Pseudoexfoliative Glaucoma

Pseudoexfoliative Glaucoma
SYMPTOMS Typically asymptomatic
In the later or more advanced stages, patients may report visual field loss such as scotomas or tunnel vision. If left untreated, complete blindness can occur
If IOP is too high (considered > 40mmHg), patient may experience Ocular pain, Pressure around the eye, Cloudy vision, Nausea, Vomiting, Headache, and Browache (these symptoms are exacerbated if patient develops a secondary angle closure)
SIGNS Typically unilateral but in 5 years, about 30% of cases eventually become bilateral
Pigment on the corneal endothelium, Pseudoexfoliative material on the corneal endothelium, Corneal edema, Pseudoexfoliative material on the pupillary ruff with loss of the pupillary ruff, Peripupillary transillumination defects, Iridodonesis (associated with lens subluxation), Miotic pupils with poor dilation, Mild uveitis (associated with breakdown of the blood-aqueous barrier), Posterior synechiae, Pseudoexfoliative material on the lens ("3 ring sign" or "bulls-eye pattern"), Lens subluxation (associated with weak zonules), Phacodonesis (associated with lens subluxation), Narrow angles on Van Herick, Shallow anterior chamber
In the presence of secondary angle closure, a Shallow anterior chamber, Bowed iris, Narrow angles on Van Herick, Fixed and mid-dilated pupil, Peripheral anterior synechiae, Circumlimbal injection, Corneal edema, Mild uveitis, and Iris atrophy may be present
Glaucomatous optic nerve damage (notching, rim tissue thinning, cupping, vessel shelving, vessel bearing, vessel bayonetting), RNFL thinning, Drance hemes, Peripapillary atrophy
With advanced glaucomatous optic nerve damage, Optic nerve pallor will be present
WORK-UP Pupils (typically presents with a decrease in light response or an APD if glaucoma is asymmetric/ fixed, mid-dilated pupil in the presence of secondary angle closure), Color vision (typically normal), Slit lamp examination, IOP with GAT or ORA (typically elevated especially in the presence of secondary angle closure where the IOP will typically be 40-80 mmHg), Dilated retinal exam, Post-dilated IOP with GAT or ORA (typically elevated compared to IOPs measured before dilation), Fundus photos, Visual field with Humphrey 24-2/30-2/24-2C threshold or 10-2 threshold in more advanced glaucoma (nasal step, arcuate defect, paracentral scotoma, tunnel vision with temporal crescent sparing), OCT analysis of the optic nerve/RNFL/GCL (RNFL and GCL thinning with GCL loss typically preceding RNFL loss), OCT-Angiography (decrease in density of the radial peripapillary capillary plexus and superficial capillary plexus), Anterior segment OCT (normal to narrow angles with a shallow anterior chamber and possible lens subluxation/ occluded angles with a shallow anterior chamber, peripheral anterior synechiae, and forward subluxation of the lens in the presence of secondary angle closure glaucoma), Gonioscopy (normal to narrow angles with an irregular, heavily pigmented trabecular meshwork and Sampaolesi line especially in the inferior angle as well as a bowed iris approach/ occluded angles with associated peripheral anterior synechiae in the presence of secondary angle closure glaucoma), Pachymetry (thin corneas carry a higher risk of glaucoma but patients with pseudoexfoliative syndrome may present with thicker corneas due to increased risk of corneal edema), Corneal hysteresis (low hysteresis carries a higher risk of glaucoma), VEP/Pattern ERG (decrease in ganglion cell function), Ultrasound biomicroscopy (normal to narrow angles with a shallow anterior chamber and possible lens subluxation/ occluded angles with a shallow anterior chamber, peripheral anterior synechiae, and forward subluxation of the lens in the presence of secondary angle closure glaucoma)
TREATMENT Pseudoexfoliative syndrome with normal IOPs and no evidence of glaucomatous optic nerve damage: No treatment needed
Pseudoexfoliative syndrome with elevated IOPs but no evidence of glaucomatous optic nerve damage or pseudoexfoliative syndrome with secondary open angle glaucoma: Begin treatment
First-line of treatment would typically be a combo drop which includes Cosopt 1 gtt BID, Combigan 1 gtt BID, or Simbrinza 1 gtt TID due to the aggressive nature of pseudoexfoliative glaucoma
If adjunct drop needed, consider Rhopressa which is to be used 1 gtt QHS
Topical carbonic anhydrase inhibitors to be used 1 gtt BID. alpha-2-agonists to be used 1 gtt BID, and/or topical beta blockers to be used 1 gtt once a day in the morning up to BID (the evening dose is thought to be minimally effective) can be considered as adjunct treatment (as long as class of medication is not currently being used in the first-line combo glaucoma drop)
Topical carbonic anhydrase inhibitors and alpha-2-agonists are to be used 1 gtt TID if utilized as stand-alone therapy
Topical apraclonidine and oral acetazolamide can be given in-office or on a short-term basis to quickly lower IOPs
Prostaglandins, pilocarpine, and Rocklatan (due to prostaglandin component) should be avoided due to high risk of uveitis, posterior synechiae, and miotic pupils in these patients
Always keep in mind the contraindications and side effects before prescribing any glaucoma medications
SLT may be used as a first-line of treatment in lieu of glaucoma drops or as an adjunct treatment along with glaucoma drops
LPI is an option for treatment as patients with pseudoexfoliative syndrome tend to have narrow angles with shallow anterior chambers (need to use caution as LPIs increase risk of zonular dehiscence in patients with pseudoexfoliative syndrome)
If using more than 2 different types of glaucoma drops in order to manage patient, co-management with a glaucoma specialist should be considered
If using more than 3 different types of glaucoma drops, refer to glaucoma specialist for co-management
Patients with advanced, severe, or end stage glaucoma should be co-managed with a glaucoma specialist as further intervention such as blebs, MIGs, or shunts may be needed (there is an increased risk of complications with glaucoma surgeries in patients with pseudoexfoliative syndrome)
Pseudoexfoliative syndrome with secondary angle closure with or without glaucoma: The goal is to lower the IOP as quickly as possible in office
Combigan (may use a separate topical beta blocker and topical alpha-2-agonist)
Cosopt (may use a separate topical beta blocker and topical carbonic anhydrase inhibitor)
Apraclonidine 1%
Pred Forte
Diamox 250mg (2 tablets given in one dose)
Prostaglandins and pilocarpine should be avoided with this type of secondary angle closure
Always keep in mind the contraindications and side effects before prescribing any glaucoma medications
IOP should be checked every 15 minutes for 1 hour while treating in-office
Once IOP is in better control, refer to ophthalmology ASAP for further evaluation and treatment which usually includes a laser peripheral iridotomy and/or cataract surgery
FOLLOW-UP Pseudoexfoliative syndrome with normal IOPs and no evidence of glaucomatous optic nerve damage: Monitor every 6 months
Pseudoexfoliative open angle glaucoma or pseudoexfoliative syndrome with elevated IOPs and no evidence of glaucomatous optic nerve damage: Patients should be seen back in 2-4 weeks after initiation of treatment in order to assess IOP. After the initial assessment of IOP with treatment, patients are typically seen back every 3-4 months for glaucoma testing in order to look for progression
Pseudoexfoliative syndrome with secondary angle closure with or without glaucoma: After seeing and being treated by ophthalmologist, monitor every 3-4 months. The patient will need to continue topical glaucoma drops
ADDITIONAL LAB | TESTS Since pseudoexfoliative syndrome is a systemic disease, patients should be referred to their PCP for additional testing which includes a cardiovascular work-up and check of homocysteine levels
ETIOLOGY Polymorphism in LOXL1 causes build-up of abnormal fibrillin and elastin (pseudoexfoliation material), which disrupts and alters function of structures it attaches to as it spreads throughout the eye and body. Pseudoexfoliative material in the trabecular meshwork leads to loss of porosity and dysfunction of the trabecular meshwork with subsequent increase in IOP
Subluxation of the lens anteriorly due to zonular weakness leads to an increase in lens-iris contact which subsequently could lead to secondary angle closure from pupillary block
DIFFERENTIAL DX Primary open angle glaucoma, Pigment dispersion glaucoma, Primary angle closure glaucoma, True exfoliation syndrome
NOTES Pseudoexfoliative syndrome is the most common cause of a unilateral glaucoma
The majority of patients with pseudoexfoliative syndrome will eventually develop glaucoma in less than 10 years
The most important indicator for development of glaucoma in patients with pseudoexfoliative syndrome is the amount of pigment and pseudoexfoliative material in the trabecular meshwork and schlemm’s canal
Pseudoexfoliative glaucoma has a poorer prognosis and is much more difficult to treat than primary open angle glaucoma
Patients with pseudoexfoliative syndrome have a higher risk of complications during cataract surgery
Pseudoexfoliative Glaucoma: "Bulls-eye pattern" or "3 ring sign" seen on the lens of a patient with pseudoexfoliative syndrome https://imagebank.asrs.org/file/25759/pseudoexfoliation-syndrome-ring
Posted in Glaucoma, Ocular Conditions