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2016 ; 25
(ä): 131
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Extra-ocular retinoblastoma: about 12 cases followed at the Mohamed VI university
hospital of Marrakech
#MMPMID28292093
Leila S
; Ibtissam H
; Hafsa E
; Abdeljalil M
Pan Afr Med J
2016[]; 25
(ä): 131
PMID28292093
show ga
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.