Under Pressure: Concomitant Median Arcuate Ligament Syndrome and Nutcracker Syndrome

Introduction: Median arcuate ligament syndrome (MALS) results from the compression of the celiac artery and its surrounding ganglion by median arcuate ligament. Nutcracker syndrome occurs when the abdominal aorta (Ao) and superior mesenteric artery (SMA) compress the left renal vein leading to entrapment. Both can result in nonspecific abdominal symptoms and are challenging to diagnose. We present a case of concomitant findings of both syndromes in a patient presenting with abdominal pain. Case Description/Methods: An 18-year-old woman presented with chronic recurrent abdominal pain in 2 sites: one is left flank/lower quadrant pain that is triggered by exertion, and a second intermittent postprandial epigastric pain. Physical examination and routine labs were unremarkable. Urinalysis showed trace hemoglobin. Magnetic resonance angiography revealed compression of celiac artery as well as compression of left renal vein between SMA and Ao (Figure 1). Ultrasound showed increased velocity of celiac artery more prominent with expiration and left renal vein compression with collaterals, consistent with a diagnosis of MALS and nutcracker syndrome. Celiac nerve plexus block was performed with improvement of symptoms. The patient successfully underwent laparoscopic surgery for MALS release. Discussion: MALS is rare with incidence of 2 in 100,000 patients and can be challenging to diagnose as it can be often incidental and asymptomatic. When symptomatic, MALS can present with nonspecific pain, including recurrent post-prandial abdominal pain or exercise induced pain, and should be considered in the differential diagnosis for such when other causes have been eliminated. Vascular imaging such as duplex arterial ultrasound, computed tomography angiography, or magnetic resonance angiography with inspiratory changes are used to determine the presence of celiac artery compression which is key to diagnose. Nutcracker syndrome is even less commonly recognized than MALS with unclear incidence. While often asymptomatic, it can cause hematuria, proteinuria, hypertension, flank pain, dyspareunia, dysmenorrhea, and pelvic congestion. There are only a few cases of concomitant MALS and Nutcracker syndrome have been described in literature. When presenting concurrently, MALS and nutcracker syndrome can be managed operatively simultaneously.Figure 1.: Magnetic resonance angiography revealing compression of celiac artery consistent with MALS (A and B). Ultrasound showing compression of left renal vein consistent with nutcracker syndrome (C and D).