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From questions on page 282
sdOCT revealed a corrugated appearance of the RPE with disorganisation of the photoreceptor inner segment—outer segment junction in both eyes. This high-resolution imaging technique, with near histological definition, confirms that the layers structurally involved in APMPPE include the photoreceptors and RPE. The abnormalities observed on sdOCT and retinal thickness notably improved with disease quiescence.
Patients with APMPPE occasionally develop cerebral vasculitis, which can be life-threatening. Cerebral MRI and MRA are non-invasive imaging tests that can detect cerebral vasculitis in patients with symptoms, which was a concern in this case due to the patient's tinnitus and headaches. If the MRI and MRA are normal, but suspicion for vasculitis is high, cerebral angiography may be performed.
Previously reported vaccine triggers of APMPPE include the hepatitis B vaccine, meningococcal C conjugate vaccine and swine flu vaccine. Various white spots syndromes such as APMPPE have been noted to follow vaccinations. One possible mechanism underlying this association may be molecular mimicry. Sequence similarities between the introduced peptides and autologous tissue may incite a host autoimmune reaction.
This report describes APMPPE in a child following administration of the live, attenuated varicella vaccine Varivax (Merck & Co., Whitehouse Station, New Jersey). Recent vaccines should be considered as potential triggers in patients who present with white spot syndromes. APMPPE is characterised by acute painless visual loss in association with multiple, flat, cream-coloured lesions with indistinct margins located in the posterior pole at the level of the retinal pigment epithelium (RPE) or choroid. The condition is typically bilateral, affects patients under the age of 30 years and has a good visual prognosis.1
APMPPE has been well documented following vaccinations, including the swine flu vaccine, hepatitis B vaccine and meningococcal C conjugate vaccine.2 3 To the best of our knowledge, APMPPE has not been reported following Varivax, a live attenuated varicella virus vaccination. Varivax was approved for use in the USA in 1995 and is now recommended for all healthy children in a two-dose regimen at age 12–15 months and 4–6 years, as well as for individuals aged 13 years or older without evidence of immunity.4
APMPPE is rare in children but has been described in a 7-year-old girl.5 Treatment in our case with both oral prednisone and valacyclovir was empirical. APMPPE has a favourable prognosis, and the utility of systemic corticosteroids has been debated.6 Imaging with fundus autofluorescence and sdOCT can be effective tools to track the restoration of anatomical disturbance at the level of the photoreceptors and RPE in APMPPE.
Varicella vaccination is rapidly increasing worldwide. This practice has great potential to avert significant systemic and ocular morbidity. However, ophthalmologists should be cognisant that Varivax may be included in the list of vaccine triggers for APMPPE.