Transcript becky - University of Nairobi

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
Preserving life.
Preserving
life.
•
•
TO
Globe salvage.
Vision
preservation
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.
Chemotherapy
(tumour reduction)
Systemic
chemotherapy16
Globe
preservation15
Focal consolidative
therapy (tumour
destruction)
•
Laser photocoagulation,
cryotherapy, radiotherapy,
local chemotherapy16
Tumour control and ocular salvage rates of more than 90% in group A and B eyes, 7090% 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
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
Records retrieval:
retinoblastoma ICD
C69.2
(1st Jan 2002 – 30th June
2014)
Unilateral
retinoblastoma.
Bilateral retinoblastoma.
1. One eye enucleated.
DATA COLLECTION
Bilateral enucleation
without globe
preservation.
ANALYSIS
Returned to records.
2. Globe preservation.
Returned to records.
Data management cont.
• Data collected using data collection forms:
HISTOLOGY OF
ENUCLEATED
EYE
HISTORY &
PRESENTATION
BIODATA
STUDY EYE
FINDINGS
TREATMENT
ANDOUTCOME
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: