Retinoblastoma: clinical picture and grouping at the time of first presentation | Siddiqui | Pakistan Journal of Medical Sciences Old Website

Retinoblastoma: clinical picture and grouping at the time of first presentation

Sorath Noorani Siddiqui, Jamshed Ahmed, Ziauddin Ahmed Shaikh


Objectives: To study the clinical picture and staging of retinoblastoma at the time of first presentation.
Methodology: This prospective study of consecutive case series was conducted in the department of ophthalmology, Dow University of Health Sciences and Civil Hospital, Karachi, from October 2007 to September 2009. Twenty patients with unilateral or bilateral retinoblastoma were examined during this period. Parents of every patient were interviewed in detail. Patients were examined under general anesthesia to measure corneal diameter, anterior chamber examination with hand held slit lamp. IOP was recorded with Perkins tonometer; fundoscopy with indentation was performed with indirect ophthalmoscope. International intraocular classification of retinoblastoma (IICR) was used for grouping of retinoblastoma. Ultrasonography and neuroimaging were performed in all cases.
Result: Twenty patients were found to have retinoblastoma with a mean age of 3.86 years (SD=2.56) with a minimum of one year and maximum of 12 years. Out of 20 patients there were 12 (60%) male and 08 (40%) female. Thirteen (65%) were from urban and 07 patients (35%) from rural areas. Family history of retinoblastoma was positive in two patients (10%). Bilateral retinoblastoma was present in 10 patients (50%) and unilateral retinoblastoma in other 10 patients (50%).The most common clinical presentation of retinoblastoma was Leukocoria that was seen in 12 patients (60%) followed by anterior segment involvement in three patients (15%), orbital Cellulitis in three patients (15%), strabismus in one patient (5%) and phthisis bulbi in one patient (5%). Among 10 patients with unilateral retinoblastoma 60% had group E, 20% had group D and another 20% with group C of IICR at the time of presentation. Twenty eyes of 10 patients with bilateral retinoblastoma showed group E in 13 eyes (65%), group D in four eyes (20%), group C in two eyes (10%) and group A in one eye (5%).
Conclusion: Leukocoria is the commonest clinical presentation of retinoblastoma. In developed countries 95% of children with retinoblastoma present with limited stage disease and are cured while in our set-up most patients still present with advanced diseases and cure rates are very low. Educational programs are required to educate general public, parents of retinoblastoma patients. Collaboration with pediatricians to check red reflex on every well baby visit is mandatory for early diagnosis.

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