구순구개열 및 가로얼굴파열을 동반한 Goldenhar Syndrome 환자에서 수술적 치료: 증례보고
Surgical Treatment of a Goldenhar Syndrome Patient with Cleft Lip and Palate and Transverse Facial Cleft: Case Report
박종열, 김준영, 박진후, 정휘동, 정영수,
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박종열 ( Park Jong-Yeol ) - 연세대학교 치과대학 구강악안면외과학교실
김준영 ( Kim Jun-Young ) - 연세대학교 치과대학 구강악안면외과학교실
박진후 ( Park Jin-Hoo ) - 연세대학교 치과대학 구강악안면외과학교실
정휘동 ( Jung Hwui-Dong ) - 연세대학교 치과대학 구강악안면외과학교실
정영수 ( Jung Young-Soo ) - 연세대학교 치과대학 구강악안면외과학교실
Abstract
Goldenhar syndrome is also knowns as Oculo-Auriculo-Vertebral Syndrome which is a rare case. It represents hemifacial microsomia, macrostomia and cleft lip and palate (CLP) in facial characteristics. It has a wide range of multiple symptoms in ophthalmic, auricular, skeletal, vertebral and cardiac system. Though the etiology of this syndrome is not fully understood, it has shown variable characteristics which are caused by uncertain factors. The incidence of this syndrome ranges from 1 in 35,000 to 56,000. The treatment within an appropriate time is able to increase the chances of a good prognosis. In this paper, we report a case of patient with Goldenhar syndrome showing macrostomia and CLP. This patient underwent two surgeries; firstly, the primary lip and nose repair for unilateral complete lip and palate, secondly, the commissure repair of transverse facial cleft and skin excision of preauricular tag. Herein we continue to emphasize the necessity of proper surgery technique along with a timely treatment in conjunction with multidisciplinary management of systemic abnormalities.
키워드
Goldenhar syndrome; Hemifacial microsomia; Macrostomia; Cleft lip and palate; Transverse facial cleft
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