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ORIGINAL ARTICLE

EPIDEMIOLOGICAL ASPECTS OF PSEUDOEXFOLIATION SYNDROME: A


STUDY
Shreya M. Patwardhan1, Mariam N. Mansuri2, Purvi R. Bhagat3,

HOW TO CITE THIS ARTICLE:


Shreya M Patwardhan, Mariam N Mansuri, Purvi R Bhagat, “Epidemiological aspects of pseudo exfoliation syndrome:
a study”. Journal of Evolution of Medical and Dental Sciences 2013; Vol. 2, Issue 51, December 23; Page: 9901-
9906.

ABSTRACT: BACKGROUND: Pseudo exfoliation glaucoma (PEX) is the most common identifiable
cause of secondary glaucoma, the prevalence of which varies considerably among different
ethnicities. AIMS: In pseudo exfoliation syndrome - To study the age and sex distribution. - To study
the laterality of involvement. - To study the spectrum of clinical findings. -To study various locations
of PEX material. SETTINGS AND DESIGN: The study was a non- interventional, observational, cross
sectional study conducted at M and J Western Regional Institute, Ahmedabad. MATERIALS AND
METHODS: This study included patients who presented during May 2006 to November 2008. The
total number of cases studied were 81 patients (160 eyes). A detailed glaucoma evaluation was done
for all these patients. RESULTS AND CONCLUSION: PEX was more common in males as compared to
females. PEX disease on presentation is unilateral, but eventually becomes bilateral. There was no
relation between maturity of cataract and appearance of PEX. The mean value of Central Corneal
Thickness (CCT) in patients with PEX showed no difference in eyes with or without PEX. PEX
material was most commonly found on the lens capsule and iris but is also seen in angles, ciliary
body and corneal endothelium. Thus proper dilatation of patients is needed to avoid missing
diagnosis of PEX because PEX material is less likely to be found on the iris as compared to the lens
capsule. In our study 76.58 % patients had normal IOP at presentation. 23.41 % had high IOP at the
time of presentation. PEX predominantly presents with open angles. PEX is an important cause for
ocular morbidity. Because of the fluctuating intraocular pressures and difficult medical management,
it stands out as a distinct and enigmatic clinical entity. Also because of its influence on cataract
surgery, it needs to be assessed carefully to avoid complications.

INTRODUCTION: Pseudo exfoliation (PEX) is an age related, systemic, elastic fibrillopathy first
described by Lindberg in 1917. [1} Despite being described nearly a century ago the exact nature of
exfoliation material and the pathogenesis still remains unknown.
Glaucoma is the most important sequel of PEX syndrome. It is the most common identifiable
cause of secondary open angle glaucoma worldwide.[2]
Intraocular pressure fluctuations are more common in glaucoma caused due to PEX. [3]
PEX glaucoma is frequently more resistant to medical management compared to primary
open-angle glaucoma.[4]
PEX not only causes chronic open angle glaucoma, but also lens subluxation, angle closure
and serious complications during cataract surgery such as zonular dialysis, capsular rupture and
vitreous loss.
An association of PEX and retinal vein occlusion has been suggested by several studies. One
potential explanation for this significant association of Central Retinal vein occlusion (CRVO) and
PEX is the secondary open-angle glaucoma caused by PEX and the higher levels of IOP associated
with PEX compared to simple glaucoma as known risk factors for CRVO. [5]

Journal of Evolution of Medical and Dental Sciences/ Volume 2/ Issue 51/ December 23, 2013 Page 173
ORIGINAL ARTICLE
Prevalence of PEX has been correlated with cardiovascular diseases such as transient
ischaemic attacks, stroke, hypertension, angina, myocardial infarction and negatively with diabetes
mellitus.[6]
Indian studies of this syndrome have been few and limited. This study aims at enhancing our
knowledge of this enigmatic disorder in relation to cataract and glaucoma.

AIMS AND OBJECTIVES:


- To study the age and sex distribution of pseudoexfoliation syndrome
- To study the laterality of involvement in pseudoexfoliation syndrome.
- To study the spectrum of clinical findings in pseudoexfoliation syndrome patients.
- To study various locations of PEX material in patients with pseudoexfoliation syndrome.

MATERIALS AND METHODS: This study was conducted at M and J Western Regional Institute,
Ahmedabad. It included patients who presented during May 2006 to November 2008. The total
number of cases studied was 81 patients (160 eyes while 2 eyes were lost eyes). Out of 160 eyes, two
eyes had corneal opacity, so limited parameters were measured in those eyes.
Patients who had evidence of present or past uveitis and those with evidence of ocular
trauma were excluded from the study. It precludes the diagnosis on the basis of signs related to
pigment dispersion alone to avoid confusion with pigment dispersion syndrome.
Demographic details of all patients were noted.
A detailed ocular and systemic history was noted. Visual acuity and Best Corrected Visual
Acuity was taken on Snellen’s chart. The anterior segments of both eyes were examined on slit lamp
before and after dilatation of pupils focusing mainly on discovering presence and distribution of
pseudoexfoliative deposits and associated pigmentary signs. Presence of PEX was defined as the
presence of exfoliative deposits on the pupillary border and/or the anterior lens capsule with or
presence of deposits elsewhere in the anterior segment.
IOP in both the eyes was measured with Perkin’s applanation tonometer. Direct and Indirect
ophthalmoscopy was also performed. Gonioscopy was done in all cases by Goldmann two mirror
indirect goniolens for angle grading (graded according to Schaffer’s criteria), pigmentation and
presence of PEX material. Visual fields were assessed with automated perimetry (Octopus 901 )
wherever possible. Ultrasonography and Ultrasound biomicroscopy were done wherever possible.

OBSERVATIONS AND RESULTS:


AGE GROUP
NO. OF PATIENTS PERCENTAGE
(in yrs)
40-50 3 4%
50-60 14 17%
60-70 40 50%
70-80 18 22%
>80 6 7%
TOTAL 81 100%
Table no 1: AGE GROUP
In the study population, 57 patients were male and 24 patients were female. Thus males
constituted 70.3% of the study population and females constituted the rest 29%.

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ORIGINAL ARTICLE
Pseudoexfoliation syndrome was unilateral in 54 patients and bilateral in 25 patients. Thus
in 68% patients the disease was unilateral and in 32% patients the disease was bilateral.

IOP
RE LE TOTAL PERCENTAGE
(mm of Hg)
<20 58 63 121 76.58%
20-30 15 6 21 13.29%
>30 7 9 16 10.12%
TOTAL 80 78 158 100%
Table no. 2: IOP MEASUREMENTS

Central corneal thickness(CCT) Value


Maximum CCT 0.580 mm
Minimum CCT 0.490 mm
Mean CCT 0.538 mm
Table no. 3: CENTRAL CORNEAL THICKNESS

Angle RE LE Total %
0 7 11 18 11
1 7 6 13 8
2 11 11 22 14
3 55 52 107 67
Table no. 4: SHAFFER ANGLE GRADES

In 77 patients (37.56%) PEX material was found on the crystalline lens. In 67 patients
(32.68%) PEX material was found on the iris. In 24 patients (11.70%) PEX material was found in the
angle. In 34 patients (16.58%) it was found on the ciliary body and in 3 patients (1.46%) it was
found on the corneal epithelium.

Fig. 1 Fig. 2

DISCUSSION: Our study shows increase in the disease pattern of pseudoexfoliation with age and is
in accordance with all the available reports. [7] The youngest patient in our series was 40 yrs old and
the oldest was 80 yrs old.

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ORIGINAL ARTICLE
A hospital based study in Pakistan showed prevalence of 4.2% in the age group 50-60, 20.8%
in age group 60-70 and 75% in age group above 70 yr. [8] Population based studies in Iran have also
shown increase of prevalence with age incidence being 15% in 50-60 yrs, 34% in 60-70 yrs and 46%
in 70-80 yrs age groups.[9]
Our study shows a male predominance (70.3%) as compared to female (29.7%).
Available data on the prevalence of PEX as related to sex shows conflicting reports.
Clements and Luntz found higher incidence among males.[10][11] A similar study in Finland
showed higher incidence in females. (14% in males and 16.2% in females) [12]
In our study, 68 % of patients had unilateral while 32 % had bilateral disease.
Our study shows that 68 % eyes had immature cataract, 28 % had mature cataract and 4%
had hypermature cataract.
This is in concordance with the studies done by Mariam and Aurora showing incidence of
immature, mature and hypermature cataract of 85 %, 9.3 % and 4.0 % respectively. [13]
In our study the mean value of CCT in patients with PEX was 0.538 mm. The lowest CCT
findings were 0.490 mm and maximum of 0.580, which is in concordance with results of Hepsen et al
[14]
.
In our study 67 % eyes had grade 3 angles, 14 % had grade 2 angle, 8% had grade 1 angle,
11% had angle 0. Thus 67% had open angle and 33% had narrow angle glaucoma.
A study of PEX in Chinese population by Young et al showed that 18 % of all eyes had narrow
angles (defined as grade 0 to 2 by Shaffer grading.)[15]
In our study PEX material was found in 37.56% of the eyes on lens capsule, iris 32.68%, angle
11.70 % eyes, ciliary body 16.58 % eyes and corneal endothelium 1.46%. Similar results were found
in a population based study in central Iran .[9]
In our study 76 % had IOP less than 20 mm of Hg. 13.29 % eyes had IOP between 20-30 mm
of Hg, the rest 10.12 % eyes had IOP more than 30 mm of Hg.

CONCLUSION: A definite increase in the disease frequency was noted with age. So PEX definitely
seems to be a disease of senility.
PEX was more common in males as compared to females.
PEX disease on presentation is unilateral, but eventually becomes bilateral.
There was no relation between maturity of cataract and appearance of PEX.
The mean value of CCT in patients with PEX showed no difference in CCT in eyes with or
without PEX.
PEX material was most commonly found on the lens capsule and iris but is also seen in
angles, ciliary body and corneal endothelium. Thus proper dilatation of patients is needed to avoid
missing diagnosis of PEX because PEX material is less likely to be found on the iris as compared to
the lens capsule.
In our study 76.58 % patients had normal IOP at presentation. 23.41 % had high IOP at the
time of presentation.
PEX predominantly presents with open angles.
PEX thus is an important cause for ocular morbidity. Because of the fluctuating IOP and
difficult medical management, it stands out as a distinct and enigmatic clinical entity.

REFERENCES:

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ORIGINAL ARTICLE
1. Tarkkanen A, Kivela T, John G. Lindberg : And the discovery of exfoliation syndrome, Acta
Ophthalmol Scand., 2002, 80:151-154.
2. Ritch R, Schloö tzer - Schrehardt U : Exfoliation syndrome, Surv Ophthalmol., 2001, 45: 265–315.
3. Nenciu A, Stefan C, Melinte D, Malcea C, Tebeanu E, Nae I., IOP diurnal fluctuations in patients
presenting with pseudoexfoliative syndrome, Article in Romanian Clinica de Oftalmologie – 2,
2006;121-125
4. Henry CJ, Krupin T, Schmitt M, et al., Long-term follow-up of Pseudoexfoliation and the
development of elevated intraocular pressure, Ophthalmology, 1987; 94: 545–52.
5. Claus Cursiefen, Thomas Hammer, Michael Kutchle, Gottfried O H Naumann, Ursula Schlotzer –
Schrehardt, Pseudoexfoliation syndrome in patients of Ischaemic Central Retinal Vein
Occlusion; Acta Ophthalmologica Scandinavica, Vol 79, Issue 5;476-47.
6. Shrum K R, Hattenhauer MG, Hodge D, Cardiovascular and cerebrovascular mortality
associated with ocular pseudoexfoliation, Am J Ophthalmol. 2000; 129 (1): 83 – 6.
7. Vassilios P. Kozobolis, Maria Papatzanakil, Ioannis G. Vlachonikolis, Ioannis G. Pallikaris' and
Ioannis G. Tsambarlakis Epidemiology of pseudoexfoliation in the island of Crete (Greece) Acta
Ophthalmologica Scandinavica Volume 75, Issue 6, 726-729.
8. Rashad Qamar Rao, Tariq Mehmood Arain and Muhammad Ali Ahad; The prevalence of
pseudoexfoliation syndrome in Pakistan. Hospital based study; BMC Ophthalmology 2006, 6:27
9. Kouros Nouri- Mahdavi, Nastaran Nosrat, Ramin Sahebghalamand Mehdi Jahanmard;
Pseudoexfoliation syndrome in Central Iran: A population based survey Acta Ophthalmol.
Scand. 1999: 77: 581-584.
10. D B Clements: Glaucoma in the Isle of Man with special reference to pseudo-capsular
exfoliation; Br J Ophthalmol. 1968 July; 52(7): 546–549.
11. Luntz MH, Prevalence of pseudoexfoliation syndrome in an urban South African Clinic
population; American Journal of Ophthalmology 1972, Vol 74, pg 581.
12. 12 . Forsius H: Prevalence of pseudoexfoliation in Finns, Lapps, Icelanders, Eskimos, and
Russians Trans Ophthalmol Soc UK 99: 296-298, 1979
13. Mariam M.K, Aurora A, Pani S.P. et al: XXV International Congress of Ophthalmology, Roma,
Immunology, 1986, 48.
14. Hepsen et al : Corneal curvature and thickness in PEX, Can J ophth 2007; 42 : 677-80.
15. A L Young, W W T Tang, D S C Lam; The prevalence of pseudoexfoliation syndrome in Chinese
population; British Journal of Ophthalmology 2004; 88: 193-195.

AUTHORS: NAME ADDRESS EMAIL ID OF THE CORRESPONDING


1. Shreya M. Patwardhan AUTHOR:
2. Mariam N. Mansuri Dr. Shreya Patwardhan,
3. Purvi R. Bhagat Room No. 7,
M & J Institute of Ophthalmology,
PARTICULARS OF CONTRIBUTORS: Civil Hospital Campus, Asarwa,
1. Resident, Department of Ophthalmology, Ahmedabad – 380016.
M & J Western Regional Institute of Email – shreyaaa215@gmail.com
Ophthalmology, Ahmedabad.
9 2. Professor & Head of Glaucoma Unit,
Department of Ophthalmology, M & J Western Date of Submission: 20/10/2013.
R Regional Institute of Ophthalmology, Date of Peer Review: 21/10/2013.
Ahmedabad. Date of Acceptance: 07/11/2013.
3. Associate Professor, Glaucoma Unit, Date of Publishing: 17/12/2013
Journal of Evolution of
Department of Medical and Dental
Ophthalmology, M&Sciences/ Volume 2/ Issue 51/ December 23, 2013
J Western Page 177
Regional Institute of Ophthalmology,
Ahmedabad.
ORIGINAL ARTICLE

Journal of Evolution of Medical and Dental Sciences/ Volume 2/ Issue 51/ December 23, 2013 Page 178