BASICS

DESCRIPTION

Sudden onset of open-angle glaucoma secondary to leakage of soluble lens proteins from an intact mature cataractous lens.

EPIDEMIOLOGY

• More common in underdeveloped countries where patients with cataract tend to present late.

• Cataract either mature (white) or hypermature (liquid cortex and free-floating nucleus)

RISK FACTORS

Elderly patient with advanced cataract

GENERAL PREVENTION

Removal of cataract before it has advanced or liquefaction has occurred.

PATHOPHYSIOLOGY

• Macrophagic response to heavy molecular weight (HMW) lens proteins that have leaked from a hypermature cataract

• Obstruction of the trabecular meshwork with HMW proteins that have leaked from the mature cataract

• Macrophages distended with engulfed lens material are found in the trabecular meshwork; however, it is now largely believed that the HMW proteins are the cause of a high intraocular pressure (IOP). The macrophagic response is associated but not causative of the glaucoma.

ETIOLOGY

Mature cataract or hypermature cataract, which is leaking HMW lens protein through an intact capsule.

COMMONLY ASSOCIATED CONDITIONS

• Uveitis is associated with the leakage of lens protein.

• Open-angle glaucoma is related to the obstruction of the trabecular meshwork by HMW lens proteins.

DIAGNOSIS

HISTORY

• Hypermature cataract with elevated IOP and white material in the anterior chamber

• History of gradually diminishing vision over months or years and recent red, painful eye

PHYSICAL EXAM

• Usually unilateral

• Redness, pain

• Elevated IOP

• Diffuse corneal edema

• Intense cell and flare in the anterior chamber

• Iridescent or hyperrefringent particles in both lens and aqueous

• White material aggregates in the anterior chamber.

In the presence of a mature cataract

• Keratic precipitates are not seen.

• Fellow eye usually has a deep chamber and a mature cataract.

DIAGNOSTIC TESTS & INTERPRETATION

Lab

Initial lab tests

Aqueous aspiration for histologic examination

Imaging

Initial approach

• B-scan ultrasound to ensure that there is no other concomitant ocular pathology

• Aggressive control of inflammation and IOP

Follow-up & special considerations

Frequent follow-up until definitive treatment is implemented, which involves removal of the inciting cataract.

Diagnostic Procedures/Other

Needle aspirate of the anterior chamber fluid and examination of the sample for macrophages laden with eosinophilic lenticular material.

DIFFERENTIAL DIAGNOSIS

• Phacoanaphylactic glaucoma

• Lens particle glaucoma

• Exacerbation of prior uveitis

• Endophthalmitis

TREATMENT

MEDICATION

First Line

• Topical steroid therapy to reduce inflammation

• Antiglaucoma therapy to control IOP: with hyperosmotics, topical carbonic anhydrase inhibitors, topical beta-blockers, and alpha-2 agonists

ADDITIONAL TREATMENT

Issues for Referral

Retina consultation and referral may be necessary, if there is associated cystoid macular edema related to the uveitis.

Additional Therapies

• Once the cataract is removed, the uveitis is usually easier to control and steroid therapy should be tapered.

• IOP must be monitored and treatment instituted if open-angle glaucoma persists.

SURGERY/OTHER PROCEDURES

• Surgical removal of the inciting cataract, the source of the HMW proteins, and wash out of proteinaceous material from the anterior chamber

• Most often cataract removal is curative and no further surgery is needed.

• If the IOP remains elevated after surgical removal of the cataract and open-angle glaucoma is uncontrolled then glaucoma filtering surgery may be indicated.

IN-PATIENT CONSIDERATIONS

Initial Stabilization

Admission and intravenous mannitol may be necessary to control IOP until definitive surgery.

Discharge Criteria

Once the IOP is controlled, the patient may be discharged with close follow-up appointments.

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Very close follow-up during the initial postoperative period is warranted to manage the uveitis and IOP.

Patient Monitoring

• Once the IOP and uveitis are controlled lifelong IOP monitoring and treatment are necessary for open-angle glaucoma.

• 3–6-month intervals depending on the severity of disease

PATIENT EDUCATION

The physiology of glaucoma and the importance of adherence to medication should be discussed.

PROGNOSIS

• Eyes with poor vision, even light perception without projection, often obtain good vision after treatment.

• The prognosis depends on the prompt treatment of the uveitis and glaucoma.

COMPLICATIONS

• Vision loss secondary to glaucoma or uveitis, that is, cystoid macular edema

• Surgical complications of complex cataract surgery and/or glaucoma filtering surgery

ADDITIONAL READING

• Allingham RR, Damji KF, Freedman S, et al. Shields textbook of glaucoma, 5th Ed. Philadelphia: Lippincott Williams & Wilkins, 2004.

• Ellant JP, Obstbaum SA. Lens-induced glaucoma. Ophthalmology 1992;81:317–338.

• Epstein DL. Diagnosis and management of lens-induced glaucoma. Am Acad Ophthalmol 1982;89(3):227–230.

CODES

ICD9

• 364.3 Unspecified iridocyclitis

• 365.11 Open-angle glaucoma

• 365.51 Phacolytic glaucoma

CLINICAL PEARLS

Suspect in patients with mature, white, or liquefied cataract

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