Sir,

A 46-year-old Caucasian man presented with vague abdominal symptoms, constipation, and painless loss of vision bilaterally. The presenting visual acuity was 6/36 in the right eye and hand movements in the left. Examination revealed bilateral vitreous haemorrhage with disc neovascularisation (NVD) in the right eye and a dense vitreous haemorrhage in the left eye precluding clear fundal angiography.

Fundal Fluorescein Angiography (FFA) of the right eye showed peripheral veno-venous shunts, extensive ischaemia as well as peripheral and disc neovascularisation with leakage (Figure 1).

Figure 1
figure 1

Pre-pegaptanib FFA of right eye: Showing NVD, new vessels elsewhere (NVE), peripheral ischaemia and veno-venous shunts.

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Numerous investigations were carried out. Full blood count, clotting, urea, and electrolytes, glucose, thyroid function tests, serum lipids, and plasma protein electrophoresis were within normal limits. Vasculitis screen including erythrocyte sedimentation rate, C-reactive protein, serum ACE, ANCA, anti-DS DNA, ANA, anti-treponemal antigen, Mantoux test, and chest X-Ray were also negative. In addition, causes of hypercoagulability such as antiphospholipid syndrome and homocysteinuria were excluded. In the absence of other findings the presence of peripheral perivascular sheathing, periphlebitis, and neovascularisation suggested idiopathic retinal vasculitis (Eales' disease).

Over a period of 1 year he had repeated vitreous haemorrhage and was treated with full bilateral panretinal photocoagulation. Owing to recurrent persistent non-clearing vitreous haemorrhage he underwent four successive pars plana vitrectomies, endolaser, and cryotherapy. He was also commenced on high-dose oral prednisolone (60 mg). Despite medical and surgical treatment he had a further vitreous haemorrhage and a single intra-vitreal pegaptanib (Macugen) 0.3 mg injection was administered to the right eye.

Two weeks post-pegaptanib his visual acuity improved to 6/18 without any evidence of vitreous haemorrhage.

An FFA of the right eye confirmed complete regression of retinal neovascularisation without leakage. (Figure 2) Following this improvement intra-vitreal pegaptanib was given in the left eye with similar success. Nine months post intra-vitreal pegaptanib and vitrectomy our patient has not had a recurrence of vitreal haemorrhage in either eye, with a final visual acuity in the left eye of 6/18.

Figure 2
figure 2

Post-pegaptanib FFA of right eye: Note complete regression of NVEs and no disc leakage.

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Eales' disease is a rare idiopathic retinal perivasculitis. It causes retinal ischaemia which may lead to neovascularisation and recurrent vitreal haemorrhage.1

Pegaptanib (Macugen) inhibits the 165 isoform of vascular endothelial growth factor, which is associated with ocular neovascularisation. It is approved to treat neovascular disease such as age-related macular degeneration.2 Furthermore, the benefit of intra-vitreal bevacizumab (Avastin) in Eales' disease has been reported.3

Histopathological examination of an eye with Eales' disease has shown intense expression of vascular endothelial growth factor compared with other conditions inducing neovascularisation. This may explain the severity of neovascularisation and vitreous haemorrhage in this condition.4

We decided to use pegaptanib as it is licensed for intraocular use, has proven efficacy and an excellent safety profile.5 This case demonstrates that pegaptanib is a safe and effective adjunctive therapy for retinal neovascularisation in Eales' disease refractory to other treatment.