양측성 구순열이 동반된 Goldenhar 증후군의 치험례: 진단 기준과 외과적 처치

• 영문명: A Case Report on Goldenhar Syndrome with Bilateral Cleft Lip: Diagnostic Criteria and Surgical Intervention
• 발행기관: 대한구순구개열학회
• 저자명: 박영욱
• 간행물 정보: 『대한구순구개열학회지』제27권 제1호, 18~22쪽, 전체 5쪽
• 주제분류: 의약학 > 일반외과학
• 파일형태: PDF
• 발행일자: 2024.06.30

국문 초록

Goldenhar syndrome, also known as oculo-auriculo-vertebral spectrum, represents a rare congenital anomaly characterized by complex craniofacial and spinal malformations. This report discusses the clinical presentation and surgical management of a 4-year-old male patient with Goldenhar syndrome, who exhibited extensive right-sided abnormalities including microphthalmia, coloboma, external auditory canal atresia, ear dysplasia with associated hearing loss, hemifacial microsomia, and an extra thumb. Notably, the patient also presented with bilateral cleft lip and palate. The diagnostic challenges and criteria are explored, particularly in light of the patient's extensive presentation. Surgical intervention comprised of bilateral cheiloplasty under general anesthesia, focusing on the functional reconstruction using the orbicularis muscles from lateral segments due to severe hypoplasia of the prolabium, columella, and nasal mucosa. The postoperative outcome was deemed successful both functionally and aesthetically. This case underscores the importance of tailored surgical approaches in managing complex manifestations of Goldenhar syndrome and adds to the body of literature guiding diagnostic criteria for this rare disorder.

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키워드

Goldenhar syndrome Bilateral cleft lip and palate Oculo-auriculo-vertebral spectrum