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I. Pseudoexfoliation/Exfoliation Syndrome (XFS) A. Demographics 1. Most common identifiable formof POAG worldwide, 20-25% of POAG worldwide a) Not just Sweden: >50% of POAG in Norway, Ireland, Greece, Saudi Arabia. Strong populations in Africa, Nepal, Native Americans 2. Common comorbidities include MI, TIA, stroke, dementia, hearing loss but there is no evidence of earlier mortality in XFS 3. 2/3 are clinically unilateral, disc changes more often observed in XFS eye even if IOP equal 4. Pigmentation of TM predicts damage 5. Autosomal dominant inheritance but with late expression and incomplete penetrance, also found in young patients after surgery B. Defining features 1. Presence of XFS imparts 5-10x risk of glaucoma 2. Fibrillar matrix either overproduced or undermetabolized, leading to accumulation in TM and increased outflow resistance 3. Ocular signs: white deposits on lens surface (central disc, intermediate clear zone, peripheral granular zone), deposits on pupil border, pigment loss from pupillary ruff/iris sphincter, pigment dispersion/IOP increase with dilation, poor pupillary dilation, early cataract formation, conjunctival and eyelid skin biopsy revealing exfoliation fibers on TEM, vessel dropout in iris, iris ischemia, increased protein levels in AC 4. Associated ocular risks: lens subluxation, angle closure, CRVO, bloodaqueous barrier impairment, intraoperative zonular dialysis, capsular rupture, vitreous loss, capsular phimosis or PCO formation C. Treatment 1. Bimatoprost produced greater IOP reduction than Latanoprost 2. Pilocarpine should reduce amount of exfoliation material reaching TM and should allow increased TM clearing by increasing aqueous flow, shown in ICEST study to work better than Cosopt 3. Aqueous suppressants should reduce TM function by decreasing TM clearing 4. ALT/SLT safe and effective, equivalent, better than with POAG but with potentially abrupt loss of effect 5. Incisional surgery (trabeculectomy, GDD) results are comparable for POAG, with added risks of lens-instability related complications D. Prognosis 1. XFS has worse prognosis than POAG, more likely to progress at a given IOP than non-XFS POAG patients, progress is more rapid in XFS, poorer response to meds, greater need for surgery than POAG, greater diurnal fluctuation E. ICD-10 Coding 1. “Capsular Glaucoma with Pseudoexfoliation of Lens” 2. Staging by eye: H40.14(1,2,3,9)(0-4) 3. Severity determination follows same guidelines as all other glaucoma (early, moderate, severe, indeterminate, unspecified)