Pseudoexfoliation Syndrome/Exfoliative Glaucoma

Definition

A systemic disorder in which grayish-white exfoliation material, along with pigment released from the iris sphincter region, block the TM and raise the IOP. Diagnosis confers a 25% risk of glaucoma, which can be poorly responsive to therapy. Pseudoexfoliation is the most common secondary glaucoma in those of European descent, but can be seen in nearly all ethnic groups.

Symptoms ⬆ ⬇

Usually asymptomatic in its early stages. Unlike POAG, more often asymmetric/unilateral at presentation.

Signs ⬆ ⬇

(See Figures 9.11.1 and 9.11.2.)

Critical

White, flaky material on the pupillary margin; anterior lens capsular changes (central zone of exfoliation material, often with rolled-up edges, middle clear zone, and a peripheral cloudy zone); peripupillary iris TIDs; and glaucomatous optic neuropathy. Bilateral, but often asymmetric.

Other

Irregular black pigment deposition on the TM more marked inferiorly than superiorly; black scalloped deposition of pigment anterior to Schwalbe line (Sampaolesi line) seen on gonioscopy, especially inferiorly. White, flaky material may be seen on the corneal endothelium, which often has a lower than normal endothelial cell density; can look like angular, irregular KP. Iris atrophy. Poor pupillary response to dilation (with more advanced cases, believed to be secondary to iris dilator muscle atrophy). Incidence increases with age. Zonular laxity can lead to anterior lens dislocation, angle narrowing, and secondary angle closure.

9-11.2 Pseudoexfoliation syndrome with white material on anterior lens capsule.

9-11.1 Pseudoexfoliation syndrome with white material on pupillary margin.

Differential Diagnosis ⬆ ⬇

Inflammatory glaucoma: Corneal endothelial deposits can be present in both exfoliative and uveitic glaucoma. Typically, IOP is highly volatile in both. The ragged volcano-like PAS of some inflammatory glaucomas are not seen in the exfoliation syndrome, but angle closure due to zonular instability can occur. Photophobia is common with uveitis. See 9.7, INFLAMMATORY OPEN-ANGLE GLAUCOMA.
Pigmentary glaucoma: Midperipheral iris TIDs. Pigment on corneal endothelium and posterior equatorial lens surface. Deep anterior chamber angle. Myopia. See 9.10, PIGMENT DISPERSION SYNDROME/PIGMENTARY GLAUCOMA.
Capsular delamination (true exfoliation): Trauma, exposure to intense heat (e.g., glass blower), or severe uveitis can cause a thin membrane to peel off the anterior lens capsule. Glaucoma uncommon.
Primary amyloidosis: Amyloid material can deposit along the pupillary margin or anterior lens capsule. Glaucoma can occur.
Uveitis/glaucoma/hyphema (UGH) syndrome: Prior surgery. See 9.16.3, UVEITIS, GLAUCOMA, HYPHEMA SYNDROME.

Work Up ⬆ ⬇

Workup

History: Family history.
Slit lamp examination. Look for white, flaky material along the pupillary margin, peripapillary TIDs; often need to dilate the pupil to see anterior lens capsular changes.
Perform baseline glaucoma evaluation. See 9.1, PRIMARY OPEN-ANGLE GLAUCOMA.

Treatment ⬆ ⬇

For medical and surgical therapy, see 9.1, PRIMARY OPEN-ANGLE GLAUCOMA. LT can be particularly effective, possibly related to higher laser uptake due to pigmentation.
The course of exfoliative glaucoma is nonlinear. Early, the condition may be benign. However, pseudoexfoliation is associated with highly volatile IOP. Once IOP becomes difficult to control, the glaucoma may progress rapidly (e.g., within months).

NOTE:

Cataract extraction does not eradicate the glaucoma. Cataract extraction may be complicated by weakened zonular fibers and synechiae between the iris and peripheral anterior lens capsule with increased risk of intraoperative vitreous loss and zonular dehiscence. Postoperative intraocular lens dislocation may occur with time.

Follow Up ⬆

Every 1 to 3 months as with POAG, but with the awareness that damage can progress very rapidly.