Harlequin Syndrome

Excerpt

To the Editor: Harlequin syndrome (HS) is characterized by unilateral flushing and hyperhidrosis induced by exercise, heat, or emotional stress [1, 2]. Here, we describe a rare case of HS in a boy who developed unilateral flushing and hyperhidrosis after esophageal atresia (EA) repair, and the symptoms persisted for more than 10 y. The patient was born via spontaneous vaginal delivery without complication at 39 wk of gestation and was diagnosed as having EA (type C). The following day he underwent primary repair for EA by the extrapleural approach via right thoracotomy at the fourth intercostal space. General anesthesia was induced uneventfully without jugular vein catheterization. He was discharged on postoperative day 21 without complications. However, his parents noticed hemifacial flushing and hyperhidrosis when he was 1-y-old. When he was 9-y-old, he started complaining of right hemifacial anhidrosis and left hemifacial flushing and sweating, pointed out by friends at school. Additionally, he noticed hyperhidrosis limited to the right side of his trunk after physical exercise. When he was 12-y-old, he was referred to our clinic. Neurological examination was not significant. However, after exercising, the right sides of his face, neck, and upper trunk were pale, and he experienced nearly complete anhidrosis with compensatory flushing and hyperhidrosis on the left sides of the same areas with sharp demarcation (Fig. 1). His chest computed tomography scan and the head and cervical magnetic resonance imaging studies were unremarkable. A diagnosis of HS was made. While the detailed pathogenesis of HS is unknown, plausible causes include damage of preganglionic or postganglionic cervical sympathetic fibers. Recent reports indicate that 3 out of 136 children with repaired EA by thoracotomy, and 2 out of 55 children using the thoracoscopic approach, experienced HS [3, 4]. The surgical sites for EA repair in our patient, specifically the extrapleural approach via right thoracotomy at the fourth intercostal space and dissection of the entire circumference of the esophagus, correspond with the area of the thoracic sympathetic ganglion (T2-T4). Although we could not completely determine whether this condition was idiopathic or acquired, iatrogenic causes might be possible.

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