Diabetic papillopathy is a form of optic neuropathy occasionally described in patients with diabetes mellitus. The typical presentation involves slowly-progressive, unilateral or bilateral, blurring of vision associated with swelling and hyperemia of the optic disc. Symptoms are usually mild, and the outcome usually favourable. The pathogenesis is unknown. No relationship has been demonstrated between diabetic papillopathy and either diabetic retinopathy or glycemic control. Hypoxia is thought to play a role: for example, fluorescein angiography has demonstrated diffuse impairment of retinal capillary perfusion in approximately half of cases. Although diabetic papillopathy has been considered a form of ischemic optic neuropathy (both Arteritic and non-arteritic), its presentation and prognosis differ profoundly from these conditions in which visual loss is sudden and profound, with a poor prognosis for recovery.
May be asymptomatic. Often presents with slowly-progressive, mild blurring of vision.
Signs are usually, but not invariably, unilateral. Diabetic papillopathy may be an incidental finding in an asymptomatic patient. Visual acuity is often mildly reduced or normal, with or without an enlarged blind spot. Optic disc swelling may be striking, with distended surface vessels. There is not the pale swelling or atrophic appearance of anterior ischemic optic neuropathy. Signs of diabetic macular edema are often present. Cotton wool spots and other signs of severe retinal ischemia are uncommon.
Rare. The classical presentation is in a young adult with Type I diabetes. Cases have been reported between ages of 17 and 79 years, in patients with Type I and Type II diabetes.
Significance and Differential Diagnosis
When a diabetic patient with intact visual function presents with swelling and hyperemia of the optic disc, the initial priorities are to exclude papilloedema (disc swelling with raised intracranial pressure) and proliferative diabetic retinopathy with neovascularisation on the disc. The importance of regular review and strict glycaemic control for the diabetic patient should be reinforced.
Arteritic Ischaemic Optic Neuropathy, Non-Arteritic Ischaemic Optic Neuropathy, Diabetes -Introduction to Retinopathy, Diabetes – Macular Edema.
Ocular Tests, Imaging investigations
The first priority on initial presentation is to exclude (i) other causes of optic nerve swelling, and (ii) proliferative retinopathy with neovascularization on the disc. Tests in this regard will depend on the clinical presentation, but may include imaging investigations of the brain and orbit (see Papilloedema). Fluorescein angiography helps differentiate dilated vessels over the optic disc in diabetic papillopathy (generalised leakage of dye at the optic disc) from neovascularization in proliferative retinopathy (focal leakage from new vessels). A variable finding is coarsening of the retinal capillary network in the posterior pole, which is consistent with ischemia.
Review and Advice
No specific treatment is indicated for diabetic papillopathy. Notably, corticosteroids provide no known benefit, and will temporarily disrupt glycemic control. Initial review is recommended at one month, three months and subsequently as indicated by the patient’s underlying diabetic retinopathy. The disc swelling usually resolves spontaneously over several months without residual damage. Rarely, a variable degree of optic atrophy develops. The patient’s long-term visual outcome will be optimized by strict glycemic control and regular review.
Marked diabetic papillopathy with disc edema and blot hemorrhages. Note the associated macula edema with blurring of the detail of the underlying tissues.
Fluorescein angiogram of the same eye as in figure 1. Note the marked leakage at the disc, widespread microaneurysms and macula edema.