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Nutcracker Syndrome (Pelvic Congestion Syndrome) – Linked with POTS and childhood CFS

Discussion in 'General Symptoms' started by kangaSue, Jan 16, 2017.

  1. kangaSue

    kangaSue Senior Member

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    A couple of things turned up in researching the links between gastroparesis, Superior Mesentery Artery Syndrome (SMAS) and Nutcracker Syndrome (a.ka, Nutcracker Phenomenon or Pelvic Congestion Syndrome) that will likely be of interest for some with Chronic Fatigue Syndrome.

    Gastroparesis is something that can occur as a result of having SMAS. SMAS can result as a consequence of having Nutcracker Syndrome (NCS) as both of these can have the same cause of onset, loss of the retroperitoneal fat pad between the aorta and the superior mesentery artery causing compression of both the duodenum and left renal vein. Compression of the left renal vein can disrupt the outflow of blood from the kidney into the inferior vena cava which can then instead reflux into the left ovarian vein which will be visible in an abdominal CT scan, often because the ovarian vein is grossly enlarged. It affects mostly women but guys can get this and have problems in the left testicle.

    I've been diagnosed with Gastroparesis and Nutcracker Syndrome, SMAS is still in debate but it fits for my clinical picture (I don't identify with having ME/CFS though). I don't get chronic fatigue and only have mild Orthostatic Hypotension but it was interesting to find that, as well as there being known atypical presentation of NCS (including no compression of the left renal, no blood and/or protein in the urine, being totally asymptomatic), a number of papers mentioned that severe POTS or OH is a symptom that some people also have with NCS, along with chronic fatigue too.

    I can't find any statistics on the frequency of this but in reading about NCS in various forums, anecdotal evidence does indicate POTS to be a known co-morbidity in some people. A post by someone in one forum who also had severe POTS said that their Neurologist had mentioned to them that they had encountered several other patients with both POTS and NCS at their clinic.

    The other aspect that cropped up in looking into this is that some papers suggest paediatric Chronic Fatigue Syndrome can also be related back to having NCS in also having symptoms of having orthostatic hypotension, postural tachycardia, or other autonomic dysfunction symptoms and chronic fatigue. Most of the papers are fairly dated now so again, not a lot of statistics are around on the subject.

    Some children just grow out of this so it begs the question, does that maybe leave them open to developing this again later in life?

    Patients' age for NCS can range from childhood to the seventh decade of life, but most symptomatic patients are in their second or third decade of life, it's more commonly reported in women and there's looking to be a second peak of NCS in middle-aged women, a somewhat similar pattern to ME/CFS too.

    Because NCS itself can be totally asymptomatic, it is something to keep in mind with having POTS or issues of gastrointestinal disturbances and/or unexplained abdominal/pelvic pain.

    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878259/ Nutcracker Phenomenon and Nutcracker Syndrome
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5101106/ Unusual Manifestation of Superior Mesenteric Artery Syndrome in a Female Patient: A Case Presentation
    https://www.ncbi.nlm.nih.gov/pubmed/10749295 Does severe nutcracker phenomenon cause pediatric chronic fatigue?
     
    Last edited: Jan 17, 2017
  2. halcyon

    halcyon Senior Member

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    Interesting. I do have nutcracker phenomenon but don't seem to have NCS.
     
  3. kangaSue

    kangaSue Senior Member

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    Which goes to the heart of what I am pointing out here. The two terms are used interchangeably in the literature but you will tend to be dismissed here as it contributing to serious symptoms if you don't have evidence of left renal vein entrapment. The only common symptom that I am finding with everyone where it can result in there being some significant issues with this an enlarged left ovarian vein but some people with this are asymptomatic too.

    In hindsight, technically, I actually have Nutcracker Phenomenon too as the left renal vein is only "of reduced calibre" but I have a "massively enlarged let ovarian vein" which different doctors have referred to as both Nutcracker Phenomenon and Nutcracker Syndrome.

    My difficulty is getting the GI motility specialist to agree that this is also causing all my GI dysfunction from SMA Syndrome when it is argued by them that I don't have this on the grounds that I no longer have an issue with vomiting (cured myself from vomiting by other unrelated measures) and I don't have evidence of compression of the duodenum so I don't pass the "classic text book presentation".

    According to individual case studies, there are many atypical presentations so I have a site in mind where I would like to insert that classic text book, somewhere that might have a significant impact on a certain specialist's colonic motility.
     
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  4. Sea

    Sea Senior Member

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    In the context of orthostatic intolerance the abbreviation NCS usually stands for Neurocardiogenic Syncope. So when reading and discussing about NCS it will be necessary to double check which condition is actually being referred to.
     
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  5. halcyon

    halcyon Senior Member

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    I wonder how many people have it and don't even know it. I've had at least 7 CT scans in the last 16 years (I have an issue with chronic kidney stones as well) and only one single scan report mentioned it.
     
  6. kangaSue

    kangaSue Senior Member

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    Several of my CT scans make no mention of this eitherso I guess it sometimes depends on what the CT was ordered to investigate.

    Poring over various discussion forums, it's obvious that this is often just an incidental finding during investigations for other issues, and often, little emphasis is placed on the relevance of it when symptoms are relatively mild when compared with classic text book presentation.

    I can see a reason why I only have mild OH here when others have debilitating POTS from it. I think it could come down to the degree of compression of the left renal vein, the less the restriction, the less severe OH. It's difficult to get a firm conclusion on it though with the interchangeable terms used by everyone.

    When there is full compression of the left renal vein (vein entrapment), it can affect the renin-angiotensin-aldosterone system balance in that, when the blood flow is "backed up" into the left kidney, the renin output from that kidney is increased but then the right kidney will compensate and reduce its output of renin so these extremes cancel each other out to show up as a normal renin result in blood tests. Aldosterone is influenced by the renin system but how it is affected then is something I haven't got into in any depth yet.
    http://www.bhj.org.in/journal/2008_5001_jan/download/retro-aortic-left-renal-vein-hypertension.pdf

    There is a kidney-brain link related to renin-angiotensin too from an ischemia-reperfusion aspect so maybe that explains brain fog and cognitive symptoms for some people too.

    https://www.ncbi.nlm.nih.gov/pubmed/28030955
    [CONCLUSIONS:These data suggest that the renal and cerebral renin-angiotensin axes are interlinked by a reno-cerebral sympathetic reflex that is activated by ischemia-reperfusion, which contributes to ischemia-reperfusion-induced brain inflammation and worsening of the acute renal injury.]
     
    Last edited: Jan 18, 2017
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  7. kangaSue

    kangaSue Senior Member

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    Actually, that could tie in with the benefit of taking captopril mentioned in reply #18 in this thread http://forums.phoenixrising.me/inde...-patient-natural-therapies.34683/#post-541322

    Also in the same context is the role of the renin-angiotensin system and renal sympathetic nerve system.
    https://www.ncbi.nlm.nih.gov/pubmed/25999788
     
  8. adreno

    adreno PR activist

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    I've noticed that certain exercises involving the core musculature can set off a crash for me, e.g. planks, hanging leg raises etc. I also suffer from pelvic pain/tightness. Hmm.
     
  9. GalaxiiGrl

    GalaxiiGrl

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    You know that phenomenon when you first encounter a word or term that you've never heard of before in your entire life, and then it shows up again shortly after in a seemingly unrelated way?

    I just had an MRI on Thursday to check for Median Arcuate Ligament Syndrome (MALS), which is also common in patients with POTS and sounds similar to SMAS/ Nurtcracker Syndrome. It's so mind-boggling that I had never heard about Nutcracker Syndrome until a few days ago when I saw it referenced on a website with information about MALS, and now I'm seeing it mentioned here for the second time in just a few days. Perhaps this is a sign that I should learn more about it!
     
  10. kangaSue

    kangaSue Senior Member

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    The symptom set for both MALS and SMAS are very similar, the only major difference is the anatomical feature being compressed. I wasn't looking into it specifically but haven't seen reports of MALS and Nutcracker Syndrome (NCS) occurring together, were you just meaning that NCS is also mentioned along with the other two as a vascular compression syndrome too?

    Definitely so. Something to be wary of for anyone who might have MALS or SMAS is that one of the complications of either one can be microvascular intestinal ischemia (non-occlusive mesenteric ischemia, NOMI). It is often mentioned in the medical literature but not a lot of emphasis is placed on it and I am of the opinion that it is a largely unknown or under reported symptom, mainly because there is no definitive test to establish you have it so you might only find out you have this for sure if and when the collateral blood flow to the bowel becomes insufficient to prevent damage to your bowel mucosa, which can go on to cause bowel necrosis. This doesn't appear to be the case too often though with this sort of chronic ischemia, not like the more insidious Acute Mesenteric Ischemia which comes on suddenly.
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2778114/ (see the references to NOMI in this paper)

    I have only seen a couple of meds suggested as a treatment for this, nicorandil, a cardiac anti-anginal nitrate which I use, or cilostazol which is another type of peripheral artery vasodilator used for Intermittant Claudication.
     
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  11. adreno

    adreno PR activist

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    I've been checked for blood in the urine several times and nothing has showed up. Wouldn't this almost certainly rule out NCS?
     
  12. GalaxiiGrl

    GalaxiiGrl

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    Yes, that's what I meant. :)

    Thank you very much for this information! I definitely have some reading to do.

    ETA: It's so disheartening that most doctors would never, ever think to check for these things. I had to bring up the question of MALS with my cardiologist after doing my own research, and while she was vaguely familiar with it, she had to do a bit of research on it herself. Thankfully she is a wonderful doctor, and is always open to considering my input on my conditions.
     
    Last edited: Jan 22, 2017
  13. kangaSue

    kangaSue Senior Member

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    Absolutely not the case. A large part of the problem in getting this diagnosed is that there is a wide variation in symptoms between cases. It's quite common in the literature for there to be a total absence of blood in the urine or it might be just microscopic. Protein in the urine is a "classic" symptom too but often not present as well. You don't even need to have a physical finding of actual compression of the left renal vein but can be deemed to be symptomatic if you have evidence of enlarged left ovarian vein (or left varicocele for the guys).
     
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  14. adreno

    adreno PR activist

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    Okay. This sounds like a nightmare to get diagnosed, as doctors will be dismissive if nothing is found in the urine. Are there any specialists who have a better chance of diagnosing this, or is it left to GPs?
     
  15. kangaSue

    kangaSue Senior Member

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    Yeah and don't I know it.

    It gets a bit messy where specialists are concerned too, it crosses the boundaries of Gastroenterologists, Gynecologists, Urologists, Nephrologists or vascular surgeons.

    My G.P. is of the opinion that a vascular surgeon is the best option and is referring me to the same but he knows that I've already been blown off by the G.I., Gyne and Urologist as to there being any link with having chronic GI dysfunction too.

    In their defense, with our mix of public and private healthcare systems, none of them have seen every test result that I have had, only me and that's only from having the diligence to apply for copies of past results after finding out they don't tell you all the details that are in some results, like that I had Osteopenia for 18 months before I found out I now have Osteoporosis.

    It's a call here that the G.I could have made on what has been available to her, if only she wasn't blinded by the insistence that you need to conform to only the typically known course of development.
     
    Last edited: Jan 25, 2017
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  16. kangaSue

    kangaSue Senior Member

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    @GalaxiiGrl I had the same experience in trying to show that I had chronic intestinal ischemia as part of the mix with GI dysmotility. The usual presentation is acute onset and more often with an occlusive cause but mine was neither.

    Having an epigastric bruit and an elevated mid superior mesentery artery blood flow velocity was the only reason I kept pursuing it. A lot of those with a compression syndrome will have an epigastric bruit, MALS included. I never had a doctor listen to my bowel sounds for sign of this until I learned to ask asked for it to be done.

    There is a lot of good information about compression syndromes in this paper;
    http://pubs.rsna.org/doi/full/10.1148/rg.341125010
     
  17. ScottTriGuy

    ScottTriGuy Stop the harm. Start the research and treatment.

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    Thanks @kangaSue - replying on this thread since it seems more appropriate for your question - I had to google a number of terms to sort of understand - quite interesting stuff you found.

    No, the vascular ultrasound did not look at my GI, it was only of my arms and front shoulder area. The technician said that my right side was more symptomatic (which is probably why I felt mild nausea in having to hold my right arm above my head for perhaps 20 seconds). She said that previously they'd send patients to the Cleveland Clinic for surgery, but they had just opened their own vascular surgery last week. Without saying so directly, she seemed to be implying I would need surgery.

    However, on the way to the hospital for the ultrasound at 7am I very nearly didn't make it because I was so light-headed. I was feeling a little unstable when I woke up, had some smoothie, then walked the 3 minutes to the streetcar and that made me worse. I had strong urges to lie down on the floor of the streetcar - it felt like the blood was pooling in my abdomen - and my right arm went slightly numb - by slouching, I tried to get as horizontal as possible without actually lying on the floor.

    I was hesitant to get on the subway for fear of fainting and for the first time asked somebody to move out of a reserved handicapped seat so I could sit down - slouching really, as I tried to get as horizontal as possible without actually lying on the subway car floor.

    By the time I got to the hospital at 7:45 I was feeling pretty normal. I was hoping the ultrasound would pick up whatever was going wrong but now my symptoms were gone. I decided to try to induce them by picking up a chair and lifting it over my head. But no symptoms of light-headed, urge to get horizontalness. I tried twice more but nothing. Perplexing.

    Yet in Dec when I threw my small suitcase into the backseat of the car it made me a total mess and, after spending 10 minutes on the floor in the middle of departures, I got a wheelchair for check in etc. I now wonder if its a specific lifting and twisting movement that induces the symptoms because I cannot shovel even a bit of snow.

    When I think of my recent experiences of light-head, urge to be horizontal, blood pooling in abdomen symptoms, they have all been very soon after breakfast (on days I had to get up earlier than usual) and exacerbated by 'activity' (walking, driving).

    Other things that may rule in / out Nutcracker Syndrome:

    I have trace blood in urine for undetermined reason
    I urinate a lot
    I don't have left testicle or vein enlargement
    Normal blood pressure when they take it
    I have an empty sella
    I had pheochromocytoma symptoms in the past during a 2 month period pre-ME (brief sensation in left upper abdomen, followed immediately by waves of adrenaline. I described it as having a panic attack without emotional panic)
    I don't have fatigue, brain fog, pain.
    I can only walk for about 10 minutes on flat ground or walk up 1 flight of stairs at a time or I will get symptoms.
    I do not get delayed malaise (anymore), it is pretty much immediate.
    I now love the heat and sun and am extremely sensitive to the cold.
    Normal thyroid labs but am doing better (less cold sensitivity) on synthroid.

    So I'm not sure I have Nutcracker, but with the variety of manifestations, it wouldn't be a total surprise.
     
  18. kangaSue

    kangaSue Senior Member

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    @ScottTriGuy
    Hard to get a line on things sometimes where there's multiple seemingly unconnected issues going on. It would be nice to be text book abnormal though, wouldn't it?

    I see a lot of mention here about a feeling of blood pooling in the abdomen but I can't say that it is something I can relate to. I don't get light headed much either even with having low b.p. and my urinary output is on the other end of the scale with very infrequent visits to the loo but that might be explained by having diarrhea instead.

    Having traces of blood in the urine for undetermined reason would give rise to checking this out anyway, just in case. I was surprised to learn that there was a connection with this being able to cause to POTS or OH. Having such a wide variation in symptoms doesn't help the cause much.
     
  19. ScottTriGuy

    ScottTriGuy Stop the harm. Start the research and treatment.

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    Oh yeah.

    Thanks for bringing Nutcracker to my attention - I've just started seeing a functional doc who seems very open so I will float the idea by him.
     
  20. Kenjie

    Kenjie Senior Member

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    I've had blood showing up in urine tests for last two or so yrs although Noone is ever concerned about it. Believe me I have asked.

    My mum also tells me when I was 2 or 3 yrs old she would take me to hospital cos I wold get very hot and have pre-convulsions... I never knew that only finding out since I came down with this suspected autonomic syndrome..
     

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