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Author: Kayoung Yi, MD, PhD, Assistant Professor of Ophthalmology, Hallym University, Kangnam Sacred Heart Hospital, Korea; Exchange Scholar, Research Fellow, Department of Ophthalmology, Glaucoma Service, Massachusetts Eye Ear Infirmary and Harvard Medical School

Kayoung Yi is a member of the following medical societies: American Society of Cataract and Refractive Surgery

Coauthor(s): Teresa C Chen, MD, FACS, Assistant Professor, Department of Ophthalmology, Harvard Medical School; Director of Clinical Affairs, Glaucoma Service, Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary

Editors: Richard W Allinson, MD, Associate Professor, Department of Ophthalmology, Texas A&M University Health Science Center, Scott and White Clinic; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Martin B Wax, MD, Clinical Professor, Department of Ophthalmology, University of Texas Southwestern Medical School; Vice President, Ophthalmology Research and Development, Head, Ophthalmology Discovery Research, Alcon Labs, Inc; Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri; Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Author and Editor Disclosure

Synonyms and related keywords: phacolytic glaucoma, PG, lens protein glaucoma, open-angle glaucoma, cataract extraction, lens-induced glaucoma, visual deficit, vision loss

Background

Phacolytic glaucoma (PG) is the sudden onset of open-angle glaucoma caused by a leaking mature or hypermature (rarely immature) cataract. It is cured by cataract extraction.

Pathophysiology

In contrast to some forms of lens-induced glaucomas (eg, lens particle glaucoma, phacoanaphylactic glaucoma), PG occurs in cataractous lenses with intact lens capsules. The available evidence implicates direct obstruction of outflow pathways by lens protein released from microscopic defects in the lens capsule that is intact clinically. The high molecular weight proteins found in cataractous lenses produce outflow obstruction in experimental perfusion studies similar to that found in PG. Although a macrophagic response is typically present, macrophages are believed to be a natural response to lens protein in the anterior chamber rather than the cause of the outflow obstruction.

Frequency

United States

PG is infrequent in developed countries, such as the United States, because of greater access to health care and earlier cataract surgery.

International

PG occurs more frequently in underdeveloped countries.

Mortality/Morbidity

Most cases resolve after cataract extraction with excellent improvement in vision.

Race

No racial predilection exists.

Sex

No sexual predilection exists.

Age

PG typically occurs in older adults. The youngest patient reported was age 35 years.



History

  • Patients typically have a history of slow vision loss for months or years prior to the acute onset of pain, redness, and sometimes further decrease in vision.
  • Vision may only be inaccurate light perception due to the density of the cataract.
  • Symptoms mimic acute angle-closure glaucoma (see Glaucoma, Angle Closure, Acute).
  • The history of slow vision loss due to advancing cataract preceding the acute onset of symptoms is a vital clue to the correct diagnosis.

Physical

  • Intraocular pressure (IOP) characteristically is elevated severely.
  • Slit lamp examination typically reveals microcystic corneal edema, and the anterior chamber contains intense flare, large cells (macrophages), aggregates of white material, and iridescent or hyperrefringent particles. The latter represent calcium oxalate and cholesterol crystals being liberated from the degenerating cataractous lens. Unlike uveitic glaucoma (such as that seen in phacoanaphylactic glaucoma), no keratic precipitates typically are present.
  • The anterior capsule of the lens frequently is dotted with patches of soft white material. In contrast to some forms of lens-induced glaucomas (eg, lens particle glaucoma, phacoanaphylactic glaucoma), the lens capsule is grossly intact.
  • Gonioscopy findings usually are normal; however, evidence of old angle recession was found in 25% of eyes in one study.

Causes

  • Mature cataract (totally opacified)
  • Hypermature cataract (liquid cortex and free-floating nucleus)
  • Focal liquefaction of immature cataract (rare)
  • Dislocated cataractous lens in vitreous



Glaucoma, Angle Closure, Acute
Glaucoma, Lens-Particle
Glaucoma, Neovascular
Glaucoma, Phacomorphic
Glaucoma, Uveitic

Other Problems to be Considered

In addition to the above diagnoses, another lens-induced glaucoma, phacoanaphylactic glaucoma, may be confused with PG. Phacoanaphylactic glaucoma is a delayed-onset granulomatous reaction to retained lens material, such as that occurring after disruption of the lens capsule from trauma or because of incomplete removal of lens material during cataract extraction (including gross loss of lens fragments into the vitreous cavity during cataract extraction). Pain, if it occurs at all, is often slow in onset and is associated with signs of granulomatous uveitis (eg, keratic precipitates) in contrast to the acute, severe pain that is typical of PG, which shows no signs of chronic inflammation.

Traumatic glaucomas, including angle-recession glaucoma and ghost cell glaucoma, also may be confused with PG. A careful history and examination of the anterior chamber angle may reveal evidence of prior trauma. A history of long-standing vitreous hemorrhage should raise suspicion of ghost cell glaucoma.

Finally, in apparently aphakic eyes, the posterior segment must be inspected carefully for a dislocated lens that, when hypermature, may begin to leak and cause PG even when a cataract is not readily visible.



Histologic Findings

In questionable cases, a diagnostic paracentesis should show characteristic swollen macrophages with engulfed lens material on phase contrast microscopy.



Medical Care

  • Initial treatment is focused upon acute lowering of IOP using a combination of topical and systemic IOP-lowering agents. Topical steroids also may facilitate IOP lowering and decrease pain.
  • Medical therapy is only a temporizing measure until cataract surgery can be scheduled.

Surgical Care

  • The definitive treatment of PG is cataract extraction. Extracapsular cataract extraction (eg, phacoemulsification) with an intraocular lens implant has largely replaced intracapsular cataract extraction as the procedure of choice.
  • If PG is caused by a lens that has dislocated into the vitreous cavity, the procedure of choice is pars plana vitrectomy with removal of the lens from within the vitreous cavity.

Diet

Instruct patients not to eat or drink if emergency cataract extraction is being considered.



Several applications of a topical beta-blocker, a topical alpha2-adrenergic, a topical carbonic anhydrase inhibitor, and a topical corticosteroid should be started in the office on presentation when possible. The IOP should be remeasured in 30 minutes to 1 hour. If the IOP is severely elevated or is nonresponsive to initial topical medications, a systemic carbonic anhydrase inhibitor and an osmotic agent also should be administered. The latter medications may be administered intravenously if the patient is nauseated or vomiting. Prostaglandin analogs (eg, Xalatan, Rescula, Lumigan, Travatan) may not be as useful in the treatment of PG because of their slow onset of action and their theoretical risk of exacerbating intraocular inflammation. The adequacy of initial response to medical therapy helps to determine the urgency of scheduling cataract extraction.

Drug Category: Beta-blockers

The exact mechanism of ocular antihypertensive action is not established, but it appears to be a reduction of aqueous humor production.

Drug NameTimolol maleate or hemihydrate (Timoptic XE, Timoptic, Betimol)
DescriptionMay reduce elevated and normal IOP, with or without glaucoma, by inhibiting inflow.
Adult Dose1 gtt of 0.25% or 0.5% in affected eye(s) qd/bid; if IOP is still not at satisfactory level, consider concomitant therapy
Pediatric DoseAdminister as in adults; try 0.25% first
ContraindicationsDocumented hypersensitivity; bronchial asthma; sinus bradycardia; second- and third-degree AV block; severe chronic obstructive pulmonary disease; overt cardiac failure; cardiogenic shock
InteractionsMay exacerbate bradycardia and asystole when used in combination with systemic beta-blockers (may cause additive effects)
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsProduct may have sulfites, which may cause allergic-type reactions in susceptible patients; may exacerbate or precipitate heart block, asthma, chronic obstructive pulmonary disease, and mental changes (especially in elderly patients)

Drug NameLevobunolol (AKBeta, Betagan)
DescriptionNonselective beta-adrenergic blocking agent that lowers IOP by reducing aqueous humor production
Adult Dose0.5% solution: 1 gtt in affected eye(s) qd
0.25% solution: 1 gtt in affected eye(s) bid
Severe or uncontrolled glaucoma: 0.5% solution bid; closely monitor patient; > 1 gtt (0.5% levobunolol) bid not shown to be more effective; if IOP not at satisfactory level on this regimen, concomitant therapy can be instituted; do not administer 2 or more topical ophthalmic beta-adrenergic blocking agents simultaneously
Pediatric DoseNot established
ContraindicationsDocumented hypersensitivity; bronchial asthma; severe chronic obstructive pulmonary disease; sinus bradycardia; second- and third-degree AV block; overt cardiac failure; cardiogenic shock
InteractionsMay exacerbate bradycardia and asystole when used in combination with systemic beta-blockers (may cause additive effects)
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsBeta-blockade may potentiate muscle weakness that is consistent with certain myasthenic symptoms (eg, diplopia, ptosis, generalized weakness); product may have sulfites, which may cause allergic-type reactions in certain susceptible persons

Drug NameCarteolol (Ocupress)
DescriptionBlocks beta1- and beta2-receptors and has mild intrinsic sympathomimetic effects.
Adult Dose1 gtt in affected eye bid
Pediatric DoseAdminister as in adults
ContraindicationsDocumented hypersensitivity; congestive heart failure; asthma; cardiac conduction defects; breastfeeding
InteractionsMay exacerbate bradycardia and asystole when used in combination with systemic beta-blockers (may cause additive effects)
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsProduct may have sulfites, which may cause allergic-type reactions in certain susceptible persons

Drug NameBetaxolol (Betoptic)
DescriptionSelectively blocks beta1-adrenergic receptors with little or no effect on beta2-receptors. Reduces IOP by reducing production of aqueous humor.
Adult Dose1 gtt in affected eye(s) bid; consider concomitant therapy if IOP is not at satisfactory level
Pediatric DoseNot established
ContraindicationsDocumented hypersensitivity; bronchial asthma; severe chronic obstructive pulmonary disease; sinus bradycardia; second- and third-degree AV block; overt cardiac failure; cardiogenic shock
InteractionsMay have additive systemic effects if patient is already on systemic beta-blockers
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsBeta-blockade may potentiate muscle weakness consistent with myasthenic symptoms; product may have sulfites, which may cause hypersensitivity reactions in susceptible persons

Drug NameMetipranolol hydrochloride (OptiPranolol)
DescriptionBeta-adrenergic blocker that has little or no intrinsic sympathomimetic effects and membrane-stabilizing activity. Has little local anesthetic activity. Reduces IOP by reducing production of aqueous humor.
Adult Dose1 gtt in affected eye(s) bid
Pediatric DoseNot established
ContraindicationsDocumented hypersensitivity; sinus tachycardia; cardiac failure; cardiogenic shock; second- and third-degree AV block
InteractionsMay exacerbate bradycardia and asystole when used in combination with systemic beta-blockers (may cause additive effects)
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsCaution in diabetes mellitus, bradycardia, asthma, cardiac failure, and AV block

Drug Category: Topical alpha2-adrenergics agonists

May reduce elevated and normal IOP, with or without glaucoma, by inhibiting inflow.

Drug NameApraclonidine (Iopidine)
DescriptionReduces elevated and normal IOP whether or not accompanied by glaucoma. Apraclonidine is a relatively selective alpha-adrenergic agonist that does not have significant local anesthetic activity. Has minimal cardiovascular effects.
Adult DoseSolution (0.5%): 1 gtt in affected eye(s) bid/tid; since apraclonidine 0.5% will be used with other ocular glaucoma therapies, use an approximate 5-min interval between instillation of each medication to prevent washout of the previous dose; this treatment is not for injection into eye
Pediatric DoseNot established
ContraindicationsDocumented hypersensitivity; patients on MAOIs or who have taken them in the past 14 d
InteractionsMonitor pulse and BP frequently when giving cardiovascular drugs; not for use concurrently with MAOIs
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsMay exacerbate or precipitate ocular irritation, topical sensitivity, vasovagal attack, and optic nerve ischemia in patients with advanced glaucomatous optic neuropathy

Drug NameBrimonidine (Alphagan)
DescriptionSelective alpha2-receptor that reduces aqueous humor formation and may increase uveoscleral outflow.
Adult Dose1 gtt in affected eye bid/tid
Pediatric DoseNot established
ContraindicationsDocumented hypersensitivity; patients receiving MAOIs
InteractionsCoadministration with topical beta-blockers may further decrease IOP; tricyclic antidepressants may decrease effects of brimonidine; CNS depressants, such as barbiturates, opiates, and sedatives, may potentiate effects of brimonidine
PregnancyB - Usually safe but benefits must outweigh the risks.
PrecautionsMay exacerbate or precipitate ocular irritation, topical sensitivity, vasovagal attack, and optic nerve ischemia in patients with advanced glaucomatous optic neuropathy

Drug Category: Carbonic anhydrase inhibitors

By slowing the formation of bicarbonate ions with subsequent reduction in sodium and fluid transport, it may inhibit carbonic anhydrase in the ciliary processes of the eye. This effect decreases aqueous humor secretion, reducing IOP.

Drug NameDorzolamide (Trusopt)
DescriptionUsed concomitantly with other topical ophthalmic drug products to lower IOP. If more than one ophthalmic drug is being used, administer the drugs at least 5 min apart. Reversibly inhibits carbonic anhydrase, reducing hydrogen ion secretion at renal tubule and increasing renal excretion of sodium, potassium bicarbonate, and water to decrease production of aqueous humor.
Adult Dose1 gtt in affected eye(s) bid/tid
Pediatric DoseNot established
ContraindicationsDocumented hypersensitivity to drug or sulfonamides
InteractionsCoadministration with high-dose salicylate therapy may increase toxicity; may have additive systemic effects if patient is already on oral carbonic anhydrase inhibitors
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsLocal ocular adverse effects, primarily conjunctivitis and lid reactions, may occur with long-term administration of dorzolamide (discontinue therapy and evaluate patient before restarting therapy)

Drug NameBrinzolamide (Azopt)
DescriptionCatalyzes reversible reaction involving hydration of carbon dioxide and dehydration of carbonic acid. May use concomitantly with other topical ophthalmic drug products to lower IOP. If more than one topical ophthalmic drug is being used, administer drugs at least 5 min apart.
Adult Dose1 gtt in affected eye(s) bid/tid
Pediatric DoseNot established
ContraindicationsDocumented hypersensitivity to drug or sulfonamides
InteractionsMay have additive systemic effects if patient is already on oral carbonic anhydrase inhibitors
PregnancyB - Usually safe but benefits must outweigh the risks.
PrecautionsLocal ocular adverse effects, primarily conjunctivitis and lid reactions, may occur with long-term administration (discontinue therapy and evaluate patient before restarting therapy)

Drug NameAcetazolamide (Diamox, Diamox Sequels)
DescriptionInhibits enzyme carbonic anhydrase, reducing rate of aqueous humor formation, which, in turn, reduces IOP. Used for adjunctive treatment of chronic simple (open-angle) glaucoma and secondary glaucoma and preoperatively in acute angle-closure glaucoma when delay of surgery desired to lower IOP.
Adult DoseOpen angle: 250 mg PO 1-4 times/d or 500 mg SR cap bid
Closed angle: 250-500 mg IV/IM; may repeat in 2-4 h to a maximum 1 g/d
Pediatric Dose8-30 mg/kg/d or 300-900 mg/m2/d PO divided q8h
Alternatively, 20-40 mg/kg/d IV/IM divided q6h; not to exceed 1 g/d
ContraindicationsDocumented hypersensitivity to drug or sulfonamides; hepatic disease; severe renal disease; adrenocortical insufficiency; severe pulmonary obstruction
InteractionsCan decrease therapeutic levels of lithium and alter excretion of drugs (eg, amphetamines, quinidine, phenobarbital, salicylates) by alkalinizing urine
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsPatients with impaired hepatic function may go into coma; may cause substantial increase in blood glucose in some patients with diabetes

Drug NameMethazolamide (Neptazane)
DescriptionReduces aqueous humor formation by inhibiting enzyme carbonic anhydrase, which results in decreased IOP.
Adult Dose50-100 mg PO bid/tid
Pediatric DoseNot established
ContraindicationsDocumented hypersensitivity to drug or sulfonamides; renal impairment
InteractionsMay increase toxicity of salicylate, digoxin; coadministration with other diuretics may induce hypokalemia; decreases effects of lithium and alters excretion of other drugs by alkalinizing urine
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsCaution in respiratory acidosis and diabetes mellitus; impairs mental alertness and/or physical coordination; hematuria, glycosuria, polyuria, hepatic insufficiency, bone marrow suppression, thrombocytopenia/purpura, agranulocytosis, urticaria, pruritus, and rash may occur

Drug Category: Hyperosmotic agents

Create an osmotic gradient between ocular fluids and plasma. Not for long-term use.

Drug NameIsosorbide (Ismotic)
DescriptionMay be used to abort an acute attack of glaucoma. In the eyes, it may create an osmotic gradient between the plasma and ocular fluids and induce diuresis by elevating the osmolarity of the glomerular filtrate. These effects may, in turn, inhibit tubular reabsorption of water. Preferred when less risk of nausea and vomiting than that posed by other oral hyperosmotic agents is desired.
Adult DoseInitial dose: 1.5 g/kg PO
Dose range: 1-3 g/kg PO bid/qid as indicated
Pediatric DoseNot established
ContraindicationsDocumented hypersensitivity; anuria; severe dehydration; frank or impending acute pulmonary edema; severe cardiac decompensation
InteractionsNone reported
PregnancyB - Usually safe but benefits must outweigh the risks.
PrecautionsUse repetitive doses with caution, particularly in patients with diseases associated with salt retention

Drug NameGlycerin (Osmoglyn)
DescriptionUsed in glaucoma to interrupt acute attacks. Oral osmotic agent for reducing IOP. Able to increase tonicity of blood until finally metabolized and eliminated by the kidneys. Maximum reduction of IOP usually occurs 1 h after glycerin administration. Effect usually lasts approximately 5 h.
Adult Dose1-2 g/kg PO and repeat q5h prn
Alternatively, 1 mL/kg PO as a 50% solution in juice
Pediatric DoseAdminister as in adults
ContraindicationsDocumented hypersensitivity; frank or impending acute pulmonary edema; anuria; severe dehydration; severe cardiac decompensation
InteractionsNone reported
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsAdminister orally, never parenterally; for oral use only; avoid in acute urinary retention in preoperative period; continued use may result in weight gain; caution in hypervolemia, diabetes, individuals who are severely dehydrated, confused mental states, congestive heart disease, and cardiac, renal, or hepatic disease

Drug NameMannitol (Osmitrol)
DescriptionReduces elevated IOP when pressure cannot be lowered by other means. Initially assess for adequate renal function in adults by administering a test dose of 200 mg/kg, given IV over 3-5 min. Should produce a urine flow of at least 30-50 mL/h of urine over 2-3 h. In children, assess for adequate renal function by administering a test dose of 200 mg/kg, given IV over 3-5 minutes. Should produce a urine flow of at least 1 mL/h over 1-3 h.
Adult Dose1.5-2 g/kg IV as 20% solution (7.5-10 mL/kg) or as 15% solution (10-13 mL/kg) over a period as short as 30 min but preferably longer
Pediatric DoseNot established
ContraindicationsDocumented hypersensitivity; anuria; severe pulmonary congestion; progressive renal damage; severe dehydration; active intracranial bleeding; progressive heart failure
InteractionsMay decrease serum lithium levels
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsCarefully evaluate cardiovascular status before rapid administration of mannitol since a sudden increase in extracellular fluid may lead to fulminating CHF; avoid pseudoagglutination; when blood given simultaneously, add at least 20 mEq of sodium chloride to each liter of mannitol solution; do not give electrolyte-free mannitol solutions with blood

Drug Category: Corticosteroids

Reduce eye pain and intraocular inflammation.

Drug NamePrednisolone ophthalmic (Pred Forte)
DescriptionTreats acute inflammation following eye surgery or other types of insults to eye. Decreases inflammation and corneal neovascularization. Suppresses migration of polymorphonuclear leukocytes and reverses increased capillary permeability. In cases of bacterial infections, concomitant use of anti-infective agents is mandatory; if signs and symptoms do not improve after 2 days, reevaluate patient. Dosing may be reduced, but advise patients not to discontinue therapy prematurely.
Adult DoseSolution: 1 gtt into conjunctival sac q1h during day; once desired response obtained, use 1 gtt q4h; may reduce to 1 gtt tid/qid to control symptoms
Suspension: Shake well before using, and instill 1 gtt into conjunctival sac 2-4 times/d; if necessary, may increase dosing frequency during initial 24-48 h
Pediatric DoseAdminister as in adults
ContraindicationsDocumented hypersensitivity; viral, fungal, or tubercular infections
InteractionsNone reported
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsCaution in hypertension; known to cause cataract formation with long-term use; suspect fungal invasion in any persistent corneal ulceration where a corticosteroid has been used or is in use (obtain fungal cultures when appropriate)



Further Inpatient Care

  • Inpatient care is not usually necessary unless the patient is briefly hospitalized following emergency cataract extraction.

Further Outpatient Care

  • In most cases, IOP begins to rapidly normalize following cataract extraction, allowing discontinuation of ocular medications. A minority of patients will have persistent elevation of IOP requiring long-term medical therapy or filtering surgery to control the glaucoma.

Deterrence/Prevention

  • Removal of mature or hypermature cataracts

Complications

  • Loss of vision from uncontrolled glaucoma and/or persistent corneal edema
  • Surgical complications, including suprachoroidal hemorrhage, capsular rupture with loss of lens material into the posterior segment, corneal injury, and vitreous prolapse

Prognosis

  • Prognosis is excellent, with most patients experiencing marked improvement in vision following cataract extraction; however, delayed treatment may cause a poor outcome.
  • Patients with PG may have a worse prognosis than patients with phacomorphic glaucoma.

Patient Education



Medical/Legal Pitfalls

  • With a mature or hypermature cataract, the eye care specialist should emphasize the importance of cataract extraction prior to the development of glaucoma.



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Glaucoma, Phacolytic excerpt

Article Last Updated: Oct 16, 2006