Charles Explorer logo
🇬🇧

Acute Posterior Multifocal Placoid Pigment Epitheliopathy in a Child

Publication |
2013

Abstract

The authors present a rare case report of APMPPE in a 12-year- old girl with a detailed documented finding of inflammatory changes in the macula, leading to a decrease in BCVA. After systemic and local steroids the BCVA improved promptly, although changes in the IS/OS junction and irregularities in the RPE layer remained.

The ophthalmological findings were not accompanied by any systemic signs or diseases.Introduction APMPPE is a very rare bilateral disease which was first described by Gass JDM in 1968. Around 30% of the patients have a prodrome similar to flu symptoms.

Rarely, APMPPE can be associated with cerebral vasculitis, inflammation of the thyroid gland, Wegener's granulomatosis or microvascular nephropathy. The majority of patients (80%) do not require treatment, and systemic steroids are reserved for patients with affected maculae.

The prognosis of the APMPPE is good unless the macular region is affected.