17.3Retinal Haemangioma and Telangiectasia

Fig. 17.7 There is a spectrum of retinal vascular anomalies which may affect the eye including Coat’s disease, isolated angiomas and von Hipple–Lindau. In this young patient there is angioma on the disc causing macular swelling. There is no effective way of treating the optic nerve disease. FFA confirms leakage (see Fig. 17.8)

Fig. 17.9 A small angioma in von Hipple–Lindau. These angiomata can produce devastating problems within the retina, such as exudative retinal detachment, vitreous haemorrhage and subretinal and intraretinal exudation

Fig. 17.11 A peripheral angioma seen on indentation

Fig. 17.12 A composite picture showing treated lesions, macular exudation and an optic nerve head angioma

Fig. 17.14 This patient had 16 procedures on his eyes for the retinal complications of von Hipple–Lindau which included rhegmatogenous retinal detachment, exudative retinal detachment, PVR and cystoid macular oedema. Only the eye shown retained useful vision of 20/120 but with untreatable peripapillary angiomas

Fig. 17.15 Parafoveal telangiectasia causing an ERM

Fig. 17.17 Multiple leaking telangiectasia sometimes called Leber’s miliary aneurysms

In VHL a mean of four tumours are seen per eye, and new lesions tend to occur before the age of 47 years. In VHL extraocular lesions occur as:

1.Central nervous system haemangioma

2.Renal cyst, renal carcinoma

3.Pancreatic cysts and adenoma, pancreatic islet cell tumours

4.Phaeochromocytoma

5.Endolymphatic sac tumour of the inner ear

6.Cystadenoma of the epididiymis and broad ligament

Retinal angiomas may cause vitreous haemorrhage, tractional, exudative or rhegmatogenous retinal detachment and macular pucker or hole (Inoue et al. 2004; Schwartz et al. 1990; Laatikainen et al. 1989; Ferguson and Singh 2002; Loewenstein 1995; Machemer and Williams 1988). Treatment may involve

1.Observation

2.Laser photocoagulation of angiomas smaller than 1.5 mm

3.Cryotherapy to larger lesions

4.Vitrectomy surgery for the complications such as vitreous haemorrhage, retinal detachment and macular pucker (Singh et al. 2002; Raju et al. 2003)

Unfortunately PVR formation is common making surgery

hazardous.

External beam radiotherapy has been tried where other treatments have failed to regress the lesions (Raja et al. 2004), but as yet an appropriate therapeutic option is not available for optic nerve lesions (Garcia-Arumi et al. 2000).