Ординатура / Офтальмология / Английские материалы / Shields Textbook of Glaucoma, 6th edition_Allingham, Damji, Freedman_2010
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associated conditions include systemic hypertension, primary clotting disorders, and the systemic use of anticoagulants and thrombolytic agents (131, 132). The mechanism of the angle closure is thought to be the abrupt, forward displacement of the lens-iris diaphragm by the massively detached retina and choroid (131). Visual prognosis is poor in these eyes, and the management is directed primarily at relief of pain through IOP control with antiglaucoma medications or cyclodestructive surgery.
Hemorrhagic choroidal detachment may also occur during or after intraocular surgery, especially filtering surgery, with associated IOP elevation and flattening of the anterior chamber. (This is discussed in Section III as a complication of trabeculectomy.)
Ciliochoroidal Effusion
In the following conditions, uveal effusion with ciliochoroidal detachment may lead to a forward rotation of the lens-iris diaphragm and angle-closure glaucoma.
Nanophthalmos
Nanophthalmos is a rare inherited disorder characterized by a small eye with a small cornea, shallow anterior chamber, narrow angle, and high lens-to-eye volume ratio (133, 134 and 135). The eyes are highly hyperopic because of the short axial length (<20 mm by most definitions) and frequently develop angle-closure glaucoma in the fourth to sixth decades of life. Additional reported retinal disorders include pigmentary retinal dystrophy and a family with pigmentary retinal degeneration and cystic macular degeneration in an autosomal recessive syndrome (136, 137). Uveal effusion and nonrhegmatogenous retinal detachment may follow intraocular surgery in these cases (133, 134 and 135, 138). There is also evidence in some patients that the uveal effusion and retinal detachment may precede the surgery and cause the angle-closure glaucoma by producing a forward shift of the lens-iris diaphragm, leading to a pupillary block mechanism (139).
Histopathologic studies reveal an unusually thick sclera with irregular, interlacing collagen bundles, fraying of collagen fibrils, reduced levels of glycosaminoglycans, and elevated levels of fibronectin (140, 141, 142 and 143). Altered metabolism of glycosaminoglycans and fibronectin may be related to the development of abnormal sclera in nanophthalmos (140, 141, 143). Tissue culture studies of sclerocytes from a patient with nanophthalmos revealed modified glycosaminoglycan metabolism, which may contribute to the abnormal packing of collagen bundles and thickening of the sclera (144). Uveal effusion may be caused by reduced scleral permeability to proteins by the thickened sclera or compression of venous drainage channels by the dense collagen around the vortex veins (145, 146). However, no collagen abnormality was seen in three patients of one study, leading the investigators to suggest that nanophthalmos may result from several distinct defects (142).
This form of glaucoma responds poorly to conventional surgical therapy and has a high complication rate that is associated primarily with the uveal effusion (134, 138). Medical therapy may be effective, although miotics may increase the pupillary block (134). Laser iridectomy and laser gonioplasty (i.e., retraction of the peripheral iris) have the highest success rates and are the procedures of choice (134, 139), although
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these are not uniformly successful. One suggested approach to managing the uveal effusion is to decompress the vortex veins by making large scleral flaps over the veins and, in some cases, draining choroidal or subretinal fluid with air injection into the vitreous cavity (146). Lamellar sclerectomy, by dissecting areas of sclera to two-thirds thickness, is also reported to relieve the IOP elevation and the shallow anterior chamber (147).
Uveal Effusion Syndrome
The uveal effusion syndrome has similarities to nanophthalmos; the main exception is an eye of normal size. The uveal effusion syndrome occurs more frequently in male patients and is characterized by dilated episcleral vessels, thickened or detached choroid and ciliary body, and nonrhegmatogenous retinal detachment (145). As with nanophthalmos, the sclera may be thickened and impermeable, although one ultrastructural study revealed increased glycosaminoglycan-like deposits between the scleral fibers and dilated, rough endoplasmic reticulum and large intracellular glycogen-like granules in
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scleral cells (148). The IOP may be normal, unless angle-closure glaucoma is present, which reportedly responds to cycloplegic agents, aqueous suppressants, and corticosteroids (149). If surgical intervention is required, subscleral sclerectomy (i.e., sclerectomy under two scleral flaps located at the equator) may be helpful in inducing resolution of the subretinal fluid, especially in eyes that are smaller than the average size (<23 mm) (150).
Other Causes of Ciliochoroidal Effusion
Several additional causes of ciliochoroidal effusion are considered in subsequent chapters: arteriovenous malformation (see Chapter 20), tumors (see Chapter 21), inflammatory conditions (see Chapter 22), trauma (see Chapter 25), and surgery (see Chapter 26). Drugs, mostly sulfa-related compounds, rarely can produce uveal effusions. These include acetazolamide and topiramate (an anticonvulsant medication) (151).
Retinopathy of Prematurity
Contracture of the retrolental mass in retinopathy of prematurity (retrolental fibroplasia) can cause progressive shallowing of the anterior chamber with eventual angle-closure glaucoma. This complication coincides with the cicatricial phase of the disease, which usually has its onset at 3 to 6 months of age. However, the angle-closure glaucoma may occur later in childhood or even in young adulthood (152, 153), and continued observation is needed. Although the typical mechanism of glaucoma is angle closure due to the retrolental mass, anterior chamber angle abnormalities, including hypopigmentation of the iris root, a translucent material in the angle, and a prominent Schwalbe line, suggest a developmental origin in some cases (154).
The glaucoma usually does not respond well to medical therapy, although some success has been reported with the use of cycloplegic agents and topical corticosteroids (155, 156). Iridectomy or trabeculectomy may be effective in some cases (153). Lens aspiration, with anterior vitrectomy, has been successful in other patients in controlling the IOP, although the procedure is usually only to relieve pain and avoid enucleation because useful vision is often lost by this stage (152). Vitrectomy techniques to reattach the retina have resulted in improved vision in some cases (157, 158), although patients with concomitant glaucoma usually have a poor visual outcome despite reattachment of the retina (159). In one case, however, an infant regained vision after treatment of the glaucoma with antihypertensive medication (160).
Persistent Hyperplastic Primary Vitreous
Retention and hyperplasia of the primary vitreous is usually unilateral and is often associated with microphthalmia and elongated ciliary processes (161). Because of anastomotic vessels between the anterior and posterior tunica vasculosa lentis, the presence of small pupillary notches may be a helpful sign of persistent hyperplastic primary vitreous, especially when the diagnosis is obscured by an opaque lens (162). The appearance of persistent hyperplastic primary vitreous by computed tomography is sufficiently characteristic as to also make this a useful diagnostic modality (163).
Glaucoma is usually a late finding with persistent hyperplastic primary vitreous. Angle-closure mechanisms are most common, resulting from anterior displacement of the lens-iris diaphragm due to contracture of the fibrous retrolenticular mass or a swollen lens. Other cases of angle-closure glaucoma may have extensive peripheral anterior synechiae. Open-angle mechanisms may include chronic uveitis, intraocular hemorrhage, and abnormal development of the anterior chamber angle.
If left untreated, most of these eyes undergo progressive deterioration (164). The recommended treatment is aspiration of the lens and removal of the fibrovascular mass with scissors or vitrectomy instruments (164, 165 and 166). Because the retina in these cases often extends as far anteriorly as the pars plicata, a pars plana incision is thought to be contraindicated (167), and success with a limbal incision has been reported (164). This treatment may prevent or eliminate the angle-closure glaucoma, although postoperative visual rehabilitation is difficult and treatment to avoid amblyopia is usually required (165, 166).
Attention should also be given to the fellow eye in these cases, because two adult patients with uncomplicated unilateral persistent hyperplastic primary vitreous were found to have open-angle glaucoma in the contralateral eye, associated with anomalous blood vessels in the entire circumference
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of the anterior chamber angle, band keratopathy, and heterochromia iridis (167). Retinal Dysplasia
This condition is usually bilateral and is associated with multiple congenital anomalies, especially in trisomy 13-15 (168). The dysplastic retina may be pulled up behind the lens, and glaucoma may result from angle closure or an associated dysgenesis of the anterior chamber angle.
Retinitis Pigmentosa
Retinitis pigmentosa has been described in association with glaucoma, which appears most often to be of the open-angle type (169). However, the association is infrequent, and a true cause-and-effect relationship has not been established.
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KEY POINTS
Neovascular glaucoma is a relatively common and serious complication of several retinal disorders, especially diabetic retinopathy, central retinal vein occlusion, and ocular ischemia, as well as certain other ocular and extraocular conditions.
The pathophysiology of neovascular glaucoma involves abnormally high levels of VEGF within the eye and growth of a fibrovascular membrane on the iris surface and in the anterior chamber angle, which initially obstructs aqueous outflow in an open-angle glaucoma and then contracts to produce an angle-closure form of glaucoma.
The most effective long-term treatment of neovascularization of the iris or neovascular glaucoma is panretinal photocoagulation in the early stages of the disease to reduce the stimulus for anterior segment neovascularization. Intravitreal or intracameral injection of anti-VEGF agents cause regression of anterior segment neovascularization and can thus be a very useful short-term adjunct.
Retinal detachments are usually associated with a reduction in IOP, although some patients may have concomitant retinal detachment and glaucoma, which may or may not have a cause-and- effect relationship.
A group of conditions in which angle-closure glaucoma may be associated with a retinal, choroidal, or vitreous disorder include central retinal vein occlusion, nanophthalmos, retinopathy of prematurity, persistent hyperplastic primary vitreous, and retinal dysplasia.
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Shields > SECTION II - The Clinical Forms of Glaucoma >
20 - Glaucomas Associated with Elevated Episcleral Venous Pressure
Authors: Allingham, R. Rand
Title: Shields Textbook of Glaucoma, 6th Edition Copyright ©2011 Lippincott Williams & Wilkins
> Table of Contents > SECTION II - The Clinical Forms of Glaucoma > 20 - Glaucomas Associated with Elevated Episcleral Venous Pressure
20
Glaucomas Associated with Elevated Episcleral Venous Pressure EPISCLERAL VENOUS PRESSURE
The episcleral venous pressure is one factor that contributes to the intraocular pressure (IOP). The normal episcleral venous pressure is approximately 8 to 10 mm Hg (1, 2, 3, 4 and 5), although recorded values vary according to the measurement technique used. (The instruments for measuring episcleral venous pressure are described in Chapter 2 (1, 2, 4, 5).)
It is commonly thought that for every mm Hg increase in episcleral venous pressure, there is an equal rise in IOP, although the magnitude of IOP rise may be greater than the rise in venous pressure (6). Studies of chronic open-angle glaucoma have revealed no significant abnormality of episcleral venous pressure (2, 5, 7). In one study, however, the episcleral venous pressure was slightly higher in patients with primary openangle glaucoma (12.1 mm Hg) or normal-tension glaucoma (11.6 mm Hg) than in control eyes (9.5 mm Hg) (8). There are, in fact, various conditions in which elevated episcleral venous pressure can produce characteristic forms of associated glaucoma. These conditions are the subject of this chapter.
