 2023, Jemima had surgery in Germany. She was approved for some funding through the Government’s high-cost treatment pool – application-based funding for private or overseas treatments.

“I went from just lying in bed to being able to go back to dance, to hang out with friends, to do everything that I was doing before I got sick.”

Jemima said she still suffered from EDS and had digestive problems but could “pretty much eat whatever I want”.

Her pain was more manageable.

“And I’m not dying any more.”

(A bit unfortunate, that bit about recurrent central line infections!)

A very recent media article here is RNZ: Abdominal Vascular Compressions Syndromes sufferers left fund raising for their own surgery —

Williams was told by healthcare professionals that she had an eating disorder, that it was a psychological issue and a gut-brain axis issue.

"This was really tough to hear because it made me feel crazy and like it was all in my head despite clear statements and scans from a vascular surgeon in Hamilton, and two doctors in Germany stating that I was clearly suffering from abdominal vascular compressions," Williams said.

 

[hibiscuswahine]

Off topic again but I read in Stuff last month, a young woman diagnosed with hEDS flew to Germany for the nutcracker surgery with her mother after doing a Give A Little page, and it didn't work.

King, who is now living at her parents’ home, is unable to eat or drink and has had no luck trying to access nutrition, such as in the form of a feeding tube, from the public health system.

“I feel like I've been left to die,” she said.

“Doctors will say that they're going to help you, but they don’t help you.”

King said if she had an eating disorder “they would let me into a psychiatric unit and force feed me”.

“But because it’s physical, I’m essentially labelled too complex and pushed aside.”

https://www.stuff.co.nz/nz-news/350343014/39kg-woman-ive-been-left-die

She, unfortunately, is misinformed about NZ psychiatric units, we do not force feed people there. (Most of our eating disorder services are in the community and are on a voluntary basis). Also it is pretty hard to get into a mental health unit, psychiatrists have a hard time getting patients a bed sometimes.

It is only in very extreme circumstances that people are "force fed" (and you would have to have a mental disorder and be sectioned first)

You would have to fulfil medical criteria for a life saving intervention and it is done on a medical ward.

It is not something physicians or psychiatrists do lightly and it is a rare occurrence.

 

[Sid]

I'm actually a bit worried about the recent proliferation of vascular compression surgeries (Nutcracker, May-Thurner etc) where patients are travelling to Germany to see one of a very small number of surgeons; it reminds me very much of what happened with CCI.

I'm seeing this more and more on social media. Risky. Leaving aside the issue of whether this is real or not, pursuing unconventional treatments, especially surgical treatments, that are not recognised by mainstream medical authorities in your country could result in the parent and the child being diagnosed with FII/Munchausen's by proxy when they return home.

 

[SNT Gatchaman]

I read in Stuff last month, a young woman diagnosed with hEDS flew to Germany for the nutcracker surgery

Yikes.

It was two years ago when her health started to deteriorate, including two kidney stones requiring to be surgically removed, with medical staff noticing she had high levels of [lipase], an enzyme made by your pancreas which helps your body digest fats.

That led to King being diagnosed with pancreatitis. Her health continued to deteriorate, with her weight plunging [to] 56kg as she experienced a range of symptoms, including nausea, fatigue, vomiting, constipation and diarrhoea.

Frustrated by a lack of help, and following an admission to the Emergency Department at Dunedin Hospital she began researching similar conditions on Google.

That led her to contact two specialists in Germany, who examined her medical records and concluded that she suffered from abdominal vascular compressions and also Hypermobile Ehlers-Danlos syndrome.

I don't think Jonathan will be at all impressed when he reads that!

In September [2023], King who was joined by her mother Cheryl King travelled to Germany for [de]compression surgery aimed to release blood vessel pressure, allowing blood to flow better and lessen her symptoms.

But her health has since worsened.

King, who is now living at her parents’ home, is unable to eat or drink and has had no luck trying to access nutrition, such as in the form of a feeding tube, from the public health system.

I'm pretty sure our surgeons try and stay well away from peri- and post-pancreatitis abdomens. They would tend to use the word "hostile". The surgery the Germans would have been doing is in the retroperitoneum, right next to the pancreas...

 

[Jonathan Edwards]

He did remark that a similar situation is the (h)EDS / vascular compression cases, some of whom have flown from NZ to Germany for treatment, as reported on in our local media.

I'm actually a bit worried about the recent proliferation of vascular compression surgeries (Nutcracker, May-Thurner etc) where patients are travelling to Germany to see one of a very small number of surgeons; it reminds me very much of what happened with CCI. I looked but couldn't find many case reports so difficult to get a handle on the kind of radiological evidence that's being used to justify surgery.

We seem to be hearing more and more of this stuff. The British Society for Gastroenterology group that are anti feeding support for 'functional' cases are weighing in with reference to hEDS. They don't mention vascular compression but I suspect it is all part of the same fashionable meme-talk.

I am pretty certain that EDS has nothing to do these people's problems. They may have ME/CFS, they may have other things we don't understand. Similarly I think it is vanishingly unlikely that vascular compression is responsible for a stream of cases going to German surgeons.

The people who are denying sensible treatment are the very people who feeding the false beliefs into the system.

 

[Jonathan Edwards]

I won't link that paper but the imaging was incontestable and the surgery was uncomplicated and definitive/permanently curative.

But that still gives us no proof even in that case that the compression was actually relevant. It was there but how do we know it caused the clinical problem? People get better after surgery for all sorts of reasons, as we know. You may be right but I have yet to see a single image relating to all this EDS talk that convinces me that an operation is justified. The only thing I know for a fact is that the rate of diagnosis of EDS has increased a hundred fold in the last twenty years and there is every reason to think that it is spurious.

 

[Jonathan Edwards]

pursuing unconventional treatments, especially surgical treatments, that are not recognised by mainstream medical authorities in your country could result in the parent and the child being diagnosed with FII/Munchausen's by proxy when they return home.

So maybe this is Munchausen by gastroenterologist?

 

[rvallee]

I'm seeing this more and more on social media. Risky. Leaving aside the issue of whether this is real or not, pursuing unconventional treatments, especially surgical treatments, that are not recognised by mainstream medical authorities in your country could result in the parent and the child being diagnosed with FII/Munchausen's by proxy when they return home.

Predictable, though. Authorities can't claim they don't understand that when they leave people stranded, they will turn to more dangerous alternatives. This is the crux of the entire opioid crisis. The flood of opioid prescriptions is what opened the door, but it's leaving people off with two problems that sealed the deal: they still have chronic pain but now with the added experience that it can be relieved. Then they turn to the black market, where nothing is controlled and deaths spiral out of control.

I would argue that the failed biopsychosocial ideology and the resulting stagnation in medical science is responsible for at least half of this. It's the same idea, really. You have pain? Suck it up. Don't look up. Overlaps precisely with gay conversion therapy. You're not gay/in pain, you just think you are.

It's the exact same problem underneath both: reactionary politics, all stick and no carrot. They're bad ideas with bad outcomes but once they're put into place, there is no turning back because those kinds of policies are enacted without any reflection or sound reasoning. Then it encourages to not look for evidence, or even to distort it, as reliable data points out the lie. And they'd rather stick with the lie than admitting they made terrible mistakes.

You can even compare how drug prohibition has sent trillions into the pockets of organized crime and tyrants with how this obsession with magical "mind-body" powers is enriching the wellness scam industry. They obviously don't compare in intent, but they are both the direct product of deliberate policies built on ideology.

 

[rvallee]

There was a comment, and others reflecting the same thing, in the Reddit thread that @SNT Gatchaman linked to, about how they hate working the GI ward because it's almost all, in their opinion, fake/functional zebra stuff. So this is a common issue, and there is no possible way to know about most outcomes because no one's checking.

I don't think they were that surprised with Maeve or anything like that. It's probably how it's commonly handled. I don't think anyone told the whole truth here. Only just enough for plausible deniability.

 

[Sid]

So maybe this is Munchausen by gastroenterologist?

Yes but the gastroenterologist will be just fine. It's the families, particularly the mothers, who will get brutalised by the state when something goes wrong.

 

[SNT Gatchaman]

But that still gives us no proof even in that case that the compression was actually relevant. It was there but how do we know it caused the clinical problem?

That was an isolated compression (vascular compressing gut), but I don't think it had anything to do with EDS. In that case it was the third part of duodenum being compressed in the aortomesenteric angle (SMA syndrome). I placed an NJT past the obstruction and the team progressed feeding for a period hoping to increase the retroperitoneal fat and bring the SMA clear. I think it worked a bit for some months but then recurred. There was no question what the problem was: food simply couldn't get through the duodenum and the stomach was massively distended. You could see this in realtime on fluoroscopy and the causative anatomy showed clearly on CT, confirmed at surgery. The surgeon re-routed the third part of duodenum through a window in the transverse mesocolon to bring it anterior the SMA. Problem solved.

These EDS cases seem to be more about ligament shifts impinging on vascular structures, eg median arcuate ligament compressing the origin of the coeliac axis (MALS). I've seen a couple of cases of coeliac axis stenosis, that weren't EDS, eg with Alagille syndrome. This is important in pre-liver transplant workup as they'll need vascular reconstruction to adequately supply the graft and prevent hepatic arterial thrombosis.

Interestingly in the series from Abdominal Arterial Anomalies in Children With Alagille Syndrome (2017, Journal of Pediatric Gastroenterology and Nutrition) most of those didn't relate to the median arcuate ligament.

In a recent study on vascular anomalies in patients with AGS, authors showed abdominal aorta stenosis associated in each case with celiac trunk stenosis and suggested the involvement of a compressive median arcuate ligament (14). From our results, we can confirm the frequency of celiac trunk stenosis. Our findings, however, suggested that MALS was not responsible for this stenosis. In addition, none of the children with AGS experienced symptoms of MALS; in our study, stenosis had different patterns and surgeons identified only 4 compressive median arcuate ligaments during LT. Furthermore, patterns of celiac trunk stenosis were similar to other abdominal arterial stenosis found in our study (eg, superior mesenteric artery) and they could not be the consequence of external compression. Nevertheless, celiac trunk stenosis predisposes to HAT decreasing the arterial blood flow (22,23).

 

[Nightsong]

Unable to sleep much tonight, I've been having another look through the literature and found this paper; two of the authors are associated with a number of the supposed EDS-III patients that have been heading to Germany for surgery. It's a case series of 169 patients who had 196 operations for compression syndromes in a single decade (2010-2020). Of the 169, 122 had been diagnosed with a hypermobility disorder. Only skimmed it, but a few relevant quotes:

We developed a sixfold measurement of the effect of the compression:
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.

As there were no data in the literature to rely on, we used these parameters 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.

Imaging (CTA, MRA, digital subtraction angiography [DS], venography) has prominence within the majority of publications related to CS. Such imaging is helpful in patients having been treated intra- abdominally for other causes and after failed attempts at treating CS, but in our experience, these forms of imaging are less sensitive and no more specific in identifying CS compared to USD examination in experienced hands. Also, they do not provide information about the grade of compression related to various body positions, which USD can.

The ultrasonographic methodology does not seem to be well-supported to me and they seem to be operating based on the symptoms & ultrasound findings alone.

In fact rare enough that we even published a case report back in the day (SMAS though not EDS-related as far as I recall). I won't link that paper but the imaging was incontestable and the surgery was uncomplicated and definitive/permanently curative.

Saw that one; there were much more clear-cut CT findings in that case & obviously very different clinically from the above.

The other worrying thing I've seen recently is that a small number of gastroenterologists are diagnosing "gastroptosis" (!) in EDS-III patients (there are a few scant reports - there's a mention in this presentation & this paper as well as the video we briefly discussed here). I sometimes feel we've learned nothing since the days of William Arbuthnot Lane...

 

[Jonathan Edwards]

These EDS cases seem to be more about ligament shifts impinging on vascular structures, eg median arcuate ligament compressing the origin of the coeliac axis (MALS).

I can believe that compression syndromes of one sort or another occur rarely as developmental problems in young children. But everything tells me that the use of this explanation in young adults with GI symptoms is likely to be 95% bogus.

I say that as much as anything because I saw so much of that bogus diagnosis in my own department over the years. The hypermobility industry has been almost entirely phoney.

Also, my experience of working on pressure gradients in connective tissue biology - like cartilage and joint lining - made me realise how easy it is to completely misinterpret the way these things workk. They can seem to work according to our intuitions but in reality the pressures simply cannot exist that way. In the 1980s we finally sorted out fluid flow dynamics in synovial joints by realising that all the equations were using the wrong terms.

 

[Leila]

I went to the specialist in Germany that might be mentioned here and I was diagnosed with May Thurner, Dunbar, Nutcracker, Pelvic Congestion by ultrasound.

The May Thurner I might believe because it was first seen by a different doctor with ultrasound and phlebography. The other diagnoses...I'm not sure.

The specialist who did the ultrasound sent me to a surgeon. I never went because his treatment would have been extremely risky and extensive vascular surgery. I later found a critical news article about this very surgeon having questionable methods.

Most of my symptoms subsided within two years so I don't think my compressions, if I do have them, were the reason. The pain in my left abdomen and leg I still have and it often does feel like my leg is a bit cut off from blood supply (May Thurner?), but also lots of endometrios in this area.

There's another trifecta of endometrios, vascular compressions and hEDS being discussed, also promoted by one particular gyn surgeon mostly online. Some patients get better, some stay the same and some get worse. A lot worse.

Like with CCI there's a handful of doctors most people go to, often with very different results and recommendations. This is from my memory of talking to other patients and I don't want to name names. But I would be very, very cautious..there might be over diagnosis and a bit of a filter bubble going on. That if you show up with certain symptoms to certain doctors you will always end up with the same diagnoses. What worries me most is the lack of aftercare if patients don't get better. Again, some do and there might even be a connection between these conditions but there's no data. Just surgeons.

 

[Leila]

@Nightsong

I was also diagnosed with gastroptosis, not by an EDS specialist but a radiologist specialized in dysphagia. She said it might be the reason for my delayed gastric emptying at that time.

Other doctors said it's just how a stomach can look like and as of today, I don't have many digestive issues anymore.

It's tricky to connect anatomical variants to certain health issues.