Fig. 13.68 Coats disease. (A) Leukocoria; (B) retinal telangiectasis; (C) intraretinal exudates; (D) subretinal exudation; (E) progressive involvement; (F) exudative retinal detachment
(Courtesy of C Barry – fig. E)
Fig. 13.69 FA in mild Coats disease; (A) FA venous phase shows hyperfluorescence of telangiectasis, (B) late phase shows extensive hyperfluorescence due to leakage and staining
(Courtesy of C Barry)
Treatment
1Observation in patients with mild, non-vision threatening disease and in those with a comfortable eye with total retinal detachment in which there is no hope of restoring useful vision.
2Laser photocoagulation to areas of telangiectasia should be considered if progressive exudation is documented. Frequently more than one treatment session is required to obliterate the peripheral telangiectasia and induce resolution of remote exudation at the macula (Fig. 13.70).
3Anti-VEGF therapy. Limited studies of anti-VEGF therapy for Coats have been carried out to date, but initial results are promising, including as an adjunctive treatment to laser. Long-term safety in childhood remains unknown.
4Cryotherapy, with a double freeze-thaw method, in eyes with extensive exudation or subtotal retinal detachment although this may result in marked reaction with increased leakage. Therefore, laser photocoagulation is still the preferred option if at all possible.
5Vitreoretinal surgery may be considered in eyes with total retinal detachment and a poor visual prognosis as successful retinal reattachment often prevents the subsequent development of neovascular glaucoma.
6Enucleation may be required in painful eyes with neovascular glaucoma.
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Fig. 13.70 (A) Hard exudates in mild Coats disease; (B) resolution several months after laser photocoagulation
Prognosis
The prognosis is variable and dependent on the severity of involvement at presentation. Young children, particularly those under 3 years of age, frequently have a more aggressive clinical course and often already have extensive retinal detachment at presentation. However, older children and young adults have a more benign disease with less likelihood of progressive exudation and retinal detachment and in some cases spontaneous regression may occur.
Differential diagnosis
Differential diagnosis includes other causes of unilateral leukocoria and retinal detachment in children such as late-onset retinoblastoma, toxocariasis, incontinentia pigmenti and retinal capillary haemangioma.
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Eales disease
The eponym ‘Eales disease’ is used to describe patients with bilateral, idiopathic, occlusive, peripheral periphlebitis and neovascularization. The disease is rare in Caucasians but is an important cause of visual morbidity in young Asian males and is strongly associated with tuberculoprotein hypersensitivity.
1Presentation is usually in the 3rd–5th decades with vitreous haemorrhage.
2Signs. The disease is characterized by three overlapping stages: (a) periphlebitis, (b) occlusion and (c) retinal neovascularization.
•Mild uveitis is common.
•Peripheral vascular sheathing associated with peripheral capillary non-perfusion, particularly superotemporally (Fig. 13.71A).
•Branch retinal vein occlusion
•Peripheral neovascularization at the junction of perfused and non-perfused retina (Fig. 13.71B), with recurrent vitreous haemorrhage (13.71C).
3Complications include tractional retinal detachment, rubeosis iridis, glaucoma and cataract.
4Treatment involving either PRP or feeder vessel photocoagulation is useful in active disease. Systemic steroids, and possibly other immunosuppressants, may be helpful in the inflammatory stage. Persistent vitreous haemorrhage or tractional detachment may require vitreoretinal surgery. As there is evidence of substantial VEGF activity in Eales disease, intravitreal VEGF inhibitors may be useful in the proliferative stage. Investigation should be carried out to exclude conditions presenting with similar features. The visual prognosis is good in the majority of cases.
Fig. 13.71 Eales disease. (A) Peripheral vascular sheathing and occlusion in the superotemporal quadrant; (B) peripheral neovascularization; (C) haemorrhage from new vessels
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Radiation retinopathy
Radiation retinopathy may develop following treatment of intraocular tumours by plaque therapy (brachytherapy) or external beam irradiation of sinus, orbital or nasopharyngeal malignancies. It is characterized by delayed retinal microvascular changes with endothelial cell loss, capillary occlusion and microaneurysm formation. As with diabetic retinopathy its progress may be accelerated by pregnancy. Affected patients may also develop cataract and keratopathy. There is some evidence that it is more likely to occur in genetically predisposed patients.
1Presentation. The time interval between exposure and disease is variable and unpredictable, although commonly between 6 months and 3 years.
2Signs
•Discrete capillary occlusion with the development of collateral channels and microaneurysms, best seen on FA (Fig. 13.72A).
•More severe capillary non-perfusion (Fig. 13.72B).
•Retinal oedema and exudates (Fig. 13.72C).
•Cotton wool spots, flame-shaped haemorrhages (Fig. 13.72D) and papillopathy.
•Proliferative retinopathy (Fig. 13.72E).
3Treatment by laser photocoagulation may be beneficial. Papillopathy may benefit from systemic steroids and macular oedema from intravitreal triamcinolone.
4Prognosis depends on the severity of involvement. Poor prognostic features include papillopathy and proliferative retinopathy, which may result in vitreous haemorrhage and tractional retinal detachment.
Fig. 13.72 Radiation retinopathy. (A) FA shows focal retinal capillary non-perfusion associated with microvascular abnormalities; (B) more severe retinal capillary non-perfusion and microvascular abnormalities; (C) microvascular abnormalities and hard exudates; (D) cotton wool spots and haemorrhages following brachytherapy for choroidal melanoma; (E) disc new vessels and arterial occlusion
(Courtesy of J Donald M Gass, from Stereoscopic Atlas of Macular Diseases, Mosby 1997 – fig. B; S Milenkovic – fig. C; B Damato – fig. D)
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Purtscher retinopathy
Purtscher retinopathy is caused by microvascular damage with occlusion and ischaemia associated with severe trauma, especially to the head and in chest compressive injury. Other causes include embolism (fat, air or amniotic fluid) and systemic diseases (acute pancreatitis, pancreatic carcinoma, connective tissue diseases, lymphoma, thrombotic thrombocytopenic purpura and following bone marrow transplantation). Cases not associated with trauma are sometimes referred to as ‘Purtscher-like retinopathy’.
1Presentation is with sudden visual loss.
2Signs. Multiple unilateral or bilateral superficial white retinal patches, resembling large cotton wool spots, often associated with superficial peripapillary haemorrhages (Fig. 13.73).
3Treatment of the underlying cause is desirable but not always possible.
4Prognosis is guarded as a result of macular or optic nerve damage; only a small proportion will regain normal vision. At least 50% achieve spontaneous visual recovery of two lines or more. The acute fundus changes usually resolve within a few weeks.
Fig. 13.73 Purtscher retinopathy
(Courtesy of J Donald M Gass, from Stereoscopic Atlas of Macular Diseases, Mosby 1997)
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Benign idiopathic haemorrhagic retinopathy
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Benign idiopathic haemorrhagic retinopathy
Benign idiopathic haemorrhagic retinopathy is rare but important because it has a good prognosis without treatment.
1Presentation is in adult life at any age with acute unilateral visual impairment.
2 Signs. Unilateral multiple large intraretinal haemorrhages at the posterior pole and around the optic disc (Fig. 13.74). 3 Course. Vision recovers within 4 months.
4Differential diagnosis
•Terson syndrome which is typically associated with subarachnoid haemorrhage.
•Benign retinal vasculitis.
•Valsalva retinopathy.
•High altitude retinopathy.
Fig. 13.74
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Valsalva retinopathy
The Valsalva manoeuvre comprises forcible exhalation against a closed glottis, thereby creating a sudden increase in intrathoracic and intraabdominal pressure (e.g. weight-lifting, blowing up balloons). The associated sudden rise in venous pressure may result in rupture of perifoveal capillaries leading to unilateral or bilateral sub-internal limiting membrane haemorrhage at the macula of varying severity (Fig. 13.75).
Fig. 13.75 Valsalva retinopathy. (A) Mild; (B) severe
(Courtesy of J Donald M Gass, from Stereoscopic Atlas of Macular Diseases, Mosby 1997 – fig. B)
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Lipaemia retinalis
Lipaemia retinalis is a rare condition characterized by creamy-white coloured retinal blood vessels in patients with hypertriglyceridaemia (Fig. 13.76). The visualization of high levels of chylomycrons in blood vessels accounts for the fundus appearance. Visual acuity is usually normal but electroretinogram amplitude may be decreased.
Fig. 13.76 Lipaemia retinalis
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