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 have no idea what is being shown in this image beside my kidney.|
Going down the Nutcracker Syndrome and Nutcracker Phenomenon rabbit hole has led to some interesting discoveries.
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
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.
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.