Pigment dispersion syndrome involves the abnormal release of pigment from the iris that is deposited throughout the eye. Some patients may develop pigmentary glaucoma. The mechanical abrasion theory proposes that iris defects allow iris pigment to enter the eye. The abiotrophy theory suggests there is a genetic component. Symptoms are typically absent early on but later include vision loss. Signs include pigment deposition in tissues like the cornea and trabecular meshwork. Treatment focuses on lowering intraocular pressure through medications, laser trabeculoplasty, or surgery if needed. About 25-50% of patients may progress to pigmentary glaucoma.
2. OVERVIEW
PIGMENT DISPERSION SYNDROME
– abnormal amounts of pigment are liberated from the
posterior surface of the iris
– pigments are deposited all throughout the anterior and
posterior chambers of the eye
PIGMENTARY GLAUCOMA
– a secondary glaucoma that develops among patients
with PDS
4. MECHANICAL ABRASION THEORY
proposed by D.G. Campbell
proposed that people with PDS often exhibits iris
transillumination defects
iris transillumination defects (slitlike defects) –
there was a corresponding packet of zonules
attached to it.
9. ABIOTROPHY THEORY
Abiotrophy is the premature degeneration of
cells or tissues, especially when it is due to
genetic defects
PDS might have a heritable component –
GPDS1 gene mapped in chromosome 7
microscopic findings of iris tissue
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12. EPIDEMIOLOGY
Diagnosed on second and third decade of life
PDS is equally prevalent among men and
female, PG is more common among males
More common among whites
More common on myopic patients
Genetic association is under study
13. SYMPTOMS
EARLY: usually no symptoms; some may
have blurred vision with jarring
exercise
LATER: loss of peripheral vision
VERY LATE: loss of central vision
14. SIGNS
CORNEA
– KRUKENBERG SPINDLE – pigment deposited on the
corneal endothelium and phagocytosed in the endothelial
cells
– pigments do not cause visual symptoms
– pigments do not harm the endothelium
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16. SIGNS
IRIS
– develops defects in pigment epithelium of the iris that can be
seen through transillumination
– pigments deposited on the anterior surface can cause iris to
appear darker
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19. SIGNS
PUPILS
– change in the function of the pupil
– in asymmetric disease, larger pupil is found in the affected
eye
ANTERIOR CHAMBER
– anterior chamber is usually deep
20. SIGNS
IRIDOCORNEAL ANGLE
– open angle
– densed trabecular pigment
– backbowed iris
* gonioscopy is of importance to visualize the angle
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23. SIGNS
POSTERIOR CHAMBER
– pigments can accumulate at the junction of the lens and
posterior zonules
– SCHEIE STRIPE or ZENTMEYER LINES
– best examined in dilated pupils
– gonioscopy in of benefit
24. In Summary ...
Classic triad of clinical signs of PDS
– Krukenberg spindle
– slitlike, radial, midperipheral iris transillumination
defects
– pigment deposition on the trabecular meshwork
25. TREATMENT
Treated like POAG
Cholinergic agonist drugs (Pilocarpine) –
induces miosis and lifts iris from the zonules
– not well tolerated by myopics
– may increase risk for developing retinal detachment
– pilocarpine Ocusert can be used to lessen side effects
26. TREATMENT
Laser Trabeculoplasty may be beneficial in PG
Laser Iridotomy – in PG patients with iris
backbowing
Not an ideal choice in the following patients
1. Patients with PDS with no Ocular Hypertension and may never
develop optic nerve damage
2. Patients with advanced glaucomatous damage taking multiple
medications
27. TREATMENT
Trabeculectomy – if medical and laser therapy
fails
– increased risk for failure
– antimetabolites should be used with caution
– myopic males is at increased risk to develop hypotony
maculopathy
28. PROGNOSIS
25 to 50% of patients with PDS will progress to
Pigmentary Glaucoma (PG)
6 to 8% of patients with PDS & PG will develop
retinal detachment
Pigment dispersion syndrome (PDS) is an ocular condition in which granules from the colored part of the eye become dislodged and accumulate in other structures within the eye.
Liberated pigment granules are borne by aqueous currents and deposited on the structures of the anterior segment.
Areas of pigment loss appear as bright spikes emanating outward from the pupil, much like the spokes on a bicycle wheel. As you can see from the figure, the pattern is striking and easily recognizable.
This work led to the theory that mechanical rubbing between anterior packets of lens zonules and the posterior iris in predisposed eyes is the cause of pigment dispersion.
One explanation stems from the observation that myopia (nearsightedness) is associated with pigment dispersion syndrome. Myopic eyes tend to have a slightly concave iris that potentially could press against the zonules, enabling mechanical abrasion between the anterior packets of zonules and the iris during normal pupillary movement. Although myopia could explain some cases of pigment dispersion syndrome, it is insufficient in explaining all instances. Not everyone with myopia has PDS, and not everyone with PDS has myopia
A second, and complementary, explanation for iris concavity is that a phenomenon called reverse pupillary block can cause the iris to be positioned against the lens. In reverse pupillary block, pressure in the anterior chamber of the eye exceeds that of the posterior chamber. This could be caused by obstruction of aqueous humor outflow through the trabecular meshwork and/or anterior movement of aqueous humor from blinking. The increased pressure forces the iris over the lens, where it acts like a ball valve in the pupillary opening, preventing the return of aqueous humor to the posterior chamber. As pressure continues to increase, it causes a backward bowing of the iris that accentuates contact between it and the Zonules of Zinn.
While mechanical abrasion theory explains several clinical features of pigment dispersion syndrome, key gaps in knowledge still remain. Primary among these is that liberation of iris pigment can occur in eyes that are not myopic and for which there is little or no evidence of zonule abrasion. For this reason, thinking about PDS has expanded to include the abiotrophy theory. Abiotrophy is the premature degeneration of cells or tissues, especially when it is due to genetic causes. Cellular degeneration is supported in pigment dispersion syndrome by two lines of evidence.There appears to be a clustering of PDS cases in a subset of families, suggesting that it might have a heritable component. In confirmation, a gene known as GPDS1 has been mapped to chromosome 7 that exhibits a dominant inheritance pattern and appears to contribute to PDS in some patients. Other gene candidates currently are being investigated.Additional evidence comes from microscopic examination of iris tissue. Within tissue disruptions throughout the iris, pigment-containing cells called melanocytes either are missing or have ruptured membranes from which pigment extrudes. The irisstroma (connective tissue) in the area of the pigment-cell disruptions is sometimes affected and typically is permeated with pigment-laden macrophages. A macrophage is a type of white blood cell that is part of the body's immune response. Its job is to ingest foreign material.
Normalhistologic section of the iris Some studies also have identified the presence of abnormal iris melanosomes. These are tiny oval-shaped organelles (specialized subunits within cells ) that synthesize melanin pigment within the melanocyte cells. Melanosome irregularity would not be expected from mechanical abrasion and suggests a different disease process, possibly related to genetic factors involved in melanin biosynthesis or programmed cell death (apoptosis).
Taken together mechanical abrasion and abiotrophy theories account for most of the cases of pigment dispersion syndrome. Moreover, evidence appears to be mounting they may represent two distinct disease processes that have a common endpoint of pigment dispersion.Once pigment is liberated from the iris, it is carried throughout the anterior chamber and is deposited in multiple locations, including the cornea, iris, lens, zonules, ciliary body, and trabecular meshwork.
The densed black pigment from the posterior iris is deposited in the pigmented trabecular meshwork as a dense black band
Cross-section of the anterior chamber showingPigment accumulation in the trabecular meshwork.
The densed black pigment from the posterior iris is deposited in the pigmented trabecular meshwork as a dense black band