„White-dot-Syndrome” im Kindesalter

Abstract

The term "white dot syndromes" describes a group of heterogeneous inflammatory disorders of the choriocapillaris. They were first described a few decades ago and our knowledge about these variable diseases is very limited, especially in regard to their overall incidence and their differential diagnostic relevance in uveitis of childhood. A retrospective analysis has been performed of all cases of white dot syndromes in 407 patients with paediatric uveitis who were examined between 1996 and 2006. The relevant literature was reviewed. The following incidence of white dot syndromes in childhood was found: acute posterior multifocal placoid pigmentepitheliopathy (APMPPE) (n = 4), multiple evanescent white dot syndrome (MEWDS) (n = 4), multifocal choroiditis and panuveitis (MCP) (n = 3) and 1 case of serpiginous choroiretinitis. The review of literature shows a different age predilection of the different white dot diseases. The relative frequency of white dot syndromes in paediatric uveitis patients is estimated to be between 1 - 5%. "White dot syndromes" are an important differential diagnosis in uveitis of childhood. ICG angiography is an important tool for the diagnosis and follow-up examinations of these inflammatory diseases of the choriocapillaris. The different entities of white dot syndromes show differences concerning incidence, prevalence, course of disease, rate of complications and therapeutic implications.