The Nutcracker Suite Revisited

  • Wed, 12/7/11 - 11:54am
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A 57-year-old woman presented with severe unrelenting pelvic pain, which had been occurring for years and led to her having a hysterectomy for dysmenorrhea and fibroids. Subsequently she had an oophorectomy on the left due to severe varicoceles. Despite these surgeries, her symptoms persisted and worsened through the years. She had no kidney or urinary symptoms. Her work-up included a CT scan that demonstrated the left renal vein coursing between the SMA and the aorta. She also had significant varicosities within the pelvis. Left renal venography showed significant compression of the vein and a large engorged and tortuous gonadal vein. One consultant recommended surgery and she presented to the Phoenix Heart Center for another opinion. After reviewing the images and discussing the options we decided to proceed with coil embolization of the gonadal vein.

The gonadal vein was easily accessed via the groin with a glide catheter (Terumo Medical) and an angled glide wire. It was with great difficulty that the catheter was advanced far enough to occlude the vein proximal to the collaterals that were present. During this process the vein was dissected and some extravasation of dye was noted in the retroperitoneum. The patient remained hemodynamically stable yet the vein no longer had any identifiable flow. Coils were not placed. The patient was discharged later that same day. On follow-up 2 months later, her symptoms had all but gone away. The nutcracker syndrome is quite a rare condition that occurs because of the compression of the distal segment of the left renal vein (LRV) between the superior mesenteric artery (SMA) and the aorta (also called left renal vein entrapment). El Sadr reported the first case in 1950, but De Schepper named it. This syndrome needs treatment when symptoms are disabling. Compression of the distal part of the LRV is generally due to the superior mesenteric artery arising from the aorta at an acute angle.This syndrome occurs most often in women in their third and fourth decades of life. Symptoms can range from left flank pain, chronic pelvic pain, dyspareunia, dysmenorrhea, and hematuria. The symptoms seem to be positional and are often worse with lying supine and standing due the weight of the bowels.The diagnosis can be made by contrast enhanced CT scan of the abdomen (Figure 1). Duplex scanning can also be helpful.

Figure 1. Contrast enhanced CT scan of the abdomen.

 

 

 

Treatment of the vein compression should only be entertained in symptomatic patients or those with disabling symptoms. There are several different approaches to this disease. Surgery has been the gold standard to date. However, there have been very few controlled trials due to the scarcity of patients. Endovascular approaches have been explored but none have proven completely effective. These include stenting of the renal vein as well as coil embolization of the enlarged gonadal vein. This can then relieve the pelvic congestion and the associated symptoms.

This case demonstrated the difficult nature in attempting coil embolization; however, the eventual dysfunction of the gonadal vein resulted in a significant reduction, if not elimination of, the patient’s symptoms.

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