Harlequin Syndrome after Thyroidectomy for Compressive Retrosternal Goiter. Case Report and Review of the Literature

Abstract A 74-year-old woman with known euthyroid multinodular retrosternal goiter necessitated an urgent intubation at home, due to acute respiratory distress evoked by tracheal compression. Extubation after a few days failed, and she underwent an urgent total thyroidectomy. During postoperative extubation the patient developed suddenly unilateral facial flushing and sweating at the left side, without ptosis of the left levator palpebrae superioris. These symptoms persisted during the next 24 hours. The skin at the right side of the face remained uninvolved. In the early postoperative period this appearance recurred at moments of emotions, exercise or heat. Beside this, the patient had a normal recovery. Six weeks later this reaction couldn’t be provoked anymore. ‘Harlequin’ syndrome (unilateral facial flushing and sweating) is caused by a lesion of the contralateral sympathetic chain at the levels T2 and T3. It is unknown if the sweating and vasodilation at the “healthy” side is normal or if it is a reaction of hyperactivity.

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