目的:研究患有Goldenhar综合征(GS)的儿童的眼科临床特征和治疗结果。
方法:对符合GS诊断标准的纳入儿童进行回顾性回顾。人口统计,眼科特征,系统异常,和治疗结果从患者档案中手动记录.ENT,儿科,心脏病学,在接受任何眼科手术之前,所有GS患者都寻求骨科咨询。专门研究了解剖(眼表和眼睑)和功能{视觉和眼外运动(EOM)}因素。我们确保了至少12个月的术后随访,我们的数据与以GS的“眼科特征”为特征的主要研究进行了比较。
结果:共纳入30名儿童(女性=18,60%),中位年龄为48个月。27例(90%)单侧眼科受累,主要特征是上眼睑缺损(n=25,75.76%),脂类(n=18,54.55%),和角膜缘皮样(n=10,30.3%)。视力下降记录在22(73.3%),5人(16.7%)有EOM限制。系统地,突出的特征包括半面部肥大(100%),耳廓异常(80%),心脏异常(10%),和脊柱侧弯(6.67%)。用直接闭合±角囊溶解技术或使用Tenzel皮瓣修复眼睑瘤。眼科手术干预后,所有儿童的解剖和功能结果令人满意,没有任何明显的并发症。
结论:定制的眼科手术干预提供了令人满意的眼睛解剖结构和功能恢复。这些孩子需要具体的多学科咨询,以进行全面管理和全面护理。
OBJECTIVE: To study the ophthalmic clinical profile and the management outcomes of children having Goldenhar syndrome (GS).
METHODS: Retrospective review of included children fulfilling the diagnostic criteria of GS was performed. The demography, ophthalmic features, systemic anomalies, and treatment outcomes were recorded manually from patient files. An ENT, pediatrics, cardiology, and orthopedics consultation was sought for all GS patients before taking up for any ophthalmic surgical procedure. The anatomical (ocular surface and eyelid) and functional {vision and extraocular movements (EOM)} factors were exclusively studied. We ensured a minimum postoperative follow-up of 12 months, and our data were compared with the major studies featuring \'ophthalmic features\' of GS.
RESULTS: Totally 30 children (females = 18, 60%) were included with a median presenting age of 48 months. Twenty-seven (90%) had unilateral ophthalmic involvement with major features being upper eyelid coloboma (n = 25, 75.76%), lipodermoid (n = 18, 54.55%), and limbal dermoid (n = 10, 30.3%). Diminution of visual acuity was recorded in 22 (73.3%), while five (16.7%) had limitation of EOM. Systemically, the prominent features included hemifacial hypotrophy (100%), auricular anomalies (80%), cardiac anomalies (10%), and scoliosis (6.67%). The eyelid colobomas were repaired with the direct closure ± cantholysis technique or using a Tenzel\'s flap. All children had satisfactory anatomical and functional outcomes after ophthalmic surgical interventions without any significant complications.
CONCLUSIONS: The tailored ophthalmic surgical intervention(s) provides satisfactory restoration of anatomy and functionality of the eye. These children need specific multi-discipline consultations for the holistic management and complete care.