Retinoblastoma and vision

Abstract

The assessment of vision has a growing importance in the management of retinoblastoma in the era of globe-conserving therapy, both prior to and after treatment. As survival rates approach 98-99% and globe salvage rates reach ever-higher levels, it is important to provide families with information regarding the visual outcomes of different treatments. We present an overview of the role of vision in determining the treatment given and the impact of complications of treatment. We also discuss screening and treatment strategies that can be used to maximise vision.

Fig. 1. The role of systemic chemotherapy as primary treatment.

a Retinoblastoma group C in the left eye at presentation. b Good response following systemic chemotherapy with calcified-treated tumour (Type 1 regression).

Fig. 2. The role of intra-arterial chemotherapy as primary treatment.

a Nasal retinoblastoma tumour in a group D eye at presentation with obscuration of the optic nerve. b Tumour treated with primary intra-arterial chemotherapy showing shrinkage of the tumour (Type III regression) with sparing of macula and good visual outcome.

Fig. 3. The impact of choroidal ischaemia from intra-arterial chemotherapy on vision.

a Healthy fovea before IAC for several relapsing peripheral lesions uncontrolled by focal therapy. b Choroidal ischaemia after IAC with visual loss.

Fig. 4. Laser treatment on a small tumour.

a Small group A tumour at presentation in the left eye. b Group A tumour in the left eye treated with laser with good response.

Fig. 5. Identification of the preferred retinal locus (PRL) superior to macular tumour scar.

Goldmann III stimuli were projected for 200 ms in a 4-2 threshold strategy. The PRL is identified as the centre of the fixation ellipse [94]. (Permission from JAAPOS).