What is Nutcracker Syndrome?
Nutcracker Syndrome (NCS) is a genetic anatomic variant. Nutcracker Syndrome occurs when the aorta or another artery going to the intestine, often the superior mesenteric artery, compresses the left renal vein (the path blood flows from the left kidney back to the heart).
NCS is present at birth, and can exacerbate due to kinking blood vessels which affect blood flow. This can happen in a variety of situations, such as weight loss and surgery. Weight loss leads to blood vessels gathered closer than normal and surgery often shifts the abdominal contents. Due to growing obesity awareness, we are seeing more NPS cases due to massive weight loss. This is because when we lose weight on the outside of our bodies, we also lose it in other areas, such as the interior of the body, our faces,etc. Fat pads in our bodies often help us maintain appropriate distances between structures like arteries and veins, and the loss of this fat allows veins and arteries to come into close contact. When this happens, the artery almost always compresses the vein as in MTS, causing kinking of the vein
The worst Nutcracker Syndrome cases present themselves in childhood. These cases often include left sided kidney problems - given the very high severity of the vein obstruction.
When NCS presents in older individuals, it is usually due to a less severe form that presents slower.
Nutcracker Syndrome is a very common condition that is present in almost 50% of women with other forms of pelvic vein disease, such as May Thurner Syndrome (MTS) and Pelvic Congestion. This is because both MTS and PCS are anatomic variants in the structure of abdominal veins that predispose the patient to all three issues.
Nutcracker Syndrome Symptoms
The signs and symptoms of NCS are mostly silent until it the condition is quite severe. Because we have two kidneys, we are often not aware of kidney failure as the other one compensates. Symptoms that are early indicators of the disease include:
- Protein in the urine
- Microscopic blood in the urine (hematuria)
- Abdominal pain
- Other early indicators of early stage kidney malfunction
Flank pain and pelvic pain occur when the lesion is quite severe, but most often this is not the case.
Nutcracker Syndrome also commonly causes pelvic congestion syndrome in women and varicocele (enlarged testicular veins) in men due to the backing up of blood in branches of the kidney veins.
The condition can also cause problems with urine flow. These cases cause a backup of urine into the left kidney due to pressure of the distended vein on the urine drainage system (hydro-nephrosis). Hydro-nephrosis can cause issues like recurrent urinary tract infections and kidney stones.
Chronic fatigue, lack of energy, and hormonal disturbances can occur due to the blockage of the adrenal gland, which is also a branch of the left renal vein in many patients.
In rare cases, NCS can be a cause of medication refractory high blood pressure.
When the kidney is truly not doing well, often it leaks microscopic amounts of blood in the urine.
Nutcracker Syndrome Treatment
In general, if a patient has positive ultrasound findings of kidney vein destruction or blood pooling in the pelvis, as well as a history of abdominal pain and pelvic symptoms, we perform a diagnostic venogram.
Venogram treatment of nutcracker syndrome is usually performed under local anesthesia, and allows doctors to clearly localize the anatomy, cause, and severity of blood pooling in the pelvis, and other associated lesions such as nutcracker syndrome. We are often able to diagnose NCS in the initial diagnostic test and we keep the patient awake preferentially so that they can understand the anatomy and why we perform these tests. In our experience, this process helps tremendously with patient satisfaction.
In almost all cases of nutcracker syndrome, pelvic congestion syndrome (PCS) or varicocele exists as well. Typically we treat the PCS first, as that is usually the cause of the patient’s symptoms.
Next, we discuss a test called IVUS or intravascular ultrasound which is typically performed at a second visit. During the ultrasound we see precisely how narrow the kidney vein is, and determine whether to treat it. We can also assess kidney function which can sometimes be useful in helping us to decide whether to treat, and how urgent it is. In most cases, radiologic evidence of NCS is not symptomatic, and does not require treatment.
Determining Whether to Treat Nutcracker Syndrome
At NYC Surgical Associates, we evaluate a number of factors to determine whether to treat a patient’s Nutcracker Syndrome.
- At least 3 episodes of hematuria (blood in the urine at 3 separate occasions). We do this to avoid false positives as most people will have blood in their urine at some point, usually from heavy exercise.
- A significant renocaval gradient (measured at venogram) that demonstrates a significant pressure difference between the kidney vein and vena cava.
- Severe narrowing of the left kidney vein on venogram and ultrasound.
- Absence of other causes of hematuria such as high blood pressure and diabetes.
- Patient desire to treat their NCS.
Nutcracker Syndrome requires treatment in most cases, although there are exceptions. In some cases we will delay treatment for other reasons, like in a woman expecting to have more children, which may put pressure on an endovascular stent.
Nutcracker syndrome treatment is very safe, benign, with almost no long-term consequences or lifestyle alterations needed.
Alternatives to Stenting
Alternatives to stenting exist but are typically more invasive, such as autotransplantation of the kidney or renal vein transposition. In general, your surgeon should be pretty firm on the approach he has chosen for you as stenting can at times make other surgeries slightly more challenging, however we have a great deal of experience with this at NYC Surgical Associates, and have never had an unsuccessful stent procedure.
NCS Recovery and Aftercare
After NCS treatment (usually immediately) there is a vague feeling of mild pain in the treated area. This is typically a result of the stent stretching the vein and the muscles it lies upon. Pain with typically resolve over several days.
As in every stent procedure, your surgeon will prescribe an anticoagulation regimen based on how severe the narrowing was. All patients are placed on aspirin, while in some special instances the physician may prescribe another medication to keep blood from clotting inside the stented area temporarily. Clots can occur because the stent shortly stretches the inside of the vein, causing microscopic tears. These tears make the blood more likely to clot for a short time interval immediately after stent placement. This risk typically goes away inside of a month.
If your stent was placed for deep vein thrombosis or clotting, it is possible you will need to remain on blood thinners permanently. If you have formed a clot already, it will make future procedures more complicated, and the outcomes less positive.
After treatment for NCS, the prognosis is excellent with very few recurrences, even long term. If the condition is corrected prior to severe kidney damage, often the kidney will recover to some extent, and progressive decline of kidney function in most cases will be stopped.
Risks of Treatment
With any medical procedure, there is a theoretical risk of bleeding as well as infection. In addition, whenever endovascular procedures are performed, such as for NCS, some unique but very rare risks include the possibility of malposition or movement of devices, as well as damage to blood vessels and organs.
In our experience, NYC Surgical Associates’ complication rate is far below most major academic institutions and the national average of less than 1 percent. Overall, the risk of these procedures is far lower than conventional surgery, but still present, and must be discussed.
Risks of Not Treating NCS
The risks to not treating Nutcracker Syndrome are progressive kidney damage and the risk of complete occlusion of the kidney vein by a clot. Once this happens, the prognosis becomes much worse, and the chance of complete resolution of the problem is far less. This makes the decision of when to treat more severe cases somewhat complex.
Very often, we delay treatment in patients. As mentioned above, this may be due to factors such as age, pregnancy, and overall kidney function in the affected kidney. These situations require close follow up with the patient and a discussion about preferences.
The key thing to remember with venous disorders such as NCS, is chronic issues have likely been present for decades. This being the case, no rushed treatment is necessary and patients have time to make a logical decision. It is often best to address NCS when it is diagnosed, but certain factors may delay treatment safely.
Nutcracker Syndrome Treatment Cost New York & New Jersey
NYC Surgical Associates is committed to helping our patients receive the best care possible. That journey always begins with a medical consultation.
Cases of Nutcracker Syndrome are unique and no two are exactly alike. Consultations enable our specialists to understand each patient’s situation and determine the best NCS treatment for them.
Prior to booking a consultation, our team will collect your insurance information. From there we will determine if our providers accept your specific plan and lay out options for your care.
If we do not accept your insurance, we will inform you of any out of pocket consultation fees. Either way, a doctor must see you before we can determine what treatment will entail, from both a medical and cost standpoint.
