Systematic review of endovascular therapy for nutcracker syndrome and case presentation




Abstract


Background


The left renal vein (LRV) entrapment syndrome is a rare condition. Here, we present a 22-year-old female presenting with recurrent nausea, vomiting, weight loss and evidence of the LRV compression by the superior mesenteric artery and the abdominal aorta. Hemodynamic assessment confirmed a pressure gradient of > 3 mmHg between the LRV and the inferior vena cava. A self-expandable stent was then deployed in the LRV. Subsequent clinical follow-ups ensure full resolution of the patient’s symptoms.


Methods


Multilingual search was performed in PubMed, Google scholar, Scielo, Korea Med and EMBASE with the medical subheadings “nutcracker syndrome”, “nutcracker phenomenon” and “compression vein syndrome” from January 1983 to September 2013.


Results


Review of the literature exhibited a plethora of individual case reports (291 citations). Importantly, few retrospective case series [5] comprising a total of 157 patients included successful endovascular interventions.


Conclusion


Endovascular therapy for nutcracker syndrome represents a safe and suitable treatment option, but prospective studies are needed to confirm these data.



Introduction


The clinical scenario of the left renal vein (LRV) entrapment syndrome (also known as nutcracker syndrome, NCS) comprises two main variants depending on the location where the vein compression occurs. When the compression takes place between the abdominal aorta and the superior mesenteric artery (SMA), it is called anterior NCS. When the LRV is compressed between the aorta and the vertebral column, it is termed as posterior NCS . It has also been described a circumaortic LRV trajectory, with concomitant vein compression both in the anterior and posterior compartment . Another variant of the NCS includes the LRV compression by the SMA to the right renal artery, also called lateral NCS . An incidental finding of the LRV compression in asymptomatic patients merits no therapy, and it is known as a nutcracker phenomenon. Noninvasive and invasive modalities have been used to evaluate the severity of the LRV compression. The different diagnostic criteria for NCS includes: the ratio of the duplex ultrasound peak systolic velocity of the aortomesenteric segment to the hiliar portion of the LRV > 4.2–5.0 , the relation between the diameter of the aortomesenteric segment to the hiliar portion of the LRV by multidetector computed tomography (< 50%) , the angle between the SMA and the aorta (normal > 41°), the presence of collaterals veins in the retroperitoneum and around the renal hilium, and the evidence of a pressure gradient between the LRV and the inferior vena cava (> 3 mmHg) . Here, we describe a symptomatic patient with LRV compression syndrome treated with an endovascular procedure and a systematic review of the literature with emphasis in the intervention studies.





Case presentation


A 21 year-old African American female with no significant medical history presented with intractable nausea, bilious vomiting, left upper quadrant abdominal pain, decreased oral intake and 10-pound weight loss. Review of symptoms was positive for dyspareunia. Her past medical history included the prior presentations to multiple community hospitals where she was diagnosed with gastroenteritis and subsequently prescribed antiemetic medications. Additionally, she had a cesarean section 3 years prior, smoking history (1 pack of cigarettes per day for the last 5 years) with occasional marijuana use, but otherwise denied ethanol or other illicit drugs consumption. Her family history was noncontributory. Laboratory data revealed hyponatremia, hypokalemia, metabolic alkalosis and negative pregnancy test. Prior imaging test included a non-diagnostic abdominal and pelvic ultrasound. The rest of the laboratory data was otherwise unremarkable. A CT scan of the abdomen with contrast revealed compression of the LRV between the superior mesenteric artery and the abdominal aorta ( Fig. 1 A ). To evaluate the severity of the stenosis, intravascular ultrasound and hemodynamic assessment of the pressure gradient between LRV and the inferior vena cava were performed. A minimal luminal area of 2.8 mm 2 and a > 3 mmHg gradient between the LRV and the inferior vena cava were found. These results prompted an intervention with a self-expandable SMART stent 14 × 40 mm deployment (Cordis, Hialeah, FL USA; Fig. 1 B–D). Repeated assessment of the pressure gradient documented a ≤ 1 mmHg difference. Additionally, intravascular ultrasound confirmed that the minimal luminal area increased to 10 mm 2 . Following the procedure, full anticoagulation with low molecular weight heparin was started. Subsequent clinical follow-ups ensure that the patient’s nausea and vomit were resolved. Also, her oral intake improved, and the patient was then discharged home. Due to the high risk of stent thrombosis reported in case series , the patient was discharged home with oral anticoagulation (warfarin) for 6 months.




Fig. 1


Imaging assessment and endovascular stenting of the left renal vein.

A, CT scan of the abdomen with intravenous contrast exhibits the left renal vein compression. Arrow = illustrates the compression of the renal vein.

B, Baseline left renal venogram in an antero-posterior projection demonstrates the left renal vein stenosis.

C, The lesion was crossed with a Rosen guide wire (Cook Medical INC, Bloomington, IN, USA) and subsequently an ANL2 flexor Ansel guiding sheath (Cook Medical INC, Bloomington, IN, USA) was advanced to the area of interest. The Rosen wire guide was exchanged with a super stiff Amplatz wire (Boston Scientific Inc., Natick, MA, USA), and the SMART stent (Cordis, Hialeah, FL USA) was then advanced over the wire and deployed. Intravascular ultrasound confirmed optimal stent apposition.

D, Final angiographic result after stenting.

E, Repeat CT scan of the abdomen at 1-year follow up documenting the patency of the stent. The arrow points the patent stent in the left renal vein.





Case presentation


A 21 year-old African American female with no significant medical history presented with intractable nausea, bilious vomiting, left upper quadrant abdominal pain, decreased oral intake and 10-pound weight loss. Review of symptoms was positive for dyspareunia. Her past medical history included the prior presentations to multiple community hospitals where she was diagnosed with gastroenteritis and subsequently prescribed antiemetic medications. Additionally, she had a cesarean section 3 years prior, smoking history (1 pack of cigarettes per day for the last 5 years) with occasional marijuana use, but otherwise denied ethanol or other illicit drugs consumption. Her family history was noncontributory. Laboratory data revealed hyponatremia, hypokalemia, metabolic alkalosis and negative pregnancy test. Prior imaging test included a non-diagnostic abdominal and pelvic ultrasound. The rest of the laboratory data was otherwise unremarkable. A CT scan of the abdomen with contrast revealed compression of the LRV between the superior mesenteric artery and the abdominal aorta ( Fig. 1 A ). To evaluate the severity of the stenosis, intravascular ultrasound and hemodynamic assessment of the pressure gradient between LRV and the inferior vena cava were performed. A minimal luminal area of 2.8 mm 2 and a > 3 mmHg gradient between the LRV and the inferior vena cava were found. These results prompted an intervention with a self-expandable SMART stent 14 × 40 mm deployment (Cordis, Hialeah, FL USA; Fig. 1 B–D). Repeated assessment of the pressure gradient documented a ≤ 1 mmHg difference. Additionally, intravascular ultrasound confirmed that the minimal luminal area increased to 10 mm 2 . Following the procedure, full anticoagulation with low molecular weight heparin was started. Subsequent clinical follow-ups ensure that the patient’s nausea and vomit were resolved. Also, her oral intake improved, and the patient was then discharged home. Due to the high risk of stent thrombosis reported in case series , the patient was discharged home with oral anticoagulation (warfarin) for 6 months.


Nov 14, 2017 | Posted by in CARDIOLOGY | Comments Off on Systematic review of endovascular therapy for nutcracker syndrome and case presentation

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