Nutcracker Syndrome

What is Nutcracker Syndrome?

Nutcracker syndrome refers to the left renal vein being compressed, most generally between the aorta and the mesenteric artery which is superior. It can lead to renal venous hypertension which results in rupturing of thin-walled veins into the collecting system with resultant hematuria. It has impaired blood outflow which is often along with distension of the vein’s distal portion. The nutcracker syndrome is a rare condition. The first case was reported in 1950 by El Sadr and was named by De Schepper.  The name is derived from the detail that superior mesenteric artery and abdominal aorta looks like a nutcracker crushing a nut.

This is a rare condition. Its first case was reported in 1950 by El Sadr and was named by De Schepper.  The name is derived from the fact that superior mesenteric artery and abdominal aorta appear to be a nutcracker crushing a nut.

Nutcracker Syndrome Symptoms

The classic symptoms of this syndrome include left flank pain with gross or microscopic hematuria. Pelvic congestion symptoms such as pelvic pain and dyuspareunia are also noticed. If the left gonad gets emptied through the left renal vein it can too lead to testicular pain in men and quadrant pain in the lower part in the case of women. Nausea and vomiting may also be seen because of compression of splanchnic veins.

Picture of Nutcracker Syndrome

Picture 1 – Nutcracker Syndrome

Formation of Varicocele and varicose veins in the legs is another unusual manifestation of NCS. In some cases, there may be development of Myelitis and syringomyelia. Other symptoms may be chronic pediatric fatigue and gastrointestinal symptoms but exact reason in these cases is not clear.

Nutcracker Syndrome Diagnosis

The disorder is diagnosed in the following ways:

  • Computed tomography
  • Left renal venography
  • Abdominal ultrasonography

Nutcracker Syndrome Differential Diagnosis

The differential diagnosis of the disease involves telling its symptoms apart from those of:

  • Renal stones
  • Loin pain of hematuria syndrome
  • Genitourinary malignancy

Nutcracker Syndrome Treatment

When symptoms starts showing up, treatments should be started immediately. Treatments depend on the aggressiveness of the condition and symptoms. They treatments are endovascular stenting, re-implantation of renal vein and embolization of Gonadal vein.

Medication is prescribed only to patients who suffer from pelvic congestion. Pelvic congestion syndrome can be treated with:

Image of Nutcracker Syndrome

Picture 2 – Nutcracker Syndrome Image

  • Medroxyprogesterone acetate
  • Goserelin acetate
  • Micronized purified flavonoid fraction

There are several surgical teuchniques are there to treat this syndrome. These include:

  • LRV transposition
  • LRV bypass
  • SMA transposition
  • Auto transplantation

Open surgical Intervention may also relieve symptoms. However, the results of such a surgery may not be impressive in patients with low pressure gradients. Hematuria gradually decreases after surgery although low pressure gradients may still be present.

References:

http://radiopaedia.org/articles/nutcracker_syndrome

http://www.phlebolymphology.org/2009/07/nutcracker-syndrome/

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878259/

http://en.wikipedia.org/wiki/Nutcracker_syndrome

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