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

Abstract

BackgroundThe 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 presentationA 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.ConclusionsThe 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.

Nutcracker Syndrome Accompanying With Superior Mesenteric Artery Syndrome: A Case Report

“…1 NCS is a historically more recent clinical phenomenon than SMAS. 4 Given that both the duodenum and the LRV lie within the aortomesenteric angle, two different types of compression syndrome can arise. 4 However, both syndromes occurring concurrently are highly rare.…”

“…4 Given that both the duodenum and the LRV lie within the aortomesenteric angle, two different types of compression syndrome can arise. 4 However, both syndromes occurring concurrently are highly rare. 3 Generally, there are two main types of NCS, described as anterior and posterior NCS.…”

“…4 When the angle becomes too acute, compression of either structure can occur. 4 Although SMAS and renal NCS share the same pathogenesis, concurrent development has rarely been reported. 3 This article presents a case of a 38-year-old female with previous history of SMAS treated by gastroduodenostomy, who presented with left loin pain and microscopic hematuria caused by NCS which was treated by LRV transposition.…”

“…2,3 The duodenum and the LRV occupy the vascular angle made by the SMA and the aorta. 4 When the angle becomes too acute, compression of either structure can occur. 4 Although SMAS and renal NCS share the same pathogenesis, concurrent development has rarely been reported.…”

Nutcracker Syndrome as an Unusual Cause of Postprandial Pain

“…An anterior nutcracker syndrome can rarely occur concomitantly with SMA syndrome, in which the SMA compresses the duodenum causing postprandial pain, early satiety, nausea, vomiting, and weight loss. [2][3][4] More commonly, patients with SMA syndrome may also have nutcracker phenomenon where the LRV is compressed but without symptoms. [5][6][7][8] We suspect that physiologic dilation of the SMA after oral intake caused further compression of the LRV and hence a worsening of the patient's left flank pain in the postprandial state.…”