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Waqas Javed Siddiqui, Abu Bakar, Muhammad Aslam, Hasan Arif, Brian A. Bianco, Alexander E. Trebelev, Ellie Kelepouris, Sandeep Aggarwal
(Department of Medicine, Division of Nephrology and Hypertension, Drexel University College of Medicine, Philadelphia, USA)
Am J Case Rep 2017; 18:754-759
The term nutcracker phenomenon (NCP) elucidates anatomical structure and hemodynamics, whereas nutcracker syndrome (NCS) refers to clinical manifestations. We present three cases of similar clinical features of hematuria and flank pain with different clinical outcomes.
CASE REPORT: Case 1: A 36-year-old Caucasian female with a past medical history (PMH) of HIV infection presented for evaluation of hematuria. Computed tomography (CT) without contrast showed pelvic venous congestion and narrowing of the extra-renal left renal vein (LRV). After the failure of conservative management, renal auto-transplantation was attempted but failed because of extensive venous collateral; the patient subsequently required a total hysterectomy due to recurrence of symptoms. Case 2: A 41-year-old Caucasian female with extensive PMH presented with chronic abdominal pain. A CT scan of the abdomen and pelvis showed pelvic venous congestion. The patient underwent angioplasty and stent placement of the LRV. Subsequently, a left ovarian vein embolization was performed. On follow-up visits, her symptoms improved. Case 3: A 36-year-old female with PMH of HIV infection, gastroesophageal reflux disease, and hypertension presented with hematuria and flank pain. Her venogram revealed 1 mm Hg pressure gradient across stenosis, suggestive of LRV hypertension. Over the months of her follow-up after discharge, her hematuria gradually decreased from daily to intermittent non-daily frequency, without any intervention.
CONCLUSIONS: The treatment of NCS includes observation, percutaneous angioplasty, open or endovascular surgery, or nephrectomy. In patients younger than 18 years of age, the best option is a conservative approach with observation for at least two years, as approximately 75% of patients have complete resolution of hematuria.