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

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
• extra-ocular
• proptosis
• orbital cellulitis
• extrascleral involvement
• enucleation