Abstract
INTRODUCTION: Renal nutcracker syndrome (NCS) is a rare cause of chronic
pain, hematuria, and urogenital issues. Most often NCS presents due to
compression of the left renal vein (LRV) between the aorta and the
superior mesenteric artery (SMA). The demographics and clinicopathologic
features are not well recognized in the medical community broadly,
leading to misdiagnosis and treatment that is ineffective. We present a
systematic review of NCS case reports and case series, with the goal of
establishing common features in presentation that will allow this
condition to be more recognizable.
METHOD: A literature search was conducted through PubMed, SCOPUS, and
Cochrane databases for the past 10 years (November 2012 to November
2022) for confirmed cases of NCS. Additionally, seven of our own
clinical cases are included as a case series and included in the review.
Data such as age, sex, symptoms, and treatment were extrapolated and
aggregated.
RESULTS: Forty studies and our own series were included in the review,
for a total of 73 individual patient cases. The mean age at diagnosis
was 36. The vast majority of patients were female (79.5%). Left flank
pain was the most common presenting symptom (61.6%) with hematuria
presenting as the second most common (52.1%). However, a significant
proportion of patients presented with pelvic pain (37.0%), abdominal
pain (15.1%), as well as nausea, vomiting, and weight loss (12.3%).
Many patients also had urogenital symptoms (24.7%), such as
dyspareunia, dysmenorrhea, testicular pain, UTI, urinary frequency, or
uterine bleeding. Diagnosis was almost always made with computed
tomography. Ultrasound generally served to augment the diagnosis.
Treatment modalities were highly varied, although endovascular stenting
was most commonly done (38.4%). Other options included left renal vein
transposition, left renal vein bypass, PTFE cuff placement, renal
autotransplantation, nephrectomy, and conservative management with pain
control and nutritional support.
CONCLUSION: NCS is an uncommon cause of hematuria, flank pain, abdominal
symptoms, and urogenital symptoms that most commonly affects young
females. It presents with a variety of symptoms but should be in the
differential for otherwise healthy patients presenting with hematuria,
left flank pain, or urogenital symptoms. CT angiography and Doppler
ultrasound are useful adjuncts in making this diagnosis. Treatment
modalities range from minimally invasive to open surgery. The
consequence of a missed diagnosis can lead to years of chronic pain and
prescription opioid use. Thus, it is essential that awareness of this
condition increases.