A young patient with concurrent splanchnic dynamic vascular compression syndromes

Abstract

A thirty-year-old lady presented with chronic, postprandial abdominal pain associated with decreased appetite and weight loss. She had a past medical history of ulcerative colitis and past surgical history of Nissen fundoplication, hysterectomy and bilateral oophorectomy for endometriosis. Enhanced computed tomography (CT) of the abdomen/pelvis displayed severe narrowing of the celiac artery (CA) at the level of its ostium (Panel A) with post-stenotic dilatation. In an abdominal vascular ultrasound with respiratory maneuvers we saw a peak systolic velocity (PSV) in the CA during inspiration of 295 cm/s. The PSV was 108 cm/s during expiration for the CA. Peak systolic velocity in the superior mesenteric artery (SMA) was 424 cm/s during inspiration. Magnetic resonance angiogram (MRA, Panel B) showed compression of the SMA with post-stenotic dilatation. No signs of perivascular inflammation or fat stranding were appreciated. Serology studies workup showed only weakly positive anti nuclear antibody (ANA) titer. A mesenteric angiography (Panels C and D) illustrated progression to occlusion of the CA, a dynamic compression of the SMA with kinking mainly during expiration, and post-stenotic dilatation. The pre-operative suspicion for dynamic mesenteric vascular compression syndromes was intra-operatively confirmed with associated scarring along the CA and SMA via the arcuate ligament.