Rare Anterior Segment Retinoblastoma Masquerading as Corneal Endotheliitis.

Rare Anterior Segment Retinoblastoma Masquerading as Corneal Endotheliitis.
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罕见的眼前节视网膜母细胞瘤伪装成角膜内皮炎。

DOI:
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发表时间:
2016
期刊:
Eye & contact lens
影响因子:
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通讯作者:
Jill S. Anderson
Jill S. Anderson
中科院分区:
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文献类型:
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作者:
A. Kelly;S. Kaufman;R. Ali;A. Grajewski;Jill S. Anderson

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我们提出一个独特的情况下,涉及一个6岁的女性与单侧角膜内皮炎样的发现,谁最终被发现有一种形式的前弥漫性浸润性视网膜母细胞瘤,没有证据表明视网膜参与。患者的假定内皮炎最初治疗局部地塞米松和口服阿昔洛韦没有改善。然后,她接受了多次细针抽吸前房液,病毒聚合酶链反应、病毒培养和流式细胞术的异常结果均为阴性。在初次就诊数月后,出现前房角肿块,活检证实为视网膜母细胞瘤细胞。患者接受了角膜斑放射治疗和全身化疗。患者视力恢复良好,13个月时无肿瘤。前部炎症是一种罕见的与视网膜母细胞瘤相关的伪装综合征;然而,当它确实发生时,它往往与弥漫性后节视网膜母细胞瘤相关。弥漫性前部视网膜母细胞瘤是一种罕见的视网膜母细胞瘤,在视网膜上没有明显的病灶。最后,我们的病人发展为前房角病变,经活检证实为视网膜母细胞瘤。对常规治疗无反应的儿童出现不寻常的角膜内皮炎样改变,应引起临床医生对恶性肿瘤的怀疑,即使没有检测到视网膜病变。
We present a unique case involving a 6-year-old female with a unilateral corneal endotheliitis-like finding, who was ultimately found to have a form of anterior diffuse infiltrating retinoblastoma with no evidence of retinal involvement. The patient's presumed endotheliitis was initially treated with topical dexamethasone and oral acyclovir without improvement. She then underwent multiple fine-needle aspirations of anterior chamber fluid, which were negative for abnormal findings of viral polymerase chain reaction, viral cultures, and flow cytometry. Months after initial presentation, an anterior chamber angle mass developed and a biopsy identified retinoblastoma cells. The patient underwent plaque radiotherapy of the cornea and systemic chemotherapy. The patient regained good vision and is tumor-free at 13 months. Anterior inflammation is a rare form of masquerade syndrome associated with retinoblastoma; however, it tends to be associated with diffuse posterior segment retinoblastoma when it does occur. Diffuse anterior retinoblastoma is a rare form of retinoblastoma with no apparent focus in the retina. Ultimately, our patient developed an anterior chamber angle lesion, which was biopsied and proven to be retinoblastoma. Unusual corneal endotheliitis-like findings in children that are not responsive to conventional treatment should raise the clinician's suspicion of malignancy, even when no retinal lesion is detected.