A 19y old male with relapsed and refractory ALL was admitted under haematology with pyrexia of unknown origin. At the time of his admission, he reported sequential, bilateral visual impairment that had started 2 days previously. There was no accompanying redness, pain, photophobia, photopsia or floaters. His past medical history involved total body irradiation, chemotherapy and, 4 months previously BMT to treat the ALL. There was no previous ocular or family history of note. His medication included cyclosporine to prevent BMT rejection, and prophylactic posaconazole and acyclovir. At the time of eye clinic review his bloods were Hb- 8.2 g/dL, plt-18 × 10 9 /L and WCC- 0.1 × 109/L. PCR from peripheral blood for viral DNA tested negative for EBV, CMV and ADV.
On examination Snellen acuity was reduced to 6/36 on the right and 6/24 on the left. The anterior segments were normal. There was no anterior chamber (AC) activity or vitritis and the intraocular pressures were normal. Dilated fundus examination showed bilateral and symmetrical retinopathy with cotton wool spots and retinal haemorrhages across the posterior pole with relative sparing of the peripheral retina. Cystoid macular oedema in both eyes, with sub-retinal fluid in the right eye, was noted on optical coherence tomography (OCT) imaging. The central sub-field thickness (CST) was 557 μm and 603 μm in the right and left eyes. Imaging findings are shown below in the colour fundus pictures and OCT images (Fig. 1).
Management
Given the pyrexia of unknown origin and the above fundus findings an urgent AC tap was arranged to exclude viral retinitis. This was negative for CMV, HSV, VZV and EBV DNA. Given the persistent visual impairment but without an obvious infective aetiology, cyclosporine was stopped under haematology guidance, as this can be retinotoxic, and 40 mg of oral steroids commenced.
Vision subjectively started to improve over the subsequent 3–4 days despite no resolution of cystoid macular oedema. Two weeks after presentation, the CST was 815 μm in the right eye and 1016 μm in the left eye despite an improvement in VA. Oral steroids were slowly tapered down and stopped over a period of 4 weeks. The cyclosporine was not restarted. The vision continued to improve to 6/9 in the right and 6/6 in the left accompanied by the resolution of macular oedema over several months. This improvement did not coincide with haematological normalisation.
Sadly over the course of the subsequent few months, the patient’s systemic health deteriorated and he died as a result of pulmonary complications.