Department of Ophthalmology, Instituto de Olhos Ciências Médicas, Belo Horizonte/ MG, Brazil.
Department of Ophthalmology, Núcleo Malta, Lagoa da Prata/ MG, Brazil.
Rom J Ophthalmol. 2020 Oct-Dec;64(4):444-448. doi: 10.22336/rjo.2020.68.
The article describes a case of Goldenhar syndrome that had been diagnosed by an ophthalmologist in a medical consultation by school bullying due to a choristoma. A 15-year-old male patient, who had a nodular lesion with hair in the inferior temporal corneal-limbo-conjunctival of the left eye, was reported. He also had a facial asymmetry, with mild mandibular hypoplasia and malformation of the left external ear, where only an auricular appendage was formed. He denied similar family history and the history of genetic diseases, but revealed that his mother had used ibuprofen during the first 3 months of pregnancy and had gestational diabetes mellitus. Excisional biopsy of the eye lesion was performed and revealed a dermoid cyst. After the exegesis and with adequate multidisciplinary monitoring, the patient reported being very satisfied with the aesthetic result, returning with more confidence to his daily activities. That was a typical case of Goldenhar syndrome that has remained undiagnosed and untreated for more than a decade due to a lack of diagnosis despite its classic presentation. The delay in the approach resulted in social stigma and profound psychosocial damage. The importance of disseminating the correct knowledge of this pathology and of having an early multidisciplinary approach in these patients is emphasized, since the impact on the quality of life is significantly high.
本文描述了一例因眼部错构瘤而被眼科医生诊断为先天性耳-颜面畸形综合征(Goldenhar 综合征)的病例。该病例为 15 岁男性,左眼颞下方角膜缘-结膜混合区有一结节性带毛病变,同时存在面部不对称,表现为轻度下颌骨发育不全和左侧外耳畸形,仅形成耳廓附器。患者否认有类似家族史和遗传病史,但透露其母亲在妊娠前 3 个月使用了布洛芬,且患有妊娠期糖尿病。眼部病变行切除活检,结果显示为皮样囊肿。经过详细检查,并进行多学科充分监测后,患者对美学结果非常满意,自信心恢复,重返日常生活。这是一例典型的 Goldenhar 综合征病例,由于缺乏诊断,尽管表现典型,但十多年来一直未被诊断和治疗。这种延迟诊断导致了社会耻辱和深刻的心理社会损害。强调了传播该病理正确知识和对这些患者进行早期多学科治疗的重要性,因为其对生活质量的影响非常大。