Enucleation and Dermato-fat Graft for Retinoblastoma Management : A Case Report

Dr. Agnes Angela Mahdalena Simanjuntak, Umboh Anne, Akay Freili
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Abstract

Introduction : Retinoblastoma is the most common intraocular malignant tumor found in children before 3 years old can occur unilaterally, bilaterally, hereditary or sporadic. Incidence of about 1:14,000-20,000 individuals. This tumor originated from embryogenic retinal cells. Retinoblastoma gene (RB1) located in the 13q14 chromosome Case Illustration : A 3-year-old girl presented with leukocoria. Her parents notice whitish in her right eye swollen, teary, painful since 2 months ago. No family history. Visual acuity (VA) on the oculus dextra ( OD) no light perception, oculus sinistra (OS) 6/6. Intraocular pressure on OD n-1/palpation, OS n/palpation. Examination under anasthesia for diagnostic found there weren’t palpebral edema, conjunctival injection, hazy cornea, pupil, lens couldn’t be evaluated on OD. Ultrasound showed sugest vitreous seeding and ptisis. CT scan showed solid mass with calcification and diagnosed retinoblastoma stage group E. Patient was treated with chemoreduction using Vincristine, Etoposide, Carboplatine combined enucleation with dermato-fat graft. We did nervus opticus dissection and histopathological examination which showed retinoblastoma. Discussion : The common signs of retinoblastoma is leukocoria. The priority is saving life followed by globe preservation. Treatment based on the stage of the disease. We did chemotherapy in this patient, followed by enucleation and dermato-fat graft. Enucleation stills the main therapy for saving life with nervus opticus dissection as possible. Dermato-fat graft has been shown to grow along with the expanding orbit in children therefore shows cosmetics. Conclusion : This report illustrates a case of Retinoblastoma. The chemotherapy and enucleation using safe dermato-fat graft procedure with implant followed by a well fitted prosthesis for better cosmetics
视网膜母细胞瘤的去核和皮下脂肪移植治疗 :病例报告
导言 :视网膜母细胞瘤是 3 岁前儿童最常见的眼内恶性肿瘤,可发生于单侧、双侧、遗传性或散发性。发病率约为 1:14,000-20,000 人。这种肿瘤起源于胚胎视网膜细胞。视网膜母细胞瘤基因(RB1)位于 13q14 染色体。她的父母发现她的右眼发白、肿胀、流泪、疼痛,两个月前开始出现这种症状。无家族史。右眼视力(VA)无光感,左眼视力(OS)6/6。外眼压 n-1/触诊,内眼压 n/触诊。在麻醉状态下进行诊断性检查,发现眼睑水肿、结膜注射、角膜混浊、瞳孔、晶状体在外侧无法评估。超声波检查显示有玻璃体播散和眼睑裂。患者接受了长春新碱、依托泊苷和卡铂类药物的化学还原治疗,同时进行了带真皮脂肪移植的去核手术。我们进行了视神经解剖和组织病理学检查,结果显示为视网膜母细胞瘤。讨论:视网膜母细胞瘤的常见症状是白斑。首要任务是挽救生命,其次是保护眼球。治疗以疾病的分期为基础。我们对这名患者进行了化疗,随后进行了去核手术和真皮脂肪移植。眼球摘除术仍是挽救生命的主要疗法,并尽可能进行视神经解剖。真皮脂肪移植可随着儿童眼眶的扩大而生长,因此具有美容效果。结论:本报告阐述了一例视网膜母细胞瘤。化疗和去核手术采用了安全的真皮脂肪移植手术,并植入了合适的假体,以达到更好的美容效果。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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