Disclosures: J. Sharif Palmer, MD: No financial relationships or conflicts of interest

Case Diagnosis: Iatrogenic Harlequin Syndrome

Case Description: A 28-year-old male presented to the emergency department on 08/26/2020 after head trauma with loss of consciousness. Workup showed unstable C5 fracture. He underwent emergent anterior C5 corpectomy with plating of C4-C6 and HALO brace placement. A very complicated postoperative hospital course followed, including a right thoracotomy/decortication for empyema. The patient was admitted to inpatient rehabilitation on 11/13/2020, weaned off tube feeds 11/19, ventilator 11/23, and decannulated 12/10. In the days prior to decannulation, asymmetrical asymptomatic erythema of the face was noted, exacerbating his already significant anxiety and affecting participation with therapy.

Setting: TIRR Rehabilitation Hospital, Inpatient SCI, Houston TXAssessment/

Results: Two days prior to decannulation, patient was noted to have right sided facial erythema with sharp demarcation at the midline, without miosis or ptosis (excluding Horner’s syndrome as a diagnosis). He denied these symptoms prior to injury. Symptoms coincided with waking in the morning, positional change and participation in therapy. Unfortunately this new finding caused the patient significant anxiety, undoubtedly due in part to his recent and very complicated hospital stay. Based on clinical presentation and literature review, iatrogenic Harlequin Syndrome was diagnosed and explained to the patient, alleviating his additional worry and allowing him to continue participating with therapies.

Discussion: Harlequin Syndrome is a rare condition first defined in 1988 as “unilateral facial flushing and sweating” without “neurological symptoms and no ocular signs of Horner’s syndrome”, resulting from deficient sympathetic innervation of the the non-flushing side. Though most often idiopathic, case reports have been published attributing the onset to thoracic surgery, high level scoliosis surgery, and thoracic epidural anesthesia. Treatments have been explored using botox and surgical approaches.

Conclusion: Though a rare and benign syndrome, basic knowledge of the etiology and presentation of Harlequin Syndrome may prevent unnecessary use of resources for workup and emotional distress to the patient.

Level of Evidence: Level V

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PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/iatrogenic-harlequin-syndrome-following-emergent-anterior-fixation-of-unstable-traumatic-c5-fracture-and-right-sided-decortication-in-a-previously-healthy-28-year-old-male-a-case-report/