S4493 Superior Mesenteric Artery Syndrome and Nutcracker Syndrome in a Patient With Ehlers-Danlos Syndrome

Introduction: The association between abdominal compression syndromes (ACS) and connective tissue disorders (CTDs), such as Ehlers-Danlos syndrome (EDS), is rarely reported. The prevailing hypothesis suggests that vascular hypermobility and laxity in CTDs predisposes patients to compression by the superior mesenteric artery (SMA). We present a case highlighting a compelling association between nutcracker syndrome (NS) and EDS, emphasizing diagnostic and therapeutic considerations. Case Description: A 46-year-old woman with EDS (hypermobility type) presented with worsening reproducible postprandial abdominal pain and a 20 lb unintentional weight loss over 4 months. Computed tomography of the abdomen revealed compression of the third part of the duodenum by the SMA, with a narrowed aortomesenteric angle of 20°-22° (reference range: 28°-65°) and a distance of 2-8 mm (reference range: 10-34 mm), consistent with SMA syndrome. Parenteral nutrition was initiated to support weight gain. During hospitalization, abdominal pain fluctuated, and new left-sided flank pain emerged. Physical examination revealed unilateral left costovertebral angle tenderness. Magnetic resonance imaging demonstrated compression of the left renal vein by the SMA and a dilated left ovarian vein, confirming NS. The patient pursued autologous renal vein transplantation for pain management. Discussion: EDS is characterized by a spectrum of symptoms, ranging from skin and joint hypermobility to severe vascular complications, such as aortic dissection. Increased vascular laxity in EDS may predispose patients to ACS, including SMA syndrome and NS. Although case reports suggest a link between CTDs and NS, the low prevalence of CTDs limits large-scale studies. This case underscores the importance of considering NS in the differential diagnosis for EDS patients with persistent abdominal or flank pain. Advances in imaging and minimally invasive interventions have improved NS diagnosis and management, yet underdiagnosis remains a challenge. Increased awareness and access to diagnostic tools could enhance recognition of ACS in CTD patients, refining treatment strategies. Furthermore, the rising use of glucagon-like peptide-1 (GLP-1) receptor agonists, associated with rapid weight loss, may increase NS incidence, warranting heightened clinical vigilance. Future research should focus on elucidating the mechanistic links between CTDs and ACS to optimize diagnostic and therapeutic approaches.