Ординатура / Офтальмология / Английские материалы / Clinical Pathways in Glaucoma_Zimmerman, Kooner_2001
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230 Glaucoma Associated with Vitreoretinal Disorders
The reported rate of neovascular glaucoma in CRVO ranges from 4% to approximately 50%.5 The Central Vein Occlusion Study Group found that anterior segment neovascularization developed in 16% of 714 eyes.6 The strongest predictors of development of iris or angle neovascularization were initial visual acuity and amount of nonperfusion seen on fluorescein angiography. If the initial visual acuity is worse than 20/200, prognosis for vision is poor (80% remain at that level or worse); the primary concern is the prevention of neovascular glaucoma by careful attention to the development of iris or angle neovascularization and prompt treatment if it occurs. Because more than one-third of initially perfused eyes with poor vision develop nonperfusion and iris/angle neovascularization, visual acuity is more important than the initial fluorescein angiogram in determining the prognosis and clinical management of the patient.6
How Common Is Glaucoma in Association with Central
Retinal Artery Occlusion?
Development of neovascular glaucoma has been reported in approximately 15% of patients with central retinal artery occlusion (CRAO). In a prospective study of 33 consecutive patients, 18.2% developed neovascularization of the iris and 15.2% went on to develop neovascular glaucoma.7
How Common Is Glaucoma Associated with Carotid Occlusive Disease?
Carotid occlusive disease is a condition in which there is low blood flow through the ophthalmic artery due to blockage in the carotid artery. This condition accounted for 13% of all cases of neovascular glaucoma in one series.8
How Common Is Glaucoma in Association with Diabetes Mellitus?
Several large epidemiologic studies have noted an association between diabetes and open-angle glaucoma.9 The reported prevalence of rubeosis iridis in diabetic patients ranges from 0.25 to 20%.10 In diabetic patients with rubeosis iridis, the reported incidence of neovascular glaucoma ranges from 13 to 22%.10–12
How Common Is Glaucoma in Association with Retinopathy of Prematurity?
Glaucoma associated with retinopathy of prematurity (ROP) often occurs at approximately 3 to 6 months of age. However, angle-closure glaucoma may occur later in childhood, so continued observation is necessary.13,14 It is estimated that this complication may occur in as many as 30% of eyes with advanced retinopathy of prematurity.15
How Common Is Glaucoma In Association with Coats’ Disease?
In one series, glaucoma was present in 36 out of 62 eyes (58%) with Coats’ disease.16
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Diagnosis and Differential Diagnosis
How Is Glaucoma Diagnosed in Patients with CRVO?
When a patient presents with acute loss of vision, shallow anterior chamber, elevated IOP, and a hemorrhagic retinal fundus picture, the diagnosis of CRVO complicated with angle-closure glaucoma should be suspected. Unilateral shallowing of the anterior chamber may be secondary to anterior lens dislocation, uveal effusion, choroidal tumors, choroidal hemorrhage, and choroidal and ciliary body detachment, and can follow panretinal photocoagulation.4 Careful examination with indirect ophthalmoscopy is helpful to make the correct diagnosis. Ultrasonography may be necessary if there is media opacity.
Neovascular glaucoma associated with a CRVO usually occurs within 3 months and has been called “90-day glaucoma.” A patient often presents with poor vision, a severely elevated IOP, an open angle with neovascularization of the iris and angle, and a hemorrhagic retinal fundus picture. It is important to perform gonioscopy in order to detect neovascularization of the angle.
How Is Glaucoma Diagnosed in Patients with CRAO?
A patient who suffers an acute central retinal or ophthalmic artery occlusion presents with sudden visual loss, an edematous white retina except in the foveal region, giving a “cherry red spot” appearance, and sluggish flow through the retinal arterioles. After a few weeks the fundus may appear normal; however, the vision remains poor. These patients may develop neovascular glaucoma.
How Is Glaucoma Diagnosed in Patients with Carotid Occlusive Disease?
Patients with carotid occlusive disease will initially present with a low IOP, chronic aqueous flare, and midperipheral dot-blot retinal hemorrhages. Some patients may progress to neovascular glaucoma from iris neovascularization due to ocular ischemia.
How Is Glaucoma Diagnosed in Patients with ROP?
The diagnosis of glaucoma in a small infant with ROP is difficult.17 When a young child with a history of prematurity presents with a shallow anterior chamber and elevated IOP, the diagnosis of glaucoma associated with ROP should be entertained. Contracture of the retrolental mass in ROP can cause forward displacement of the lens-iris diaphragm with progressive shallowing of the anterior chamber and eventual angle-closure glaucoma.14 Developmental abnormalities in the anterior chamber may contribute to glaucoma in these patients. In addition, chronic retinal detachment may lead to retinal ischemia and neovascular glaucoma.
How Is Glaucoma Diagnosed in Patients with Coats’ Disease?
Coats18 described a childhood exudative retinopathy in which the primary feature was hemorrhage and exudation from the retinal blood vessels in the sub-
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retinal space. Coats’ disease was later described as a unilateral, exudative retinopathy resulting from telangiectasia of retinal vessels.19 This disease usually affects young boys before the age of 8 and occurs unilaterally.16 Glaucoma associated with Coats’ disease was secondary to angle closure, with IOP’s ranging from 21 to 60 mm Hg.16 In one study, 11% of patients with Coats’ disease presented with painful glaucoma of sudden onset due to angle closure.16 The mechanism of angle closure in these patients appears to be due to forward displacement of the lens-iris diaphragm. Neovascular glaucoma may also develop and result in a blind, painful eye.20
Treatment and Management
How Is Glaucoma Associated with CRVO Treated?
When CRVO is associated with transient angle-closure glaucoma, the treatment should be medical because the angle returns to normal depth over a few weeks. Unlike primary angle-closure glaucoma, for which iridectomy is the definitive therapy, the treatment of choice for secondary angle-closure glaucoma is medical.4 Treatment with cycloplegics and aqueous suppressants is recommended. Miotics such as pilocarpine cause a forward shift of the iris lens diaphragm and may worsen the angle closure.
The Central Vein Study Group recommended that patients be followed monthly or bimonthly depending on their visual acuity for the first 6 months to examine the iris and angle for any development of neovascularization.6 It is important to be aware that angle neovascularization can occur without pupillary margin involvement in CRVO, implying the necessity of screening gonioscopy.21 Panretinal photocoagulation (PRP) was recommended promptly at the first sign of definite neovascularization, but not prophylactically.6 Prophylactic PRP did not totally prevent anterior segment neovascularization. Prompt regression of anterior segment neovascularization in response to PRP was more likely to occur in eyes that have not been treated previously.22 Because of the widespread areas of nonperfusion, posterior segment neovascularization rarely occurs with ischemic CRVO. In 10 of 117 eyes in patients with iris/angle neovascularization, the new vessels failed to regress in response to photocoagulation, and neovascular glaucoma developed in the eye that could not be controlled medically.6 The treatment of neovascular glaucoma is discussed in Chapter 14.
How Is Glaucoma in Relation
to Retinal Arterial Occlusion Managed?
Although PRP has been proposed as a treatment for prevention of neovascular glaucoma in patients with CRAO, in one study, five of six patients with neovascularization of the iris went on to develop neovascular glaucoma despite PRP.7 However, ablation of the ischemic retina, which may be the source of angiogenic factors, must be undertaken. If the fundus view is adequate, then
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thorough PRP should be undertaken promptly. Cryotherapy or transscleral diode laser is useful if the view is poor.
How Is Glaucoma in Relation
to Carotid Occlusive Disease Managed?
Carotid occlusive disease may ultimately result in neovascular glaucoma. The role of carotid endarterectomy in preventing neovascular glaucoma is unclear. There are cases of resolution of ocular ischemic findings, but others could not document improvement or stabilization of vision.23
How Is Glaucoma in Association with Diabetes Managed?
Management of open-angle glaucoma in association with diabetes is similar to its management in nondiabetic patients. In is prudent to avoid epinephrine and prostaglandin agents in patients with diabetic macular edema.24–28 One should avoid using oral glycerin to acutely reduce IOP in diabetic patients. Oral isosorbide does not raise blood sugar and is preferable. Advanced diabetic retinopathy with widespread retinal ischemia may result in neovascular glaucoma. The ischemic retina, which is presumed to be producing angiogenic factors, must be treated for anterior segment neovascularization to regress. PRP is the easiest and least invasive method; however, this method is not possible if the view to the posterior segment is obscured by cataract or vitreous hemorrhage. Retinal cryotherapy, which does not require a posterior pole view, may be performed as an office procedure. Any retinal detachment that is present must be surgically repaired followed by laser photocoagulation.
How Is Glaucoma in Relation to ROP Managed?
Management of glaucoma in cases of ROP is difficult.17 Lens extraction, alone or combined with vitrectomy, has proven helpful in patients with angle closure.13,29 Cycloplegics may initially be helpful in some cases of angle closure related to pupillary block; however, most cases require lensectomy and vitrectomy for definitive cure.30 When a chronic retinal detachment is present, neovascular glaucoma often complicates ROP.31 Treatment may include seton devices and cyclophotocoagulation; however, enucleation may be necessary if the eye becomes blind and uncomfortable.
How Is Glaucoma Associated with Coats’ Disease Managed?
Multiple sessions of photocoagulation or cryotherapy of the peripheral retinal telangiectasias is the preferred treatment of eyes with Coats’ disease.32 Angleclosure glaucoma associated with Coats’ disease is extremely difficult to treat. Success has been reported using vitreoretinal techniques in order to reattach the retina, destroy the extensive retinal telangiectasias, and drain the subretinal exudates.20,33,34
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Future Considerations
What Future Therapies May Be Available to Treat Glaucoma Associated with the Retinal Vascular Disorders?
Antiangiogenic drug delivery may be used during or independently of surgery to reduce neovascularization. Endocyclophotocoagulation has become a useful adjunct in treatment of chronic glaucoma.
RETINAL DEGENERATION AND DYSTROPHY
Definition
How Is Glaucoma Associated with Retinal Degenerations and Dystrophies Defined?
Familial exudative vitreoretinopathy, vitreoretinal syndromes such as Stickler’s syndrome, retinitis pigmentosa (RP), and age-related macular degeneration (ARMD) are associated with glaucoma (Table 11–1).
Epidemiology and Importance
How Common Is Glaucoma Associated with Retinal Degenerations and Dystrophies?
The incidence of angle-closure glaucoma with ARMD (secondary to massive substantial hemmorhage) is rare. The prevalence of POAG in patients with RP has been reported to range from 2 to 12%,35 whereas angle-closure glaucoma has been reported to be 1.03% in patients with RP over age 40.36 In one study of 39 patients with Stickler’s syndrome, 10% had ocular hypertension.37
Diagnosis and Differential Diagnosis
How Is Glaucoma in Association with Age-Related
Macular Degeneration Diagnosed?
Age-related macular degeneration (ARMD) is a common disorder characterized by decreased visual acuity with drusen in the macula occurring in elderly patients. Late forms of this disorder affect approximately 7% of individuals over the age of 75.38 Patients with exudation may experience massive vitreous, subretinal, or suprachoroidal hemorrhage. Rapid expansion of the subretinal or suprachoroidal space leads to an increase in the posterior segment volume and anterior displacement and rotation of the lens-iris diaphragm, which may result in angle-closure glaucoma. This diagnosis should be suspected in the elderly patient presenting with angle-closure glaucoma and forward displacement of the lens-iris diaphragm without the anticipated shallow chamber in the fellow eye. Ghost cell glaucoma has also been reported following vitreous hemorrhage from ARMD.39
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How Is Glaucoma in Relation
to Retinitis Pigmentosa Diagnosed?
Retinitis pigmentosa (RP) is an inherited retinal degeneration characterized by nyctalopia, attenuated retinal arterioles, and presence of pigmentary changes in the retinal periphery. The diagnosis of glaucoma in the presence of RP can be challenging because both diseases may give a similar field defect. Because most forms of RP are rod-cone degenerations, a generalized constriction of the visual field is seen, which makes detecting glaucomatous scotomas difficult. In addition, the waxy pallor observed in RP can make evaluation of the optic nerve head difficult.
How Is Glaucoma in Relation
to Stickler’s Syndrome Diagnosed?
Stickler’s syndrome, or hereditary arthro-ophthalmopathy, is characterized by arthritis, cleft palate, midfacial hypoplasia, and ocular defects including radial perivascular lattice degeneration, vitreous degeneration, glaucoma, cataracts, and frequent retinal detachments.40 Phelps41 noted that several of his patients with Stickler’s syndrome had mild to moderate elevation of IOP. The anterior chambers were open without obvious structural malformation. The diagnosis of glaucoma is difficult because lens opacities and high myopia impair the view of the optic disc. In addition, the discs are often tilted, and cupping invariably is shallow. Areas of retinal degeneration may cause visual defects similar to those of glaucoma, and IOP may be the only reliable criterion on which to base treatment.41
Treatment and Management
How Is Glaucoma Associated with ARMD Treated?
Angle-closure glaucoma secondary to exudative complications of ARMD may be treated medically with cycloplegia and aqueous suppressants. Cycloplegia may reverse angle-closure glaucoma in this and other posterior segment causes of angle closure, whereas miotics may exacerbate the condition.
How Is Glaucoma Associated with RP Treated?
There is a tendency to treat patients with RP earlier with aqueous suppressants than might otherwise be done in the absence of a definitive diagnosis of glaucoma because of the difficulty in interpreting the visual fields and the optic nerve heads. Because posterior subcapsular cataracts are common in patients with RP, miotic therapy may lead to decreased visual acuity.
How Is Glaucoma Associated with Stickler’s Syndrome Treated?
The treatment of glaucoma in Stickler’s syndrome is mainly medical. The high IOPs respond well to antiglaucoma medications. Miotics should be avoided, as
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they may reduce vision in the presence of an axial lens opacity or may induce retinal detachment in these highly susceptible patients.42
Future Considerations
What Future Therapies May Be Available for Glaucoma
Associated with Retinal Degenerations and Dystrophies?
Gene therapy may alter the course of inherited retinal degenerations and dystrophies. Genetic testing may also help diagnose these conditions earlier.
RHEGMATOGENOUS RETINAL DETACHMENT (SCHWARTZ SYNDROME)
Definition
How Is Glaucoma Associated
with Rhegmatogenous Retinal Detachment Defined?
Most patients with a retinal detachment have decreased IOP in the affected eye as compared to their fellow eye. This phenomenon is attributed to increased uveoscleral outflow associated with a retinal break. The magnitude of the decrease is directly proportional to the size of the detachment.43 Schwartz syndrome refers to unilateral elevation of the IOP associated with retinal detachment.44
What Is the Mechanism of Glaucoma
in Schwartz Syndrome?
A retinal break allows communication between the subretinal space and the anterior chamber. Photoreceptor outer segments with few inflammatory cells obstruct the trabecular meshwork to cause open-angle glaucoma.45
Epidemiology and Importance
How Common Is Glaucoma Associated with Retinal Detachment?
Phelps and Burton46 examined 817 cases of retinal detachment and found that open-angle glaucoma was present in 4%, and an additional 6.5% had elevated IOP without glaucomatous damage. They also discovered that that 2.1% of 817 patients with retinal detachment had features consistent with Schwartz syndrome.46
Diagnosis and Differential Diagnosis
How Is Schwartz Syndrome Diagnosed?
Features of Schwartz syndrome include unilateral elevation of IOP, rhegmatogenous retinal detachment, iridocyclitis, and normalization of the
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IOP following retinal detachment repair.47 The probable etiology of this condition is obstruction of the trabecular meshwork with photoreceptor outer segments.45
Treatment and Management
How Is Glaucoma Associated with Schwartz Syndrome Treated?
The management of this condition is aqueous suppression and repair of the retinal detachment. In the majority of cases, glaucoma medications may be discontinued within a few weeks of reattachment of the retina.48
Future Considerations
How Have Recent Advancements Contributed to the Treatment of Schwartz Syndrome?
With the advancement of surgical techniques for retinal detachment repair, the reattachment rates for chronic retinal detachments have improved dramatically. In addition, with the advent of vitrectomy for retinal detachment repair, the majority of diffused photoreceptor elements may be removed from the eye at the time of the operation.
CILIOCHOROIDAL EFFUSION (UVEAL EFFUSION SYNDROME)
Definition
How Is Glaucoma Associated
with Ciliochoroidal Effusion Defined?
Angle-closure glaucoma is a known complication of ciliochoroidal effusion. These eyes are nanophthalmic and hyperopic due to a short axial length. The sclera in these eyes are thicker than normal and contain unusually disordered collagen fibrils.49 Uveal effusion may result from either a reduced scleral permeability to proteins or vortex vein compression. Uveal effusion causes forward rotation of the lens-iris diaphragm, inducing angle-closure glaucoma.
Epidemiology and Importance
How Common Is Glaucoma Associated with Uveal Effusion Syndrome?
This syndrome usually occurs in patients with small, hyperopic eyes in the fourth to sixth decades of life. This is a rare condition.
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Diagnosis and Differential Diagnosis
of the Problem
How Is Glaucoma in Association
with Uveal Effusion Syndrome Diagnosed?
In the setting of an elevated IOP, shallow anterior chamber, and narrow angle in a small, hyperopic eye, one should consider angle closure due to ciliochoroidal effusion. These eyes are highly hyperopic due to their short axial length. One should perform a fundus exam to look for a choroidal detachment. An exudative retinal detachment may be observed.
Treatment and Management
The management of glaucoma with uveal effusion syndrome is complex. Angle-closure glaucoma may be treated topically with cycloplegics, aqueous suppressants, and corticosteroids.50 Miotics may improve some cases but worsen others.51 Laser iridotomy and peripheral iridoplasty may be tried to eliminate any component of pupillary block. The choroidal effusion is treated with drainage sclerotomies in abnormally thickened sclera.52,53
Future Considerations
Future studies may better define the surgical management of these patients. Better diagnostic instruments such as biomicroscopic ultrasonography may help when the diagnosis is not clear.
VITREOUS HEMORRHAGE
Definition
How Is Glaucoma Associated
with Vitreous Hemorrhage Defined?
Vitreous hemorrhage may cause glaucoma by three mechanisms: ghost cell glaucoma, hemolytic glaucoma, and hemosiderotic glaucoma.
What Is Ghost Cell Glaucoma?
Campbell et al54 described a form of glaucoma in which degenerated red blood cells (ghost cells) develop in the vitreous cavity and subsequently enter the anterior chamber where they temporarily obstruct aqueous outflow.
What Is the Mechanism of Ghost Cell Glaucoma?
When red blood cells are present in the vitreous cavity by various mechanisms (trauma, surgery, diabetes, etc.) they degenerate into ghost cells in a matter of weeks. Ghost cells are tanor khaki-colored, spherical, less pliable structures
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that have thin walls and appear hollow except for clumps of denatured hemoglobin, called Heinz bodies. These ghost cells do not readily pass through the trabecular meshwork. Thus, once they gain access into the anterior chamber via a disrupted anterior hyaloid face, they accumulate in the trabecular meshwork and cause elevation of IOP.
What Is Hemolytic Glaucoma?
Hemolytic glaucoma is a condition in which the trabecular meshwork is blocked by hemoglobin-laden macrophages.
What Is Hemosiderotic Glaucoma?
Hemosiderotic glaucoma is a rare type of glaucoma associated with a longstanding vitreous hemorrhage that may lead to trabecular meshwork damage secondary to iron accumulation.
What Is the Etiology of Vitreous Hemorrhage?
The most common causes of spontaneous vitreous hemorrhage are diabetic retinopathy, and retinal break with or without detachment, and retinal vein occlusion.55,56 Trauma and surgery are commonly associated with vitreous hemorrhage.
Epidemiology and Importance
What Is the Incidence of Glaucoma Associated with Vitreous Hemorrhage?
The incidence of spontaneous vitreous hemorrhage is approximately 7 cases per 100,000 population.57 The incidence of glaucoma associated with vitreous hemorrhage is not known.
Diagnosis and Differential Diagnosis
How Is Ghost Cell Glaucoma Diagnosed?
Patients with ghost cell glaucoma present with pain, perilimbal injection, and an elevated pressure with a history of a vitreous hemorrhage. There are ghost cells visible in the anterior chamber. Ghost cells are spherical, khaki-colored cell walls of the red blood cell. They are produced 1 to 2 weeks following a vitreous hemorrhage when the red blood cells degenerate. Ghost cells clog the trabecular meshwork because they are less rigid than the normal red blood cell. An anterior chamber paracentesis may be performed to examine the fluid for ghost cells if the diagnosis is uncertain.
How Is Hemolytic Glaucoma Diagnosed?
Hemolytic glaucoma is a condition in which the trabecular meshwork is blocked by hemoglobin-laden macrophages. Because the breakdown of the red