'Winged' Eagle's Syndrome - a Rare Cause of Spinal Accessory Nerve Palsy
Kitty Wu, MD1, Fawaz Siddiqi, MD MBA FRCSC2, John Yoo, MD FRCSC2, Douglas C Ross, MD MEd FRCSC3 and Thomas Miller, MD FRCPC2, (1)Roth McFarlane Hand & Upper Limb Centre, London, ON, Canada, (2)Western University, London, ON, Canada, (3)Roth | McFarlane Hand and Upper Limb Centre, Division of Plastic Surgery, The Peripheral Nerve Clinic, London, ON, Canada
Introduction: The eponymous Eagle's syndrome results from an elongated and calcified styloid process or stylohyoid ligament; symptoms include dysphagia, globus sensation, and otalgia Carotid artery and internal jugular vein compression are described vascular variants of Eagle's syndrome, but spinal accessory nerve (SAN) involvement has not been previously described.
Materials & Methods: We describe two patients (both male, ages 61 and 71) presenting with spinal accessory nerve palsy resulting from compression by a posteriorly angulated and/or calcified styloid process. Both patients exhibited atrophy of the trapezius and sternocleidomastoid muscles causing lateral scapular winging, accentuated by shoulder abduction. MRI Magnetic resonance imaging excluded a skull base tumour and computed tomography demonstrated extrinsic compression of the SAN by the styloid process and significant narrowing of the stylo-atlantoid space. Electrophysiologic testing showed a few, large amplitude motor units in the upper trapezius and no motor units in the middle and lower trapezius. Both patients underwent surgical exploration, styloidectomy, and SAN decompression through a trans-cervical approach.
Results: At 4 months post-operative, both patients had improved pain with overhead shoulder activity and improved shoulder range of motion. Electromyography testing demonstrated evidence of reinnervation to the upper fibres of trapezius with nascent units and improvement in compound muscle action potentials to both the upper and middle trapezius.
Conclusions: We report the first case of an atypical 'winged' variant of Eagle's syndrome, with SAN palsy resulting from compression by the styloid process, resulting in lateral scapular winging. This represents a rare but important diagnosis to include in the differential for SAN palsy. We believe that assessment by a multi-disciplinary team of physiatry head and neck surgery and neurosurgery was important in ensuring timely diagnosis and surgical success.
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