RetCam Ⅱ小儿视网膜检查系统在婴幼儿眼底病筛查中的应用

目的评价RetCamⅡ小儿视网膜检查系统在婴幼儿眼底病筛查中的应用价值。方法对我院568例（1136只眼）婴幼儿患者,进行眼底筛查,将RetCamⅡ小儿视网膜检查系统的眼底检查结果与间接检眼镜确诊结果相比较。结果RetCamⅡ小儿视网膜检查系统检查发现有较大临床意义的眼底病变56例（98只眼）,间接检眼镜检查确诊眼底病变56例（96只眼）。1例患者,RetCamⅡ诊断为2期早产儿视网膜病变（ROP）,被间接检眼镜证实为3期ROP。RetCamⅡ对眼底疾病筛查的敏感度达到100％,特异度达97．9％。结论RetCamⅡ为婴幼儿眼底疾病的筛查提供了一条简便、高效的途径。     

RetCamⅡ早产儿眼底筛查仪在婴幼儿眼病筛查中的应用价值

目的探讨RetCamⅡ早产儿眼底筛查仪在婴幼儿各种眼病筛查中的应用价值,了解婴幼儿眼病的发病情况。方法收集2009年7月到2010年3月在我院行RetCamⅡ眼底检查的婴幼儿,记录其出生胎龄、出生体重、年龄、家族疾病史等临床资料。结果共有32例婴幼儿完成眼部检查,其中,双眼角膜白斑1例、双眼颞侧巩膜化角膜1例、瞳孔区出血1例（1只眼）、先天性白内障3例（3只眼）、小儿黄疸2例（2只眼）、先天性脉络膜缺损1例（1只眼）,双眼视网膜母细胞瘤2例、视神经胶质瘤术后视神经前新生血管1例（1只眼）、双眼先天性色素不均1例、双眼视网膜出血1例。其中1例双眼视网膜母细胞瘤患儿行RetCamⅡ荧光素眼底血管造影检查。结论 RetCamⅡ不仅可用于早产儿视网膜病变的筛查,并且可用于婴幼儿的眼病筛查、术后复查、眼底荧光血管造影等,可提高婴幼儿眼病的诊断率,有利于其早期发现,早期治疗。     

眼科手术或联合系统化学疗法治疗视网膜母细胞瘤的短期观察

观察视网膜母细胞瘤（RB）患者全身化学药物治疗联合局部治疗的临床效果。方法回顾分析2006年8月至2007年9月本院收治的68例84只眼行全身化疗联合局部治疗并随访3个月以上的RB患儿的临床资料。所有患儿均进行眼部超声（B型超声或彩色多普勒超声）、影像学（CT/MRI）以及眼底照相检查（RetCam婴幼儿眼底照相机）,确诊为RB的患儿共66例82只眼,双眼16例,单眼50例;男性36例,女性30例。按照国际眼内视网膜母细胞瘤分期（IIRC）系统分期,A期选择局部治疗（包括激光光凝和冷冻疗法）,B、C、D、E期在系统化疗（CCTV方案,环孢霉素、卡铂、替尼泊苷、长春新碱）的基础上,联合手术（包括局部治疗、眼球摘除和眶内容剜出）治疗。随访时间6~13个月,平均8.6个月。结果 按IIRC分期,A期5只眼,B期6只眼,C期5只眼,D期15只眼,E期51只眼（包括球外转移的）;治疗结束后22只眼得以保存,其中,A~E期分别为5、6、5、4、2只眼,分别占同期治疗眼的100%、100%、100%、26.7%、3.9%;死亡5例,均为E期患者,占总人数的7.6%。结论 眼科手术或联合系统化学治疗RB是有效的,其疗效与患者肿瘤的临床分期有关,A、B、C期患者治疗效果较好,D、E期次之。     

晚期视网膜母细胞瘤视盘遮蔽与视神经侵犯程度的相关性研究

目的评估晚期视网膜母细胞瘤(RB)眼球摘除术前眼底照相检查中视盘遮蔽与眼球摘除术后视神经侵犯程度的相关关系。方法回顾性系列病例研究。收集2012年1月1日至2015年12月31日在北京大学人民眼科诊断为RB且分期为D期或E期并进行眼球摘除术的77例(77只眼)患者资料, 所有患者均在眼球摘除术前首次诊断及随后每3周1次复查时全身麻醉下使用RetCam眼底照相机拍摄眼底图像, 记录视盘遮蔽(眼底像中视盘结构完全不可见)情况。眼球摘除术后以组织病理学诊断是否出现肿瘤侵犯筛板前视神经、肿瘤侵犯筛板后视神经和视神经断端存在肿瘤组织为主要观察指标。比较视盘遮蔽患者与视盘可见患者间肿瘤组织视神经侵犯程度及高危病理因素。采用χ2检验进行统计学分析。结果 77例患者中男性46例, 女性31例, 首次诊断RB年龄为(27.1±22.1)个月。首次诊断RB时77只患眼中62只眼(80.5%)存在视盘遮蔽。29只眼(37.7%)为首次诊断后即进行眼球摘除术;48只眼(62.3%)为首次诊断后先行保眼治疗后行眼球摘除术。77只眼行眼球摘除术前末次眼底照相时, 16只眼(20.8%)视盘可见, 61只眼(79....     

广角数码儿童视网膜成像系统进行早产儿视网膜病变筛查研究

目的：探讨广角数码儿童视网膜成像系统( RetCamⅡ)进行早产儿视网膜病变( ROP)筛查的临床价值。
　　方法：选择2012-01/2015-12产科符合筛查标准的200例400眼早产儿采用RetCamⅡ进行ROP筛查,以双目间接眼底镜检查结果作为金标准,计算RetCamⅡ筛查早产儿ROP的价值。
　　结果：本次筛查200例400眼早产儿,双目间接眼底镜检查共检出ROP病变63眼,ROP患病率为15.8%,其中正常337眼、ROPⅠ期42眼、Ⅱ期14眼、Ⅲ期7眼、Ⅳ期0眼、Ⅴ期0眼;RetCamⅡ共计筛查出ROP病变64眼,其中误诊5眼、诊断级别降低6眼。 RetCam Ⅱ检出结果与双目间接眼底镜检查结果的一致性Kappa值为0.814( P<0.05)。 RetCam Ⅱ筛查早产儿 ROP 病变的灵敏度为93.7%、特异度为98.5%、漏诊率为6.4%、误诊率为1.5%、阳性预测值92.2%、阴性预测值98.8%。
　　结论：RetCam Ⅱ进行早产ROP筛查具有较高的临床实用价值。     

RetCamⅢ数字化广域成像系统在婴幼儿眼病中的应用

目的：：探讨儿童数字化广域成像系统RetCamⅢ在婴幼儿眼病筛查中的应用。方法：收集2013-07/2015-07在我院行RetCamⅢ检查的婴幼儿89例,年龄6~24月龄。所有病例均行眼底照相检查,2例在全身麻醉下行荧光素眼底血管造影检查。结果：家族性渗出性玻璃体视网膜病变19例、永存玻璃体增生症16例、视网膜母细胞瘤12例、外层渗出性视网膜病变12例、先天性脉络膜缺损9例、双眼球震颤8例、视神经先天发育异常6例、早产儿视网膜病变晚期5例、视神经炎2例。其中2例患儿在全身麻醉下行眼底荧光血管造影检查,1例诊断为双眼神经炎、1例诊断为家族渗出性玻璃体视网膜病变。结论：RetCamⅢ为婴幼儿眼病的早期诊断提供了可靠依据,对于指导婴幼儿眼病的早期治疗和视觉康复具有重要的临床意义。     

北京儿童医院2009年至2017年眼内视网膜母细胞瘤患儿死亡原因分析

目的:观察并分析眼内期视网膜母细胞瘤（RB）死亡患儿的临床特征。方法:回顾性临床分析。2009年至2017年于北京儿童医院接受治疗后死亡的眼内期RB患儿14例23只眼纳入研究。其中,男性7例10只眼,女性7例13只眼;单眼5例,双眼9例。患儿年龄（17.2±15.5）个月。所有患儿均行数字化广域眼底成像系统检查。参照眼...     

视网膜母细胞瘤的治疗

近十余年来,视网膜母细胞瘤(RB)的治疗有了新的进展。治疗的目的是保存生命,保留眼球及有用视力。因此目前趋向于保守治疗。如果肿瘤的大小和位置能够允许视力发育,则不摘除眼球。选择非手术治疗并不一定导致死亡率增加。在保守治疗时,应仔细检查病人,早期和确切的诊断无疑是很重要的。在全麻下,应用间接眼底镜,巩膜压陷等方法,可以早期发现位於周边部视网膜的小肿物。Ellsworth观察75％肿瘤是位於赤道部前。对于有RB家族史的婴幼儿应从6周起检查眼底,以后逐渐增加眼底检查的间隔时间。     

筛查发现无症状视网膜母细胞瘤的临床特征、治疗及预后分析

目的:观察分析筛查发现的无症状视网膜母细胞瘤（RB）患儿的临床特征、治疗及预后。方法:回顾性系列病例研究。2006年1月至2019年3月于复旦大学附属眼耳鼻喉科医院眼科确诊的无症状RB患儿8例11只眼纳入研究。其中,男性6例,女性2例;确诊年龄2 d~20个月,中位数确诊年龄6个月;单眼5例,双眼3例。国际眼内RB分期...     

RetCamⅡ在先天性青光眼前房角检查的应用

目的:评价RetCamⅡ在先天性青光眼前房角检查的应用价值。方法:用RetCamⅡ在全身麻醉下对18例可疑先天性青光眼患儿进行双眼前房角检查并照相。结果:通过RetCamⅡ检查前房角,并结合其它眼部检查,确诊原发性婴幼儿型青光眼13例26眼,Axenfeld-Rieger综合征3例6眼,无虹膜性青光眼1例2眼,先天性大角膜1例2眼。结论:RetCamⅡ可以客观记录前房角的结构图像,对先天性青光眼的诊断具有重要价值。     

Metachronous tumor development in unilateral retinoblastoma

PURPOSE: A series of 205 retinoblastoma (RB) patients referred to the Department of Ophthalmology at the University of Siena (Italy) was evaluated in order to assess the proportion of unilateral cases later developing tumors in the companion eye ("metachronous" bilateral retinobastoma) (MBRB). METHODS: The total number of unilaterally affected patients developing tumors in the fellow eye was recorded and the risk factors assessed for the development of asynchronous bilateral retinoblastoma, i.e., family history, tumor multifocality and early age at diagnosis. RESULTS: Only two out of 133 (1.5%) unilateral retinoblastoma patients in our series could be considered affected by MBRB. CONCLUSIONS: The incidence of MBRB in our series was negligible (1.5% of all unilateral cases) compared to other reports. None of the reported risk factors for the development of tumors in the fellow eye was relevant in the present series. Although close follow-up of some unilateral cases is still recommended, thorough examination of the fellow eye, to search for lesions in the peripheral retina, is essential in all cases of unilateral RB. MBRB may be a distinctive clinical entity with specific clinical, genetic and prognostic features. However, all these aspects need to be better investigated in larger series.     

Nature and risk of neovascularization in the fellow eye of patients with unilateral retinal angiomatous proliferation

PURPOSE: To determine the nature and risk of neovascularization in the fellow eyes of patients with unilateral retinal angiomatous proliferation (RAP), a neovascular form of age-related macular degeneration (AMD). METHODS: A consecutive series of 52 patients diagnosed with unilateral RAP were studied retrospectively. Clinical biomicroscopic examination, fluorescein angiography, and indocyanine green angiography were used to evaluate all patients for the development of neovascular manifestations in the fellow eye. RESULTS: Neovascularization developed in the fellow eye in 52 patients over the follow-up period (range, 2-36 months). All patients developed neovascular manifestations of RAP in the fellow eye. Twenty-one patients (40%) developed a RAP lesion within 1 year; 29 (56%), within 2 years; and 52 (100%), within 3 years. At the time of diagnosis of neovascularization in the fellow eye, 8 patients (15%) had a stage I RAP lesion, 36 (70%) had a stage II RAP lesion, and 8 (15%) had a stage III RAP lesion. Other characteristic findings in these patients included the presence of preretinal, intraretinal, and subretinal hemorrhages in 49 patients (94%) and pigment epithelial detachments in 41 patients (79%). CONCLUSIONS: In patients diagnosed with unilateral RAP lesions, the form of neovascularization that develops in the fellow eye is virtually always RAP. The annual and accumulative risk of neovascularization in the fellow eye is higher in patients with RAP than in those with other forms of neovascular AMD. These new findings enhance our understanding of the clinical spectrum of RAP in terms of its natural course and visual prognosis and may possibly offer useful information to establish future treatment options.     

Foveal pseudocyst as the first step in macular hole formation: a prospective study by optical coherence tomography

OBJECTIVE: To establish the natural history of a series of impending macular holes presenting as foveal pseudocysts using optical coherence tomography (OCT). DESIGN: In a prospective observational case series, patients exhibiting a foveal pseudocyst on biomicroscopy were examined with OCT and were followed up for 3 to 26 months (mean, 9.4 months) PARTICIPANTS: Twenty-two eyes of 20 consecutive patients examined for a macular hole in the fellow eye or reporting visual symptoms in only one eye, in whom a foveal pseudocyst was diagnosed on OCT. METHODS: In all cases, fundus biomicroscopy and OCT findings were compared. MAIN OUTCOME MEASURES: Biomicroscopic fundoscopy, OCT scans, and visual acuity. RESULTS: Eight foveal pseudocysts occurred in the fellow eye of an eye with a macular hole, and 14 were diagnosed in patients with unilateral visual symptoms. In four of the 22 eyes, the macula was considered normal on biomicroscopy. In the 18 others, biomicroscopy detected a foveal pseudocyst, radial striae, a yellow spot or ring, or a combination of these findings. No posterior vitreous detachment was seen on biomicroscopy in any of the eyes. On OCT, the cystoid space occupied the inner part of the foveal tissue in the stage 1A impending hole; a stage 1B impending hole corresponded to a cystoid space that extended posteriorly, disrupting the outer retinal layer. During the follow-up period, three pseudocysts evolved into full-thickness macular holes, four turned into lamellar holes, seven resolved completely after detachment of the posterior hyaloid, and eight remained unchanged for a long time. CONCLUSIONS: Foveal pseudocysts are a specific entity occurring either as a primary ocular involvement or in the fellow eye of an eye with a macular hole. Foveal pseudocysts are the first step of full thickness macular hole formation, but they also may evolve into a lamellar hole, may persist unchanged for months, or may resolve completely. Foveal pseudocyst formation may be the result of the incomplete separation of the vitreous cortex at the foveal center and the particular structure of the foveal Müller cells.     

Parents with unilateral retinoblastoma: their affected children.

BACKGROUND: A retrospective analysis of all patients with retinoblastoma on file at the Ophthalmic Oncology Center of the New York Hospital, Cornell University Medical Center was performed to examine the characteristics of retinoblastoma in children with a unilaterally affected parent. METHODS: Data were analysed for laterality of disease, age at initial diagnosis, number of tumour foci at diagnosis, ocular outcome, and patient survival. RESULTS: There were 54 patients, including nine pairs of siblings, diagnosed with retinoblastoma, each of whom had one unilaterally affected parent. The retinoblastoma was bilateral in 49 (91%) patients and unilateral in the remaining five (9%). The mean age at diagnosis was 10.5 months (range 1-36 months) and 35 patients (65%) were diagnosed within the first year of life. The mean number of initial tumour foci was 1.7 per eye; 1.6 foci per eye in bilaterally affected patients, and 3.7 foci per eye in unilaterally affected patients (p = 0.36). Retention of the eye was possible for 51 of 103 eyes affected with retinoblastoma. The mean follow up for patients was 9.0 years, with a range of 1 month to 30.5 years. There were a total of 12 deaths (22%): from retinoblastoma (two), sarcoma (five), cutaneous malignant melanoma (one), cancer type unknown (two), and cause unknown (two). CONCLUSION: Overall, the characteristics of retinoblastoma patients with a unilaterally affected parent, were similar when compared with all retinoblastoma patients with a positive family history.     

Bilaterality of idiopathic macular holes

Background: There has been wide variation in estimates of the incidence of bilateral idiopathic macular holes in the literature. This report of a large series of patients with macular holes provides further information regarding incidence of bilaterality, interval between onset in the first and the second eye, and visual outcome. Methods: A retrospective chart review was done of 550 patients with idiopathic macular holes examined at the Bascom Palmer Eye Institute between 1968 and 1994. The incidence of bilaterality was estimated from 365 patients in whom the fellow eye was normal at the initial examination. The rate of onset in the fellow eye was evaluated by survival analysis. Mean follow-up was 31 months (median 17 months). Results: Patients with incomplete macular holes (stage 1, aborted stage 1, lamellar) or full-thickness holes had a 19% incidence of bilaterality at 48 months follow-up. In the subset of 32 patients with full-thickness macular holes in the first eye, 13% developed full-thickness holes in the fellow eye within 48 months. The median interval between the onset in the first and in the second eye was 17.5 months. Visual acuity was excellent and stable in eyes with aborted stage 1 and lamellar holes. The visual acuity in the first eye with full-thickness macular hole decreased to 20/200 or worse in 79% of cases within 36 months' follow-up. Conclusions: The incidence of bilaterality and poor visual function in the majority of full-thickness idiopathic macular holes by 3 years' duration should be considered when advising patients and planning management.     

Incidence of fellow eye retinal detachment resulting from macular hole

PURPOSE: To determine the incidence of retinal detachment resulting from macular hole (MHRD) in fellow eyes. DESIGN: Observational case series. METHODS: The medical records of 59 normal fellow eyes of cases with MHRD diagnosed between 1994 and 2004 at Osaka Rosai Hospital were retrospectively reviewed. Thirty-nine of the 59 fellow eyes were highly myopic. The average follow-up period was 42 months with a range of seven to 132 months. The incidence of MHRD in fellow eyes, and the interval between the onset of MHRD in the first eye and the fellow eye were determined. The Kaplan-Meier method was used to estimate the probability of the fellow eye developing MHRD at 18 months and five years, and the probability of high myopia in the development of MHRD with log-rank test. RESULTS: Five eyes (8.5%) developed MHRD during the follow-up period. All affected eyes were highly myopic, and the incidence of MHRD among the highly myopic fellow eyes was 12.8%. The average interval was 51 months. The Kaplan-Meier estimated probability of the fellow eye developing MHRD was 3.7% (0% to 8.7% for 95% confidence interval [CI]) at 18 months and 8.0% (0% to 17.7% for 95% CI) at five years. The probability of the fellow eyes with high myopia developing MHRD was significantly higher than that of eyes without high myopia (P = .0304). CONCLUSIONS: The high incidence of MHRD developing in the fellow eye indicates that the fellow eyes should be examined and followed carefully.     

Retinoblastoma: 21-year review

The natural history and prognosis of retinoblastoma were analysed using data from 73 medical records with diagnosis of retinoblastoma registered during 1963-1983 at University Hospital of Kinshasa (Za?re). Of the 73 cases, 24 children (33%) were bilaterally affected. There were 38 (52%) boys and 35 (48%) girls. Ages at diagnosis ranged from 1/2 year to 6 years with an average age of 2.4 years. The white pupil was the most common initial sign. Many of our patients were severely affected with massive tumor filling the entire eye with vitreous seeding and poor general condition. Because of the more advanced stage of the tumor at time of diagnosis, surgery was the usual form of treatment. Only 5 children in our series showed a long-term survival, longer than 3 years after surgical treatment for retinoblastoma.     

The fellow eye of patients with rhegmatogenous retinal detachment

OBJECTIVE: To determine how often the fellow eyes of patients with rhegmatogenous retinal detachment (RRD) without proliferative vitreoretinopathy (PVR) harbor vision-threatening conditions at presentation and during follow-up. DESIGN: Retrospective, observational case series. PARTICIPANTS: Two hundred forty-eight patients with RRD without PVR were studied. INTERVENTION: The authors observed the fellow eye of patients with RRD for vision-threatening pathology. MAIN OUTCOME MEASURE: Detection of vision-threatening pathology in the fellow eye of patients with RRD without PVR. RESULTS: Two hundred forty-eight patients were observed for an average of 5.2 years. One hundred fifty-seven vision-threatening events or diagnoses occurred in the fellow eye, including pre-existing and newly diagnosed conditions, 97 (62%) of which were rhegmatogenous in nature. Fifty-six patients (23%) had retinal detachments in their fellow eye, including those with a history of RRD repair (28 eyes), those presenting with bilateral RRD (4 eyes), and those developing new RRD during follow-up (24 eyes). In addition, 1 patient developed recurrent retinal detachment in the fellow eye during follow-up. Retinal tears were diagnosed and treated with laser retinopexy or cryotherapy in 30 (12%) fellow eyes (4 before the initial examination, 7 upon initial examination, and 19 on follow-up). Lattice degeneration was present in 27 (11%) fellow eyes. CONCLUSIONS: Patients who develop RRD are at considerable risk for developing vision-threatening events, particularly rhegmatogenous events, in the fellow eye.     

Clinical Presentation and Outcome of Retinoblastoma among Children Treated at the National Cancer Institute (NCI) in Gezira,Sudan: A single institution experience

Background: Retinoblastoma (RB) is a rare and unique cancer that affects the eyes of very young children. There are few reports on RB in Sudan.

Materials and Methods: We performed a retrospective study of data from patients diagnosed with retinoblastoma between January 1999 and December 2009 at the National Cancer Institute in Gezira (NCI-Gezira).

Results: Of the 519 cases of childhood cancer treated at NCI-Gezira during the study period, 25 (4.8%) were retinoblastoma. Of these 25 patients with retinoblastoma, there were 13 boys and 19 cases were unilateral. The median age at diagnosis was 36 months (range, 8–60 months). The disease was localized in 9 patients, regional in 5 patients, and metastatic in 11 patients. The most frequent symptoms were enlarged eye (n?=?14) and leukocoria (n?=?8). Nine patients (36%) have been lost to follow-up; 9 were alive at last follow-up (7 in remission, 2 progressed); and 7 have died (5 from disease and 2 from unrelated causes). Twenty-two eyes were enucleated in 16 patients (6 bilateral and 10 unilateral). Pathologic examination of the enucleated eyes could only be completed in 11 patients. Diagnostic imaging in the form of computerized tomography scans or ultrasonography of the brain and orbit was done for 10 patients (40%).

Conclusions: Although these findings are not surprising, and similar to reports from other developing countries, we hope our work will provide a foundation for strategies to improve outcome for retinoblastoma in our center such as proper training, public awareness, team approach, and twinning.