Purpose To present the clinical characteristics and management outcomes of Coats disease with retinoschisis (retinal cyst). Methods This was a retrospective review of 13 eyes from 13 consecutive Coats disease patients with retinal cyst. Clinical findings from multimodal ophthalmic imaging were recorded to characterize retinal cyst in Coats disease. All eyes were initially treated with endolaser photocoagulation combined with external drainage of cystic fluid and intravitreal ranibizumab injection. Patients were subsequently followed up to analyze anatomic and visual outcomes. Results All retinal cysts were associated with extensive retinal exudation; 3 eyes (23.1%) presented with exudative retinal detachment. A total of 92.3% (12/13) of the retinal cysts were located in the inferotemporal quadrant. Fluorescein angiography demonstrated telangiectasia surrounding and/or at the edge of the retinal cyst. Eleven eyes (84.6%) underwent endolaser photocoagulation via a nonvitrectomy approach, and 2 eyes (15.4%) underwent endolaser photocoagulation after vitrectomy due to the presence of epiretinal membrane. An average of 1.5 additional laser photocoagulation sessions was performed per patient. After a median follow-up of 23 months (range, 11–33 months), all eyes demonstrated retinal cyst resolution and no active disease. Subretinal fibrosis was observed in 9 eyes (69.2%); of these, 8 had macular subretinal nodules. In total, 30.8% of the eyes had a final vision ≥20/200. Conclusion The presence of retinoschisis (retinal cyst) in Coats disease is strongly associated with retinal telangiectasia and exudation and is indicative of active vascular disease requiring effective ablative treatment. We propose a novel approach, endolaser photocoagulation combined with external drainage of cystic fluid and intravitreal ranibizumab injection, which was effective in achieving final anatomic improvement with retinal cyst resolution.
// Shuxian Chen 1, * , Xunda Ji 2, * , Ming Liu 1 , Zhengrong Xia 1 , Hui Zheng 1 , Qiufeng Yin 1 , He Wang 3 , Yuhua Li 1 1 Department of Radiology, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai 200092, China 2 Department of Ophthalmology, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai 200092, China 3 Institute of Science and Technology for Brain-Inspired Intelligence, Fudan University, Shanghai 200433, China * These authors have contributed equally to this work Correspondence to: Yuhua Li, email: liyuhua10@sina.com He Wang, email: hewang@fudan.edu.cn Keywords: retinoblastoma, intra-arterial chemotherapy, MRI, ADC value, children Received: December 23, 2016 Accepted: March 14, 2017 Published: March 21, 2017 ABSTRACT Retinoblastoma is the most common intraocular malignant tumor of childhood. Intra-arterial chemotherapy (IAC) is a recently popularized technique used for the treatment of retinoblastoma, to decrease mortality, increase preservation of the eye, and prevent blindness. Along with the extensive use of IAC, it is important to apply noninvasive examination methods to assess the activity of the tumor and the risk factors for disease dissemination without histopathological confirmation. There are few studies that have assessed the value of magnetic resonance imaging (MRI) in evaluating the efficacy and complications of IAC for retinoblastoma. We retrospectively analyzed the MRI features of 60 patients with unilateral retinoblastoma given the primary treatment of IAC from January 2014 to February 2016 in our hospital. Our study showed that MRI could well assess the decreased activity of the tumor after IAC, presenting with diminished tumor size, increased apparent diffusion coefficient (ADC) values (from 0.94 ± 0.24 × 10 −3 mm 2 /s to 2.24 ± 0.40 × 10 −3 mm 2 /s), and a reduced degree of enhancement of the tumor. Our study also showed that MRI can monitor the risk factors of abnormal enhancement of the postlaminar optic nerve, to avoid unnecessary enucleation. Meanwhile, the results showed that the main late complications after IAC included affected eyeball volume reduction, subretinal hemorrhage, vitreous hemorrhage, vitreous opacity, cataractous len, and choroidal vascular ischemia.
This study aimed to evaluate the efficacy and complications of intra-arterial chemotherapy (IAC) as a primary treatment for advanced unilateral retinoblastoma in Chinese patients.This study was a retrospective review of patients with advanced unilateral retinoblastoma treated with IAC as the primary treatment. The IAC procedures were performed using a balloon-assisted technique. The clinical status and treatment complications were recorded at each visit. Kaplan-Meier analysis was performed to estimate recurrence-free survival and ocular survival.In total, 116 eyes of 116 patients with advanced unilateral retinoblastoma were enrolled, including 66 eyes (57%) in group D and 50 eyes (43%) in group E. All treated eyes received a mean of 3 cycles of IAC (range, 3-5), and 66% of the eyes were combined with local consolidation therapy. The median follow-up time was 39 months (range, 22-57 months). The 3-year recurrence-free survival and ocular survival rates were 68.8% (95% CI, 59.2-76.6%) and 88.5% (95% CI, 80.9-93.2%), respectively. Moreover, the 3-year ocular survival rate in group D was significantly higher than that in group E (96.9%, 76.3%; P < 0.01). The common ocular complication was vitreous hemorrhage (19.8%). No deaths or severe systemic complications occurred.Primary intra-arterial chemotherapy is effective for the treatment of advanced unilateral retinoblastoma, especially in group D, with acceptable toxicity.
The risk of extraocular extension from injecting chemotherapy into eyes with retinoblastoma is minimally understood; however, understanding this risk is important because of the increasing use of intravitreous chemotherapy.To evaluate the risk of extraocular extension in eyes with retinoblastoma that have received intravitreous chemotherapy injections.This retrospective cohort study was performed in 655 patients at 10 retinoblastoma centers in North and South American, European, Israeli, and Chinese centers. Physicians at the retinoblastoma centers administered more than 120 intravitreous chemotherapy injections in eyes with retinoblastoma from February 1, 1999, through February 28, 2017.Risk of extraocular extension with secondary observational variables, including injection and precautionary techniques.A total of 3553 intravitreous chemotherapy injections (3201 melphalan hydrochloride, 335 topotecan hydrochloride, and 17 methotrexate sodium) were administered to 704 eyes in 655 patients with retinoblastoma (mean [SD] age of patients at the time of the initial injections, 31.6 [11.6] months; 348 male [53.1%]). There were no extraocular tumor events related to prior intravitreous injections. This finding resulted in a calculated proportion of zero extraocular events per eye. According to the rule of 3, the risk is no greater than 0.08% injections. All 10 centers included in this study used at least 2 presumed precautionary injection methods (lowering of intraocular pressure, cryotherapy, ocular surface irrigation, ultrasonic biomicroscopy surveillance of the injection site, and subconjunctival chemotherapy deposition).With use of at least 2 presumed precautionary safety methods, no extraocular extension of tumor events occurred. According to the rule of 3, this finding suggests that the risk is no greater than 0.08% injections.
Our study aimed to comprehensively investigate the age of onset, magnetic resonance imaging (MRI) features, and prognosis of children with trilateral retinoblastoma (TRB).We included 14 patients with TRB diagnosed or followed up in our hospital. The age of onset and MRI features of the intraocular tumor and intracranial lesions were evaluated. A follow-up study was also conducted.A total of 11 participants were diagnosed with concurrent TRB at the age of 11.1±7.4 months, and 3 participants had late-onset TRB at age 37±19.1 months. The incidence of TRB with unilateral eye involvement was 7.1% (1/14). The intraocular tumors showed intense enhancement in contrast-enhanced T1-weighted images (WI) and significant diffusion restriction in diffusion WI (DWI) with an apparent diffusion coefficient (ADC) of (0.619±0.22)×10-3 mm2/s. The intracranial lesions showed similar DWI aspects with an ADC value of (0.680±0.206)×10-3 mm2/s. Therapeutically, 8 participants had a period of intraocular tumor stabilization and significant intracranial lesion volume reduction after chemotherapy, and 6 participants had given up treatment. Only 2 participants who simultaneously received high-dose chemotherapy and autologous hematopoietic stem cell rescue were still alive with no recurrence at 24 and 54 months of follow-up. The 1-, 2-, and 3-year overall survival (OS) rates were 80%, 18.75%, and 12.5%, respectively.Patients with unilateral or bilateral RB can develop TRB. The intraocular and intracranial tumors showed slightly different ADC values. High-dose chemotherapy, combined with stem cell rescue can significantly improve survival. A long term and scheduled follow-up before 60 months of age is necessary for screening later-onset TRB patients.
Abstract Background/Objectives Retinoblastoma is a common childhood intraocular malignancy, the bilateral form of which most commonly results from a de novo germline pathogenic variant in the RB1 gene. Both advanced maternal age and decreasing birth order are known to increase the risk of de novo germline pathogenic variants, while the influence of national wealth is understudied. This cohort study aimed to retrospectively observe whether these factors influence the ratio of bilateral retinoblastoma cases compared to unilateral retinoblastoma, thereby inferring an influence on the development of de novo germline pathogenic variants in RB1 . Subjects/Methods Data from 688 patients from 11 centres in 10 countries were analysed using a series of statistical methods. Results No associations were found between advanced maternal age, birth order or GDP per capita and the ratio of bilateral to unilateral retinoblastoma cases ( p values = 0.534, 0.201, 0.067, respectively), indicating that these factors do not contribute to the development of a de novo pathogenic variant. Conclusions Despite a lack of a definitive control group and genetic testing, this study demonstrates that advanced maternal age, birth order or GDP per capita do not influence the risk of developing a bilateral retinoblastoma.
Objective
To evaluate the efficacy of intra-arterial chemotherapy (IAC) for advanced retinoblastoma (RB) after failure of intravenous chemotherapy (IVC).
Methods
Fifteen eyes of 13 patients with advanced RB were treated with IAC (1 - 5 cycles) after failure of IVC (2 - 8 cycles). The patients included 10 boys and 3 girls, with the mean age of (15.67±8.16) months. Six patients had bilateral RB and 7 patients had unilateral RB. There were 14 eyes (93.33%) in stage D, 1 eye (6.67%) in stage E according to the International Classification of intraocular retinoblastoma. The main reasons for failure of IVC were recurrent primary tumor in 3 eyes (20.00%), subretinal seeds recurrence in 9 eyes (60.00%), viable vitreous seeds in 2 eyes (13.33%) and poor response of primary tumor in 1 eye (6.67%). The mean interval between IVC completion and IAC start was 3 months. The mean follow-up was 19 months (ranged from 3 to 52 months).
Results
After IVC and secondary IAC, the retinoblastoma and seeds were regressed in 12 eyes (80.00%). Three eyes required enucleation for severe vitreous seeds, subretinal seeds recurrence and primary tumor recurrence. There was no evidence of metastasis in any case.
Conclusion
IAC can achieve high global salvage rate (80.00%) for patients with advanced retinoblastoma after failure of IVC.
Key words:
Retinoblastoma/drug therapy; Chemotherapy, cancer, regional perfusion; Treatment outcome
'/%3e%3cuse xlink:href='%23a' transform='rotate(23.036 .093 25.536)'/%3e%3cuse xlink:href='%23a' transform='rotate(45.87 1.273 16.18)'/%3e%3cuse xlink:href='%23a' transform='rotate(69.945 .996 12.078)'/%3e%3cuse xlink:href='%23a' transform='rotate(20.66 -19.689 31.932)'/%3e%3c/svg%3e)
'%3e%3cpath fill='%23012169' d='M0 0v30h60V0z'/%3e%3cpath stroke='%23fff' stroke-width='6' d='m0 0 60 30m0-30L0 30'/%3e%3cpath stroke='%23C8102E' stroke-width='4' d='m0 0 60 30m0-30L0 30' clip-path='url(%23b)'/%3e%3cpath stroke='%23fff' stroke-width='10' d='M30 0v30M0 15h60'/%3e%3cpath stroke='%23C8102E' stroke-width='6' d='M30 0v30M0 15h60'/%3e%3c/g%3e%3c/svg%3e)
'/%3e%3c/g%3e%3cuse xlink:href='%23b' transform='matrix(1 0 0 -1 0 549)'/%3e%3c/svg%3e)
