治疗戈登哈尔综合征患者的肢端皮样瘤:病例系列

Anchal Tripathi, Shalini Mohan, Lav Pathak
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摘要

目的研究被诊断为戈登哈尔综合征的角膜缘皮样瘤患者的临床特征、相关系统特征和治疗结果:本病例系列包括眼科门诊中根据系统评估确诊为戈登哈尔综合征的患者。记录了人口统计学数据、眼科评估和系统评估。根据角膜缘皮样瘤的程度采取了不同的手术干预措施,术后随访一年:结果:共纳入七名患者(九只眼),平均年龄(7.71±4.15)岁。2名患者为双侧睑缘皮赘,5名患者为单侧。观察到 1 级和 2 级眼球皮赘。BCVA的平均值为0.50±0.11(logMAR),6名患者存在散光。戈登哈尔综合征的全身特征包括脊椎异常、耳部异常、面部异常和下肢畸形。手术治疗方法多种多样,包括单纯切除术、板层角膜移植术和羊膜移植术。术后,所有单纯切除的病例都形成了角膜瘢痕:本系列病例强调了在戈登哈尔综合征的背景下发生角膜缘畸形的罕见性,以及早期手术干预对处理这些病例的重要性。通过分享我们的经验和结果,我们希望能帮助人们更广泛地了解这种疾病及其最佳治疗方法。
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Managing Limbal Dermoids in Patients with Goldenhar Syndrome: A Case Series.

Objective: To investigate the clinical characteristics, associated systemic features, and management outcomes of patients with limbal dermoids diagnosed with Goldenhar syndrome.

Methods: This case series included patients from the eye outpatient department, diagnosed with Goldenhar syndrome based on systemic evaluation. Demographic data, ophthalmological assessments, and systemic evaluations were recorded. Various surgical interventions were employed based on the extent of limbal dermoids, and postoperative follow-up spanned one year.

Results: Seven patients (nine eyes) were included, with a mean age of 7.71±4.15 years. Limbal dermoids were bilateral in two patients and unilateral in five. Grade 1 and grade 2 limbal dermoids were observed. The mean BCVA was 0.50±0.11 (logMAR), and astigmatism was present in six patients. Systemic features of Goldenhar syndrome included vertebral anomalies, ear abnormalities, facial anomalies, and lower limb deformity. Surgical interventions varied, with simple excision, lamellar keratoplasty, and amniotic membrane transplantation utilized. Postoperatively, corneal scar formation occurred in all simple excision cases.

Conclusion: This case series underscores the rarity of limbal dermoids in the context of Goldenhar syndrome and the importance of early surgical intervention in managing these cases. By sharing our experiences and outcomes, we hope to contribute to the broader understanding of this condition and its optimal treatment.

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