Abstract
Objectives: To assess the clinical picture and grouping of retinoblastoma at first presentation to the oncology ward at Muhimbili National Hospital. Methods: A cross sectional descriptive hospital based study was conducted from April to December 2018. Seventy two patients who presented for the first time to Muhimbili National Hospital (MNH) with a diagnosis of retinoblastoma were consecutively sampled and recruited in the study. Visual acuity, horizontal corneal diameter, intraocular pressure, anterior and posterior segments of the eye were assessed and each eye was classified according to the International Classification of Retinoblastoma (ICRB) group. Ultrasonography, neuroimaging and histology were performed. Results: A total of 72 patients comprising of 90 affected eyes were recruited and analyzed: 39(54.2%) males and 33(45.8%) females. Majority of patients were residents of the Coastal and Lake Zones. Family history of Retinoblastoma was positive in only one patient. Fifty four (75.0%) patients had unilateral disease. Leukocoria (77.8%) and proptosis (41.7%) were the commonest first clinical signs noted by the family and health workers at MNH respectively. At MNH, both bilateral and unilateral cases presented with advanced disease of group E and extra ocular extension where 84(93.3%) eyes were indicated for enucleation. The median lag time from disease onset to presentation at MNH was 4 months. Conclusion: Leukocoria was the commonest first sign of retinoblastoma presentation at home. However, despite early presentation to primary health facilities, most patients presented to the tertiary centre of MNH late with advanced disease. Efforts to raise awareness on retinoblastoma to both health workers and the community are important for early case detection, referral, diagnosis and treatment in order to improve visual outcomes and survival rates of retinoblastoma patients.
Highlights
Retinoblastoma is the most common and important life-threatening intraocular malignancy in children which originates in immature retinal cells in one (60%) or both eyes (40%) [1]
Inclusion criteria All patients who presented to Muhimbili National Hospital (MNH) for the first visit and who were confirmed to have retinoblastoma by a pediatric ophthalmologist were consecutively recruited into the study
A total of 312 children were diagnosed with different cancers in the oncology ward during the study period
Summary
Retinoblastoma is the most common and important life-threatening intraocular malignancy in children which originates in immature retinal cells in one (60%) or both eyes (40%) [1]. It results from genetic mutations in one or more cells of the retina due to mutational inactivation of the retinoblastoma (RB1) gene. An inactive RB1 gene leads to tumor formation by allowing uncontrolled retinal cell division. The RB1 gene may be inherited leading to familial disease in 6% or may commonly develop at random leading to sporadic disease in 94% [2]. The incidence of retinoblastoma is similar in both males and females [3] and is about 1/15000 live births.
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