Retinoblastoma in Asia: Clinical Presentation and Treatment Outcomes in 2112 Patients from 33 Countries.

PURPOSE: To describe the clinical presentation and treatment outcomes of children who received a diagnosis of retinoblastoma in 2017 throughout Asia. DESIGN: Multinational, prospective study including treatment-naïve patients in Asia who received a diagnosis of retinoblastoma in 2017 and were followed up thereafter. PARTICIPANTS: A total of 2112 patients (2797 eyes) from 96 retinoblastoma treatment centers in 33 Asian countries. INTERVENTIONS: Chemotherapy, radiotherapy, enucleation, and orbital exenteration. MAIN OUTCOME MEASURES: Enucleation and death. RESULTS: Within the cohort, 1021 patients (48%) were from South Asia (SA), 503 patients (24%) were from East Asia (EA), 310 patients (15%) were from Southeast Asia (SEA), 218 patients (10%) were from West Asia (WA), and 60 patients (3%) were from Central Asia (CA). Mean age at presentation was 27 months (median, 23 months; range, < 1-261 months). The cohort included 1195 male patients (57%) and 917 female patients (43%). The most common presenting symptoms were leukocoria (72%) and strabismus (13%). Using the American Joint Committee on Cancer Staging Manual, Eighth Edition, classification, tumors were staged as cT1 (n = 441 [16%]), cT2 (n = 951 [34%]), cT3 (n = 1136 [41%]), cT4 (n = 267 [10%]), N1 (n = 48 [2%]), and M1 (n = 129 [6%]) at presentation. Retinoblastoma was treated with intravenous chemotherapy in 1450 eyes (52%) and 857 eyes (31%) underwent primary enucleation. Three-year Kaplan-Meier estimates for enucleation and death were 33% and 13% for CA, 18% and 4% for EA, 27% and 15% for SA, 32% and 22% for SEA, and 20% and 11% for WA (P < 0.0001 and P < 0.0001), respectively. CONCLUSIONS: At the conclusion of this study, significant heterogeneity was found in treatment outcomes of retinoblastoma among the regions of Asia. East Asia displayed better outcomes with higher rates of globe and life salvage, whereas Southeast Asia showed poorer outcomes compared with the rest of Asia. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Outcome of Injection Botulinum Toxin in Blepharospasm.

INTRODUCTION: Blepharospasm is a condition of involuntary spasm of the orbicularis oculi muscle which leads to intermittent or complete closure of the eyelids. Botulinum toxin is the currently recommended first line treatment for such blepharospasm. This study aims to find out the outcome of injection Botulinum toxin Type A in Blepharospasm. MATERIALS AND METHODS: It was a hospital based, prospective, interventional study conducted on patients diagnosed as Benign essential blepharospasm (BEB), Meige syndrome (MS) and Hemifacial spasm (HFS) by oculoplastic surgeon at Oculoplasty department OPD, Tilganga Institute of Ophthalmology, from December 2018 to November 2019. After taking all standard precautions for botulinum toxin injections, 6 to 8 sites for injecting 2.5 to 5 IU of the toxin were given. All the patients were evaluated before and after injections according to Jankovic spasm grading and improvement in functional impairment scale and followed on one week, one month, three month and when the symptoms reappeared. RESULTS: A total of 43 cases which included 32 cases of Benign essential Blepharospasm, 9 Hemifacial spasm and 2 Meige syndrome. The mean Jankovic severity score was 3.51 ± 0.51 (range 3-4). The mean improvement in functional score was 2.60 ± 0.54 (range 1-3), was statistically significant (p-value <0.001).The effective period of injection was 130 ± 20.82 (93 - 189) days.38 patients had repeated injections after reappearance of symptoms. 4 patients had side effects of redness and hematoma at one site. CONCLUSION: This study concludes that Botulinum toxin type A is effective in the management of Benign essential blepharospasm, Hemifacial spasm and Meige syndrome. This along with a good safety profile justifies its role as a first line treatment therapy in blepharospasm. However, it is a temporary treatment option where the effect lasts for a short period of time and repeated injections are required.

Ocular involvement in metastatic and systemic malignancies is not rare.

BACKGROUND: Metastatic disease to the eye most commonly involves choroid followed by orbit leading to varied ocular manifestations. By comparison, it is relatively rarer than primary malignancies of eye as well as metastasis in other parts of the body. AIM: The aim of this study is to evaluate the common eye and orbital structures involved in secondary ocular and metastatic disease, to describe its clinical manifestations and outline the management done. METHODS: A retrospective study of newly diagnosed cases of ocular metastasis in last 2 years conducted in our recently established department of ocular oncology at a tertiary eye care hospital in Nepal. Demography, age and sex distribution were noted. The patients were segregated into those with secondary or metastatic ocular malignancies. Detail study on the metastatic disease to eye was made in regards to presenting symptoms, signs, primary site of cancer, and the treatment done. Details of the investigations done, like biopsy and imaging were also recorded. RESULTS: There were a total of 28 patients, whose age group ranged from 9 years to 69 years with median age of 43 years. Females constituted 46% of total patients. Both the eyes were involved in 9 patients (32%). Eye was secondarily involved by paranasal sinus tumors and Non Hodgkin lymphoma (7 patients each). Ocular metastasis was commonly seen from broncogenic carcinoma in four and breast carcinoma in three patients. Simultaneous metastasis to other parts of the body was also seen in 61% of our patients. Diminution of vision in 49% was the most common presenting feature followed by proptosis in 16% and palpable mass in 14% of patients. Orbit in 43% cases is the commonest ocular structure involved. Histopathologic diagnosis was done in 32% only while rest was based on imaging alone. The most common treatment done was chemotherapy in 57% patients. CONCLUSION: Ocular metastasis can display a wide variety of clinical and imaging features and therefore a high degree of suspicion is required. It is often associated with simultaneous metastasis to other parts of the body as well, hence the importance of earlier diagnosis and metastatic workup.

Clinicopathologic Presentation and Management of Malignant Orbito-ocular Tumors at a Tertiary Care Hospital in Nepal.

INTRODUCTION: This study was conducted to evaluate the clinical presentations and management of various Orbito-ocular malignancies in Nepal. MATERIALS AND METHODS: This is a prospective study of ocular malignant cases conducted at a tertiary care eye hospital in Kathmandu, Nepal. One hundred and seventeen newly diagnosed eyes with ocular malignancy of 106 patients presenting to the clinic in a year from September 2018 to August 2019 were included for analysis. Patients' demography, clinical features, various types of malignancies seen in eyes and their management were evaluated. RESULTS: The mean age of patients was 30.35 years with a range from 1 month to 84 years and standard deviation of 26.63. 56% of the total were male. Red eyes and visible mass were the most common symptoms in 23% with intraocular contents in 48% being the most common site involved. Different types of ocular malignancies were detected, among which retinoblastoma was the most common in 38% of patients. Diagnosis in most patients was clinical and/or radiological; incision biopsy was required in 1.7% patients only. Surgical excision of the tumor was the most common treatment done in 29% followed by enucleation in 27% of eyes. Bilateral involvement of the eyes was seen in 10% of the patients. CONCLUSION: Malignant orbito-ocular tumors can be seen in any age group. Retinoblastoma is the commonest malignancy seen. With the help of imaging, diagnosis is near accurate; hence biopsy is not required in most cases before commencing treatment.

Choroidal Melanoma: Our Experience.

BACKGROUND: Choroidal melanomas are diagnosed in approximately 6 out of one million Americans per year, and although their incidence is low, they are the most common primary intraocular tumor in adults. METHODS: Choroidal melanoma is rare tumors and till date no such reports with sclera involvement has been reported from Nepal. It is a prospective case series. The aim of this study is for awareness of the severity of the Choroidal melanoma and it's management. Besides, it is also to study the demography, presentation, histopathological variations and management of cases of choroidal melanoma. All the consecutive cases of Choroidal melanoma presenting between Jan 2017 to May 2018 and those who were within the inclusion criteria were included in this study. RESULTS: There were eight patients, five male and three female, within age range of 18-73, median age was 47. All patients presented with decreased vision ranging from 6/24 to PL of less then three months to 2 years duration. Fund us showed choroidal mass associated with Vitreous hemorrhage (VH), and retinal detachment (RD). Two patients were managed with enucleation with External beam radiotherapy (EBRT). Four underwent only enucleation. One patient with lesion size less then 10mm under went plaque brachy therapy. One patient underwent initially Plaque brachy therapy but later had to undergo Enucleation. Histopathological examination (HPE) of enucleated patient revealed epitheloid cell melanoma grade three in four and Spindle cell melanoma in three patients. Each one patient of epitheloid cell melanoma and spindle cell melanomahad scleral involvement. Indication for radiotherapy was scleral involvement. CONCLUSION: With 8 cases of Choroidal melanoma in a single year in a single hospitalgives us a clue that there may be much more undiagnosed cases of Choroidal melanomain Nepal that should be taken seriously. Going for annual eye examination with routine dilated fund us exams can help in prevention and early diagnosis of this life and sight threatening condition and to reduce the mortality rate.