This article is more than 5 years old. Information may no longer be current.
Woman presents with acute onset of decreased vision in right eye
Seven weeks before presentation, the patient developed right-sided facial pain and a subsequent facial rash.
Click Here to Manage Email Alerts
A 45-year-old woman was referred to our office for a new onset dark curtain and flashing lights affecting her right eye for the past 24 hours. At the time of presentation, she described new floaters, intermittent shooting pain in the right eye for the past week and decreased vision.
Her medical history included systemic lupus erythematosus, anti-phospholipid syndrome, hypertension, Nocardia, cutaneous mycobacterium avium infection and a recent ongoing flare of herpes zoster affecting the right V1 dermatome. Her medications included prednisone, mycophenolate mofetil, Bactrim, subcutaneous heparin, lisinopril, atorvastatin, acyclovir, colchicine and gabapentin.
She reported that 7 weeks before presentation she developed right-sided facial pain and a subsequent facial rash. Her primary ophthalmologist examined her at that time and diagnosed her with herpes zoster ophthalmicus with corneal involvement. She was prescribed a 10-day course of famciclovir (500 mg orally three times a day). Two weeks later, after completion of the famciclovir, she was found to have persistent corneal involvement on exam and was started on acyclovir (500 mg orally five times a day). Three days after initiation of acyclovir, she was reported to have improved clinically and acyclovir was decreased to 500 mg orally twice daily. She remained on this dose for 3 weeks at which time she was again noticed to have worsening corneal involvement. Her ophthalmologist increased her acyclovir again to 500 mg orally five times a day. Three days later, she noticed the curtain in the vision in her right eye, which led to the referral to our office.
Examination
In our office, the patient’s best corrected visual acuity was 20/40 in the right eye and 20/20 in the left. IOPs in the right and left eyes were 12 mm Hg and 11 mm Hg, respectively. Pupils were round and reactive to light with no afferent pupillary defect. External exam revealed no facial rash. Slit lamp biomicroscopy was remarkable for 1+ conjunctival injection in the right eye; however, there was no anterior chamber inflammation.
Dilated funduscopy of the right eye revealed 3+ vitritis with peripheral retinal whitening and necrosis for 5 consecutive clock hours from 7 o’clock to 2 o’clock in a clockwise direction with a temporal retinal detachment and fluid extending to the macula (Figure 1). Multiple large retinal holes in the areas of necrosis were identified with several smaller peripheral holes. Dilated fundus exam of the left eye was normal. Optical coherence tomography of the macula of the right eye highlighted the presence of the temporal retinal detachment with subretinal fluid approaching the fovea (Figure 2).
Figure 1. Moderate vitritis with peripheral areas of retinal whitening and necrosis with several large retinal holes noted superotemporally in the area of necrosis.
Images: Tawse KL, Rogers A
Figure 2. OCT of the macula of the right eye showing temporal retinal detachment arising from the area of peripheral necrosis with fluid extending into the temporal macula.
What is your diagnosis?
Retinitis with necrosis
The differential diagnosis for retinitis with necrosis includes acute retinal necrosis, progressive outer retinal necrosis, cytomegalovirus retinitis, syphilitic retinitis, intraocular lymphoma, sarcoidosis, tuberculosis, atypical toxoplasmosis, fungal infection and retinal vasculitides, particularly Behçet’s disease. In our patient, the clinical history of a vesicular facial rash helped to solidify the diagnosis of acute retinal necrosis based on the clinical appearance at the time of presentation.
Discussion
Acute retinal necrosis (ARN) is a rare presentation of herpetic eye disease. The most common culprits are varicella zoster virus followed by HSV-1 and HSV-2. It is characterized by large areas of peripheral retinal whitening and necrosis, which tend to spread centripetally. Vitritis, with or without anterior segment inflammation, is seen in addition to optic neuritis, arteriolitis, vascular occlusions and, in some cases, retinal detachment. Progressive outer retinal necrosis, in contrast to ARN, is characterized by a lack of intraocular inflammation and earlier and more severe macular involvement; it is limited to severely immunocompromised patients. In some cases, ARN can be seen in patients with some level of immune dysfunction, as in our patient. PCR-based analysis of intraocular fluid has a very high sensitivity and specificity for both HSV and VZV and may aid in the diagnosis of unusual presentations, although the diagnosis of ARN is usually made clinically.
Treatment should be initiated promptly because fellow eye involvement occurs within 1 month in one-third of patients. The current gold standard for treatment of ARN is 10 mg/kg to 15 mg/kg of intravenous acyclovir three times a day for 5 to 10 days followed by 400 mg to 800 mg of oral acyclovir five times a day for 6 to 12 weeks, although a variety of regimens including oral valacyclovir and oral famciclovir, both of which have superior plasma bioavailability to acyclovir as oral preparations, have been described. Also, the addition of intravitreal foscarnet or ganciclovir can be considered.
Given the extreme rarity of the disease process, randomized trials comparing the efficacy of treatment regimens would be too difficult to adequately power. Confluent prophylactic laser photocoagulation to the affected retina to prevent retinal detachment remains controversial, as the rate of retinal detachment remains high with or without treatment. However, this may in part be due to a delay in diagnosis and hazy media at the time of treatment.
Clinical course
The patient was taken emergently to the operating room for repair of the retinal detachment with pars plana vitrectomy with 360° of peripheral endolaser, fluid-gas exchange, drainage of subretinal fluid and infusion of 1,000 cs of silicone oil. Postoperatively, she was admitted to the infectious disease service and started on intravenous acyclovir 10 mg/kg three times a day. Vitreous biopsy sent for viral PCR was positive for varicella zoster virus.
During her hospitalization, the patient was found to have a CD4 count of 4, and mycophenolate mofetil was held per infectious disease’s recommendations. On postoperative day 4, the patient’s best corrected visual acuity was 20/30 with resolution of the vitritis. Dilated exam showed the retina to be attached with laser surrounding the prior areas of necrosis with some intraretinal and pre-retinal hemorrhage in the areas of necrosis (Figure 3). A peripherally inserted central catheter line was placed, and she was discharged on intravenous ganciclovir twice daily.
Figure 3. Exam on postoperative day 4 showed improvement in vitritis and attached retina with laser surrounding the peripheral areas of previous necrosis. Small intraretinal and pre-retinal hemorrhage was present in areas of necrosis.
Twelve months after presentation, the patient’s retina remains attached, and best corrected visual acuity measures 20/60 with a slow–growing posterior subcapsular cataract from the silicone oil. Her left eye remained unaffected throughout the course.