Case Presentation: An 18-year-old woman who presented with a year history of recurrent painless gross hematuria and a 3-year history of anemia requiring continuous oral iron replacement therapy and subsequent blood transfusions. On further evaluation, she was also found to have significant proteinuria with preserved renal excretion function and a negative autoimmune panel. A renal biopsy was performed which revealed marginal thinning of the basement membrane; which was thought not to be significant enough to explain the degree of anemia and the hematuria. A cystoscopy was further carried out and this localized the left kidney as the possible source of the hematuria. Sequel to the suspicious cystoscopy findings, a renal vein angiogram was ordered, and it revealed a patent renal vein however with elevated pressures of 13/3 mm mercury at the renal vein and 3/1 mm mercury at inferior vena cava, confirming the presence of a renal vein entrapment. She was adequately counselled on the possible different modalities of treatment and the decision was made to go ahead with an open renal auto-transplantation. She was managed under a multidisciplinary team and continues to be under close follow up post auto-transplantation.
Discussion: The Nutcracker syndrome is a rare clinical condition which occurs when there is compression of the left renal vein between the abdominal aorta and the superior mesenteric artery. The exact prevalence of this condition however it is thought to be extremely rare. It is thought to be somewhat more in the Far Eastern part of the world. It has also slightly been more reported in females especially in the 2nd-3rd decades of life, like in our patient. Although, both symptomatic and asymptomatic cases have been reported anywhere from childhood to the 7th decade of life. With the advent of new and continued growth of technology, it has become relatively easier to diagnose but still albeit, a conundrum. The various modalities of management include conservative management with watchful waiting and weight gain to augment retroperitoneal fat and subsequently a change in the position of the left kidney. Other invasive modalities of management include endovascular stenting, open surgery with left renal vein transposition and auto-renal transplantation. Regardless, our patient remains under close follow-up and to the best of our knowledge, this is one of the cases that has been reported in the United States.
Conclusions: With hematuria being one of the main presenting symptoms, a high index of suspicion is required by Physicians for the diagnosis and prompt referral and or management of nutcracker syndrome. Renal auto-transplantation is one of the management modalities of the nutcracker syndrome although rarely performed for this purpose; in part ppossibly due to the rare nature of the disease. We present a rare case of renal auto-transplant for the management of this syndrome in an 18-year-old Hispanic woman. To the best of our knowledge, no such case has been reported in a Hispanic woman in literature.