OUTCOME OF GLOBE PRESERVATION THERAPY IN PATIENTS WITH BILATERAL RETINOBLASTOMA AT THE

KENYATTA NATIONAL HOSPITAL, KENYA.

DR REBECAH NAMWEYI NANDASABA 1ST Supervisor : DR LUCY NJAMBI

2ND 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, 2011-2012.2. Nyamori JM, Kimani K, Njuguna MW, Dimaras H. "The incidence and distribution of retinoblastoma in Kenya. British Journal of ophthalmology. 2012;96(1):141-142.

Age at presentation

INTERNATIONALLY AGE AT PRESENTATION

> 90 % of cases < 3 years

Positive family history 4 months

Unilateral disease 24 months

Bilateral disease 12 months

1.American Academy of Ophthalmology, Opthalmic Pathology and Intraoccular Tumours, 2011-2012. 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 2013 1: 15-19

Age at presentation cont.

KENYA AGE AT PRESENTATION

Unilateral disease 35.9 – 39.89 months

Bilateral disease 24.34 – 26 months

Familial disease 32.8 months

Non – familial 33.1 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 2013 1: 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, 2011-2012.13. 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 2013 23(4) : 532-538.

MANAGEMENT EVOLUTION

• Drop in enucleation rates from 36 %(1956-1976) to 7% (1990-2000) 15

• 1990 to 2000 globe preservation : 62% of preserved eyes had vision >20/40 15

15. Ramasubramanian A, Shields CL. Retinoblastoma. Jaypee brothers medical publishers. 2012. New-Delhi.

Globe preservation.

• Tumour control and ocular salvage rates of more than 90% in group A and B eyes, 70-90% in group C eyes and 40-50 % in group D eyes. 18

15 Ramasubramanian A, Shields CL. Retinoblastoma. Jaypee brothers medical publishers. 2012. New-Delhi.16 Bhavna C, Amit J, Rajvardhan A. Conservative treatment modalities in retinoblastoma. Indian Journal of Ophthalmology Sept. 2013 61

(9): 479-485. 18 National Cancer Institute. PDQ retinoblastoma Treatment. Bethesda, D: national Cancer Institute. Date last modified 12/6/2013.

Available at http://cancer topics/pdq/treatment/ retinoblastoma. Accessed 3/4/14

Globe preservation15

Chemotherapy(tumour reduction)

Focal consolidative therapy (tumour

destruction)

Systemic chemotherapy16

Laser photocoagulation, cryotherapy, radiotherapy,

local chemotherapy16

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 1st January 2002 – 30th 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 1st September to 31st

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 1st 2002 – June 30th 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

Data management cont.

• Data collected using data collection forms:

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: