Yenidoğan Döneminde Bilateral Katarakt İle Bulgu Veren Goldenhar Sendromu Olgusu

Goldenhar sendromu veya okuloaurikulovertebral spektrum, göz-kulak anomalileri, hemifasyal mikrozomi vevertebral anomaliler ile karakterize gelişimsel bir bozukluktur. Sendrom, birinci ve ikinci brakial yarıktan köken alan yapıların doğumsal defektlerinden oluşur. Sendromun nedeni tam olarak bilinmemektedir ancak heterojen olduğu düşünülmektedir. Olguların çoğunda tek taraflı tutulum görülürken %4-8 inde iki taraflı olabilmektedir. Bu sendromun yönetimi multidisipliner yakalaşım gerektirir. Burada sendromun tüm klasik bulgularına çift taraflı kataraktın eşlik ettiği prematüre doğan bir olgu sunulmuştur.

Anahtar Kelimeler:

Goldenhar sendromu, konjenital katarakt, fasyal asimetri

Goldenhar Sydrome Presented Bilateral Cataract In Neonatal Period

Goldenhar syndrome also known as Oculo-auriculo-vertebral spectrum is a complex developmental disorder characterised mainly by anomalies of the ear and eye, hemi facial microsomia and vertebral anomalies. It is associated with anomalous development of the first branchial arch and second branchial arch. The aetiology of this rare sydrome is largely unknown and seems to be heterogeneous. The malformations affect usually one side of the body, however 4-8 percent of affected individuals have bilateral involvement. The management of this condition requires a multi-diciplinary approach.We report a pretem neonate of Goldenhar sydrome who presented bilateral cataract in addition to all classical signs of the condition.

Keywords:

Goldenhar syndrome, congenital cataract, facial asymmetry,

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