Morbus Coats

Advanced Coats' disease is one of the most difficult differential diagnoses of retinoblastoma in early childhood. We describe the clinical and histological findings in two boys, ages 9 months and 21 months, with unilateral leucocoria. Despite comprehensive diagnostics that included examination under general anaesthesia, magnetic resonance imaging, and ultrasound, retinoblastoma could not be excluded, and the eyes were enucleated. Histological diagnosis of Coats' disease was confirmed. Because differentiation between retinoblastoma and Coats' disease may be difficult, enucleation seems to be indicated in uncertain cases due to the reduced visual prognosis and the risk of secondary complications in advanced Coats' disease.     

儿童白瞳症的超声诊断价值

探讨不同眼内疾病所致儿童白瞳症的B型超声表现，为临床诊断及鉴别诊断提供客观依据。方法对26例（35只眼）白瞳症进行B型超声检查。结果不同的疾病所致的白瞳症患儿B型超声检查结果不同。结论 B型超声检查为儿童白瞳症的临床诊断及鉴别诊断提供客观、可靠的依据，具有极其重要的价值。     

Echography in the diagnosis of retinoblastoma

Echography was performed on 38 eyes enucleated for suspected retinoblastoma. The two major criteria for the echographic diagnosis of retinoblastoma were the presence of intratumoral calcifications and normal axial length of the eye with respect to age. No errors in diagnosis were made on the 25 eyes considered to be cases of retinoblastoma by echographic criteria. Two doubtful cases turned out to be pseudogliomas and in 11 cases the diagnosis of pseudoglioma by ultrasonic examination was confirmed by histopathology. The results are compared with those of 67 cases of retinoblastoma examined from 1964 to 1977 by one of the authors and to other published series. This study helps to demonstrate the importance of echography in the diagnosis of retinoblastoma and in the differentiation between retinoblastoma and pseudoglioma .     

Clinical presentation of retinoblastoma in Eastern Nepal

BACKGROUND: The purpose of the present study was to determine modes of presentation of retinoblastoma and the association between proptosis with orbital extension and histopathologically detectable optic nerve infiltration by the tumour. METHODS: Clinical records and histopathological reports of patients with retinoblastoma were studied retrospectively. The work was undertaken at a tertiary care hospital in eastern Nepal during the period from September 1995 to August 2002. RESULTS: A total of 43 patients with retinoblastoma were studied. Among them unilateral involvement was found in 39 patients (90.70%) and bilateral involvement in four patients (9.30%). The mean age of presentation was 3.04 +/- 1.80 years (95% confidence interval = 2.49-3.59). The commonest mode of presentation was proptosis with orbital extension in 19 eyes (40.42%) followed by leucokoria in 14 eyes (29.78%). Histopathological reports of 42 eyes (one patient died shortly after admission and did not have an autopsy) confirmed the diagnosis of retinoblastoma and revealed that the optic nerve cut section had been infiltrated in 18, of which 16 had the clinical finding of proptosis with orbital extension. This was statistically significant (relative risk = 21.33, P < 0.001). CONCLUSION: The commonest mode of presentation of retinoblastoma in Nepal is proptosis. Presence of proptosis with orbital extension is associated with optic nerve cut end infiltration by the tumour. The diagnosis or presentation of retinoblastoma in Nepal is delayed and there is tremendous scope for improvement in its management.     

Neonatal or juvenile leucocoria

The differential diagnosis between the diseases which cause leucocoria is discussed. Among them congenital cataract, persistence of the primary vitreous, retrolental fibroplasia, retinoblastoma, Coats' disease, von Hippel's disease, inflammatory pseudoglioma, Norrie's disease, and organized intraocular haemorrhage are mentioned.     

Retinal dysplasia with hydrocephalus (author's transl)

A Study is described of a family in which four children of the same generation died during early childhood; three of them had an obvious hydrocephalus. Two of the latter could be examined clinically, and their eyes removed for pathology. One of them presented clinically bilateral leucocoria; histology showed a total detachment of a dysplastic retina, with absence of development of the vitreous. The other child had apparently normal eyes. Yet on microscopical examination there was a discrete retinal dysplasia without retinal detachment in both eyes, and a persistent hyaloid artery, in one. This association of hydrocephalus with retinal dysplasia is clinically and genetically different from other types of retinal dysplasia.     

视网膜母细胞瘤并发白内障的手术效果

目的：分析视网膜母细胞瘤（Rb）患儿并发白内障的诊断时间、手术时间,手术方式以及手术效果,并评估其行白内障手术的时机和安全性。方法：回顾性分析2010年1月至2016年12月在北京儿童医院、北京同仁医院和泉州市儿童医院行白内障摘除术的Rb患儿23例（25眼）。其中男11例（11眼）,女12例（14眼）。7眼行透明角膜25G切口,18眼行睫状体平坦部25G或者27G巩膜微切口。随访1～72个月,平均（24±16）个月。统计手术时间,术前Rb治疗方式及次数,术后眼底检查是否有肿瘤复发或转移。结果： 患儿平均手术年龄（35±19）个月。自确诊Rb到发现白内障的时间（中位数）为19 个月,发现白内障到白内障手术时间（中位数）为5个月,最后一次Rb治疗到行白内障手术的时间（中位数）是10个月。行白内障摘除术前全身化疗22例（102次）,局部光凝12眼（62次）,眼内注药11眼（19次）,放疗1眼（1次）,眼外冷冻6眼（10次）,玻璃体切割手术17眼（20次）。2眼在Rb治疗过程中不能查见眼底,而眼B超检查示肿瘤复发,故行白内障手术,术后局部激光治疗复发肿瘤,随访期内未再复发。23眼为肿瘤局限钙化后行白内障手术,术后19眼无肿瘤复发及转移,未做治疗;4眼出现肿瘤晚期并发症（1眼继发青光眼,2 眼前房出血,1 眼眼内肿瘤复发）,行眼球摘除术。术后行病理检查,摘除眼未见眼球外有Rb转移。结论：对于Rb患儿,必要的白内障手术为眼底检查提供了清晰的视野。对于肿瘤局限钙化病情稳定的患眼,在无肿瘤生长的角膜或者睫状体平坦部做切口行白内障手术是安全的。     

The National Registry of Retinoblastoma in Japan (1983–2014)

Purpose

To review the clinical findings of retinoblastoma recorded over a period of 32 years by the National Registry of Retinoblastoma in Japan.

Study design

Retrospective.

Methods

We reviewed the diagnoses, clinical pictures, and treatment data recorded on a yearly basis from 1983 until 2014 by major Japanese medical facilities.

Results

A total of 2360 patients (1225 boys, 1135 girls) were analyzed. Of those, 67.3% had unilateral retinoblastoma, 32.7% had bilateral retinoblastoma, and 6.7% had a family history of retinoblastoma. The average occurrence frequency for retinoblastoma was 1:16,823 births/year. At diagnosis, 89.0% of the patients were aged younger than 3 years and 41.0% were aged younger than 1 year. The most common initial symptom was leukocoria (48.9%), which was followed by cat’s eye (17.1%) and strabismus (14.8%). Of the total 3131 eyes, 53.0% were Reese-Ellsworth group V. As per the International Classification of Retinoblastoma, 33.7% of the eyes were group D, and 30.1% were group E. Enucleation was performed in 1545 eyes (52.2%), and subsequent treatment was administered in 379 eyes. Conservative therapy was performed in 1415 eyes (47.8%) of 926 patients. The number of eyes receiving conservative therapy increased every year, even in about 30% of those with advanced group V eyes. Radiation therapy was the primary conservative therapy before the year 2000 and was thereafter replaced by chemotherapy. In the eyes receiving chemotherapy, anticancer agents were administered systematically in one-third of the eyes, locally in another one-third of the eyes, or as a combination of both in the other one-third. Conservative therapy successfully preserved in over 90% of the eyes of groups I to IV and in 78.1% of the eyes of group V.

Conclusions

Retinoblastoma is often diagnosed at an advanced stage. With the development of systemic and local chemotherapy, the number of eyes with advanced retinoblastoma preserved by conservative therapy has been increasing.

     

66例视网膜母细胞瘤患儿临床资料分析

目的观察西南地区视网膜母细胞瘤(RB)患眼的临床特点。方法回顾性临床研究。2010年1月至2017年12月在四川大学华西医院眼科检查确诊的RB患儿66例82只眼纳入研究。患儿均行眼部B型超声、眼眶CT或MRI检查,行广角数码视网膜成像系统检查10例。组织病理学检查确诊29例,临床症状结合影像学检查确诊37例。根据肿瘤是否侵及眼眶和视神经分为眼外期和眼内期;后者根据国际眼内期RB分期标准分为A^E期。根据不同分期行相应治疗。回顾分析患儿基本情况、就诊年龄、病程、就诊原因、分期、治疗方案和保眼率。结果66例患儿来自四川、云南、贵州省分别为56、2、2例,西藏自治区6例。可明确常住地43例,其中来自农村27例(62.8%)。男性38例(57.6%);单、双眼分别为50(75.8%)、16(24.2%)例。初诊51例(77.3%),复诊15例(22.7%)。初诊51例中,平均就诊年龄(20.9±14.4)个月;单、双眼患儿平均就诊年龄分别为(23.2±14.7)、(11.2±7.6)个月。可明确病程及就诊原因41例,患儿平均病程(90.6±115.2)d。瞳孔区发白32例(62.7%),眼部红肿4例(9.8%),其他原因5例(12.2%)。复诊15例中,平均就诊年龄为(63.6±46.8)个月;平均病程(32.8±45.5)个月。复发、手术后并发症分别为5(33.3%)、3(20.0%)例;瞳孔区发白4例(26.7%);眼球突出、眼部红肿分别为2(13.3%)、1(6.7%)例。82只眼中,入院治疗50只眼;其中,初诊37只眼,复诊13只眼。初诊37只眼中,眼内期31只眼(83.8%),包括A^C期5只眼(13.5%)、D^E期26只眼(70.3%);眼外期6只眼(16.2%)。A^C期5只眼均行激光光凝和(或)冷冻联合全身化学药物治疗(化疗);D期4只眼行眼动脉介入化疗;其余D^E期22只眼、眼外期6只眼中,行眼球摘除、眶内容物剜除手术分别为19(51.3%)、2(5.4%)只眼,放弃治疗7只眼(18.9%)。复诊13只眼中,既往已行眼球摘除6只眼(46.2%),其中手术后复发5只眼;眼外期4只眼(30.8%);D^E期3只眼(23.1%)。行眶内容物剜除、眼球摘除手术分别为5(38.5%)、4(30.8%)只眼;行眼整形手术1只眼(7.7%);放弃治疗3只眼(23.1%)。入院治疗患儿保眼率为18.0%,眼内期保眼率29.0%,眼外期保眼率0.0%。结论西南地区RB患儿就诊时病程长、分期晚、保眼率低。     

Enucleation for retinoblastoma: the experience of a single center in Jordan

Despite multiple advances in the management of retinoblastoma, enucleation remains an essential therapeutic modality. We studied patients who underwent enucleation at the King Hussein Cancer Center in Jordan. We retrospectively reviewed medical records of children with retinoblastoma who were treated at our center from June 2002 to February 2008. Twenty-eight eyes from 27 patients were enucleated. Median age at diagnosis was 1.1 years (range, 0.3–6.3 years). Twenty-six eyes (93%) had advanced disease (RE groups IV and V). Seventeen patients (61%) had unilateral retinoblastoma, and 11 (39%) had bilateral retinoblastoma. The median time from diagnosis to enucleation was 0.45 months (range, 0–45 months; mean, 4.4 months) and was longer for patients with bilateral retinoblastoma (median, 2.2 vs. 0.2 months; P = 0.034). Twenty enucleated eyes (71%) did not show high-risk pathologic features. Seventeen eyes with advanced intraocular disease were enucleated at the time of presentation, whereas chemoreduction was attempted for the other 19 eyes with advanced intraocular disease. Enucleation was then recommended for nine (47%) of those eyes. Enucleation at the time of diagnosis was feasible for most patients with advanced disease. Attempted salvage of eyes with advanced disease is justified, particularly in patients with bilateral disease. We were able to salvage almost half of these eyes. We hope our study provides new insights for counseling patients.     

The effect of initial recognition of abnormalities by physicians on outcome of retinoblastoma.

PURPOSE: The purpose of this study was to assess the effect of early diagnosis by physicians on outcome of retinoblastoma. METHODS: This was an observational case series in which the records of 36 patients with retinoblastoma were reviewed. The age at onset of first symptoms and diagnosis, identity of the individual who first recognized an abnormality, treatment, and outcome were analyzed. RESULTS: Twenty-two patients had unilateral retinoblastoma. Both eyes were preserved in only 2 of these patients, both of whom had abnormalities first noted by their pediatricians. Fourteen patients had bilateral retinoblastoma. Both eyes were preserved in the 5 bilateral patients who were monitored from birth with serial examinations under anesthesia because of family histories of retinoblastoma. Enucleation of at least 1 eye was required in 8 of the 9 bilateral patients whose families first noted abnormalities. Enucleation of at least 1 eye was required in 27 of 28 patients (96%) in whom abnormalities were first noted by parents. CONCLUSION: In this series, the only patients with unilateral retinoblastoma who did not require enucleation were those in whom pediatricians, rather than family members, first noted ocular abnormalities. Both eyes were preserved in all patients with bilateral retinoblastoma who underwent serial examinations under anesthesia because of family histories of retinoblastoma. The prognosis for retinoblastoma appears to be greatly improved when physicians initially detect abnormalities. Educational efforts directed at early detection of retinoblastoma by pediatricians and other primary care providers should be encouraged.     

Delay in diagnosis of retinoblastoma: risk factors and treatment outcome

BACKGROUND: Delay in diagnosis of retinoblastoma causes considerable parental distress; however, the primary healthcare professional (PHP) may have difficulty detecting the most common presenting symptom-leucocoria. Alternatively, the PHP may not appreciate that retinoblastoma is the pathology underlying more common ocular symptoms in infants and young children. METHOD: The parents of 100 recently diagnosed patients with retinoblastoma were interviewed to establish the extent of diagnostic delay, ascertain any associated risk factors, and to determine whether or not delay influenced treatment outcome. RESULTS: Although nearly 50% of patients were referred to an ophthalmologist within 1 week of first consulting a PHP, one quarter waited more than 8 weeks. There was a significantly increased risk of diagnostic delay in younger patients, those presenting with squint rather than leucocoria, and those first presenting to a health visitor rather than to a general practitioner. The risk of local tumour invasion was significantly increased by diagnostic delay. Treatment with primary enucleation was not increased by diagnostic delay. There were no deaths during the study period. CONCLUSION: Primary healthcare professionals require education about the importance of ocular symptoms, especially squint, in paediatric patients.     

Computed tomography in the diagnosis of retinoblastoma.

The role of orbital computed tomography (CT) in the management of retinoblastoma is reviewed. All 21 patients with retinoblastoma studied with high-resolution computed tomography had intraocular calcification demonstrable in at least one eye. 83% of tumours showed evidence of calcification on CT scan. The degree of calcification appeared to depend on tumour size; only small tumours were devoid of calcification. The amount and distribution of calcification was similar on both histological study and CT scan. In patients under 3 years old in whom a retinoblastoma is suspected the presence of calcification on CT is virtually diagnostic of it. Patients with leucocoria without calcification on CT probably have a simulating lesion.     

Ultrasound biomicroscopy in the management of retinoblastoma

Purpose

To determine the role of ultrasound biomicroscopy (UBM) in the management of children affected with retinoblastoma.

Methods

A review of clinical records of children with the diagnosis of retinoblastoma at the Hospital for Sick Children from January 1995 to December 2007, for whom UBM was used to determine the extent of intraocular tumor. Clinical characteristics were compared with UBM. Pathological correlation was performed for enucleated eyes.

Results

In total, 101 eyes of 75 patients were included in the final analysis. Only 11 eyes were diagnosed on UBM to have extension of the tumor anterior to the ora serrata, and were enucleated. Histopathological examination confirmed the anterior extension in all the 11 eyes. In total, 50 eyes were enucleated because of various reasons, such as poor visual prognosis (12 eyes), unilateral group D or E (23 eyes), recurrences (8 eyes), and treatment failure (7 eyes). None of those patients were found to have anterior extension of the disease on histopathological examination. UBM did not yield any false negative (0/50) or any false positives (0/11).

Conclusions

The UBM provided a sensitive and reproducible visualization of the anterior retina, ciliary region, and anterior segment allowing a better staging of the advanced disease process. Primary assessment of the true extent of retinoblastoma is critical for the selection of an optimal management approach.     

Retinoblastoma. HLA expression induced by retinoic acid

The full expression of Class 1 antigens of the major histocompatibility complex (HLA A,B,C,) on ocular cell surfaces appears to be developmentally regulated; HLA is undetectable in retina from 16-week-old fetal eyes but is present in adult retina. Cultured retinoblastoma (RB) may serve as a useful model of retinal cell development. SF81 is an undifferentiated subculture of in vitro retinoblastoma with little evidence of spontaneous differentiation and lack of expression of HLA, attesting to the "undifferentiated" character of this cell line. RB SF81 cells were treated with retinoic acid, a known stimulator of differentiation (optimal concentration 5 X 10(-8) M). HLA expression was evaluated with immunofluorescence, immunoperoxidase, and radioimmunoassay, using monoclonal antibodies. RB SF81 treated with retinoic acid showed a significant, persistent increase in HLA levels by 24 hours compared with controls. Thus, retinoic acid has been documented to induce the surface expression of HLA in undifferentiated retinoblastoma. Developmental parallels between embryonic retina and in vitro retinoblastoma treated with retinoic acid exist.     

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,A期、B期、C期分别为4、3、4只眼。有RB家族史1例。行Rb1基因检测4例。8例患儿的16名父母及3名兄弟姐妹均行常规眼部检查及超广角眼底照相检查。全身静脉化学药物治疗(IVC)采用卡铂、长春新碱和依托泊苷联合方案;眼动脉灌注化学药物治疗(IAC)采用马法兰和卡铂;眼局部治疗包括冷冻和经瞳孔温热疗法(TTT)。平均随访时间47.25个月。结果8例患儿均无任何眼部症状。行IVC 5~6次者6例,IAC 3次者1例,仅眼局部治疗者1例。11只眼中,行眼局部冷冻治疗9只眼,TTT治疗8只眼。随访中发现新瘤体2例,发现新瘤体时间为化疗结束后平均6.3个月。末次随访时,瘤体消退11只眼,稳定11只眼;无眼球摘除者,保眼率100%(8/8)。最佳矫正视力0.1、1.0者分别为3、5只眼;未查及者3只眼。行Rb1基因检测的4例患儿中,未检测到明确致病变异3例,检测到Rb1基因1c.35_69del(p.T12fs)杂合突变1例。16名父母中,双眼眼球痨1名,为双眼RB患儿的母亲。3名兄弟姐妹中,双眼RB 1例,为双眼RB患儿的哥哥。结论眼部筛查有助于发现早期RB,经IVC、IAC等全身或局部化学药物治疗及冷冻、TTT等眼局部治疗后保眼率高,长期视力预后较好。     

视网膜母细胞瘤化学减容加局部治疗的临床疗效分析

目的 总结、探讨视网膜母细胞瘤化学减容加局部治疗的初步疗效.方法 回顾性系列病例研究.分析2005年1月至2007年12月间29例(40只眼)视网膜母细胞瘤(RB)接受化学减容加局部治疗患者的临床资料,化学减容加局部治疗以VEC方案化学缩瘤,辅以冷冻、TTT等局部巩固治疗.随访时间为12～50个月,平均38个月.结果 其中4例单眼发病,25例双眼发病.40只眼参照眼内型RB国际分期,A期14只眼(35%)、B期9只眼(22.5%)、c期4只眼(10%)、D期10只眼(25%)、E期3只眼(7.5%).17只眼存在玻璃体、视网膜下种植.总体眼球保存率为75%(30/40).瘤体呈现退缩模式并稳定的治愈率依分期不同呈现递减趋势,A期100%(14/14)、B期100%(9/9)、C期75%(3/4)、D期40%(4/10)、E期0%(0/3).4只眼肿瘤复发,从化疗结束至复发眼球摘除的平均时间为5.7个月.10只眼最终行眼球摘除,其中C期1只眼、D期6只眼、E期3只眼.随访中无1例死亡或产生白血病等严重并发症.结论 化学减容加局部治疗可以有效保存部分RB患儿的眼球,乃至有用视功能.眼内型RB的国际分期对临床工作具有指导意义.     

Full-field electroretinography under general anesthesia in retinoblastoma

Purpose

To investigate the electrical responses of the retina in retinoblastoma (RB), by recording full-field electroretinography (ERG) under general anesthesia.

Methods

The ERG was recorded using Ephios hand-held portable ERG system, according to International Standards for Clinical Electrophysiology of Vision. Forty-eight eyes of 43 cases and 33 eyes of 33 controls were enrolled. The cases were classified based on international intraocular retinoblastoma classification (IIRC). Forty-eight eyes of cases were divided into 30 cases with active RB and 18 cases with regressed RB.

Results

The amplitudes of a- and b-waves were decreased as compared to controls in all subgroups. The implicit times of all RB patients from group A to C differed statistically from controls (p value < 0.05) except for single-flash rod response. The ERG waveforms in group E eyes were non-recordable. The comparison of ERG parameters between active and regressed groups (IIRC groups A and B) was statistically insignificant. Single case follow-up of unilateral RB after systemic chemotherapy showed improvement in amplitudes compared to baseline parameters.

Conclusions

Reduced amplitudes and delayed implicit times were noted in advanced disease. The ERG of RB cases did not follow any specific pattern of waveform. ERG appears to be a dynamic parameter to observe changes following treatment for RB. Although ERG is not a diagnostic test for RB, it can be used as a complementary test to assess the residual retinal function in RB eyes.     

Vitrectomy in eyes with unsuspected retinoblastoma

OBJECTIVE: To analyze patient management and prognosis after vitrectomy in eyes with unsuspected retinoblastoma. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Eleven consecutive patients who had undergone vitrectomy on an eye with unsuspected retinoblastoma. MAIN OUTCOME MEASURES: The two main outcome measures were ultimate patient management and the development of retinoblastoma metastasis. RESULTS: Of more than 900 consecutive patients with retinoblastoma managed on the Ocular Oncology Service at Wills Eye Hospital in Philadelphia, 11 (1%) had prior vitrectomy in an eye with viable tumor before referral to us for suspected retinoblastoma. The main preoperative diagnoses included vitreous hemorrhage in seven patients (64%), toxocariasis in two patients (18%), toxoplasmosis in one patient (9%), and endophthalmitis in one patient (9%). In no case was retinoblastoma suspected before vitrectomy. The mean patient age at vitrectomy was 6 years. Retinoblastoma was later suspected during vitrectomy in two patients (18%), on cytologic examination of the vitrectomy specimen in eight patients (73%), and after referral in one patient (9%). The mean interval between vitrectomy and referral to us was 23 days. On examination, the globe was classified as Reese-Ellsworth group Vb in all 11 patients (100%). Anterior chamber tumor cells were clinically visible in four eyes (36%), hyphema in two eyes (18%), and iris neovascularization in two eyes (18%). Retinoblastoma cells were visualized in the vitreous in seven eyes (64%) and not visualized in four eyes (36%) that had vitreous blood. Enucleation was necessary in all 11 patients (100%). Adjuvant treatment was delivered in 10 patients (91%), using orbital radiotherapy in nine patients (82%) and chemotherapy in nine patients (82%). Histopathologic evidence of retinoblastoma invasion was documented in the episclera (two eyes; 18%), anterior chamber (seven eyes; 64%), iris (five eyes; 45%), ciliary body (five eyes; 45%), choroid (three eyes; 27%), and optic nerve (four eyes; 36%; prelaminar, two eyes; postlaminar, two eyes). The vitrectomy ports, Tenon's fascia, cut end of the optic nerve, and orbit were free of tumor. Of the 10 patients who received prophylactic chemotherapy, radiotherapy, or both in addition to enucleation for prevention of retinoblastoma metastasis, none (0%) experienced metastasis or orbital recurrence during the mean follow-up of 7 years (range, 0.2-24 years) from the time of retinoblastoma diagnosis. However, one patient was referred to us after the development of metastatic retinoblastoma, and despite aggressive chemotherapy and radiotherapy after enucleation, died 24 months later. CONCLUSIONS: Retinoblastoma may present with atypical features such as vitreous hemorrhage or signs of vitreous inflammation, particularly in older children. Vitrectomy should be avoided in these cases until the possibility of underlying retinoblastoma is excluded. If vitrectomy is performed in an eye with unsuspected retinoblastoma, enucleation combined with adjuvant chemotherapy, radiotherapy, or both without delay is advised to prevent systemic tumor dissemination.