Asociación Sindromática: Poland, Goldenhar, Moebius, Klippel-Feil. Presentación de un Caso Clínico

Abstract

ABSTRACT Poland, Goldenhar, Moebius, Klippel-Feil Syndrome. A Clinical Case Poland Syndrome is a congenital defect characterized by a unilateral absence of the clavicular and sternocostal portion of the pectoralis muscles associated to abnormalities of other muscles of the thoracic wall, ribs, breast and upper extremity. It is found in one of 20 to 32 thousand newborns. It is found sometimes associated to other syndromes, most often with Moebius Syndrome, and rarely with Goldenhar and Klippel-Feil. Due to the association, a common pathogenic cause has been postulated, that being an anomaly of vascularization during embrionary development. Clinical Case: A newborn male was seen who presented with Poland, Goldenhar, Moebius and Klippel-Feil Syndromes. Clinically, he presented left hemifacial mycrosomy, microtia, shorten-ing and paralysis of the facial nerve; his neck was short and movement was limited due to C4-C5 fusion; agen-esis of left pectoralis, hypoplasia of left radius and hand. There was no known additional family cases, being thus, a sporadic syndromatic association.(