Happy Holidays: Pefect Time for a Nutcracker Not-Suite

A while back, one of my readers mentioned something to me called Nutcracker Syndrome and recommended looking into it as a possible cause for my flank pain. With every new document I read, I became more and more convinced I needed to be tested for the syndrome. The description of the pain was exactly as I described my flank pain: writhing, hot, twisting, deep. Also, hematuria is a symptom, meaning blood in the urine with no known cause. I have hematuria. The only problem, Nutcracker Syndrome usually occurs on the left side of the body, and my flank pain is on the right side. Then, I found two case studies of a man and a woman, both of whom had Inverted Nutcracker Syndrome, meaning on the right side. I jumped for joy at the thought of a possible diagnosis for my four-year-long adventure in the land of flank pain. I took all of the studies and piles of paperwork to my Primary Care Physician, aka Amazing PCP, and he sent me on to the nephrologist (kidney doctor).

My nephrologist is awesome. Beyond awesome. Not only did he believe me when I told him about my symptoms, but, he agreed that it seemed like I could very well have Nutcracker Syndrome, complimented me on my research skills, and then tried to convince me to go to med school and become a doctor. Ha! I am happy helping people from my couch, when able, which is all I can do, but I greatly appreciated that he took me seriously. It is so nice working with a doctor that respects that I know my body better than anyone, and, in turn, that I know the doctor is smart. That way, we are both able to work together for the betterment of my health. It made for such a nice pairing.

About to get my IV line inserted at the hospital.

I went in to the hospital for the tests, a CT Angiogram of my pelvis and a CT Angiogram of my abdomen. Both of these were necessary to get a grasp on how my veins and arteries formed and how they enter and exit my kidneys. The test was easy, just an IV line for contrast and a quick jaunt in the CT machine. Twenty minutes later, I was back in my car and waiting to hear from my doctor about the results. Less than a week later, it was confirmed: I have Nutcracker Syndrome. But…here’s the catch: Nutcracker of the left renal vein, not the right. What?! So…now, it is confirmed that my veins and arteries are entirely messed up by my left kidney, but the nephrologist doesn’t think that could cause my right-sided flank pain. Oh no! Now, I have another rare and random diagnosis, and still no answers regarding my flank pain. Gah! I am frustrated to say the least; disappointment and anger also followed suit. I prayed for the diagnosis of Nutcracker Syndrome because I thought it would lead to a fix for my flank pain, but I forgot to specify which side of my body. Ha!

IV line ready for contrast when I get the CT Angio.
Bruising from the IV line.

Now, I know you are wondering what exactly Nutcracker Syndrome is, so here are all the details:

According to Wiki, Nutcracker Syndrome (NCS), aka Nutcracker Phenomenon, Renal Vein Entrapment Syndrome, and Mesoaortic Compression of the Left Renal Vein, is the compression of the left renal vein between the Abdominal Aorta (AA) and the Superior Mesenteric Artery (SMA). Basically, this means the left renal vein is being squished in between the Aorta and the SMA. The gonadal vein drains from the kidney, and because of the compression, my gonadal vein is dilated (enlarged, swollen), and they suspect reflux (fluid leaking backward) as well.

Here is an example; this is from my CT Angiogram:

I altered this image below (unknown source) to help you look at this from two different angles. My renal vein looks like the “squished renal vein,” it is being pinched in between the Aorta and SMA. From this image, you can see where they got the name Nutcracker, as the AA and SMA are like the handles and they are pressing in on the renal vein like a nutcracker would squish against a nut to crack it open.
Here are a few more images from my CT Angiogram:
I have no idea what is being shown in this image beside my kidney.
So what does all of this mean? Treatment is varied, depending on the patient, so I will have to wait and see what route we are going to take. It could be as simple as just leaving it alone and monitoring it. I might need to have the renal vein stented. Or, I may need major surgery to redo the actual anatomy. In order to determine the best route of treatment, my nephrologist is sending me to the best kidney and urology hospital in the country, The Cleveland Clinic’s Glickman Kidney and Urological Center. I will be seeing a doctor that specializes in Nutcracker Syndrome, and he will help me determine what course of action is needed. I am still holding out hope that somehow this will be related to my right flank pain and that treating this will ease the pain, or that the doctor has another idea of what might be causing my pain. Either way, I am really looking forward to this appointment so I have some answers. Waiting is hard, especially when you know there is something wrong with you and you don’t know how to fix it.
I feel very confident in the hospital I am headed to. For 2012/2013 they are ranked as the #4 hospital overall nationwide, and the #1 hospital for Kidney and Urological Disorders, and recognized globally for their kidney care:

Going down the Nutcracker Syndrome and Nutcracker Phenomenon rabbit hole has led to some interesting discoveries.

Dinet reports a link between Postural Orthostatic Tachycardia Syndrome (POTS) and Nutcracker.

Nutcracker has reportedly produced POTS symptoms in some individuals. Nutcracker phenomenon (NC) is the congestion of the left renal vein due to its compression by the aorta and the superior mesenteric artery (Takahashi, Ohta, Sano, Kuroda, Kaji, Matusuki & Matsuo, 2000). The main and common findings of one study on pediatric NC patients were chronic fatigue associated with orthostatic hypotension and/or postural tachycardia (Takahashi, Ohta, Sano, Kuroda, Kaji, Matusuki & Matsuo, 2000). The authors of this study point out that “the originally reported symptom of NC is renal bleeding. However, reported ‘renal bleeding’ patients, including ours, have no complaints of chronic fatigue and our ‘chronic fatigue’ (NC) patients have no renal bleeding”. Some of these patients did report fibromyalgia type pain. Some patients had proteinuria,others had no urinary abnormalities.

The authors of this study explain the various ways in which NC might affect autonomic function: First, severe congestion in the kidney may cause the expansion of the renal venous bed, which would affect the renin-angiotensin system. Secondly, severe congestion in the adrenal medulla, which is innervated by sympathetic nerves, may disturb a complex set of central neural connections controlling the sympathoadrenal system. On the other hand, overproduction or night retention of catecholamines might be responsible for the various symptoms of pediatric chronic fatigue syndrome (Takahashi, Ohta, Sano, Kuroda, Kaji, Matusuki & Matsuo, 2000). The nutcracker phenomenon occurs in adults as well as children. Transluminal balloon angioplasty has successfully been used to treat compression of the left renal vein between the aorta and superior mesenteric artery (Takahashi, Sano & Matsuo, 2000).

The methods used to diagnose nutcracker phenomenon include Doppler US, MRI and three-dimensional helical computed tomography. Dr. Takahashi (personal communication, September 8, 2002) explains the procedures for testing as follows: Conventional ultrasound requires patients to be examined for left renal vein obstruction in 4 positions: supine, semisitting, upright and prone. Nonvisualization of the left renal vein lumen or absence of the left renal vein wall between the aorta and superior mesenteric artery is regarded as signifying left renal vein obstruction. Doppler color flow imaging can be used to locate a blue-colored blood stream flowing to the dorsal direction. This is a collateral vein flowing from the left renal vein into the paravertebral vein. With MRI, oblique coronal images along the left renal vein, and also axial images, are recommended to visualize the collateral veins around the left renal vein. Read more

NCBI reports a link between childhood chronic fatigue, POTS, and Nutcracker.

Does severe nutcracker phenomenon cause pediatric chronic fatigue?

Background: In the past five years we experienced 9 fatigued disabled children who were intermittently or persistently absent from school.

Patients: They had been suspected to be burdened with psychosomatic disorders, having orthostatic hypotension, postural tachycardia, or other autonomic dysfunction symptoms.

Results: Investigating the cause of moderate orthostatic proteinuria in some of them, we found by chance severe typical nutcracker phenomenon (NC), which was present in all 9 children complaining of chronic fatigue.

Conclusion: Their symptoms filled the criteria of childhood fatigue syndrome or idiopathic chronic fatigue (CFS/CF). An association between severe NC and autonomic dysfunction symptoms in children with CFS/CF has been presented.

There’s a reported link between CCSVI (Chronic Cerebrospinal Vein Insufficiency) and Nutcracker Syndrome:

From the nutcracker-phenomenon of the left renal vein to the midline congestion syndrome as a cause of migraine, headache, back and abdominal pain and functional disorders of pelvic organs.

Dr. Diana Driscoll reports a link between CCSVI (Chronic Cerebrospinal Vein Insufficiency) and Ehlers-Danlos Syndrome (EDS):

On January 2nd, I head up to The Cleveland Clinic, so I will keep you posted on the progress. Until then, I will be sitting on my heating pad and popping my Norco. Hope you all have a wonderful holiday(s)!


  1. Anonymous

    One reason the pain can be on the right is if you have your ovaries. Nutcracker syndrome will cause Pelvic congestion syndrome if you have your ovaries and thus cause right sided pelvic, abdominal, back pain, etc. When this happens you may have left sided pain also but the right side may hurt even worse which will make the left side not noticeable. ALSO, (I suspect the following more so in you) Right sided pain can also be caused by Nephroptosis (kidney dropping when you go upright.) It also causes hematuria. It is not uncommon to find Nephroptosis (especially on the right side) along with Nutcracker syndrome on the left especially in people that are slim or underweight. Is your pain worse when you are upright and gets better the longer you lay down? Nephroptosis can be diagnosed with an UPRIGHT combined with a laying down IVP study to see how far the kidney(s) drops when upright. This is why I know so much about these conditions. I have Nutcracker syndrome, Pelvic congestion syndrome, Superior messenteric artery syndrome, (which is all not uncommon to happen together) Nephroptosis of both kidneys (right much worse than left which is common), gastroptosis (stomach drops when upright) and enteroptosis (intestines drop when upright). All of the ptosis problems have to be tested for by upright radiology studies. These organs go back into place when lying down so CT's, MRI's etc will no show ptosis of organs. If a person with nephroptosis of the right kidney has had an upright and lying down regular flat panel x-ray sometimes you can compare them and find that the right kidney is actually laying on it's back on top of the intestines and pushing the intestines down in the upright x-ray. That's how I found my nephroptosis of my right kidney after 4 years of radiologist missing it 5 times on 5 sets of upright and laying down x-rays. I then asked my doctor for and upright and lying down IVP study and that test confirmed the nephroptosis. I also have neurological problems from the waist down from the Nutcracker syndrome due to the venous blood from the left kidney re-fluxing into my spine and around my spinal cord. This was confirmed by venogram. Hope something here helps you out.


  2. Anonymous

    Also, I should have noticed before I wrote my first January 17th comment that you also have EDS. Ptosis of organs such as the kidneys, stomach, intestines.etc are common in EDS. I bet with the correct upright radiology test it will be found that you at the least have Nephroptosis of your right kidney. In case you do some searching for EDS and these ptosis problems, Visceroptosis is also a term used when a person has many organs that drop and I have found Visceroptosis used when found in EDS patients.


  3. Nicole

    Does anyone have NC and Superior Mesenteric Artery Syndrome (these came after 1.5 years of symptoms for my daughter. Originally after 4 months of SMAS symptoms ( but we had never heard of this until a few months ago) my daughter was diagnosed with Othostatic intolerance. Looking for specialists that can handle these complicated medical cases. Anyone know of any close to Wisconsin? But willing to travel if that is best for her. Thanks


  4. Anonymous

    Nicole, My son has also been diagnosed with both after two years of dealing with the issue. We are in Arizona and as far as I know nobody I have talked to has ever seen SMAS other than in the textbooks.Shannon


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