Approximately 5–7 % of all infants are born prematurely, and birth before 37 weeks is the most common cause of neonatal mortality, morbidity and long-term disability. Premature infants are poorly equipped for life outside the womb, and oxidant stress has been implicated in the aetiology of visual impairment in these infants, who are often exposed to increased O2 concentrations and high light intensity in neonatal units. The carotenoids lutein and zeaxanthin, which give the macular area of the eye its yellow colour, are located in the retinal pigment epithelium of the eye, and are believed to play a role in protecting it against oxidative and light damage. The macular pigments are of dietary origin, and green leafy vegetables are the primary source of lutein and zeaxanthin. Lutein is one of the five most common carotenoids found in the diet. There is current interest in the macular pigment in relation to age-related macular degeneration, but these pigments may also have a protective role in the retinal pigment epithelium of the newborn infant. Little information is available on blood lutein and zeaxanthin levels in neonates. Levels of lutein in human milk are two to three times higher than those of β-carotene, whereas their concentrations in the mothers’ blood are approximately the same. Human milk is the main dietary source of lutein and zeaxanthin for infants until weaning occurs. The biochemical mechanisms which mediate the transport of the macular carotenoids into the eye are not known, but tubulin has been identified as the major carotenoid-binding protein, and may play a role in the physiology of the macula.

Age-related maculopathy (ARM) has become the major cause of blindness in the Western World. Currently its pathogenesis and primary site of functional damage is not fully understood but ischemia is believed to play a major role. Early detection and precise monitoring of progression of ARM are main goals of current research due to lack of sufficient treatment options, especially in the dry, atrophic form of this disease. We applied the multifocal electroretinogram (mfERG) that can detect any local functional deficit objectively in the central retina. We recorded two paradigms in early ARM patients, the fast flicker and the slow flash paradigm which both represent fast adaptation processes of the proximal retina but under differing photopic conditions and stimulation rates. By subtracting the waveform responses we extracted a late component in the difference waveform that was significantly reduced in the early ARM group compared to a healthy control group (p ≤ 0.05). We propose that this multifocal nonlinear analysis permits the detection of adaptative deficits and provides topographic mapping of retinal dysfunction in early ARM. The difference waveform component we extracted with this novel approach might indicate early functional loss in ARM caused by ischemia in postreceptoral layers such as bipolar cells and inner plexiform regions.

This paper aims to provide an overview of age related macular degeneration (ARMD) and its radiotherapy management.

ARMD is already the leading cause of adult blindness in the western world. The neovascular form of ARMD is responsible for some 85% of blindness caused by ARMD as a whole. Its aetiology is unknown. The natural course of the disease is rapid progression from onset to disciform scarring and accompanying loss of visual acuity. Laser photocoagulation is only suitable for 10% of patients with neovascular ARMD. Initial studies using low dose fractionated external beam radiotherapy have demonstrated stable visual acuity over the range of 40–93% at short term follow-up. Only transient acute side effects have been observed. No radiation-induced retinopathy or vasculopathy have been observed. Common radiotherapy macula techniques are reviewed, field sizes examined and criteria for a simple macula technique proposed. A subset of patients with rapidly progressing neovascular ARMD may be candidates for rapid treatment starts. Although follow up times have been short, radiotherapy is a promising option for those patients facing imminent blindness due to rapidly progressive neovascular ARMD.

Sorsby's fundus dystrophy (SFD) is a rare autosomal dominant disorder that results in degeneration of the macular region of the retina, with onset usually in the fourth to fifth decade of life. It leads to the rapid loss of central vision, often followed by further loss of peripheral vision. SFD shares several pathological features commonly found in the ‘wet’ or exudative form of age-related macular degeneration (AMD), the most common cause of blindness in the elderly in developed countries. These phenotypic similarities have led to SFD being proposed as an acceptable genetic model for AMD. Whereas AMD appears to have a complex aetiology, with both genetic and environmental factors playing a role, SFD has been shown to be a single-gene disorder, linked to mutations in exon 5 of the tissue inhibitor of metalloproteinases 3 (TIMP3) gene on chromosome 22q12-q13. This review confines itself to a discussion of the known biochemical properties of the wild-type and SFD TIMP3 proteins and attempts to relate these to the pathology encountered in SFD patients. We also discuss briefly how, despite the lack of inherited mutations in the structural gene, the TIMP3 protein might play a role in the onset and progression of AMD.