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Case series
Extra-ocular retinoblastoma: about 12 cases followed at the Mohamed
VI university hospital of Marrakech
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Ophthalmology Department, Mohamed VI University Hospital, Marrakech, Morroco
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Corresponding author: Soltani Leila, Ophthalmology Department, Mohamed VI University Hospital, Marrakech, Morroco
Key words: Retinoblastoma, extra-ocular, proptosis, orbital cellulitis, extrascleral involvement, enucleation
Abstract
Retinoblastoma is the most frequent childhood intraocular tumor. The aim of our study is to evaluate the clinical features and management of
extra-ocular retinoblastoma in the Mohamed VI university hospital of Marrakech. Retrospective case series, the patient's records were reviewed for
patient and tumor features, ocular management, histopathological findings, and patient survival. Over a period of three years, 35 eyes were
diagnosed with retinoblastoma; 12 children (16 eyes) (46%) had extra-ocular retinoblastoma. Mean age was 27 months, 60% were males. Six
cases had unilateral tumor, five bilateral and one case of trilateral retinoblastoma. There was no positive family history, proptosis was the mean
mode of presentation (41,6%) followed by staphyloma (25%) orbital cellulitis (25%) and hyphema(8,3%). The median lag period was 18 months.
On imaging and histopathological analysis, there was extrascleral involvement in 41.6%, involvement of orbital part of optic nerve (75%), of orbital
muscles (50%) and eyelids in 16.6%. the surgical treatment included according to the degree of extension enucleation (75%) or exenteration
(25%) associated to chemotherapy in all cases and one case of external beam radiation. There were 2 cases of orbital recurrence, one death and
no metastases at 30 months follow-up.Orbital retinoblastoma still stands as a tall challenge requiring multi-modal and multi-disciplinary approach.
Although the survival has increased over the last few years, lack of access to medical facilities, lack of education about the need for early medical
attention and cultural resistance to enucleation continue to contribute to an epidemic of extra ocular disease at diagnosis in the developing world.
© Soltani Leila et al. The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution
License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is
properly cited.
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Table 1: Lag period between first symptom and diagnosis of extra-ocular retinoblastoma (months)
parameters Lag period Range P-value
Lag period Not significant
Lag period according to laterality 10+/-2 2-18
Unilateral 9 2-16
bilateral 12 6-18
Lag period according to age Not significant
0-2 years 10.5 3-18
2-4 years 8 2-14
More than 4 years 6.5 3-10
Table 3: Comparative frequency of common presenting signs of retinoblastoma in different parts of world
Author Year Country No.of Leucocoria Strabismus Proptosis
patients (%) (%) (%)
Badhu et al [9] 2005 Nepal 43 32.5 - 44.2
Sumitha et al [13] 2005 India 355 74.6 6.2 25.3
Sow et al [15] 2014 Senegal 53 22.6 5.6 52.8
Abramson et al [16] 1998 USA 1265 56.1 23.6 0.5
Present study 2015 Morocco 12 40 20 41.6
Marrakech
Figure 1: clinical appearance of orbital cellulitis of the right eye revealing an orbital
retinoblastoma
Figure 3: CT scan axial cuts of a bilateral orbital mass, optic nerve extension and
orbital cellulitis of the right eye revealing a bilateral retinoblastoma with extra-ocular
involvement