Co-occurring superior mesenteric artery syndrome and nutcracker syndrome requiring Roux-en-Y duodenojejunostomy and left renal vein transposition: a case report and review of the literature.

BACKGROUND: The duodenum and the left renal vein occupy the vascular angle made by the superior mesenteric artery and the aorta. When the angle becomes too acute, compression of either structure can occur. Each type of compression is associated with specific clinical symptoms that constitute a rare disorder. If clinical symptoms are mild, conservative treatment is implemented. However, surgery is often the only solution that can improve quality of life and/or avoid life-threatening complications. This report describes a case of a patient with both types of aortomesenteric compression that required two separate surgeries to alleviate all symptoms.

CASE PRESENTATION: A 20-year-old white woman presented to the Emergency Room complaining of sudden onset severe left flank and lower left quadrant abdominal pain, nausea, and vomiting. A clinical work-up revealed elevated white blood cells and hematuria. She was discharged with a diagnosis of urinary tract infection. Symptoms continued to worsen over the subsequent 2 months. Repeated and extensive clinical work-ups failed to suggest evidence of serious pathology. Ultimately, an endoscopy revealed obstruction of her duodenum, and barium swallow identified compression by the superior mesenteric artery, leading to the diagnosis of superior mesenteric artery syndrome. She underwent a Roux-en-Y duodenojejunostomy. Six weeks later she continued to have severe left-sided pain and intermittent hematuria. Venography revealed compression of the left renal vein, extensive pelvic varices, and significant engorgement of her left ovarian vein. A diagnosis of nutcracker syndrome was made and a left renal vein transposition was performed. Significant improvement was seen after 8 weeks.

CONCLUSIONS: The disorders associated with aortomesenteric compression can lead to serious symptoms and sometimes death. Diagnosis is challenging not only because of the lack of awareness of these rare disorders, but also because they are associated with symptoms that are similar to those seen in less serious diseases. Guidance for health care professionals with respect to relevant radiological and clinical markers needs to be reconsidered in order to clarify the etiology of the diseases and create better diagnostic protocols.