Pigment Dispersion Syndrome (PDS) involves abnormal amounts of pigment released from the iris that deposit throughout the eye. It can sometimes progress to Pigmentary Glaucoma (PG). PDS is more common in young, white, myopic males and has genetic links. Theories for its pathogenesis include mechanical abrasion between the iris and lens zonules or abnormal iris cell degeneration. Over time, PDS can cause increased eye pressure and vision loss from PG if not properly treated with medications, laser procedures, or surgery.

1. Pigment Dispersion Syndrome/
Pigmentary Glaucoma
Dr shylesh B dabke
Glaucoma Fellow
Aravind Eye hospital
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2. Overview
Pigment Dispersion syndrome(PDS)
Abnormal amount of pigments are liberated from posterior
surface of iris, which are deposited throughout the anterior and
posterior chamber of the eye.
Pigmentory Galucoma
A type of secondary open glaucoma that develops among
patients with PDS.

3. History
In 1940, Sugar briefly described one such case with pigment
dispersion and glaucoma.(1)
In 1949 - Sugar and Barbour(2)
- Reported details of this entity
- Referred this condition as “Pigmentary Glaucoma”
1.Sugar HS. Concerning the chamber angle. I. Gonioscopy.Am J Ophthalmol 1940;23:853-866.
2. Sugar HS, Barbour FA. Pigmentary glaucoma: a rare clinical entity. Am J Ophthalmol. 1949;32(1):90-92.

4. Epidemology
Corresponds to 1-1.5% of all Glaucoma.
Onset < 40yrs
Male : Female – 2:1
More frequent in whites
Strong association between Pigmentary Glaucoma and Myopia.

5. “Young White Myopic Male”

6. ▪ Usually Bilateral
▪ No typical Hereditary Pattern – Sporadic
▪ Significant linkage between disease phenotype and genetic markers
located on 7q 35-36.
▪ Probability of PDS converting to PG*
10% in 5 years
15% in 15 years
* SiddiquiY., Hulzen R.D.T., Cameron J.D., et al:What is the risk of developing pigmentary glaucoma from
pigment dispersion syndrome?. Am J Ophthalmol 2003; 135:794-799

7. Pathogenesis
Anatomical Predisposition
- Myopia
- Deep AC
- Posterior insertion of iris into ciliary body
- Mechanical abrasion theory
- Abioptrophy theory
 Pathophysiology

8. Mechanical Abrasion Theory
▪ D G Campbell
▪ This theory is based on finding of slit like trans illumination
defect on iris.

9. Slit like trans illumination defect
 Mechanical rubbing
between anterior packets
of lens zonules &
posterior iris in
predisposed eyes
 Cause of pigment
dispersion.

10. PDS and Myopia
 This association can be
explained with mechanical
abrasion theory.

11. PDS and reverse pupillary block
 Second & complementary
explanation for iris
concavity.

12. PDS and Blinking
 Campbell proposed blinking
initially deforms the cornea
Pushing iris against zonules
Transient increase in IOP

13. PDS and Accommodation
 Accommodation in patients
with PDS – increased
posterior bowing of iris
 Forward movement of lens
– increasing pressure in
anterior chamber

14. Abiotrophy Theory
 Premature degeneration of cells or tissue, especially when it is
due to genetic defect
 While mechanical abrasion theory explains several clinical features
of pigment dispersion syndrome, key gaps in knowledge still
remain.
 Cellular degeneration is supported in pigment dispersion syndrome
by two lines of evidence.

15. Heritable Component
 There appears to be a clustering of PDS cases in a subset of families
 GPDS1 has been mapped to chromosome 7
 Dominant inheritance pattern
 Other gene candidates currently are being investigated.

16. 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.

17. Mechanical
Abrasion
Theory
Abiotrophy
Theory
PDS
 Evidence appears to be mounting that the two 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.

19. Clinical Features
 Early - Asymptomatic
 Episodes of pain/blurred
vision with haloes
following strenuous exercise
 Advanced – Loss of
central vision
 Later - Loss of peripheral
vision

20. Signs
 Cornea – Krunkenberg
Spindle*
 Ant Chamber
-Deep
-Posterior bowing of
peripheral iris
-Pigments in AC
 Iris – Slit like/Spoke like
transillumination defect*

21. Infrared Videography Technique

22.  Fine surface pigment
granules that may extend
onto the lens
 Partial loss of pupillary
ruff (frill)
 Heterochromia
 Anisocoria

23.  Gonioscopy
- Wide open angle
- Mid peripheral iris
concavity*
- Trabecular
hyperpigmentation
 Lens
- Annular ring of pigment
on posterior lens surface
“Scheie Strip/Zentamayer
Ring”*
 Fundus
- Retinal Breaks & Lattice
degeneration(20%)
- Retinal detachment (6-7%)

25. Classic Clinical Triad
Krukenberg Spindle Dense trabecular
pigmentation
Midperipheral iris transillumination defects

26. Differential Diagnosis
 Exfoliation Syndrome
 Uveitis
 Pigment dispersion with PCIOL
 POAG
 Pigmented Tumors

27. Other conditions with pigment dispersion
 Acute angle closure glaucoma
 Rhegmatogenous RD
 Ocular Haemorrhage
 Trauma(Surgical/nonsurgical)

28. Conditions with iris trans-illumination
defects
 Albunism

29. Mid peripheral / Peripupillary
Homogenous / Patchy
<40 / >60
Absent / Present
PDS vs Pseudoexfoliation syndrome
Trans-
illumination
defect
TM
Pigmenta
- tion
Age
Granular
deposits

30. Investigation
 UBM – Posterior iris insertion
Iris concavity
Iridozonular contact

31. Medical Miotics
Physical
activity
Laser
Trabeculoplasty
Treatment
• Similar to POAG
• Rx typically begins with medical therapy
• All ocular hypotensive medications are effective
• PG Analogues are drug of choice*
• Theoretically of benefit
• Decreases iridozonular contact
• Facilitates aqueous outflow
• Disadvantage – Exacerbation of myopia
Precipitate RD in Myopia
Not well tolerated by young patients
• Alfa adrenergic antagonist thymoxamine produces miosis without
cyclotropia
• Exercise increases pigment dispersion and causes IOP spike*
• If significant pressure rise is observed, 0.5% Pilocarpine during exercise may
be beneficial
• Heavy trabecular pigmentation allows increased absorption of laser energy
• In turn allowing lower energy level for trabeculoplasty
• Starting with 300mW per spot if using ALT and 0.4mJ per spot if using SLT
• Ritch et al reported 80% success rate at 1yr followup
• Trabeculoplasty responds well initially but IOP control tends to decline wit time
and surgery is in effective in patients who are older or who had glaucoma
for a long time

32. Laser
Iridotomy
Trabeculectomy
• Theoretical effects –
- Relives the reverse pressure gradient
- Relieves posterior bowing of peripheral iris
- Relieves rubbing
- Reduces liberation of pigment
- Allows meshwork to clear pigment & recover normal outflow function
(if changes are not already irreversible)
• When medical therapy and laser trabeculoplasty have failed to
adequately control IOP
• Filtration surgery is usually successful
• Use of adjunctive antimetabolites may improve surgical outcome
• Higher percentage of patients with pigmentary glaucoma than POAG and men
appear to require it at a earlier stage.
• Success rates are similar to POAG.
• Trabeculectomy in young myopic patients warrants extra care -
prone for Hypotony Maculopathy

33. Temporal Evolution of PDS
Conversion of PDS to Pigmentary Glaucoma
(Slow & may take years)
Slow spontaneous
resolution
Irreversible damage to angle
Transillumination defect
may disappear
IOP may return to normal
Trabecular pigmentation may decrease
Pigment reversal
sign
“burnt out”

34. With age pupil becomes small & lens becomes thicker
Decreased iridozonular contact
Decreased dispersion
Occasionally glaucoma may “Burn Out”

35.  Look carefully for PDS/PG in patients who present as
Normal Tension Glaucoma
 Look for
“Pigment Reversal”