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Ординатура / Офтальмология / Учебные материалы / Vitreoretinal Surgery Second Edition Springer.pdf
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168

7 Different Presentations of Rhegmatogenous Retinal Detachments

 

 

eye. Often, these eyes are stable after surgery even with a persistent shallow total RRD and maintain some navigation vision. These young patients do not want the risk of an ugly phthisical eye from multiple operations.

7.6Giant Retinal Tear

7.6.1Clinical Features

A giant retinal tear (GRT) is defined as a tear of more than three clock hours of the retina (or 90°) with PVD with an incidence 0.091 per 100,000 in the UK (Ang et al. 2010). The patients are often in the third to fifth decades of age, more often male and present early, frequently with the macula still on (55 %) (Ang et al. 2010). The vitreous is detached from the posterior pole and is attached to the anterior portion of the retinal tear, thereby distinguishing a GRT from a dialysis. Because the posterior flap of the tear is not attached to the vitreous, this can fold over on itself onto the retina (Fig. 7.3). In addition, there may be radial slits at either end of the tear extending posteriorly, which also aid the folding of the retina. There may be satellite U-shaped breaks elsewhere in the retina that should be searched for (Fig. 7.4).

A giant retinal tear may occur in isolation, usually in a myopic patient, but may also be present in hereditary vitreoretinal disorders such as Stickler’s syndrome (14 %) (Billington et al. 1985) or rarely in Marfan’s syndrome (Sharma et al. 2002). In Sticker’s syndrome, in particular, there is a risk of

Fig. 7.4 A wide angle view of a superior giant retinal tear

bilateral retinal tears up to 40 %, and for this reason, some surgeons advocate prophylactic 360° cryotherapy to the fellow eye (Wolfensberger et al. 2003). A giant retinal tear can also occur as a result of trauma either penetrating or nonpenetrating (Duguid and Leaver 2000; Aylward et al. 1993). It possible for the tear to appear during complicated anterior segment surgery when traction on the vitreous pulls on the vitreous base (McLeod 1985; Aaberg et al. 1997). A careless vitreoretinal surgeon may induce a GRT during insertion of instruments through the vitreous base at the pars plana.

7.6.2Stickler’s Syndrome

 

This syndrome is characterised by myopia, paravascular pig-

 

mentary changes and dragging of the major vessels at the

 

optic disc, ‘veils’ or condensations of cortical vitreous around

 

large lacunae or dehiscence’s in the gel and multiple posterior

 

vitreoretinal adhesions. Differentiation from the Wagner syn-

 

drome is dubious. Stickler’s syndrome has an autosomal

 

dominant inheritance and has highly variable penetrance.

 

A possible genetic abnormality has been identified at COL2A1

 

(Richards et al. 2000, 2005; Snead and Yates 1999). Systemic

 

associations are very variable and include high palate, charac-

 

teristic facies with a flattened nasal bridge, short mandible

 

and long philtre and arthralgia (Spallone 1987). Retinal

 

detachments are related to posterior paravascular vitreoretinal

 

adhesions or to radially orientated post-equatorial lattice

Fig. 7.3 Giant retinal tears can often fold over onto the posterior retina

degeneration. Bilateral giant retinal tears are common.

7.6 Giant Retinal Tear

169

 

 

Fig. 7.7 A high-arched palate in Stickler’s syndrome

Fig. 7.5 The flattened nasal bridge typical of Stickler’s syndrome

Fig. 7.8 Arthropathy in Stickler’s syndrome

Fig. 7.6 The flattened nasal bridge in Stickler’s syndrome

170

7 Different Presentations of Rhegmatogenous Retinal Detachments

 

 

Fig. 7.9 Lattice degeneration in a patient with Stickler’s syndrome

Fig. 7.11 The vitreous in Stickler’s syndrome has veils of condensed vitreous within an otherwise optically empty vitreous cavity

Fig. 7.10 Lattice is seen around a blood vessel typically in Stickler’s syndrome

Fig. 7.12 Vitreous veils are seen in these images from a father and son with Stickler’s syndrome (see Fig. 7.13)

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