Physician Froedtert Thedacare Health Neenah, Wisconsin
Disclosure(s):
Mac Weninger, MD: No financial relationships to disclose
Case Diagnosis: A 47-year-old female with a history of postural orthostatic tachycardia syndrome (POTS) and vascular Ehlers-Danlos syndrome (vEDS) presenting with symptoms consistent with thoracic outlet syndrome (TOS) and May-Thurner syndrome (MTS).
Case Description or Program Description: The patient presented with chronic radicular pain from the chest to both extremities, numbness in the hands, and left lower extremity swelling, redness, and allodynia. Vascular ultrasound demonstrated dampened arterial waveforms in provocative positions, consistent with TOS, and ultrasound-guided scalene blocks provided near-complete symptom relief. Venography and intravascular ultrasound confirmed MTS, revealing >50% stenosis of the left common iliac vein. Given the failure of conservative management, she underwent bilateral scalene/pectoralis minor release, first rib resection, and iliac vein stenting.
Setting: Outpatient Physiatry clinic and Vascular Surgery center.
Assessment/Results: Following first rib resection, the patient experienced reduced upper extremity numbness and improved sensation, though she developed a mild medial cord plexopathy identified on Electromyography (EMG). Despite this, she elected to proceed with contralateral TOS surgery due to significant pain relief. Iliac vein stenting resulted in near-complete resolution of lower extremity swelling and 80% pain reduction.
Discussion (relevance): This case illustrates the complex interplay of vascular, neurological, and musculoskeletal dysfunction in vEDS, where structural fragility predisposes patients to neurovascular compression syndromes. The use of ultrasound-guided scalene blocks was pivotal, not only confirming TOS but also predicting surgical success. Given the high procedural risks in vEDS, targeted diagnostics are critical to minimizing complications and optimizing outcomes. Similarly, iliac vein stenting for MTS proved effective, reinforcing the need to recognize vascular compression syndromes in connective tissue disorders. A multidisciplinary approach integrating physiatry, vascular surgery, and rehabilitation medicine was essential for achieving functional improvement and symptom relief.
Conclusions: Comprehensive evaluation and a multimodal approach, including targeted diagnostic blocks, surgical decompression, and endovascular intervention, can optimize function and quality of life in patients with vEDS and associated neurovascular syndromes.