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双侧弥漫性葡萄膜黑素细胞增生症(BDUMP)的处理 - 病例报告。

Management of Bilateral Diffuse Uveal Melanocytic Proliferation (BDUMP)-A Case Report.

机构信息

Svjetlost Eye Clinic and University Hospital, 10000 Zagreb, Croatia.

Department of Ophthalmology, General Hospital Pula, 52100 Pula, Croatia.

出版信息

Medicina (Kaunas). 2023 Dec 13;59(12):2158. doi: 10.3390/medicina59122158.

Abstract

: This study reports a case of a 62-year-old patient experiencing a significant decline in vision over the past three months. The initial best-corrected visual acuity (BCVA) of 20/20 in both eyes diminished to 20/200 in the right eye (RE) and counting fingers (CF) in the left eye (LE) within this timeframe. The patient was diagnosed with stage 4 ovarian cancer just one month before the significant vision deterioration. : A thorough ophthalmologic examination revealed a notable progression of cataracts and the presence of subretinal fluid on the posterior pole, accompanied by choroidal thickening. The right eye exhibited multifocal, orange-pigmented, and elevated choroidal lesions, while the left eye's fundus examination was impeded by dense cataracts. Optical coherence tomography (OCT) revealed bilateral choroidal thickening with overlying folds and subretinal fluid, and ultrasound imaging of the choroidal lesions indicated moderate homogenous internal reflectivity. : The patient received a diagnosis of BDUMP (bilateral diffuse uveal melanocytic proliferation), a paraneoplastic syndrome marked by simultaneous, bilateral, painless vision loss and the rapid onset of bilateral cataracts with serous retinal detachments. Despite cataract extraction, the expected visual recovery was not achieved (RE: CF; LE: 2/200, respectively). Plasmapheresis showed some success in stabilizing vision loss attributed to serous retinal detachments. : BDUMP necessitates addressing the underlying malignancy for effective treatment. Left untreated, it can lead to near blindness within a year. The prognosis remains grim, with an average survival time ranging from 12 to 15.7 months from the time of diagnosis. Considering this case report, it is crucial to establish effective management plans and further investigate potential treatment methods and predictive markers centered around BDUMP. Collaboration between healthcare professionals and researchers is crucial in addressing the complexities of BDUMP, as the timely diagnosis and treatment of the disease remains a top priority.

摘要

这是一份 62 岁患者的病例报告,患者在过去三个月中视力显著下降。该患者右眼(RE)初始最佳矫正视力(BCVA)为 20/20,在这段时间内下降至 20/200,左眼(LE)视力仅能数指(CF)。在视力显著恶化前一个月,该患者被诊断为 4 期卵巢癌。

全面的眼科检查显示,患者右眼有明显的白内障进展,后极部有视网膜下液,脉络膜增厚。右眼有多发性、橙色、隆起的脉络膜病变,而左眼眼底检查因白内障致密而受阻。光学相干断层扫描(OCT)显示双眼脉络膜增厚,伴有隆起和视网膜下液,脉络膜病变的超声成像显示中等均匀的内部反射。

患者被诊断为双侧弥漫性葡萄膜黑色素瘤增殖(BDUMP),这是一种副肿瘤综合征,其特征是双侧、无痛性视力丧失,双侧白内障迅速发生,并伴有浆液性视网膜脱离。尽管进行了白内障摘除术,但并未达到预期的视力恢复(右眼:CF;左眼:分别为 2/200)。血浆置换术在稳定浆液性视网膜脱离引起的视力丧失方面取得了一些成功。

BDUMP 需要针对潜在的恶性肿瘤进行治疗,否则会在一年内导致近乎失明。如果不治疗,预后仍然很差,从诊断到平均存活时间为 12 至 15.7 个月。考虑到这个病例报告,制定有效的管理计划并进一步研究 BDUMP 的潜在治疗方法和预测标志物至关重要。医疗保健专业人员和研究人员之间的合作对于解决 BDUMP 的复杂性至关重要,因为及时诊断和治疗疾病仍然是重中之重。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7f93/10745080/ddd37d2bdd41/medicina-59-02158-g001.jpg

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