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1 Dundee Medical School, Dundee, UK
2 Department of Medicine, Dundee Medical School, Dundee, UK
3 Department of Ophthalmology Ninewells Hospital and Medical School, Ninewells Road, Dundee DD1 9SY, UK
Keywords: parenteral nutrition; vitamin E; macular dysfunction; age related macular degeneration
A variety of nutrient deficiencies may predispose to the development of age related macular degeneration (AMD).1 Patients receiving parenteral nutrition (TPN) may be at particular risk of early onset AMD, because of inadequate or excess nutritional supplementation.1 Studies including the Eye Disease Case-Control Study and Beaver Dam Eye Study have evaluated the relation between antioxidant and micronutrient levels, and the risk of AMD.2–4 A protective effect of high plasma vitamin E levels was convincingly demonstrated.5
We describe a patient treated with parenteral fluid support who developed visual symptoms and signs of AMD, in conjunction with longstanding vitamin E deficiency. Isolated cases of visual disturbance in patients undergoing TPN have been reported in the literature6,7; however, to our knowledge, no case of visual disturbance attributed to vitamin E deficiency has been reported in this context.
Case report
A 57 year old man received parenteral fluid five times a week at home because of short bowel syndrome secondary to Crohn’s disease. It was thought he had undergone bowel adaptation to meet macronutrient and micronutrient needs in the 13 years since his surgery. He presented with subacute visual disturbance. He described altered colour perception in situations analogous to macular stress testing (moving from dark adapted situations to bright lights) and enlarging central scotomata. Visual acuity was 6/6 in the right eye, 6/12 in the left. Visual fields, intraocular pressures, and neurological examination were normal. Funduscopy revealed macular soft drusen, and extensive subretinal basal laminar deposits in the macular region, more marked in the right than left eye (fig 1). Electroretinogram was normal.
Figure 1 Macular soft drusenosis, characterised by subretinal basal laminar deposits in the macular region.
The patient was receiving electrolyte support 6 days a week at time of presentation. Measured haematological parameters and urea and electrolyte levels revealed a low haemoglobin level (11.0 g/dl), and a mild degree of macrocytosis (102.3 fl). Because hypervitaminosis and/or deficiency in trace minerals were suspected, serum values of vitamins A, E, B1, B2, B6, plasma zinc, copper, selenium, manganese, caeruloplasmin, and red cell GSH activity were measured. Results revealed vitamin E deficiency (12 μmol/l, normal range: 14–39 μmol/l). A retrospective survey of previous serum vitamin E levels suggested longstanding deficiency, with levels of 10 μmol/l, and 13 μmol/l, 6 months and 1 year respectively, before onset of symptoms. Treatment with vitamin supplementation lead to complete resolution of symptoms in 3 weeks. Vitamin E levels returned to normal; however, fundal appearances remained unchanged.
Comment
The presence of bilateral hard and soft drusen and pigmentary abnormalities in the macula are the clinical hallmarks of AMD.8 The early onset of morphological changes at Bruch’s membrane/retinal pigment epithelium (RPE) interface may relate to vitamin or micronutrient deficiency, associated with parenteral nutrition.7
Cumulative oxidative damage may have an important role in the pathogenesis of AMD, since accumulation of lipofuscin pigments may arise as a consequence of antioxidant deficiency, or under pro-oxidant conditions.5 Evidence exists for an association between atrophic AMD and excessive lipofuscin accumulation.8,9 Compromised RPE in this context is believed to be due to the amphiphilic structure and photoreactivity of the di-retinal conjugate A2E, the major constituent of lipofuscin.5,9 Antioxidant vitamins have been shown to aid in the defence against AMD.5 Vitamins E and C suppress A2E epoxidation, suggesting one mechanism by which these vitamins may protect the ageing macula.9
Vitamin E deficiency was present consistently over the 12 month period preceding symptom onset, reinforcing the likelihood that the clinical presentation had been caused by vitamin E deficiency. Vitamin E deficiency results in retinal degeneration, excessive RPE lipofuscin, and decrease in the polyunsaturated fatty acid content of rod outer segments and the RPE.5 Furthermore, vitamin E deficiency may cause mild macrocytic anaemia and accumulation of ceroid lipofuscin in nerves, affecting function of central and peripheral nervous systems.6 Patients with sufficient gut length for protein calorie nutrition receiving parenteral fluids may run the risk of micronutrient deficiency despite a normal diet, and may present to the ophthalmology department. We recommend formal micronutrient screening in patients with extensive small bowel resection.
References
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The Eye Disease Case-Control Study Group. Antioxidant status and neovascular age-related macular degeneration. Arch Ophthalomol 1993;111:1366, 1499, and 104-9.
Lyle BJ, Mares-Perlman JA, Klein BEK, et al. Antioxidant intake and risk of age-related nuclear cataracts in the Beaver Dam Eye Study. Am J Epidemiol 1999;149:801–9.
Seddon JM, Ajani UA, Sperduto RD, et al. Dietary carotenoids, vitamins A, C and E, and advanced age-related macular degeneration. JAMA 1994;272:1413–20.
Beatty S, Hui-Hang K, Henson D, et al. The role of oxidative stress in pathogenesis of age-related macular degeneration. Surv Ophthalmol 2000;45:115–34.
Kawakubo K, Matsumoto T, Mochizuki Y, et al. Progressive encephalopathy in a Crohn’s disease patient on long-term total parenteral nutrition: possible relationship to selenium deficiency. Postgrad Med J 1994;70:215–9.
Yassur Y, Snir M. Melamed S. Bilateral maculopathy simulating "cherry-red spot" in a patient with Crohn’s disease. Br J Ophthalmol 1981;65:184–8.
Zurdel J, Richard G. Clinical manifestations and natural history and ARMD. ARMD Current Treatment Concepts. Medical radiology. London: Springer, 2001.
Sparrow JR, Fishkin N, Zhou J, et al. A2E a by-product of the visual cycle. Vis Res 2003;43:2983–90.