A Tailored Approach to Treatment of Nutcracker Syndrome
Naiem Nassiri, MD RPVI1, Lauren A. Huntress, MS1, Adrian Balica, MD1, Sammy Elsamra, MD1, Susan Murphy, MD2, Gloria A. Bachmann, MD MMS1.
1Rutgers-RWJMS, New Brunswick, NJ, USA, 2Cancer Institute of New Jersey, New Brunswick, NJ, USA.
Purpose: Left renal vein (LRV) compression or Nutcracker Syndrome (NCS) is a rare though likely underdiagnosed clinical entity associated with various symptoms that depend on severity and duration of compression and the resultant local hemodynamic milieu. While LRV transposition with or without venoplasty is considered the gold standard therapy, a less invasive approach tailored to type and severity of symptoms is suggested.
Methods: Over 24 months, patients with NCS diagnosed on computed tomography venography (CTV), venous duplex, venogram, and/or intravascular ultrasound (IVUS) underwent tailored endovascular treatment based on presenting symptoms which comprised of chronic refractory gross hematuria (CRGH) and/or pelvic congestion syndrome (PCS). PCS was defined as chronic (≥ 6 months), persistent pelvic and/or lower abdominal pain accompanied by one or more of the following: dysmenorrhea, menorrhagia, dyspareunia, and urinary frequency. Data was retrospectively reviewed for technical details, perioperative complications, resolution of symptoms, and adjunctive interventions.
Results: Seven patients with NCS underwent treatment (1 male; mean age = 43.1 years [range 18-66]). Presenting symptoms were CRGH in 1, CRGH and PCS in 1, and PCS in 5. One patient had a retroaortic LRV. All underwent CTV which was suggestive of NCS. Renal vein duplex (RVD) was obtained in 4 with equivocal findings. Compression was confirmed on selective LRV venography with IVUS. IVUS criteria for a hemodynamically significant stenosis was LRV cross-sectional area reduction ≥ 50%. Those with CRGH underwent LRV stenting. If concomitant PCS was present, ipsilateral gonadal vein coil embolization was performed. Those with PCS alone underwent ipsilateral gonadal vein coil embolization. There were no perioperative or delayed complications. Mean follow-up was 13.4 months (range 2 to 48). Resolution of symptoms without recurrence was achieved in all. None required reintervention.
Conclusion: NCS is an underdiagnosed cause of PCS and CRGH. CTV is suggestive. Venography with IVUS is confirmatory. RVD has been an equivocal diagnostic modality and is best used for surveillance. In absence of CRGH, gonadal vein coil embolization is an effective and durable treatment option for NCS-mediated PCS. With appropriate technical considerations, IVUS-guided LRV stenting can safely and effectively address CRGH. Larger series and longer follow-ups can further verify current findings.
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