A 20-year-old white man presented to the emergency room with severe, left-sided, upper abdominal pain that began upon awakening two days previously.
The pain was described as aching, intermittently worse with movement, radiating to left flank, and associated with nausea. There were no obvious triggers or recent trauma. He denied any past medical history. There was no recent illness or travel.
He endorsed a family history of hypertension but not kidney disease. He had a negative medication history, denied illicit drug use, and was not sexually active. He did have one episode of similar pain two months prior. At that time, the pain was less severe, and passed with rest.
On exam, after pain is controlled with narcotics, his blood pressure was 110/60 mm Hg. Exam was normal except for pain on light palpation of the left upper quadrant and flank.
Labs, including serum creatinine, were within normal limits. Urinalysis showed no protein, and 60 RBC. Urine sediment revealed many intact, mostly non-dysmorphic, red blood cells. A computed tomography scan of the abdomen and pelvis was performed and representative image is shown.
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Nutcracker syndrome is also known as the left renal vein entrapment syndrome. As this latter name suggests, the clinical syndrome results from the compression of the left renal vein as it courses between the abdominal aorta and the proximal portion of the superior mesenteric artery. Such entrapment or compression then leads to distal renal vein tortuosity that is evident on imaging.
The nutcracker syndrome presents with left sided abdominal and flank pain of varying severity, usually in concert with micro- or macroscopic hematuria of uncertain origin. Timing of initial presentation is quite variable but tends to occur in the second or third decade. Symptoms may be precipitated by physical exertion that leads to a spike in renal vein blood pressure and resultant bleeding into the collecting system. Some patients may also have evidence of orthostatic proteinuria. Diagnosis is made through imaging of the renal vasculature.
Treatment of left renal vein entrapment is directed at alleviating the associated pain syndrome. Management options include endovascular stenting of the compressed segment of the left renal vein, or repositioning of the left renal vein in relation to the superior mesenteric artery.
Answer: Nutcracker syndrome
This case was prepared by Kevin T. Harley, MD, Assistant Clinical Professor of Medicine, Division of Nephrology & Hypertension, University of California Irvine.
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