Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2016 Nov 2:25:131.
doi: 10.11604/pamj.2016.25.131.8599. eCollection 2016.

Extra-ocular retinoblastoma: about 12 cases followed at the Mohamed VI university hospital of Marrakech

Soltani Leila  1 , Hajji Ibtissam  1 , Essafi Hafsa  1 , Moutaouakil Abdeljalil  1
Affiliations

Extra-ocular retinoblastoma: about 12 cases followed at the Mohamed VI university hospital of Marrakech

Soltani Leila et al. Pan Afr Med J. .

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.

Keywords: Retinoblastoma; enucleation; extra-ocular; extrascleral involvement; orbital cellulitis; proptosis.

PubMed Disclaimer

Conflict of interest statement

The authors declare no competing interest.

Figures

Figure 1
Figure 1
Clinical appearance of orbital cellulitis of the right eye revealing an orbital retinoblastoma
Figure 2
Figure 2
Leucocoria as a mode of presentation of an Unilateral retinoblastoma of the right eye
Figure 3
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
Figure 4
Figure 4
CT Scan axial cuts of a trilateral retinoblastoma with a secondary location in the pineal gland
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. Mohammad J, Santosh G. Orbital retinoblastoma: Present status and future challenges. Saudi J Ophthalmol. 2011;25(2):159–67. - PMC - PubMed
    1. Elkettani A, Aderdour S, Daghouj G. Retinoblastoma: resultats preliminaries du protocol national de prise en charge au CHU de Casablanca. J Fr Ophtalmol. 2014;37(2):115–24. - PubMed
    1. Bouguila H, Malek I, Boujemâa C, Mouelhi M, Daghfous F, Nacef L. Le pronostic du retinoblastome : à propos de 50 cas. J Fr Ophtalmol. 2001;24(10):1053–6. - PubMed
    1. Doz F. Retinoblastoma: A review. Arch Pediatr. 2006;13(10):1329–37. - PubMed
    1. Abramson DH, Beaverson K, Sangani P. Screening for retinoblastoma: presenting signs as prognosticators of patient and ocular survival. Paediatrics. 2003;112(6):1248–55. - PubMed

LinkOut - more resources