(See also Overview of Glaucoma Overview of Glaucoma Glaucomas are a group of eye disorders characterized by progressive optic nerve damage in which an important part is a relative increase in intraocular pressure (IOP) that can lead to irreversible... read more .)
Angle-closure glaucoma accounts for about 10% of all glaucomas in the US.
Etiology of Angle-Closure Glaucoma
Angle-closure glaucoma is caused by factors that either pull or push the iris up into the angle (ie, junction of the iris and cornea at the periphery of the anterior chamber), physically blocking drainage of aqueous and raising intraocular pressure (IOP) (see table Open-Angle Glaucoma Primary Open-Angle Glaucoma Primary open-angle glaucoma is a syndrome of optic nerve damage associated with an open anterior chamber angle and an elevated or sometimes average intraocular pressure (IOP). Symptoms are a... read more ). Elevated IOP damages the optic nerve.
Pathophysiology of Angle-Closure Glaucoma
Angle closure may be primary (cause is unknown) or secondary to another condition (see table Open-Angle Glaucoma Primary Open-Angle Glaucoma Primary open-angle glaucoma is a syndrome of optic nerve damage associated with an open anterior chamber angle and an elevated or sometimes average intraocular pressure (IOP). Symptoms are a... read more ) and can be acute, subacute (intermittent), or chronic.
Primary angle-closure glaucoma
Narrow angles are not present in young people. As people age, the lens of the eye continues to grow. In some but not all people, this growth pushes the iris forward, narrowing the angle. Risk factors for developing narrow angles include family history, advanced age, and ethnicity; risk is higher among people of Asian and Inuit ethnicity and lower among people of European and African ethnicities.
In people with narrow angles, the distance between the iris at the pupil and the lens is also very narrow. When the iris dilates, forces pull the iris centripetally and posteriorly causing increasing iris–lens contact, which prevents aqueous from passing between the lens and iris, through the pupil, and into the anterior chamber (this mechanism is termed pupillary block). Pressure from the continued secretion of aqueous into the posterior chamber by the ciliary body pushes the peripheral iris anteriorly (causing a forward-bowing iris called iris bombe), closing the angle. This closure blocks aqueous outflow, resulting in rapid (within hours) and severe (> 40 mm Hg) elevation of intraocular pressure (IOP).
Because of the rapid onset, this condition is called primary acute angle-closure glaucoma and is an ophthalmic emergency requiring immediate treatment. Non-pupillary block mechanisms include plateau iris syndrome in which the central anterior chamber is deep, but the peripheral anterior chamber is made shallow by a ciliary body that is displaced forward.
Intermittent angle-closure glaucoma occurs if the episode of pupillary block resolves spontaneously after several hours, usually after sleeping supine.
Chronic angle-closure glaucoma occurs if the angle narrows slowly, allowing scarring between the peripheral iris and trabecular meshwork; IOP elevation is slow.
Pupillary dilation (mydriasis) can push the iris into the angle and precipitate acute angle-closure glaucoma in any person with narrow angles. This development is of particular concern when applying topical agents to dilate the eye for examination (eg, cyclopentolate, phenylephrine) or for treatment (eg, homatropine) or when giving systemic drugs that have the potential to dilate the pupils (eg, scopolamine, alpha-adrenergic agonists commonly used to treat urinary incontinence, drugs with anticholinergic effects).
Secondary angle-closure glaucomas
The mechanical obstruction of the angle is due to a coexisting condition, such as proliferative diabetic retinopathy Diabetic Retinopathy In patients with diabetes mellitus, years of poorly controlled hyperglycemia lead to multiple, primarily vascular, complications that affect small vessels (microvascular), large vessels (macrovascular)... read more (PDR), ischemic central vein occlusion Central Retinal Vein Occlusion and Branch Retinal Vein Occlusion Central retinal vein occlusion is a blockage of the central retinal vein by a thrombus. It causes painless vision loss, ranging from mild to severe, and usually occurs suddenly. Diagnosis is... read more , uveitis Overview of Uveitis Uveitis is defined as inflammation of the uveal tract—the iris, ciliary body, and choroid. However, the retina and fluid within the anterior chamber and vitreous are often involved as well.... read more , or epithelial down-growth. Contraction of a neovascular membrane (eg, in PDR) or inflammatory scarring can pull the iris into the angle.
Symptoms and Signs of Angle-Closure Glaucoma
Acute angle-closure glaucoma
Patients have severe ocular pain and redness, decreased vision, colored halos around lights, headache, nausea, and vomiting. The systemic complaints may be so severe that patients are misdiagnosed as having a neurologic or gastrointestinal problem. Examination typically reveals conjunctival hyperemia, a hazy cornea, a fixed mid-dilated pupil, and anterior chamber inflammation. Vision is decreased. Intraocular pressure (IOP) measurement is usually 40 to 80 mm Hg. The optic nerve is difficult to visualize because of corneal edema, and visual field testing is not done because of discomfort. For primary mechanisms of angle-closure (eg, pupillary block and plateau iris), examination of the uninvolved contralateral eye can indicate the diagnosis.
Pearls & Pitfalls
Chronic angle-closure glaucoma
This type of glaucoma manifests similarly to open-angle glaucoma Primary Open-Angle Glaucoma Primary open-angle glaucoma is a syndrome of optic nerve damage associated with an open anterior chamber angle and an elevated or sometimes average intraocular pressure (IOP). Symptoms are a... read more . Some patients have ocular redness, discomfort, blurred vision, or headache that lessens with sleep (perhaps because of sleep-induced miosis and posterior displacement of the lens by gravity). On gonioscopy, the angle is narrow, and peripheral anterior synechiae (adhesions between the peripheral iris and angle structure causing blockage of trabecular meshwork and/or ciliary body face), also called peripheral anterior synechiae (PAS), may be seen. IOP may be normal but is usually higher in the affected eye.
Diagnosis of Angle-Closure Glaucoma
Acute: Measurement of intraocular pressure (IOP) and clinical findings
Chronic: Gonioscopy showing peripheral anterior synechiae and characteristic optic nerve and visual field abnormalities
Diagnosis of acute angle-closure glaucoma is clinical and by measurement of IOP. Gonioscopy may be difficult to do in the involved eye because of a clouded cornea with friable corneal epithelium. However, examination of the other eye reveals a narrow or occludable angle. If the other eye has a wide angle, a diagnosis other than primary angle-closure glaucoma should be considered.
Diagnosis of chronic angle-closure glaucoma is based on the presence of peripheral anterior synechiae on gonioscopy and characteristic optic nerve and visual field changes (see symptoms and signs of primary open-angle glaucoma Symptoms and Signs Primary open-angle glaucoma is a syndrome of optic nerve damage associated with an open anterior chamber angle and an elevated or sometimes average intraocular pressure (IOP). Symptoms are a... read more ).
Treatment of Angle-Closure Glaucoma
Acute: Timolol, pilocarpine, and brimonidine drops, oral acetazolamide, and a systemic osmotic drug, followed by laser peripheral iridotomy
Chronic: Similar to primary open-angle glaucoma except that laser peripheral iridotomy should be done if the ophthalmologist feels that the procedure may slow mechanical closing of the angle. Cataract Cataract A cataract is a congenital or degenerative opacity of the lens. The main symptom is gradual, painless vision blurring. Diagnosis is by ophthalmoscopy and slit-lamp examination. Treatment is... read more removal helps delay the progression of chronic angle-closure glaucoma.
Acute angle-closure glaucoma
Treatment must be initiated immediately because vision can be lost quickly and permanently. The patient should receive several drugs at once. A suggested regimen is timolol 0.5% one drop every 30 minutes for 2 doses, pilocarpine 2 to 4% one drop every 15 minutes for 2 doses, brimonidine (0.15 or 0.2%) one drop every 15 minutes for 2 doses, acetazolamide 500 mg orally initially (IV if patients are nauseated) followed by 250 mg every 6 hours, and an osmotic agent, such as oral glycerol 1 mL/kg diluted with an equal amount of cold water, mannitol 1.0 to 1.5 mg/kg IV, or isosorbide 100 g orally (220 mL of a 45% solution). (NOTE: This form of isosorbide is not isosorbide dinitrate.) Response is evaluated by measuring intraocular pressure (IOP). Miotics (eg, pilocarpine) are generally not effective when IOP is > 40 or 50 mm Hg because of an anoxic pupillary sphincter.
Definitive treatment is with laser peripheral iridotomy (LPI), which opens another pathway for fluid to pass from the posterior to the anterior chamber, breaking the pupillary block. It is done as soon as the cornea is clear and inflammation has subsided. In some cases the cornea clears within hours of lowering the IOP; in other cases, it can take 1 to 2 days. Because the chance of having an acute attack in the other eye is 80%, LPI is done on both eyes.
The risk of complications with LPI is extremely low compared with its benefits. Glare, which can be bothersome, may occur.
Chronic angle-closure glaucoma
Patients with chronic, subacute, or intermittent angle-closure glaucoma should also have LPI. Additionally, patients with a narrow angle, even in the absence of symptoms, should undergo prompt LPI to prevent angle-closure glaucoma. If a cataract is present, cataract removal can dramatically delay the progression of chronic angle-closure glaucoma.
The drug and surgical treatments are the same as with open-angle glaucoma. Laser trabeculoplasty is relatively contraindicated if the angle is so narrow that additional peripheral anterior synechiae may form after the laser procedure. Typically, partial-thickness procedures are not indicated.
Angle-closure glaucoma can develop acutely, intermittently, or chronically.
Suspect acute angle-closure glaucoma based on clinical findings and confirm it by measuring intraocular pressure.
Confirm chronic angle-closure glaucoma by peripheral anterior synechiae and optic nerve and visual field changes.
Treat acute angle-closure glaucoma as an emergency.
Consult an ophthalmologist to arrange laser peripheral iridotomy for all patients with angle-closure glaucoma.