17.8 Uveal Effusion Syndrome

Zones

•Zone 1: The centre of zone 1 is the optic nerve. The zone extends twice the distance from the optic nerve to the macula in a circle.

•Zone 2: Is a circle surrounding the zone 1 circle with the nasal ora serrata as its nasal border.

•Zone 3: Is the crescent that the circle of zone 2 did not encompass temporally.

Stages

•Stage 0: Characterised by immature retinal vasculature. No clear demarcation of vascularised and nonvascularised retina is present.

•Stage 1: A fine, thin demarcation line can be seen between the vascular and avascular regions.

•Stage 2: A broad, thick ridge exists between the vascular and the avascular retina.

•Stage 3: Neovascularisation is present on the ridge, on the posterior surface of the ridge or anteriorly towards the vitreous cavity.

•Stage 4: A subtotal retinal detachment is present, beginning at the ridge.

•Stage 4A: Does not involve the fovea.

•Stage 4B: Involves the fovea.

•Stage 5: This stage is a total retinal detachment in the shape of a funnel.

•Stage 5A: An open funnel.

•Stage 5B: A closed funnel.

Plus disease is defined as dilation and tortuosity of the

peripheral retinal vessels, iris vascular engorgement, pupillary rigidity and vitreous haze.

Vitrectomy methods have been used for the more severe grades of retinopathy, stages 4A–5B. Although there are reports of patients with better vision, up to 20/25 (Fuchino et al. 1995), most successful anatomical outcomes result in ‘fixing and following’ acuities (Capone and Trese 2001; Chong et al. 1986; Seaber et al. 1995; Trese and Droste 1998). Electrophysiology has tended to confirm poor retinal function (Cherry et al. 1995). In this eventuality functional blindness is highly likely even with surgical intervention. Initially scleral buckling (Machemer 1993; Ricci et al. 1996; Topilow et al. 1985) was used; then vitrectomy was combined with lensectomy (Machemer 1983; Mintz-Hittner et al. 1997) because of the small size of the eye and the lack of a pars plana. More recently lens-sparing techniques have been described (Prenner et al. 2004; Ferrone et al. 1997; Lakhanpal et al. 2005). Plus disease appears to be associated with a poorer chance of success (Hartnett 2003). It has been questioned whether patients who receive vitrectomy do any better than those who have not been operated upon (Quinn et al. 1991). PPV for stage 5 ROP achieves a flat macula in only 28–45 % (Lakhanpal et al. 2006; Cusick et al. 2006).

Adults who suffered ROP in infancy may present in adulthood with RRD related to myopia, or early PVD RRD occurs in early adulthood, mean 23 years, and bilaterality is common (Terasaki and Hirose 2003). Lattice degeneration is commonly seen (Tasman 1979), and tractional retinal detachments

may appear. The retinas can be successfully repaired, although with a higher chance of multiple procedures of 23–50 % (Kaiser et al. 2001; Sneed et al. 1990; Tufail et al. 2004) with final success rates of approximately 83 %.

17.8Uveal Effusion Syndrome

17.8.1 Clinical Features

The uveal effusion syndrome is an unusual condition often mistaken for either a rhegmatogenous detachment complicated by choroidal detachment, or a ‘ring melanoma’ of the anterior choroid. It is characterised by deep ciliochoroidal detachments, mottling of pigment epithelium (leopard spots) and serous retinal detachment which exhibits marked ‘shifting fluid’ (movement of subretinal fluid with gravity). Eyes may be nanophthalmic or hypermetropic. Spontaneous resolution may occur over a period of months.

Fig. 17.49 Choroidal elevation in uveal effusion syndrome (see Fig. 17.50)

Fig. 17.50 See previous figure

Fig. 17.51 Macular changes in uveal effusion syndrome

Fig. 17.52 This patient has an exudative retinal detachment from uveal effusion syndrome

Fig. 17.54 Coat’s disease in an 8-year-old boy with counting fingers vision. In general with unilateral Coat’s disease, treatments are not worthwhile as the other eye is good, and any intervention may disturb the anatomy of the eye, producing cosmetic problems

Table 17.1 Causes of exudative retinal detachment

Exudative retinal detachment, common causes