A 53- year old gentleman presented to the emergency eye service with a 1 week history of right visual disturbance with constant multi-coloured photopsia and reduced visual acuity. There was no significant past ocular or medical history. He was involved in a road traffic accident (RTA) 3 weeks earlier when his car was hit from the back but he sustained no injuries at that time. He was otherwise systemically well.
The best-corrected visual acuity (BCVA) was 6/12 in the right and 6/6 in the left eye. There were no anterior chamber or vitreous cells and no vitreous haze. In the right eye a large white deep retinal lesion, with an opaque appearance, was identified in the temporal retina, extending into the macular area and transecting the fovea (Fig. 1a). The lesion had a unique shape and margins: the nasal aspects of the lesion had a geometric like shape, with defined linear straight and curved edges, and a pronounced bright white border; the remaining border was less defined and homogenous with the lesion. Spectral domain optical coherence tomography (SD-OCT, Topcon Medical Systems Inc., Newbury, UK) within the lesion showed hyper-reflective sub-retinal change (Fig. 1a). Beyond the observed lesion edge, the ellipsoid zone (EZ) was disrupted in the nasal macular (Fig. 1a) Small, well-defined creamy white sub-retinal lesions were also evident in the superior macular area of the right eye (Fig. 1a). The left eye was unremarkable. (Fig. 1d).
The history of RTA led to consideration of commotio retinae, secondary to presumed whiplash injury from the car accident. Investigations for inflammatory and infective pathologies were negative including normal inflammatory markers, autoimmune screen, serum angiotensin enzyme, chest x-ray and syphilis testing.
Six weeks later, the patient reported full resolution of symptoms, including photopsia but the right BCVA remained the same. The area of retinal whitening and corresponding hyper-reflective sub-retinal OCT changes had resolved. Partial restoration of the EZ in the nasal macular was noted compared to earlier OCT. Multiple small macular sub-retinal pigment epithelium (RPE) deposits had appeared, with corresponding hyper-autofluorescence on fundus autofluorescence (FAF). The left eye remained normal.
The patient was lost to follow-up and re-presented 4 months later with recent onset blurred vision. The BCVA had deteriorated to 6/15 right and 6/19 left. There was no cellular activity in the vitreous but the posterior vitreous face appeared condensed and thickened. New retinal lesions were evident in the maculae of both eyes: in the right, a long, vertical lesion with ill-defined edges and different orientation, shape and margin appearance to the original lesion, was present; (Fig. 1b) in the left, a similar, ill-defined white retinal lesion inferior to the fovea was noted (Figure 1e). OCT within these lesions demonstrated hyper-reflective sub-retinal change, similar to the original lesion (Fig. 1b and e). More numerous bilateral sub-retinal and sub-RPE deposits were seen. (Fig. 1b, e).
One week later, significant evolution of the right lesion morphology was observed: the temporal margin appeared more defined with re-emergence of a bright linear edge; the lesion had increased in size; new sub-retinal deposits were identified (Fig. 1c) and the lesion appeared to have migrated supero-nasally across the peri-papillary area, maintaining the outline shape temporally of the earlier lesion. Within the previously involved area, the sub-retinal lesion clinically appeared to have resolved and OCT demonstrated resolution of the hyper-reflective change with the RPE intact. (Fig. 1c, 2a-c ) In the left, the lesion inferior to the fovea had evolved and was replaced by multiple small defined sub-retinal and sub-RPE deposits. (Figs. 1f, 2g-i) Fundus fluorescein angiogram (FFA) and indocyanine green angiography (ICG) showed multiple hypofluorescent spots corresponding to the sub-retinal/ sub-RPE deposits. (Fig. 2d-e, j-k) These lesions were more numerous than those clinically detectable. On FAF, there were hypo- and hyperautofluorescent spots corresponding to sub-retinal and sub-RPE deposits respectively. (Fig. 2f, l) The patient was referred to the uveitis service for opinion.
Prior to review, the patient developed acute confusion and impaired neurological function. Magnetic resonance imaging (MRI) of the brain, lumbar puncture and urgent brain biopsy confirmed the diagnosis of primary CNS diffuse large B-cell lymphoma.
He underwent MATRix (methotrexate, cytarabine, thiotepa, rituximab) chemotherapy and brain and orbital radiotherapy. During treatment the patient noticed a significant and improvement in his visual symptoms. His last recorded BCVA, at 12 months follow up, was 6/7.5 right and 6/6 left. Clinical examination and repeat imaging demonstrated resolution of the large retinal lesions and reduced number of sub-RPE lesions in the both eyes with complete restoration of the outer retinal architecture on OCT (Fig. 3a to d).