Vrundaben Patel1 and David Kasongole1
1University Teaching Hospitals – Eye Hospital, Lusaka, Zambia
Abstract
A 27 year old female presented with poor vision in both eyes for two months with gradual progression in one eye and sudden loss of vision in the other eye. She had been commenced on Anti-Retroviral therapy for Human Immunodeficiency Virus two weeks prior to presentation. Presenting visual acuity was hand movement in both eyes. Ocular examination revealed mild anterior uveitis with widespread areas of retinal mid peripheral and posterior pole vascular sheathing, retinal necrosis with haemorrhages and macula oedema in both eyes. A diagnosis of Cytomegalovirus retinitis was made in both eyes and commenced on bi-weekly intravitreal ganciclovir injections followed by weekly maintenance injections. Visual acuity improved only in the right eye to 6/12. She developed neovascularisation of the disc in both eyes and a tractional retinal detachment in the left eye.
INTRODUCTION
Cytomegalovirus (CMV) retinitis is a ubiquitous DNA virus that infects the majority of the adult population [1]. It is the commonest intraocular infection associated with Human Immunodeficiency Virus (HIV) affecting an average of 25% of patients [2], particularly those with CD4 cell count less than 50/microL. It also occurs as an opportunistic infection in other immunocompromised states such as post organ transplant patients and those on chemotherapy [3]. With the widespread use of anti-retroviral therapy, the incidence of CMV retinitis has reduced [4]. The case presented highlights some of the challenges faced with treating patients with CMV retinitis in our local setting.
CASE SUMMARY
A female aged 27 years presented with complaint of poor vision in both eyes. The problem started with gradual loss of vision in her left eye over the preceding two months and then she had sudden loss of vision in the right eye two weeks prior to presentation. It was not associated with pain. She had been using lubricating and dexamethasone eye drops prescribed at another eye centre. She had no previous history of ocular disorder. Medical history was significant for HIV infection for which she had been on antiretroviral treatment (ART) for two weeks. Baseline CD4 count results were not available.
On examination, her visual acuity was hand movement (HM) in both eyes. IOP was 10mmHg in both eyes. Anterior segment examination was significant for mild anterior chamber reaction with 2+ cells. Vitreous was clear in both eyes. Retinal exam showed widespread areas of vascular sheathing, retinal necrosis with haemorrhages involving the posterior pole and mid periphery with disc and macula oedema in both eyes.
A diagnosis of fulminant CMV retinitis in both eyes was made. The treatment plan included bi-weekly intravitreal injection of 2.5mg ganciclovir/0.1mls in both eyes that she received for 3 weeks. She had weekly fundus photos done and CD4 count was at 98 cells at the end of 3 weeks with a viral load of 53,262copies/ml. Due to the unavailability of oral valganciclovir, the patient was put on weekly intravitreal ganciclovir injection as maintenance treatment. After a total of 12 injections in each eye, visual acuity improved to 6/18 in the right eye and remained HM in the left eye. She developed neovascularisation of the disc (NVD) in both eyes and a dispersed vitreous haemorrhage with tractional retinal detachment (TRD) in the left eye. She received intravitreal bevacizumab 1.25mg/0.05mls in both eyes. She also had panretinal photocoagulation (PRP) to the extent possible using laser indirect ophthalmoscope in the left eye.
After a total of 20 intravitreal ganciclovir injections, 1 dose of intravitreal bevacizumab in both eyes and PRP in the left eye, visual acuity was 6/12 in the right eye and HM in the left eye. She had regressed NVD in both eyes. The right eye also had some disc pallor and sclerosed vessels while the left eye had TRD threatening macula. Viral load had dropped to 21,138 copies/mL. Maintenance therapy was stopped, ART continued as she awaits left eye pars plana vitrectomy with endolaser and silicon oil implant.
Figure 1: Colour fundus photos at diagnosis
Figure 2: Colour fundus photos at end of intensive phase
Figure 3: Colour fundus photos during maintenance phase
DISCUSSION
Patients with CMV retinitis present with painless, progressive loss of vision such as the patient in this case presented with. Sometimes floaters and visual field defects may also be noted [5]. On retinal exam, 3 main forms of CMV retinitis have been identified: fulminant (as in the case presented), perivascular and granular [6].
A major component of the treatment of patients with CMV retinitis involves counselling in terms of compliance to long term treatment and in advanced cases on the guarded visual prognosis. A multidisciplinary treatment team involving the ophthalmologist, low vision specialist, infectious disease specialist and counsellors is vital to complete care. Frequently, CMV retinitis is a clinical diagnosis though supportive tests include presence of anti-CMV antibodies or CMV antigens in body fluids including ocular fluids [7]. Serial fundus photography is useful in monitoring response to treatment and progression of the disease.
Medical treatment for CMV retinitis includes local (intravitreal) and/or systemic antiviral therapy. Drugs such as ganciclovir, valganciclovir, foscarnet, and cidofovir may be used. Disadvantage of local therapy include that there is no protection of the other eye in case of initial unilateral retinitis and that it does not treat concurrent systemic disease either [7]. For bilateral disease systemic therapy is more useful. Treatment is given for a three week long induction phase followed by a period of maintenance determined by the patient response and systemic immune recovery. ART also is key in the treatment of HIV patients with CMV retinitis and also prevent recurrence. Generally, CD4 count levels above 200 cells/microL may be used to stop maintenance anti-CMV therapy for those on ART [8].
Complications that may arise due to CMV retinitis include vitreous haemorrhage, retinal detachment, macula oedema, retinal atrophy and optic neuropathy [7]. These may lead to irreversible vision loss.
For the case presented, the diagnosis of CMV retinitis was made on presentation to the ophthalmologists. Primary care physician did diagnose HIV and start ART. However, baseline immune status by way of CD4 count was not done and even the patient only managed to get one result during the whole treatment course though it was repeatedly requested. At least two HIV viral load results were available showing reduced viraemia over time. Use of ganciclovir or oral valganciclovir was not possible in this patient due the extremely high cost of such therapy that was not sustainable. Thus, treatment was limited to intravitreal ganciclovir and ART. Other patients are apprehensive about any invasive ocular treatment and thus refuse this treatment also. The cause of poor vision in this patient included optic atrophy, macula atrophy and retinal detachment. Retinal surgery services are also not widely available as yet in the public sector in this setting and thus patient has not yet had the surgery as at publication date. Low vision services are also a challenge at present.
Despite the challenges, the patient managed to improve to 6/12 vision in one eye that enabled her to move around independently by the last follow up. She was consistent with treatment and review appointments which enabled the success.
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