OUTCOME OF GLOBE PRESERVATION THERAPY IN PATIENTS WITH BILATERAL RETINOBLASTOMA AT THE KENYATTA NATIONAL HOSPITAL, KENYA. DR REBECAH NAMWEYI NANDASABA 1 ST Supervisor : DR LUCY NJAMBI 2 ND Supervisor : DR KAHAKI KIMANI

INTRODUCTION Retinoblastoma is the most common primary intra-ocular malignancy of childhood. Accounts for 3 % of all childhood cancers. Occurs in 1: 17, 000 live births with a range of 1:14,000 to 1:20,000 live births. Kenya 1: 17,030 live births. No sex predilection. Occurs bilaterally in 30 – 40 % of cases. 1.American Academy of Ophthalmology, Opthalmic Pathology and Intraoccular Tumours, Nyamori JM, Kimani K, Njuguna MW, Dimaras H. "The incidence and distribution of retinoblastoma in Kenya. British Journal of ophthalmology. 2012;96(1):

Age at presentation INTERNATIONALLYAGE AT PRESENTATION > 90 % of cases< 3 years Positive family history4 months Unilateral disease24 months Bilateral disease12 months 1.American Academy of Ophthalmology, Opthalmic Pathology and Intraoccular Tumours, Nyawira G, Kahaki K, Karuiki-Wanyoike M, Survival among retinoblastoma patients at the Kenyatta National Hospital, Kenya. Journal of ophthalmology of Eastern Central and Southern Africa. Aug : 15-19

Age at presentation cont. KENYAAGE AT PRESENTATION Unilateral disease35.9 – months Bilateral disease24.34 – 26 months Familial disease32.8 months Non – familial33.1 months 3. Nyawira G, Kahaki K, Karuiki-Wanyoike M, Survival among retinoblastoma patients at the Kenyatta National Hospital, Kenya. Journal of ophthalmology of Eastern Central and Southern Africa. Aug : 15-19

Clinical presentation. Varies with different age groups. 1 Most common presentation: leucokoria 13 < 5 years> 5 years Leucokoria 60% leucokoria 35% Strabismus 20% decreased vision 35% Inflammation 5% strabismus 15 % Hypopyon, hyphema, anisocoria. Floaters 5 % Pain 5 % 1. American Academy of Ophthalmology, Ophthalmic Pathology and Intraoccular Tumours, Dongsheng H, Yi Zhang, Weiling Z, Yizhou W, Pinwei Z, Lian H, Yan Z, Tao H, Tian Z. Study on clinical therapeutic effects including symptoms, eye preservation rate, and follow up of 684 children with retinoblastoma. European Journal of Ophthalmology Mar (4) :

MANAGEMENT EVOLUTION Drop in enucleation rates from 36 %( ) to 7% ( ) to 2000 globe preservation : 62% of preserved eyes had vision >20/ Ramasubramanian A, Shields CL. Retinoblastoma. Jaypee brothers medical publishers New-Delhi. Preserving life. TO Preserving life. Globe salvage. Vision preservation

Globe preservation. Tumour control and ocular salvage rates of more than 90% in group A and B eyes, % in group C eyes and % in group D eyes Ramasubramanian A, Shields CL. Retinoblastoma. Jaypee brothers medical publishers New-Delhi. 16 Bhavna C, Amit J, Rajvardhan A. Conservative treatment modalities in retinoblastoma. Indian Journal of Ophthalmology Sept (9): National Cancer Institute. PDQ retinoblastoma Treatment. Bethesda, D: national Cancer Institute. Date last modified 12/6/2013. Available at topics/pdq/treatment/ retinoblastoma. Accessed 3/4/14 Globe preservation 15 Chemotherapy (tumour reduction) Focal consolidative therapy (tumour destruction) Systemic chemotherapy 16 Laser photocoagulation, cryotherapy, radiotherapy, local chemotherapy 16

STUDY JUSTIFICATION 1. No study has yet been done to determine the outcome of globe preservation therapy in our setting. 2. This study will shed light on modes and outcomes of the globe salvage therapy at KNH and give guidance on future management where gaps may be found

BROAD OBJECTIVE To determine the outcome of globe preservation therapy in patients with bilateral retinoblastoma at the KNH.

SPECIFIC OBJECTIVES 1.To describe the globe preservation therapy modalities in children with bilateral retinoblastoma treated at the KNH. 2.To determine the rate of globe preservation in patients with bilateral retinoblastoma who underwent globe preservation therapy. 3.To determine the rate of relapse and development of new tumours in patients with bilateral retinoblastoma undergoing globe preservation therapy at the KNH.

METHODOLOGY STUDY DESIGN The study will be a descriptive retrospective case series report. STUDY POPULATION All patients with bilateral retinoblastoma who have had one eye enucleated and the remaining eye undergone globe preservation therapy between 1 st January 2002 – 30 th April 2014.

STUDY AREA The study will be carried out at the Kenyatta National Hospital a national referral and teaching hospital (in association with The University of Nairobi) located in Nairobi county, Kenya. STUDY PERIOD The study period will be from 1 st September to 31 st December 2014 subject to ethics committee approval.

INCLUSION CRITERIA All patients with bilateral retinoblastoma with one eye enucleated who underwent globe preservation therapy for the remaining eye at the Kenyatta National Hospital between January 1 st 2002 – June 30 th 2014 will be included in the study. SAMPLE SIZE/ CASE DEFINITION All patients who meet inclusion criteria will be included in the study.

DATA MANAGEMENT Records retrieval: retinoblastoma ICD C69.2 (1st Jan 2002 – 30th June 2014) Bilateral retinoblastoma. 1. One eye enucleated. 2. Globe preservation. DATA COLLECTION ANALYSIS Bilateral enucleation without globe preservation. Returned to records. Unilateral retinoblastoma. Returned to records.

Data management cont. Data collected using data collection forms: HISTOLOGY OF ENUCLEATED EYE STUDY EYE FINDINGS TREATMENT ANDOUTCOME BIODATA HISTORY & PRESENTATION

Data management cont. Stored in Microsoft access and. Data will be analyzed using STATA version 13. Descriptive analysis will be done to determine the frequencies and the proportions of the various variables.

ETHICAL CONSIDERATION Approval will be sought from the KNH– UoN Ethics Committee before research is carried out. Information gathered will be accessed by the primary investigator, supervisors and statistician only. Data will be stored in a computer’s Microsoft access database. Thereafter data collection forms will be stored in a secure place for a period of time (5 years) before eventual destruction.

STUDY LIMITATIONS Missing patient records. Incomplete and missing information / data in patient records. Patients who were lost to follow up during the planned study period may bias the eventual results.

STUDY DEFINATIONS Primary failure: failure of primary treatment to control tumour. Unresponsive tumour or persistence of tumour despite treatment. Regression: complete resolving of the tumour upon treatment. Relapse /Recurrence - re-growth of intraretinal tumours, vitreous seeding or sub-retinal seeds after initial favorable response. New tumour: tumour developing in a previously disease free area.

DATA COLLECTION FORM: