RESUMO
We report three pediatric cases of concomitant congenital Horner and Harlequin syndromes. This association suggests a lesion at the superior cervical ganglion or just inferior. Often, no underlying lesion is documented.
Assuntos
Doenças do Sistema Nervoso Autônomo/complicações , Doenças do Sistema Nervoso Autônomo/diagnóstico , Rubor/complicações , Rubor/diagnóstico , Síndrome de Horner/complicações , Síndrome de Horner/diagnóstico , Hipo-Hidrose/complicações , Hipo-Hidrose/diagnóstico , Doenças do Sistema Nervoso Autônomo/congênito , Criança , Pré-Escolar , Feminino , Rubor/congênito , Síndrome de Horner/congênito , Humanos , Hipo-Hidrose/congênito , Lactente , Masculino , Prognóstico , Doenças Raras , Estudos de AmostragemRESUMO
UNLABELLED: Harlequin syndrome (HS) is a rare autonomic disorder due to a hemifacial cutaneous sympathetic denervation. It is characterized by unilateral diminished sweating and flushing of the face even though after heat or prolonged exercise. It is typically acquired. Congenital cases only represent a 6% of all individuals with HS. All congenital HS cases reported so far showed a concomitant Horner syndrome, probably due to a stellate ganglion involvement. HS represents an uncommon autonomic disorder due to a hemifacial cutaneous sympathetic denervation. It is clinically characterized by a dramatic alteration in facial appearance: ipsilateral denervated pale and dry half from the other intact red and moist half. CONCLUSION: We present, to the best of our knowledge, the first case of a patient with a congenital HS as an isolated phenomenon.
Assuntos
Doenças do Sistema Nervoso Autônomo/congênito , Doenças do Sistema Nervoso Autônomo/diagnóstico , Rubor/congênito , Rubor/diagnóstico , Hipo-Hidrose/congênito , Hipo-Hidrose/diagnóstico , Doenças Raras/congênito , Doenças Raras/diagnóstico , Face , Humanos , Lactente , MasculinoAssuntos
Alimentação com Mamadeira , Face/inervação , Rubor/etiologia , Sistema Nervoso Parassimpático/fisiopatologia , Glândula Parótida/inervação , Reflexo/fisiologia , Pele/inervação , Feminino , Rubor/congênito , Rubor/fisiopatologia , Humanos , Lactente , Recém-Nascido , Salivação/fisiologia , Fatores de TempoRESUMO
Two patients with congenital Horner's syndrome had unilateral facial flushing. Both showed pupillary supersensitivity to epinephrine as well as anhidrosis on the affected side of the face and neck. Facial skin temperature after exercise increased on the intact side, but decreased on the affected side. Thermal vasodilation in the major portions of the face is regulated by sympathetic vasodilator fibres, and less predominantly by adrenergic vasoconstrictor fibres. The asymmetry of facial flushing may have been caused by impaired sympathetic vasodilation and further intensified by active vasoconstriction due to supersensitivity to circulating catecholamine on the affected side.
