Department of Pediatrics, Ghent University Hospital, Ghent, Belgium.
Department of Pediatrics/Pediatric Neurology, AZ Groeninge Hospital, President Kennedylaan 4, 8500, Kortrijk, Belgium.
Acta Neurol Belg. 2021 Jun;121(3):625-631. doi: 10.1007/s13760-021-01593-6. Epub 2021 Jan 30.
Harlequin syndrome is a rare condition, presenting with unilateral facial flushing and hyperhidrosis in response to physical exercise, heat or emotional stressors and has scarcely been reported in pediatric patients. It is caused by a dysfunction of vasomotor and sudomotor sympathetic fiber activity inhibiting the ability to flush on the affected side, causing the neurologically intact side to appear red. We present three pediatric cases of this uncommon syndrome, each of them of different origin and displaying distinct associated (neurological) symptoms, and review medical literature. Insight into the anatomical structure of the thoracocervical and facial sympathetic nervous system is pivotal as it dictates symptomatology. About half of Harlequin syndrome cases are complicated with ocular symptoms and a minority may be part of more extensive partial dysautonomias affecting facial sudomotor, vasomotor and pupillary responses, such as Holmes-Adie syndrome and Ross syndrome. Etiology is generally idiopathic, however, cases secondary to surgery, trauma or infection have been described. Considering its predominantly self-limiting nature, treatment is usually unnecessary and should be restricted to incapacitating cases.
亨氏综合征是一种罕见的病症，表现为单侧面部潮红和多汗，对体力活动、热或情绪应激有反应，在儿科患者中几乎未见报道。它是由血管运动和汗腺交感纤维活动的功能障碍引起的，这种功能障碍抑制了受影响侧的潮红能力，导致神经完好的另一侧变红。我们提出了三个这种不常见综合征的儿科病例，每个病例的起源不同，并表现出不同的相关（神经）症状，并回顾了医学文献。深入了解胸颈和面部交感神经系统的解剖结构至关重要，因为它决定了症状。大约一半的亨氏综合征病例伴有眼部症状，少数可能是更广泛的部分自主神经功能障碍的一部分，影响面部汗腺、血管运动和瞳孔反应，如霍姆斯-阿迪综合征和罗斯综合征。病因通常是特发性的，但也有继发于手术、创伤或感染的病例。鉴于其主要为自限性，通常不需要治疗，应仅限于使能力丧失的病例。
