MyMedR

Fulltext Retinoblastoma Registry report--Hospital Kuala Lumpur experience

Jamalia R, Sunder R, Alagaratnam J, Goh PP

Med J Malaysia, 2010 Jun;65 Suppl A:128-30.

Retinoblastoma is a childhood ocular cancer. The aim of this paper is to describe the clinical and epidemiological characteristics of patients with retinoblastoma in a major paediatric ophthalmology center in the country. Retrospective information was collected through the retinoblastoma registry. Late presentation with advanced staging is a major problem.

Fulltext Trilateral retinoblastoma

Limn YS, Juraida E, Alagaratnam J, Menon BS

Med J Malaysia, 2011 Jun;66(2):156-7.

PMID: 22106703 MyJurnal

Trilateral retinoblastoma is the association of hereditary bilateral or unilateral retinoblastoma with a pineal neuroblastic tumour. We describe two cases of trilateral retinoblastoma from a total of 141 cases of retinoblastoma seen over an 8.5 year period. Both had a fatal outcome, with survival times of only 4 and 8 months respectively.

Fulltext Leucocoria in a boy with Kawasaki disease: a diagnostic challenge

Che Mahiran CD, Alagaratnam J, Liza-Sharmini AT

Singapore Med J, 2009 Jul;50(7):e232-4.

PMID: 19644606

Retinoblastoma, the most common primary intraocular malignancy of childhood, usually presents in the first three years of life. Atypical presentation of retinoblastoma can masquerade as virtually any ocular or orbital pathology, which may lead to diagnostic dilemmas especially in the presence of other systemic diseases. We report a 20-month-old boy who was diagnosed with coronary aneurysm as a complication of Kawasaki disease, and presented with sudden left eye redness. His mother noticed the presence of white pupillary reflex three months earlier. Atypical acute ocular presentation secondary to Kawasaki disease was initially suspected, but the presence of multiple calcification and mild proptosis on imaging suggested characteristics of advanced retinoblastoma. Histopathological examination of the enucleated eye, which revealed a classical rosette pattern appearance, confirmed the diagnosis. Atypical presentations of retinoblastoma are usually associated with advanced disease. The presence of other systemic conditions further complicates the diagnosis. Early diagnosis is important to reduce the mortality and morbidity.

Fulltext Tay-Sach disease with "cherry-red spot"--first reported case in Malaysia

Chan LY, Balasubramaniam S, Sunder R, Jamalia R, Karunakar TV, Alagaratnam J

Med J Malaysia, 2011 Dec;66(5):497-8.

PMID: 22390110

We present a rare case of Tay-Sachs disease with retinal 'cherry-red spots' in a 19-month-old Malay child. Molecular genetic studies confirmed the diagnosis. The case highlights that 'cherry-red spot' is a useful clinical clue in Tay-Sachs disease and several other lysosomal storage disorders. It serves as an ideal illustration of the eye as a window to inborn error of metabolism.

Fulltext Von Hippel angioma

Chong PK, Loo AV, Alagaratnam JV

Med J Malaysia, 2007 Oct;62(4):343-4.

PMID: 18551944 MyJurnal

We report a three year follow up of a 35-year-old Indian gentleman who presented with sudden, painless blurring of left (L) eye vision with initial visual acuity (VA) of 6/60. Fundoscopy revealed (L) vitreous haemorrhage and subsequently confirmed a (L) inferotemporal capillary haemangioma. The adjacent area of capillary haemangioma was treated with barricade argon laser photocoagulation to prevent progression of exudative retinal detachment inferiorly. Subsequent follow up showed mild regression of capillary haemangioma with maintenance of (L) eye vision at 6/9.

Fulltext Presentation of retinoblastoma patients in Malaysia

Subramaniam S, Rahmat J, Rahman NA, Ramasamy S, Bhoo-Pathy N, Pin GP, et al.

Asian Pac J Cancer Prev, 2014;15(18):7863-7.

PMID: 25292078

BACKGROUND: Retinoblastoma is a rare type of cancer that usually develops in early childhood. If left untreated it can cause blindness and even death. The aim of this study is to determine sociodemographic and clinical features of retinoblastoma patients and also to determine the treatment pattern and outcome in Malaysia.

MATERIALS AND METHODS: Data for this study were retrieved from the Retinoblastoma Registry of the National Eye Database (NED) in Malaysia. Hospital Kuala Lumpur, Hospital Umum Kuching, Sarawak and Hospital Queen Elizabeth, Kota Kinabalu were the major source data providers for this study. Data collected in the registry cover demography, clinical presentation, modes of treatment, outcomes and complications.

RESULTS: The study group consisted of 119 patients (162 eyes) diagnosed with retinoblastoma between 2004 and 2012. There were 68 male (57.1%) and 51 (42.9%) female. The median age at presentation was 22 months. A majority of patients were Malays (54.6%), followed by Chinese (18, 5%), Indians (8.4%), and indigenous races (15.9%). Seventy six (63.8%) patients had unilateral involvement whereas 43 patients had bilateral disease (36.1%). It was found that most children presented with leukocoria (110 patients), followed by strabismus (19), and protopsis (12). Among the 76 with unilateral involvement (76 eyes), enucleation was performed for a majority (79%). More than half of these patients had extraocular extension. Of the 40 who received chemotherapy, 95% were given drugs systemically. Furthermore, in 43 patients with bilateral involvement (86 eyes), 35 (41%) eyes were enucleated and 17 (49%) showed extraocular extension. Seventy-two percent of these patients received systemic chemotherapy. The patients were followed up 1 year after diagnosis, whereby 66 were found to be alive and 4 dead. Sixteen patients defaulted treatment and were lost to follow-up, whereas follow-up data were not available in 33 patients.

CONCLUSIONS: Patients with retinoblastoma in this middle-income Asian setting are presenting at late stages. As a result, a high proportion of patients warrant aggressive management such as enucleation. We also showed that a high number of patients default follow-up. Therefore, reduction in refusal or delay to initial treatment, and follow-up should be emphasized in order to improve the survival rates of retinoblastoma in this part of the world.

Late presentation of retinoblastoma in Malaysia

Menon BS, Alagaratnam J, Juraida E, Mohamed M, Ibrahim H, Naing NN

Pediatr Blood Cancer, 2009 Feb;52(2):215-7.

PMID: 18855905 DOI: 10.1002/pbc.21791

The aims of this study were to review the presenting features, treatment and outcome for Malaysian children with retinoblastoma currently.

Visual impairment and amblyopia in Malaysian pre-school children - The SEGPAEDS study

Chew FLM, Thavaratnam LK, Shukor INC, Ramasamy S, Rahmat J, Reidpath DD, et al.

Med J Malaysia, 2018 02;73(1):25-30.

PMID: 29531199 MyJurnal

INTRODUCTION: Little is known regarding the extent of visual impairment amongst pre-school children in Malaysia.

OBJECTIVE: To determine the prevalence of visual impairment and amblyopia in Malaysian preschool children.

METHODOLOGY: A cross-sectional, population-based study was conducted on children aged four to six years from 51 participating kindergartens in the district of Segamat, Johor, Malaysia from 20 March 2016 to 6 April 2016. All subjects had initial eye screening consisting of LogMar visual acuity, orthoptics examination and Spot vision screener assessment. Subjects who failed the initial eye screening were invited for a formal eye assessment consisting of cycloplegic refraction and a comprehensive ocular examination. Definitions of visual impairment and amblyopia were based on the Multi-Ethnic Pediatric Eye Disease Study criteria.

RESULTS: A total of 1287 children were recruited. Mean subject age was 5.03 (SD:0.77) and males represented 52.3% of subjects. Subjects by ethnicity were Malay (54.8%), Chinese (27.7%), Indian (15.6%) and Orang Asli (1.9%). Formal eye assessment was required for 221 subjects and 88.8% required ophthalmic intervention. Refractive error, representing 95.4% of diagnosed ocular disorders, comprised of astigmatism (84%), myopia (9%) and hypermetropia (6.9%). With-the-rule astigmatism was present in 93.4% of the subjects with astigmatism. Visual impairment was present in 12.5% of our subjects, with 61% having bilateral visual impairment. Of the subjects with visual impairment, 59.1% had moderate visual impairment. The prevalence of amblyopia was 7.53%, and 66% of the amblyopic subjects had bilateral amblyopia.

CONCLUSION: Our study highlights an urgent need for initiation of preschool vision screening in Malaysia.

Fulltext Treatment of diffuse choroidal haemangioma using photodynamic therapy

Poh KW, Wai YZ, Rahmat J, Shunmugam M, Alagaratnam J, Ramasamy S

Int J Ophthalmol, 2017;10(3):488-490.

PMID: 28393045 DOI: 10.18240/ijo.2017.03.26

Chemoreduction for intraocular retinoblastoma in Malaysia

Menon BS, Juraida E, Alagaratnam J, Mohammad M, Ibrahim H, George TM, et al.

J Pediatr Hematol Oncol, 2007 Jan;29(1):2-4.

PMID: 17230058

In the last decade, chemotherapy in combination with focal therapy (chemoreduction) has been increasingly used in intraocular retinoblastoma to avoid enucleation and radiotherapy. The aim of this study was to assess the feasibility and outcome of chemoreduction in Malaysian children with retinoblastoma. This was a prospective study from August 2001 to January 2006. Twenty children (25 eyes) were given 4 cycles of chemoreduction, after which the response was assessed. Fourteen eyes showed a complete response, 10 eyes showed a partial response, and 1 eye had progressive disease. Twelve eyes developed progressive disease later, 9 after an initial complete response and 3 after a partial response. Overall, progressive disease occurred in 52%. There were 2 treatment failures, in Reese-Elsworth groups 3 and 4. Both eyes required enucleation. One eye in group 5 required second line chemotherapy to achieve a complete response. No eyes were irradiated. Five children (25%) defaulted follow-up, one of whom returned with disseminated disease. In conclusion, 4 cycles of chemoreduction achieved a durable complete response in only 12% of eyes. Chemoreduction is feasible in Malaysia but requires good patient compliance and close follow-up.

Spectrum of germ-line RB1 gene mutations in Malaysian patients with retinoblastoma

Mohd Khalid MK, Yakob Y, Md Yasin R, Wee Teik K, Siew CG, Rahmat J, et al.

Mol Vis, 2015;21:1185-90.

PMID: 26539030

The availability of molecular genetic testing for retinoblastoma (RB) in Malaysia has enabled patients with a heritable predisposition to the disease to be identified, which thus improves the clinical management of these patients and their families. In this paper, we presented our strategy for performing molecular genetic testing of the RB1 gene and the findings from our first 2 years of starting this service.

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