DOS_feb_2009

Contents

E5 ditorial Photo Essay

27 Orthokeratology 55 Bilateral Anophthalmos in Two Brothers: A Case Report

Kirti Singh Garima Agrawal MS, D.C. Mehta MS

Retina Clinical Monthly Meeting

9 OCT in Diabetic Macular Edema 61 Case 1- Management Dilemma in a case of Traumatic Glaucoma

Jatin Ashar, Pradeep Venkatesh, Bhavin Shah Julie Pegu, Suneeta Dubey, Manisha Agarwal

15 Outcome of Endophthalmitis Cases in North East India 65 Case 2- Posterior Sympathetic Ophthalmia in a Child

Harsha Bhattacharjee, Satyen Deka, Hemlata Deka, Manav Jyoti Barman, Ritesh Narula, Manisha Agarwal, S.P. Chaudhary, Priyata Seth
Lokesh Jain, Kruto Kalita
69 Clinical Talk - Future of Retinal Imaging, Lasers and Therapy
21 Benign Intracranial Hypertension
Cyrus M. Shroff
Ronak P. Bhandari, Nayana A. Potdar, Chhaya A. Shinde,
Pallavi G. Sarate A77 bstract

Cornea & External Disease M81 embership Form

35 Goldenhar Syndrome: A case report & review of literature T85 earsheet

Uday Gajiwala, Rajesh Patel, Manoj Gangwal Target IOP
Shibal Bhartiya, Subha Bansal
41 Pseudophakic Bullous Keratopathy

Shreekant A. Damgude, B.P. Guliani

Neuro-Ophthalmology

49 Relative Afferent Pupillary Defect

Sumita Sethi, Mridula Mehta, Shibal Bhartiya, Sonia Bhargav

www.dosonline.org 3

4 DOS Times - Vol. 14, No. 8, February 2009

Editorial

Teachers in Ophthalmology

Teaching in ophthalmology has changed in the recent times. This can be attributed to various factors.

Learning earlier used to pass from the seniors to the juniors. However availability of the internet has facilitated procurement
of information, but at the same time the person to person teaching is decreasing, as the students and teachers are depending
more on the jargon of information available on the internet. The focussed teaching from person to person and the passing
of skills is missing. There are no dedicated teaching sessions for post graduates and students who are left to themselves to
learn the surgical skills. Similarly the clinical acumen in passing the diagnostic as well as the therapeutic skills from a
senior to junior is missing. The investigative modalities are available which are analysing the anatomical structures at
microscopic level, sometimes reading too much between the lines, sometimes making us aware of the subtle and preclinical
cases.

Research should not be machine based, it cannot be machine based; it has to be based on the benefit versus risks of a
therapeutic trial or a particular procedure. We should not be dependent on machines and technologies for making a
diagnosis or performing a surgical procedure.

Similarly topics in conferences should not be machine or tool based but should be problem oriented.

I would like to end with a quote that I read somewhere

“The greatest danger in modern technology isn’t that machines will begin to think like people, but that people will begin to
think like machines”
Thanking you,

Namrata Sharma
Secretary,
Delhi Ophthalmological Society

www.dosonline.org 5

Welcome to the NEW www.dosonline.org

Within one year of the successful re-launch of the DOS website, the site has again been completely revamped after taking into
account feedback from the DOS members.

Features of the new site include:
1. A signup feature, by which all new registered DOS members can receive the login details immediately in their email.
2. Forget password – DOS members who had forgotten their login password, can get it back in real time.
3. The DOS Times periodical has been uploaded as an online journal at www.dostimes.org. Registered members can access

the various articles, as well as search for articles of their interest.
4. The various DOS Times videos have also been uploaded and can be viewed online.
5. Online Registration and online submission of abstracts and faculty topic form for the forthcoming DOS conference can be

done at the DOS website.

The NEW look Online DOS TIMES at www.dostimes.org

Sign-up facility for new DOS members Online registration for the DOS Conference

We encourage all members to use the DOS website and enjoy all the benefits. We welcome your suggestions and feedback
in this regard. Please mail your opinions to [email protected].

6 DOS Times - Vol. 14, No. 8, February 2009

Book Review

The book "Clinical Ophthalmology
Contemporary perspectives" edited
by: Dr. A.K. Gupta and Dr. V. Krishna
is a revised and updated version of
previous edition. The book has
chapters contributed by
ophthalmologists of national and
international repute.

The book is divided into sections
which include Ocular refraction,
Ocular therapeutics, Cornea and
Ocular surface disorders, Pediatric
ophthalmology, Lens and Glaucoma,
UVEA, Vitreous, Retina, Optic
Nerve and Pathways, Ocular trauma,
Orbit and Oculoplasty, Ocular
diagnosis and Community
Ophthalmology.

Several new chapters on current
topics have been included such as
Intravitreal VEGF inhibitors and
corticosteroids, Evaluation of a
Patient with Orbital Disease, Basic
Principles of Oculoplastic Surgery,
Advances in Lacrimal Surgery,
Fundus Fluorescein Angiography of
Retinochoroidal Disorders and
Determination of Intraocular Lens
Implant Power.

It is indeed a very useful
conglomeration of both the basics of
ophthalmology which help in
understanding the fundamentals as
well as the most recent
developments in the field of
ophthalmology. The book is highly
informative, well organized and easy
to read. This book on contemporary perspectives serves as unique teaching resource for all ophthalmologists especially for the
budding ones.

I think it is a wonderful book and should be a part of every resident’s library. It should be read by every senior resident and post
graduate in ophthalmology. We owe a debt of thanks to Dr. A.K. Gupta & V. Krishna for an excellent contribution to the field
of ophthalmology.

Reviewed by: Dr. Namrata Sharma
MD, DNB, MNAMS

www.dosonline.org 7

OCT in Diabetic Macular Edema Retina

Jatin Ashar MBBS, Pradeep Venkatesh MD, Bhavin Shah MBBS

Macular edema is the most common cause of moderate visual Based on OCT DME can be classified into different pattern such
loss in patients with diabetes. as cystoid macular edema, spongy swelling of the retina, hard
exudates, serous detachment, macular traction, taut posterior
Macular edema is defined as a focal or diffuse area of retinal hyaloid membrane.
thickening which may or may not be associated with hard exudates.
Depending on its location and size it can either a clinically significant Sponge like Thickening
or non-significant.
• most common change (Figure 1)
A number of clinical methods and investigations have been used
for long for the assessment of macular edema in diabetic patients. • mostly in outer retinal layers while internal layers maintain
The slitlamp biomicroscopy has been the gold standard clinical their normal reflectivity
method for looking for macular edema. Investigative modality
like the fluorescein angiography have been used to quantify the • cross-sectional scans show swelling of the retina giving it a
intraretinal leakage in these patients, though FA is useful only as a spongy appearance with increased retinal thickness, from a
guide for focal or grid treatment of thickened areas1. It is the retinal normal value of 174 ± 18µm in an area 500microns in diameter
thickening and not the amount of leakage that is important in the centered on fovea and a central foveal thickness of 152 ± 21
determination of final visual outcome of patients with diabetic µm 2
macular edema.
• expanded areas of low reflectivity
OCT is a non contact, non invasive, micron resolution cross-
sectional study of retina which correlates very well with the retinal • backscattering seen from intraretinal fluid accumulation
histology.
• ring of hard exudates with shadowing effect
Role of OCT in DME
Cystoid Spaces

• second most common pattern (Figure 2)

Diagnosis and Quantification of retinal thickening, extension of • intraretinal cystoid spaces
involves variable depth of retina
retinal thickness, macular volume, retinal morphology and vitreo- •
retinal relationship.

• Defining the indication of surgery

• Determine prognosis

• Research tool

• Follow up of patients and determining and quantifying the
response to therapy (laser, medication, or surgery).

Retinal Map Analysis

Colour Map Figure 1: OCT showing sponge like
diabetic macular edema
It is a topographic representation of the retinal thickness calculated
from the linear scans.

Mean Thickness Map

It provides an average thickness of each of 9 different sectors of Figure 2: OCT showing cystoid
macula and has a high reliability and reproducibility. diabetic macular edema

Diabetic macular edema may present in different ways, either
singly or in combinations of various patterns.

The main pathology in DME is accumulation of fluid intraretinally.
This is seen as reduced backscattering, seen most commonly in
the outer retinal layers.

Dr. Rajendra Prasad Centre for Ophthalmic Sciences,
All India Institute of Medical Sciences, New Delhi

www.dosonline.org 9

• has intervening septa in between Besides the above mentioned patterns other features such as hard
• progresses gradually to involve the whole of retina exudates, epiretinal tractional membranes may also be found in
Serous Detachment patients with DME
• increased macular thickness
• hyporeflective areas corresponding to cysts in retina Hard Exudates
• may cause subfoveal retinal detachment
• high reflectivity spots with backscattering

• can be located in the neurosensory retina or in the subretinal
space

Tractional Retinal Detachment Epiretinal Membranes

• foveo vitreal traction causing peak shaped detachment of fovea • ERMs exerting an anteroposterior or tangential traction on
the macula can be noted on the OCT scan.
(Figure 3)

• sometimes satellite retinal detachment may also be found

• laser can worsen this condition

Taut Posterior Hyaloid Membrane

• macular edema with foveal detachment

• highly reflective signal arising form the inner retinal surface
and extending towards the optic nerve or periphery

• taut, thickened, shiny, glistening hyper-reflective membrane Figure 3: OCT showing vitreomacular traction
with striations on retina over the posterior pole with
attachment to the disc and the top of the elevated macular
surface

• retinal thickness is greatly increased with intraretinal
hyporeflective cyst like cavities (corresponding to fluid
accumulation)

Summary of OCT based classification of DME8

Retinal Thickness Volume Morphology Epiretinal Traction

1. Fixation point: Meaningful only for E1: simple thickening-Compact well-defined and continuous
retinal thicknening, no clinically hyper-reflecting line over the
Normal: 150 + 20m diffuse edema involving visible cystoid spaces inner retinal surface with at
E2: cystoid thickening - RT with least one point of adhesion to
Borderline: 170- at least the center and cysts the retina in at least one of the
E2a: mild 2-4 central small retinal map.
210 m Edema: the 1st ring, and not cysts (horizontal diameter 150-
200m, vertical diameter 400 m) T0: absence of epiretinal
> 210 m for focal edema E2b: intermediate-cysts with hyper-reflectivity.
petaloid configuration or with
2. Central Zone: Normal: 6.5 mm 3 + 1 central cysts (horizontal diameter T1: presence of a continuous
less than 300m, vertical diameter line of flat hyper-reflectivity and
Normal: 170 + 20 m Borderline: up to less than 600 m). adherent to the retina without
E2c: severe-with coalescence of significant retinal distortion.
Borderline: 190 8.0 mm 3 abnormal: cysts with retinoschisis appearance
E3: neuroepithelial detacment T2: presence of continuous line
-230 m > 8.0 mm3 presence of subretinal of hyper-reflectivity with multiple
liquid (non-reflecting space) points of adhesion to the retina
Edema: > 230 m Abnormal: > 8.0 mm 3 above the hyperrflecting line of distortion.
the pigmented epithelium
3. Perifoveal and isolated or associated with simple T3: antero-posterior traction
peripheral areas: or cystoid retinal thickening. with “gull wings” configuration.
Normal: 230 + 20m
Boarderline: 250-
290m Edema: more
than 290m

10 DOS Times - Vol. 14, No. 8, February 2009

Correlation of OCT with Histopathologic Changes in DME

Histopathology

• Intracytoplasmic swelling of Muller cells (a)
• Marked swelling of outer plexiform layer or Henle fiber layer

• Liquefaction necrosis of the Muller cells

OCT

• Sponge-like swellings

• Prominent space with low reflectivity (b)
• Cystoid cavity formation

CSME is categorized by Kang et al6 into following categories

Type 1: Thickening with homogenous optical reflectivity (55.2%)

Type 2: Thickening with markedly decreased optical reflectivity in Figure 4 (a-b): Pre and post triamcinolone injection OCT
outer retinal layer (30.3%) showing significant decrease in macular edema

Type 3: Foveolar detachment without traction (11.7%)

Type 4: Foveolar detachment with apparent vitreofoveal traction • Tractional detachments
(2.8%)
• Superficial folds with ERM
CSME corresponds to a RT more than 300 microns involving the
central zone and / or RT more than 320 micron in the 1st ring. Posterior Hyaloid Membrane
Edema confined to second ring is not considered clinically
significant. • Not visible if completely attached or completely detached
and too far
These patterns can be used in determining the treatment protocols
for macular edema (Figure 4) • can present with perifoveolar detachment, double convexity
on cross sectional scan
• Sponge like swelling
• can thickken as a sequale of fibrovascular proliferation
• focal grid / laser
Preretinal Hemorrhage
• cystoid swelling
Mask the features of edema on OCT
• IVTA
Summary of OCT based classification of DME 8
• Serous detachment
Ultra Resolution (UR) OCT4
• laser & ppv
Advantages over Stratus OCT
• tractional retinal detachment and taut posterior hyaloid
• Ultrahigh resolution make the small distortion in retinal
• ppv contour are more evident.

Changes on OCT in DME • Axial resolution of 2 to 3 µm making the visualization of intra
retinal layers simpler.
Intraretinal changes
• Better visualization of hard exudates on UR-OCT
Diffuse swelling: retinal swelling, no cystoid space, outerplexiform
and outer nuclear layers thicken and show hyporeflectivity • Small cystic changes in the ganglion cell and nuclear cell layers
is evident better than stratus OCT
Cystoid spaces: hyporeflective spaces, in Henie’s and outer
plexiform layer • Thin epiretinal membrane not evident on Stratus OCT can
be visualized using UR-OCT.
Foveolar detachment: foveal or extrafoveal cystic spaces may lead
to detachment of foveola. • Small disruption in the photoreceptor inner segment outer
segment region is also evident on UR OCT.
Hard Exudates: hyperrective intraretinal deposits, outer plexiform
layer Role of OCT vs FA in DME

Inner Retinal Border FA assess the vascular leakage and quantifies it in macular edema,
however, it is the actual thickness of the retina that correlates with
• Foveolar edema-flattening of foveal pit the final visual outcome of the patient3.

• Dome shaped elevation of inner retinal boundary

• Concave slopes due to rise of macular center

www.dosonline.org 11

Correlation of OCT and FA Morphologic Mean Visual Acuity Mean Visual Acuity

According to the modified Otani scheme9, OCT findings of macular (logMAR) (Snellen)
edema are characterized into four types:
DRT 0.69 + 0.41 20/97
Type 1: thickening of the fovea with homogenous optical reflectivity
CME 0.79 + 0.39 20/123
Type 2: thickening of the fovea with markedly decreased optical
reflectivity in outer layers SRD without PHT 0.82 + 0.34 20/132

Type 3: thickening of the fovea with subfoveal fluid accumulation PHR with TRD 1.0 + 0.24 20/200
and distinct outer border of detached retina
PHT without TRD 0.77 + 0.44 20/118
Type 3A: without foveal traction
DRT: diffuse retinal thickening; CME: diabetic cystoid macular
Type 3B: with apparent vitreofoveal traction edema; SRD: serous retinal detachment; PHT: posterior hyaloidal
traction; TRD: traction retinal detachment.
Fluorescein angiography findings can be categorized into three
types: References

Type 1: focal leakage with well defined focal areas of leakage 1. Giacomo Panozzo, Elena Gusson, Barbara Parolini and Andrea
Mercanti.. Role of OCT in the diagnosis and follow up of diabetic
Type 2: diffuse leakage which is widespread and ill defined macular edema. Seminars in Ophthalmology 2003, Vol. 18, No. 2,
pp. 74–81
Type 3: diffuse cystoid leakage with pooling in cystic spaces of
macula 2. Bandello, D. Roman Pognuz,, A. Polito, A. Pirracchio, F. Menchini,
M. Ambesi,. Diabetic macular edema: classification, medical and
Focal leakage on FA is mostly associated with non proliferative laser therapy. Seminars in Ophthalmology2003, Vol. 18, No. 4, pp.
diabetic retinopathy while diffuse leakage with proliferative diabetic 251–258
retinopathy.
3. Ozdek SC, Erdinc MA, Gurelik G, Aydin B, Bahceci U, Hasanreisoglu
The eyes with previous macular focal or grid photocoagulation B. Optical coherence tomographic assessment of diabetic macular
tend to show an increased proportion of OCT type 2 and OCT edema: comparison with fluorescein angiographic and clinical
type 3A compared with eyes without prior photocoagulation. findings.. Ophthalmologica. 2005 Mar-Apr;219(2):86-92.

Focal leakage on FA is closely related with type 1 or homogenous 4. Srinivasan VJ, Wojtkowski M, Witkin AJ, Duker JS, Ko TH, Carvalho
foveal thickening on OCT, while diffuse or cystoid leakage is related M, Schuman JS, Kowalczyk A, Fujimoto JG. High-definition and
to either type 2 or type 3A or fluid collection predominantly in 3-dimensional imaging of macular pathologies with high-speed
outer retinal layers or subretinal space. ultrahigh-resolution optical coherence tomography.
Ophthalmology.2006 Nov;113(11):2054.e1-14.
OCT vs RTA5
5. Goebel W, Franke R. Retinal thickness in diabetic retinopathy:
• OCT has excellent sensitivity and considerable specificity comparison of optical coherence tomography, the retinal thickness
making it an excellent general screening tool for diabetic analyzer, and fundus photography. Retina. 2006;26:49–57.
macular edema.
6. The correlation between fluorescein angiographic and optical
• RTA has a higher specificity making it the tool to confirm the coherence tomographic features in clinically significant diabetic
indication for laser therapy. macular edema. Kang SW, Park CY, Ham D-I. Am J Ophthalmol
137:313–322
Problems in Performing OCT in Diabetic Patients
7. Kim BY, Smith SD, Kaiser PK. Optical coherence tomographic
• Poor papillary dilatation patterns of diabetic macular edema. Am J Ophthalmol. 2006
Sep;142(3):405-12
• Media opacities in form of cataract, vitreous hemorrhage
may affect the scan quality 8. Panozzo G, Parolini B, Gusson E, Mercanti A, Pinackatt S, Bertoldo
G, Pignatto S. Diabetic macular edema: an OCT-based classification..
Mean Visual Acuity Association with Morphological Semin Ophthalmol. 2004 Mar-Jun;19(1-2):13-20.
Subtypes of DME7
9. Otani T, Kishi S, Maruyama Y: Patterns of diabetic macular edema
V/A for patients without CME increased by 0.16 log MAR units/ with optical coherence tomography. Am J Ophthalmol 1999;127:688-
100 micron increase in retinal thickness. This reflects on the Snellen 693.
acuity from 20/20 to 20/30
First Author
In the presence of CME, the mean increase is of 0.40U on logMAR Jatin Ashar MBBS
due to CME, corresponding to a change in Snellen acuity from 20/
20 to 20/50. However, increased retinal thickness along with CME
also reduces the acuity, but to a lesser extent than without CME, in
such a way that a 100micron change in retinal thickness corresponds
to change in log MAR of 0.085U, corresponding to a visual acuity
change from 20/20 to 20/25.

12 DOS Times - Vol. 14, No. 8, February 2009

Outcome of Endophthalmitis Cases in Retina
North East India

*Harsha Bhattacharjee MS, *Satyen Deka MS, DNB, *Hemlata Deka MS, *Manav Jyoti Barman DO, DNB,
**Lokesh Jain MS, *Kruto Kalita MS

In the study 178 patients were evaluated and treated by retina along with a positive fungal culture from a single or subsequent
specialists at our centre between 2003-2008. The diagnosis of sample from the same eye.
endophthalmitis was based on presence of pain, inflammation
including anterior chamber cellular reaction and flare and vitreous Intravitreal and topical antibiotics were given to all patients
cells,corneal involvement, decrease in visual acuity and loss of following standard protocol. For empiric gram negative organism
media clarity with or without the presence of hypopyon. At all coverage intravitreal amikacin (0.4 mg in 0.1 ml) or ceftazidime
visits, the ocular examinations included best corrected visual acuity, (2.25 mg in 0.1 ml) were given. For empiric gram positive organism
slitlamp biomicroscopy (anterior segment examination and 90 D coverage Vancomycin (1mg in 0.1 ml) was given. In the case of
examination of posterior segment) and indirect ophthalmoscopy fungal micro organism being isolated intravitreal amphotericin B
for posterior segment examination. Posterior segment evaluation (0.005 mg in 0.1 ml) was given.
with B scan USG was carried out where ever possible.Information
collected include history of the precipitating cause,visual status at Results
presentation,interval between development of symptoms and
treatment at our centre,microbiological profile of organisms Out of 178 endophthalmitis cases 104 (58% ) cases were post
cultured , interventions undertaken and final visual outcome and operative, 53 (30% ) post traumatic, 11 (6%) endogenous and 10(6%)
ocular status. The primary treatment was always as per the post corneal ulcer perforation cases. Out of 104 postoperative
recommendation of the Endophthalmitis Vitrectomy Study (EVS). endophthalmitis cases 9 cases were diagnosed as fibrinous uveitis.
Briefly, this included injection of intravitreal antibiotics to all There were 3 post operative cases from our centre.
patients where the presenting visual acuity was hand movement
close to face (HMCF) or better and eyes with vision less than Postoperative endophthalmitis
HMCF but with corneal involvement severe enough to preclude
vitreous surgery. In vitrectomy cases the primary goal of surgery The predisposing surgeries included phacoemulsification surgery
was to remove the core vitreous along with vitreous debris. Before (21 cases, 22%), ECCE (31 cases, 33%), SICS (29 cases,31%), phaco
vitrectomy was started 0.5 to 0.1 ml of undiluted vitreous sample with trabeculectomy (1 case,1%), trabeculectomy with MMC (11
was collected from the mid vitreous by manual aspiration with a 2 cases,12%) and VR procedures (2 cases, 2%) (Table 1). The duration
ml syringe for microbiological study. After that the infusion fluid between the precipitating surgery and presentation at the institute
was switched on and vitrectomy started. Intravitreal antibiotics was less than 24 hours in 4 (4.2%), between 1 and 7 days in 33
were injected at the end of the procedure. (34.7%) and greater than 7 days in 58 (61%) of cases (Table 2). The
presenting vision was light perception or worse in 45(47.3%), hand
Immediate inoculation of the undiluted vitreous samples were movement in 25 (26.3%) and better than hand movement in 25
done onto blood agar, chocolate agar, non-nutrient agar, (26.3%) of cases (Table 3). Vitreous biopsy culture was positive in
Sabouraud’s dextrose agar, thioglycolate and brain heart infusion 52 (54.7%) cases, with bacteria cultured in 50 (52.6%) and fungi in
broth. Sabouraud’s agar plates were incubated at 25 deg celcius to 8 (8.4%) of cases (sum is greater than the total of 54.7% because of
enhance growth of fungi while rest of the plates were incubated at polymicrobial cultures). The microbiological profile is presented
37 deg celcius. Blood agar plates were incubated under aerobic
and anaerobic conditions, and chocolate agar was incubated with Table 1: Types of Surgery No: of cases
5% carbon dioxide. Gram’s stain, Giemsa stain and KOH stain
with calcoflour white were done. A culture was considered positive Type of surgery 21
when there was growth of the same organism on two or more 31
media, confluent growth at the site of inoculation on one solid Phaco IOL 29
medium, or growth in one medium with consistent direct ECCE IOL 1
microscopy findings. The final diagnosis was made as bacterial, SICS IOL
fungal, polymicrobial or negative based on the above criteria. Phaco + Trab
Polymicrobial infection was defined as growth on culture of more
than one species or strains of bacteria or a positive bacterial culture

*Sri Sankaradeva Nethralaya Trab + MMC 11
BeltolaGuwahati – 78102 (Assam) VR procedures 2
Total 95
**Jain Eye Hospital
204-A GT Road,Shahdara, Delhi

www.dosonline.org 15

Table 2: Duration of presentation of cases Table 4: Microbiological spectrum of post operative
(Postoperative endophthamitis cases) endophthalmitis cases

Table 3: Presenting visual acuity and final visual acuity Type of micro organism No: cultured
(Post operative endophthalmitis cases)
Gram pos. Cocci 20
Gram neg. Cocci 1
Gram pos. Bacilli 8
Gram neg. Bacilli 15
Fungi 8
Negative 43 (45.2%)
Polymicrobial 5.63%

Table 5:Presenting visual acuity and final outcome

in Table 4. Out of 104 cases 53 cases received intravitreal antibiotics Table 6: Duration of presentation and final outcome
only while 42 cases received both intravitreal antibiotics and
vitrectomy.

At followup 38 (40%) of 95 patients had improvement in vision
while in 57 (60%) cases the vision remained the same or
deteriorated. The anatomical outcome was poor (pthisis bulbi,
evisceration or enucleation) in 12 (12.63%) of cases. In the study it
is seen that the final visual outcome depended on the vision at
presentation and the time interval between the precipitating
surgery and the initiation of treatment at the institute.

Post traumatic endophthalmitis The number of post traumatic At followup 18 (34.9%) of 53 patients had improvement in vision
endophthalmitis cases in the study is 53. The presenting vision while in 35 (65.1%) cases the vision remained same or deteriorated.
was light perception or worse in 36 (67.9%), hand movement in 15 The anatomical outcome was poor (phthisis bulbi, evisceration or
(28.3%) and better than hand movement in 2 (3.7%) of cases (Table enucleation) in 14 (26.4%) of cases.
5). The duration between the precipitating trauma and
presentation at the centre was less than 24 hours in 5 (9.4%), Endogenous endophthalmitis
between 1 and 7 days in 26 (49%) and greater than 7 days in 22
(41.5%) of cases (Table 6). Vitreous biopsy culture was positive in The number of endogenous endophthalmitis cases in the study is
39 (73.5%) cases, with bacteria cultured in 36 (67.9%) and fungi in 11. The presenting vision was light perception or worse in 7 (63.6%),
3 (5.7%) of cases (sum is greater than the total of 73.5% because of hand movement in 3 (27.2%) and better than hand movement in
polymicrobial cultures). Out of 53 cases 26 cases received
intravitreal antibiotics only while 27 cases received both intravitreal
antibiotics and vitrectomy. Table 7 shows the microbiological profile
of organisms cultured from post traumatic endophthalmitis cases.

16 DOS Times - Vol. 14, No. 8, February 2009

Table 7: Microbiological spectrum (Post traumatic perception or worse in 6 (60%), hand movement in 2 (20%) and
endophthalmitis cases) better than hand movement in 2 (20%) of cases (Table 11). The
duration between the development of symptoms and presentation
Type of micro organism No: cultured at the institute was between 1 and 7 days in 5 (50%) and greater
than 7 days in 5 (50%) of cases (Table 12). Vitreous biopsy culture
Gram pos. Cocci 16 was positive in only 5 (50%) cases, with bacteria cultured in 4 (40%)
Gram neg. cocci 5 and fungi in 1 (10%) of cases. Out of 10 cases 8 cases received
Gram pos. Bacilli 12 intravitreal antibiotics only while 2 cases received both intravitreal
Gram neg. bacilli 3 antibiotics and vitrectomy. Table 13 shows the microbiological
Fungi 3 profile of organisms cultured from endophthalmitis cases following
Negative 12 (30.8%) corneal ulcer perforation.
Polymicrobial 9.5%
At followup 3 (30%) of 10 patients had improvement in vision
1 (9%) of cases (Table 8). The duration between the precipitating while in 7 (70%) cases the vision remained same or deteriorated.
surgery and presentation at the institute was between 1 and 7 days The anatomical outcome was poor in 3 (30%) cases.
in 5 (45.5%) and greater than 7 days in 6 (54.5%) of cases (Table 9).
Vitreous biopsy culture was positive in only 2 (18.1%) cases, with Discussion
bacteria cultured in 2 (18.1%) and fungi in 1(9%) of cases (sum is
greater than the total of 18.1% because of polymicrobial cultures). The main objective was to study the profile of endophthalmitis
Out of 11 cases 7 cases received intravitreal antibiotics only while cases presenting at the institute and to observe any clinical
4 cases received both intravitreal antibiotics and vitrectomy. Table presentation features associated with poor visual outcome. The
10 shows the microbiological profile of organisms cultured from microbiological profile of organisms cultured from the involved
post traumatic endophthalmitis cases. eyes was also studied. Among the clinical features at presentation,
importance was laid on visual acuity at presentation and duration
At followup 5 (45.4%) of 11 patients had improvement in vision between onset of symptoms and presentation at the institute and
while in 6 (54.5%) cases the vision remained same or deteriorated. to observe whether these two factors had any bearing on the final
The anatomical outcome was poor in 3 (27.2%) cases. outcome. For the purpose of analysis the presenting visual acuity
was divided into three groups. They constituted vision less than or
Endophthalmitis following corneal ulcer perforation equal to light perception, hand movement and better than hand
movement. The duration of presentation was also divided into
In this study the number of cases of endophthalmitis following three categories, which were a duration of less than 24 hours,
corneal ulcer perforation is 10. The presenting vision was light between 1 and 7 days and greater than 7 days.

Among the post operative cases the number of ECCE cases were
highest (31 cases) followed by SICS (29 cases). Visual acuity of less
than or equal to light perception was present in 45 (47.3%) cases
while vision of hand movement and greater than hand movement
accounted for 25 cases (26.3%d”) each. Out of 45 cases having an

Table 8: Presenting visual acuity and final visual acuity (Endogenous endophthalmitis )

Presenting visual acuity No: of cases Improved vision Same/deteriorated vision Poor anatomical outcome

d” LP 7 (63.6%) 3 4 3
HM 3 (27.2%) 2 1 3 (27.2%)
> HM 1
Total cases 1 (9%) 5 (45.4%) 6 (54.5%)
11

Table 9: Duration of presentation and visual outcome (Endogenous endophthalmitis)

Duration of presentation No: of cases Improved vision Same/deterioratedvision Poor anatomical outcome

< 24 hours 5 (45.5%) 3 2 3
1 – 7 days 6 (54.5%) 2 4 3 (27.2%)
> 7 days 5 (45.4%) 6 (54.5%)
Total cases 11

www.dosonline.org 17

Table 10: Microbiological spectrum (Endogenous presenting between 1 and 7 days, 19 (73%) of the cases had a poor
endophthalmitis) outcome. Among the culture positive cases (39) there was a poor
outcome in 27(69.2%) cases while improvement occurred in12
Type of micro organism No: cultured (30.8%) of the cases. As such poor presenting visual acuity and a
culture positivity for any organism are indicative of an unfavourable
Gram pos. Cocci 2 outcome in post traumatic cases. This is in agreement with
Gram neg. Bacteria numerous studies conducted in different parts of the world.
Gram pos. Bacilli 1
Gram neg. Bacilli 9 (81.8%) In the endogenous and post corneal ulcer perforation cases the
Fungi study groups were too small to detect any specific trends.
Negative
Among the different causes of endophthalmitis the two most
important are ocular surgery and trauma. The two however are
different entities. In post traumatic endophthalmitis, the outcome
will largely depend on the circumstances of injury vis a vis the
extent of wound, type of injury, retained IOFB, nature of the
IOFB- factors which have not been included in this study. However

Table 11: Presenting visual acuity and final visual acuity ( endophthalmitis following corneal ulcer perforation )

Presenting visual acuity No: of cases Improved vision Same/deteriorated vision Poor anatomical outcome

d” LP 6 (60%) 1 5 3
HM 2 (20%) 1 1 3 (30%)
> HM 2 (20%) 1 1
Total cases 3 (30%) 7 (70%)
10

Table 12: Duration of presentation and visual outcome (endophthalmitis following corneal ulcer perforation)

Duration of presentation No: of cases Improved vision Same/deterioratedvision Poor anatomical outcome

< 24 hours 52 3 3
1 – 7 days 51 4 3 (30%)
> 7 days 10 3 (30%) 7 (70%)
Total cases

initial visual acuity d” LP there was visual improvement in only 5 Table 13: Microbiological spectrum (endophthalmitis
(11.1%) cases while in 40 (88.8 %) cases the vision remained the following corneal ulcer perforation)
same or deteriorated. There was poor anatomical outcome in 7
(15.5%) cases. Majority of the cases 58 (61 %) presented at the Type of micro organism No: cultured
institute after 7 days and among them the outcome was poor in 41
(70.6 %) cases. Among culture positive cases 67% had a poor Gram pos. Cocci 3
outcome while improvement was present in 33% cases. Therefore
three factors indicative for an unfavourable outcome in Gram neg. cocci 1
postoperative cases in this study were a poor presenting visual
acuity, delayed presentation and culture positivity for any organism. Gram pos. Bacilli 1
This has been established in a large number of studies.
Gram neg. bacilli 1
Post traumatic cases presenting with a vision of d” LP was 36
(67.9 %) out of which 27 (75 %) cases had a poor visual outcome. Fungi 1
The anatomical outcome was poor in 11 (30.5 %) cases. The
number of cases presenting late (> 7 days) were 22 (41.5%) out of Sterile culture 5 (50%)
which 15 (68.5%) cases had no improvement of vision. The
anatomical outcome was poor in 8 (36.3%) cases. The outcome At followup 3 (30%) of 10 patients had improvement in vision
was also poor in cases presenting within 7 days. Out of 26 cases while in 7 (70%) cases the vision remained same or deteriorated.
The anatomical outcome was poor in 3 (30%) cases.
18
DOS Times - Vol. 14, No. 8, February 2009

as regards post operative Endophthalmitis one can go a long way 7. Gupta A, Gupta V, Gupta A, Dogra MR, Pandav SS. Spectrum and
in saving the eye by early recognition of the problem and timely clinical profile of post cataract surgery endophthalmitis in North
intervention. India. Indian J Ophthalmol 2003;51:139-45.

References 8. Javitt JC, Vitale S, Canner JK, Street DA, Krakauer H, McBean M,
et al. National outcomes of cataract extraction. Endophthalmitis
1. Jalali S, Das T, Gupta S. Presumed noninfectious endophthalmitis following in-patient surgery. Arch Ophthalmol 1991;109:1085-89.
after cataract surgery. J Cataract Refract Surg 1996;22:1492-97.
9. TThompson WS, Rubsamen PE, Flynn HW, Schiffman J, Cousins
2. Speaker MJ, Menikoff JA. Prophylaxis of endophthalmitis with SW. Endophthalmitis after penetrating trauma: risk factors and
topical povidone- iodine. Ophthalmology 1992;98:1769-75. visual acuity outcomes. Ophthalmology 1995;102:1696-701.

3. Endophthalmitis Vitrectomy Study Group. Results of the 10. Williams DF, Mieler WF, Abrams GW, Lewis H. Results and
endophthalmitis study group. A randomized trial of immediate prognostic factors in penetrating ocular injuries with retained
vitrectomy and of intraocular antibiotics for the treatment of post- intraocular foreign bodies. Ophthalmology 1988;95:911-16.
operative bacterial endophthalmitis. Arch Ophthalmol
1995;113:1479-96. 11. Thompson JT, Parver LM, Enger CL, Mieler WF, Liggett PE.
Infectious endophthalmitis after penetrating injuries with retained
4. Wisniewski SR, Hammer ME, Grizzard WS, Kelsey SF, Everett D, intraocular foreign bodies. Ophthalmology 1993;100:1468-74.
Packo KH, et al. An investigation of the hospital charges reltaed to
the treatment of endophthalmitis in the endophthalmitis vitrectomy 12. Alfaro DV, Roth D, Liggett PE. Post-traumatic endophthalmitis:
study. Ophthalmology 1997;104:739-45. Causative organisms, treatment, and prevention. Retina
1994;14:206-11.
5. Kunimoto DY, Das T, Sharma S, Jalai S, Majji AB, Gopinathan U, et
al. Microbial spectrum and susceptibility of isolates. Part I. Post- 13. Jalali S, Das T, Majji AB. Hypodermic needles: a new source of
operative endophthalmitis. Am J Ophthalmol 1999;128:240-42. penetrating ocular trauma in Indian children. Retina 1999;19:213-
17
6. Anand AR, Therese LK, Madhavan HN. Spectrum of aetiological
agents of postoperative endophtalmitis and antibiotic susceptibility 14. Das T,Kunimoto DY,Sharma S,Jalali S, Majji AB,Gopinathan U,Rao
of bacterial isolates. Indian J Ophthalmol 2000;48:123-28. TN:Relationship between clinical presentation and visual outcome
in postoperative and post traumatic endophthalmitis in South
Central India.IJO 2005;53:5-16

First Author
Harsha Bhattacharjee MS

Answer Quiz No. 8

www.dosonline.org
4. TURNER 3. HORNER 2. BROWN 1. DUANE
8. WAARDENBURG 7. CENTURION 6. MOBIUS 5. REITER
Extra Word: SYNDROME

19

Benign Intracranial Hypertension Retina

Ronak P. Bhandari MS, Nayana A. Potdar MS, Chhaya A. Shinde MS, Pallavi G. Sarate MS

The presentation of a patient with increased intracranial abduction. It was bilateral sixth nerve palsy (Figure 2, 3). Anterior
pressure and papilloedema is an emergency. Although this segment was within normal limits except pupils were bilaterally
scenario may be the harbinger of a brain mass, other clinical entities equal, 3 mm in size and sluggishly reacting to light. On fundus
should be considered. A significant number of patients presenting examination both eyes showed that the disc margins were blurred,
in the above fashion are diagnosed with the syndrome of discs were hyperemic (Figure 4). Retinal veins were dilated,
pseudotumor cerebri. Although idiopathic intracranial tortuous. Spontaneous venous pulsations were absent. There were
hypertension, pseudotumor cerebri, and benign intracranial flame shaped retinal hemorrhages near the disc. There was bilateral
hypertension are synonymous with this diagnosis, the preferred severe papilloedema. Visual fields was not possible due to
term is idiopathic intracranial hypertension. uncooperative patient.

The diagnostic criteria include the following: symptoms and signs A CT scan brain was done which was within normal limits
restricted to those of elevated intracranial pressure; normal findings (Figure 5). Routine blood investigations like complete blood count,
on neuroimaging studies, excluding nonspecific findings of raised blood sugars, liver, renal functions and serum electrolytes were
intracranial pressure; and increased cerebrospinal fluid pressure within normal limits. X-ray chest was within normal limits. Patient
with a normal composition.

Case Report

A 19 year old male presented with frontal headache usually more
in the morning since 1 month, which aggravated 2 days before
presentation. He had deviation of eyes since 1 week which was
noticed by his parents followed by diplopia and blurring of vision
then. There was no history of fever/ loss of consciousness/
convulsion. There was no history of trauma or any medication.

On examination the vitals parameters were stable. Vision of the
patient was FC 10 ft in both eyes.

On external ocular examination, both eyes showed evidence on Figure 2: Right Lateral Rectus Palsy
non comitant convergent squint 20 degrees (Figure 1). Abduction
was restricted in both eyes. In the right eye there was moderate
restriction and in the left eye there was severe restriction of

Figure 1: Pre-Op Photo on Presentation Figure 3: Left Lateral Rectus Palsy

Lokmanya Tilak Memorial General Hospital, 21
Sion Mumbai

www.dosonline.org

Figure 4: Papilloedema Figure 5: Normal CT Scan

was transferred to neuromedicine. CSF cytology and biochemical brain tumor without a brain tumor being present (pseudo meaning
studies done were within normal limits. CSF culture was negative false). The space around the brain is filled with water-like fluid.
and meningitis was ruled out. However CSF manometry showed This space containing the fluid cannot expand. It is high pressure
pressures greater than 45 cm H2O. (Normal is between 15-25 cm in this space that produces the symptoms of idiopathic intracranial
H2O). The patient was started on intravenous mannitol and oral hypertension (IIH).
glycerol as measures to reduce the intra cranial pressure. The
ventricular system was normal on MRI brain with orbit. A Incidence
diagnosis of pseudotumor cerebri was established and patient
was taken for Right sided theco-peritoneal shunt surgery. Studies of American-based populations have estimated that the
(Figure 8) On post op day 2 the CSF pressure was recorded as 22 incidence of pseudotumor cerebri ranges from 0.9-1.0 per 100,000
cm H2O and the symptoms of the patient improved dramatically in the general population. This incidence rate increases to 1.6-3.5
(Figure 6). The vision on day 7 was both eyes 6/24. On fundus per 100,000 in women, and 7.9-19 per 100,000 in women who are
examination the disc edema was less in both eyes (Figure 7). Retinal overweight.1
hemorrhages were getting resolved. Disc hyperemia was reduced.
Papilloedema was resolving in both eyes. A strong predilection of this disease exists for women. In the
literature, the female-to-male ratio ranges from 2:1 to 10:1.
The vision after 6 weeks was 6/9 uncorrected in both eyes. The
patient was advised regular follow up. His vision and field changes Although idiopathic intracranial hypertension may affect
were monitored. individuals of any age, most patients with this disease present in
the third decade of life. Idiopathic intracranial hypertension does
Discussion occur in the pediatric population; these younger patients are often
not obese.
Idiopathic Intracranial Hypertension is a condition of high pressure
in the fluid around the brain. It is also known as pseudotumor Symptoms and Signs
cerebri because there are some of the signs and symptoms of a
The symptoms most commonly reported by IIH patients followed
by their frequency are

Figure 6: Post Operative Photo
22 DOS Times - Vol. 14, No. 8, February 2009

Figure 7: Post-Op Fundus Figure 8: Scar of Surgery

Headache (94%): Headache is present in nearly all patients with Table 1: Modified Dandy Criteria2
IIH and is the usual symptom for which patients seek medical
attention. The headaches of the IIH patient are usually severe • Signs & symptoms of increased ICP - CSF pressure
throbbing and daily. They are different from previous headaches, >25 cmH2O
may awaken the patient and usually last for hours. Nausea and
vomitting less common. The headache is often the worst head • No localizing signs with the exception of abducens
pain ever experienced. Although uncommon, the presence of pain nerve palsy
behind the eyeball that is worsened with the movements of the
eyes can occur. With Valsalva maneuver the intra cranial pressure • Normal CSF composition
increases and hence the symptoms.
• Normal to small (slit) ventricles on imaging with no
Transient visual obscurations: Visual obscurations are episodes of intracranial mass
transient blurred vision that usually last less than 30 seconds and
are followed by full recovery of vision. Visual obscurations occur to subretinal hemorrhages are poor visual prognostic signs. In
in about 3/4 of IIH patients. The attacks may be involving one or some instances, the disc swelling is asymmetric, or, rarely, the
both eyes. They are not correlated with the degree of intracranial appearance of the optic nerve may be relatively normal.
hypertension or with the extent of optic nerve swelling. Visual
obscurations do not appear to be associated with poor visual If left untreated, chronic disc swelling eventually leads to clinically
outcome. significant visual loss. Although all patients present with enlarged
blind spots during their initial perimetry, uncontrolled papilledema
Pulsatile intracranial noises: Pulsatile intracranial noises or pulse- results in progressive peripheral visual field constriction or nerve
synchronous tinnitus is common in IIH. The sound is often fiber bundle defects (eg, nasal depression, nasal steps, arcuate
unilateral. In patients with intracranial hypertension, compression scotomas). The central visual field is affected in end-stage chronic
of the jugular vein on the side of sound abolishes it. The periodic papilledema.
compressions were thought to convert the laminar blood flow to
turbulent. Sudden loss of central vision may result from an associated anterior
ischemic optic neuropathy, a vascular occlusion, or an associated
Visual loss: The most serious problem patients have is vision loss. subretinal neovascular membrane.
About 5% of patients go blind in at least one eye. These are usually
patients who do not return for follow-up evaluation. The diplopia noted in patients with idiopathic intracranial
hypertension is invariably due to unilateral or bilateral sixth nerve
Diplopia: Patients who present with double vision most frequently palsy. These cranial nerve palsies diminish with the lowering of
complain of horizontal displacement of the images. Vertical diplopia the intracranial pressure. Occasionally, patients with diplopia
is rare, but it has been reported present with oculomotor or trochlear nerve palsy. In rare instances,
vertical diplopia is due to a skew deviation.
Physical
Causes
The most significant finding in patients with this disease is bilateral
disc edema secondary to the increased intracranial pressure. This Most cases of pseudotumor cerebri occur in young women who
papilledema varies from patient to patient and is indistinguishable are obese and, less frequently, in men who are otherwise healthy.
from optic nerve swelling caused by intracranial space-occupying If this disorder presents in an individual who is not overweight,
lesions. In more pronounced cases of disc swelling, macular ruling out associated risk factors is necessary.
involvement with subsequent edema and diminished central vision
may be present. High-grade and atrophic papilledema in addition

www.dosonline.org 23

Risk factors • Drusen of the optic nerve heads

Exogenous substances • Malignant hypertension

• The list of exogenous substances associated with idiopathic • Bilateral infiltrative/infectious/inflammatory optic neuropathy
intracranial hypertension is extensive. Although an association
exists between these substances and this disorder, the exact • Bilateral anterior ischemic optic neuropathy
causal relationship is somewhat lacking in the literature.
• Bilateral optic nerve papillitis
• Exogenous substances associated with idiopathic intracranial
hypertension include amiodarone, antibiotics (eg, nalidixic • Bilateral optic nerve tumors (eg, glioma, meningioma).
acid, penicillin, tetracycline), carbidopa, levodopa,
chlordecone, corticosteroids (eg, topical, systemic), Complications
cyclosporine, danazol, growth hormone, indomethacin,
ketoprofen, lead, leuprolide acetate, levonorgestrel implants, The main complication of this disorder is progressive optic
lithium, oral contraceptives, oxytocin, perhexiline, phenytoin, neuropathy. Despite timely treatment, some patients develop an
and vitamin A (>100,000 U/d)/retinoic acid. optic neuropathy related to the optic nerve edema. Generally, this
dysfunction presents in a progressive fashion with constriction of
• Ironically, withdrawal from corticosteroids may result in the peripheral visual field; worsening nerve fiber bundle visual
idiopathic intracranial hypertension. field defects; a decrease of color vision; and, in end-stage disease,
a drop in the central visual function. Occasionally, a patient may
Systemic diseases develop an acute loss of vision due to ischemic optic neuropathy
associated with papilledema.
• The following diseases have been associated with idiopathic
intracranial hypertension: anemia, chronic respiratory Pathophysiology
insufficiency, familial Mediterranean fever, hypertension,
multiple sclerosis, polyangiitis overlap syndrome, psittacosis, The pathophysiology of this disorder is unclear. A relative
renal disease, Reye syndrome, sarcoidosis, systemic lupus resistance to the absorption of cerebrospinal fluid across the
erythematosus, and thrombocytopenic purpura. arachnoid villi is widely presumed to be present. Other theories
support an abnormality in the cerebral circulation with a resulting
Disorders of cerebral venous drainage increase in the brain's water content. The subsequent increase in
the intracranial pressure is transmitted to the structures within
• Cerebral venous compression by extravascular tumors or the intracranial cavity, including the optic nerves. The disease
secondary thrombosis. commonly occurs in women who are overweight. The role of
obesity in this disorder is unclear. Obesity has been proposed to
• Radical neck dissection, even if completed only on the right increase intra-abdominal pressure, which, in turn, raises cardiac
side (predominant drainage from the head is via the right filling pressures. This rise in pressure leads to impeded venous
jugular vein). Spontaneous recanalization usually occurs, but, return from the brain (due to the valveless venous system that
if delayed, chronic papilledema may result. exists from the brain to the heart) with a subsequent elevation in
intracranial venous pressure. If not treated appropriately, chronic
Endocrine disturbances interruption of the axoplasmic flow of the optic nerves with ensuing
papilledema due to this pressure may lead to irreversible optic
• Pregnancy is occasionally associated with idiopathic neuropathy.3
intracranial hypertension. This disorder can present at any
stage of pregnancy. Mortality and Morbidity

Differential Diagnosis The morbidity of this disorder is mainly related to the effects of
papilledema. If left untreated, long-standing disc edema results in
Bilateral optic nerve head edema is presumably due to increased an irreversible optic neuropathy with accompanying constriction
intracranial pressure secondary to an intracranial tumor until of the visual field and loss of color vision. In end-stage papilledema,
proven otherwise. Ruling out malignant hypertension prior to central visual acuity is also involved.4
pursuing the diagnostic trail of pseudotumor cerebri is always
worthwhile. Conclusion

If the patient's blood pressure is normal or minimally to moderately The diagnosis of IIH is made by identifying the typical symptoms
elevated, obtaining urgent neuroimaging studies to rule out a of the disease along with documentation of a high spinal fluid
space-occupying lesion or a dural sinus thrombosis is essential. If pressure (done during a gaurded spinal tap). The neurologic
the neuroimaging study does not demonstrate any pathology and examination is normal except for the presence of papilledema.
if a lumbar puncture demonstrates a raised opening pressure, Sometimes double vision occurs, caused by limitation of lateral
then one of the above-mentioned causes of increased intracranial eye movement. Lastly, neuroimaging procedures such as CT scans
pressure must be considered. or MRI scanning are normal.The best way to prevent visual loss is
to test vision regularly as well as assess visual fields. Patients should
In the absence of a specific offending agent, the diagnosis of the be followed frequently with tests of vision until the doctor is
patient is presumably idiopathic intracranial hypertension. confident that there is no vision loss occurring. Vision testing
should then be done once or twice a year or whenever new
• Pseudopapilledema

24 DOS Times - Vol. 14, No. 8, February 2009

symptoms occur. Unfortunately, IIH is a life-long disease and tends Procedures for Idiopathic Intracranial Hypertension in the United

to occur during periods of weight gain. The symptoms though are States, 1988-2002". Neurosurgery 57 (1): 97-108. PubMed. Retrieved
very treatable and, if treatment is started early enough, the vision on 2006-11-08.

loss is reversible. 2. Friedman, Deborah I.; Daniel M. Jacobson (November 26, 2002).

Although rare a strong suspicion of IIH in chronic severe headaches "Diagnostic criteria for idiopathic intracranial hypertension".
and immediate investigations followed by proper surgery can Neurology 59 (10): 1492-1495. PubMed. Retrieved on 2006-11-08.

salvage the vision of the patient even in established papilloedema. 3. Brazis PW, Lee AG: Elevated intracranial pressure and pseudotumor

Besides the vetriculo peritoneal shunt another surgical option cerebri. Curr Opin Ophthalmol 1998 Dec; 9(6): 27-32

would have been optic nerve sheathotomy. 4. Friedman DI, Jacobson DM: Idiopathic intracranial hypertension. J

References Neuroophthalmol 2004 Jun; 24(2): 138-45.

1. Curry, William T. Jr.; William E. Butler, Fred G. Barker (July 2005).
"Rapidly Rising Incidence of Cerebrospinal Fluid Shunting

First Author
Ronak P. Bhandari MS

Congratulations

Congratulations to Dr. A.K. Grover who has been conferred Padma Shri by the Government of India.

Congratulations “Dr. Amit Tarafdar” for receiving International ophthalmologist Education
Award from American Academy of Ophthalmology.

Congratulations “Dr. Ranjeet Kishore Rana” for receiving “Best Paper Award” in cataract and
refractive surgery at the SAARC conference 2008 held at Delhi.

www.dosonline.org 25

Orthokeratology Cornea

Kirti Singh MD, DNB, FRCS(E)

It is the temporary reduction or modfification of myopia achieved How does it work?
by the programmed application of specially designed contact
lenses which reshape the cornea. A reversible technique it involves Tear film meniscus behind the contact lens hydraulically
use of an overnight lens-wearing protocol. Orthokeratology has redistributes epithelial cells under the lens from the centre towards
been around since 1960 when George Jessen created the first periphery.
orthokeratology design from PMMA material, These early lenses
used were conventional flat-fitting, rigid contact lenses and induced The central flat base curve exerts a positive pressure on the central
significant with-the-rule corneal toricity due to decentration. 1, 2 corneal epithelium. As a consequence of this central corneal
With the advent of reverse-geometry lens designs made of high epithelium is thinned out. The steep secondary curve , creates an
Dk material in late 1980 this undesirable effect has decreased annular tear pool which by virtue of negative pressure leads to
drastically3,4,5,6 mid peripheral corneal epithelial and stromal thickening (Figure
2)8, 9,10, 11 Reduction in corneal epithelial thickness by 20 microm
Reverse-geometry lenses, propagated by Wlodyga and Stoyan, or more has been documentedover a 3 month period of ortho K
are fitted with a base curve flatter than the central corneal curvature. wear.12
The flat base curve applies pressure to the central treatment zone,
achieving sphericalization of the prolate cornea and resulting in The end result is a reduction in corneal convexity and sagittal
reduction of myopic refractive error. To aid centration a secondary corneal height, which effectively reduces myopic error. Age plays
curve is ground which is steeper than the central base curve. 7 a direct role in this corneal shaping as this response has been
noted to be rapid in children and young adults but reduced and
delayed in older people. 11 (Figure 3)

Reverse geometry design of Ortho-k LK lenses

Central Back Optic Zone Radius (BOZR)

The radius of curvature of this zone is calculated as the flat K
minus the desired amount of correction minus the compression
factor of 0.75D.

Figure1: 5 Curve Reverse geometry Lens Reverse curve 2nd Curve

The steep reverse curve is calculated to align back surface of the
lens with cornea.

Alignment curve or 3rd and 4 th Curve

This 1.0 to 1.5 mm wide bearing zone is made flatter than the
reverse curve, thereby closely aligning the peripheral cornea. This
curve helps in centering the lens and enhancing the orthokeratology
effect.

Figure 2: Corneal reshaping Figure 3: 5 Curve Reverse geometry
Lens bulls eye fluorescein pattern
Guru Nanak Eye Centre,
Maulana Azad Medical College, 27

New Delhi

www.dosonline.org

Peripheral curve Select initial lens from Trial Kit

This aids lens lift, ensures adequate tear exchange and helps in This initial lens is based on the adjusted flat K. Flat k is adjusted

removal of lens. with eccentricity “e “as per table provided.

Material Eccentricity range 0.46 to 0.54 , flat K value is not adjusted.

Since this lens is worn during sleep only high Dk material ( > 85) Centration of the shaping lens is evaluated on the
is compatible with allowing sufficient oxygen transmission to fluorescein pattern
prevent corneal hypoxia like Boston XO
Centration is critical to achieve the ortho-k effect. Decentered
It can be used for which types of patients: lenses do not produce the desired myopia reduction, in addition
they distort the cornea. A good fluorescein pattern is a bull’s eye
• Age - juvenile to adult myopes pattern , with a central zone of light touch of 3.5 - 4.0 mm in
diameter, a mid peripheral ring of fluorescein pooling, or ‘tear
• Range : -1.00D to -6.00D reservoir’, under the steeper secondary curve and a peripheral
circular band of alignment tapering to the edge lift. (Figure 3)
• Cylindrical error -2.00D or less with-the-rule corneal
astigmatism OR < -0.75D against-the-rule astigmatism. A well fitting lens should move approximately 1mm with blinking

• Flat K range - 40.00D(8.44mm)~45.50D(7.42mm) Vision Examination

Refractive endpoint is typically reached with these lenses after 7 to Remove lenses after 1 hour of wearing. Evaluate patient’s visual
10 days of wear with use of an overnight 7-8 hour lens-wearing acuity before and after lens wearing.
protocol.3,4

Fitting Process Variation of effect on the basis of Eccentricity “e “

Corneal topography The closer the “e” value is to zero, the more spherical the cornea

Evaluate corneal size, Flat-K value and eccentricity. • Higher the “e” value, more rapidly does the cornea flatten
towards the periphery, thus the potential to flatten central
Flat-K value: Radius of curvature for centre. Radius of curvature corneal curvature is greater.
for periphery also needs to be assessed by adapting corneal
eccentricity with flat-k value. • In low eccentricity, oblate corneas the effect of Ortho K is
reduced.
Corneal Eccentricity “e “: defined as the rate at which the cornea
flattens from the central area to the peripheral area. Some examples of ortho K trial fits;

Corneal size: this determines which part of cornea comes in contact Good Centration- fluorescein fit and topography picture (Figure 4a
with the alignment curve. Normal corneal size of over 11.6 mm & 4b)
requires a 10.6 mm diagnostic/ inventory lens.
• Lens movement is quick and proper as natural. (1-2mm)

Figure 4a: Good Centration Figure 4b: Fluorescein fit and
28 topography picture

DOS Times - Vol. 14, No. 8, February 2009

Figure 5a: Poor Figure 5b: Fluorescein fit and
Centration (Steep Fit) topography picture

• Base Curve Zone Touch: Central black part (4~6mm) Some examples

• Reverse Curve Zone: Green colored ring with equal width Case I: 21-year-old female, -3.50D myopia.

• Alignment Zone : Black part with equal width • Keratometry: (Rh):- 7.48mm (Rv):-7.61mm (flat-K value)

Poor Centration (Steep Fit) fluorescein fit and topography picture • Corneal topography: Cornea size - 12.3mm.
(Figure 5a & 5b) • Eccentricity - 0.49(0.46-0.54)
Thus trail lens selected would be :
• Lens movement is slight (1mm“!) and rides low • Eccentricity as less than 0.5 therefore No need to need to

• Base curve zone Touch: Touch zone narrow. ( < 4mm) adjust flat-K value.
• Initial trial lens selected = 7.61
• Reverse Curve Zone: Air bubble/ thick ring • Initial lens selected
• 10.6mm/7.63/-3.50D OR 10.6mm/7.58/-3.50D
• Alignment Zone: Very black part

Poor Centration ( Flat Fit) fluorescein fit and topography picture
(Figure 6a & 6b)

• Lens movement is excessive • Fluorescein pattern: check centering and alignment.

• Base curve zone Touch zone is wide over 5mm • Tear meniscus size and shape, lens movement

• Reverse Curve Zone: Green ring has irregular width If lens adheres, air bubbles would be noted in the reverse curve
• Alignment Zone: Not black, with fluorescein (reservoir) zone then - change the central bearing (make the fit
steeper)

Eccentricity Trial set - Flat K Flat K Adjustment Power Adjust

Eccentricity 0.50-0.75D steep 43.50(7.76)-43.75(7.72) -3.50~-3.75D
0.00-0.25D steep 43.00(7.85)-43.25(7.80) -3.00~-3.25D
0.30 - 0.39 Flat K 43.00D(7.85mm) -3.00D
0.40 - 0.45 0.25-0.50D flat 42.75(7.90)-42.50(7.94) -2.75~-2.50D
0.46 - 0.54 0.50-0.75D flat 42.50(7.94)-42.25(7.99) -2.50~-2.25D
0.55 - 0.60 0.75-1.00D flat 42.25(7.99)-42.00(8.04) -2.25~-2.00D
0.61 - 0.65
0.66 - 0.70

www.dosonline.org 29

Figure 6a: Poor Figure 6b: Fluorescein fit and
Centration (Flat Fit) topography picture

Figure 7: Topography Image after One Hour
(a) Initial Map (b) After 1H Wearing

Evaluate ortho-k effect Lens prescription and dispensing

• After 1 hour wearing, remove initial lens. Finally the lens with Corneal Diameter 10.6mm/7.58/-3.50D has

• Check unaided visual acuity, Slit Lamp examination and been ordered and was dispensed to the patient.

corneal topography. Does this technology work?

• Perform Over-refraction to determine lens’ target power Unaided vision improvement and reduction of the myopic
refractive error have been documented now by many authors in
It is observed that her visual acuity before lens wearing was 6/36) different ethnicities.13,14,15,16
and after lens wearing is 6/6 .
The average percentage reductions in spherical equivalent have
Topography Image after One Hour been noted to be in the range of 84 - 92% and errors of till -5D
have been corrected.15,16
We can see that here red rounded part (corresponds to the
alignment curve) is centered after 1hour of wear (Figure 7a & 7b). A multicentre study recently found evidence to state that overnight
After 3 days, this red rounded part becomes more evident. orthokeratology is efficacious and safe for young myopic
(Figure 8) patients.17,18

30 DOS Times - Vol. 14, No. 8, February 2009

2. Binder PS, May CH, Grant SC. An evaluation of orthokeratology.
Ophthalmology 1980;87:729-44.

3 Nichols JJ, Marsich MM, Nguyen M, et al. Overnight
orthokeratology Optom Vis Sci 2000;77: 252-9.

4. Swarbrick HA, Alharbi A. Overnight orthokeratology induces central
corneal epithelial thinning. Invest Ophthalmol Vis Sci 2001;42:S597

5. Mountford J. An analysis of the changes in corneal shape and
refractive error induced by accelerated orthokeratology. ICLC
1997;24:128-44.

6. Lui W-O, Edwards MH. Orthokeratology in low myopia. Part 1:
efficacy and predictability. Cont Lens Anterior Eye 2000;23:77-89.

Figure 8: After 3 Days 7. Wlodyga RJ, Bryla C. Corneal molding: the easy way.Contact Lens
Spectrum 1989;4:58-65.

8. Swarbrick HA. Orthokeratology (corneal refractive therapy): what
is it and how does it work? Eye Contact Lens. 2004 Oct; 30(4):181-
5; discussion 205-6.

9. Alharbi A, Swarbrick HA. The effects of overnight orthokeratology

This has been confirmed by a large cohort studies evaluated over lens wear on corneal thickness. Invest Ophthalmol Vis Sci. 2003

a long term period of at least 12 months.14,19 Jun;44(6):2518-23.

Problems encountered with this lens 10. Walline JJ, Holden BA, Bullimore MA et al. The current state of
corneal reshaping. Eye Contact Lens. 2005 Sep;31(5):209-14.
Corneal staining was the most commonly observed complication
with ortho-k and more than 80 per cent of patients were advised 11. Jayakumar J, Swarbrick HA. The effect of age on short-term
to apply ocular lubricants.20 orthokeratology. Optom Vis Sci. 2005 Jun;82(6):505-11.

Another optical problem noted is decreased corneal optical quality 12. Soni PS, Nguyen TT, Bonanno JA. Overnight orthokeratology:
in terms of increased higher-order aberrations and decreased visual and corneal changes. Eye Contact Lens. 2003 Jul;29(3):137-
contrast sensitivity. This correlates with the magnitude of myopic 45
correction.21, 22,23 However, these induced optical higher order
aberrations along with the improvement in uncorrected visual 13. Johnson KL, Carney LG, Mountford JA, Collins MJ, Cluff S, Collins
acuity are fully reversible after discontinuation of the Ortho K PK.Visual performance after overnight orthokeratology. Cont Lens
lens even after 1 year of use.23 Anterior Eye. 2007 Mar;30(1):29-36. Epub 2007 Jan 9.

Lens decentration during overnight wear can lead to diplopia, 14. Soni PS, Nguyen TT; XO Overnight Orthokeratology Study Group.
glare and reduction in the orthokeratology effect. In a study Overnight orthokeratology experience with XO material. Eye
involving 270 eyes over a 6 month period of OrthokK lens wear Contact Lens. 2006 Jan;32(1):39-45.
decentration was more in patients with greater initial astigmatism
and use of smaller lenses. 24 15. Cheung SW, Cho P, Chui WS, Woo GC. Refractive error and visual
acuity changes in orthokeratology patients. Optom Vis Sci. 2007
Microbial keratitis is the most severe, potentially vision-threatening May;84(5):410-6.
adverse response associated with orthokeratology contact lens
wear. The organisms identified have been Pseudomonas 16. Chan B, Cho P, Cheung SW Orthokeratology practice in children in
aeruginosa and Acanthamoeba species. 25,26 It has been associated a university clinic in Hong Kong. Clin Exp Optom. 2008
with the use of tap water in care regimens for overnight Sep;91(5):453-60. Epub 2008 Mar 18.
orthokeratology and the patient must be forewarned against using
tap water. 17. Walline JJ, Rah MJ, Jones LA The Children’s Overnight
Orthokeratology Investigation (COOKI) pilot study. Optom Vis
Conclusions Sci.2004 Jun;81(6):407-13.

Ortho-K lenses are effective for myopia correction by overnight 18. Rah MJ, Jackson JM, Jones LA, Marsden HJ, Bailey MD, Barr JT
wearing about 7 hours. Refractive endpoint is typically reached Overnight orthokeratology: preliminary results of the Lenses and
after 7 to 10 days of wear with the use of an overnight lens-wearing Overnight Orthokeratology (LOOK) study. Optom Vis Sci. 2002
protocol. Under close monitoring, overnight ortho-k is effective Sep;79(9):598-605.
and safe for reducing low to moderate myopia and the treatment
is well accepted by the children. 19. Van Meter WS, Musch DC, Jacobs DS, Kaufman SC, Reinhart WJ,
Udell IJ.Safety of overnight orthokeratology for myopia: a report
References by the American Academy of Ophthalmology. Ophthalmology.
2008 Dec;115(12):2301-2313.e1. Epub 2008 Sep 20.
1. Kerns RL. Research in orthokeratology. Part VIII: results, conclusions
and discussion of techniques. J Am Optom Assoc 1978;49:308-14. 20. Chan B, Cho P, Cheung SW.Orthokeratology practice in children in
a university clinic in Hong Kong. Clin Exp Optom. 2008
Sep;91(5):453-60. Epub 2008 Mar 18.

21. Hiraoka T, Okamoto C, Ishii Y, Kakita T, Okamoto F, OshikaT.
Time course of changes in ocular higher-order aberrations and contrast

www.dosonline.org 31

sensitivity after overnight orthokeratology. Invest Ophthalmol Vis 24. Yang X, Zhong X, Gong X, Zeng J. Topographical evaluation of the
Sci. 2008 Oct;49(10):4314-20. Epub 2008 May 23. decentration of orthokeratology lenses. Yan Ke Xue Bao. 2005
Sep;21(3):132-5, 195.
22. Hiraoka T, Okamoto F, Kaji Y, Oshika T. Optical quality of the
cornea after overnight orthokeratology.Cornea. 2006 Dec;25(10 25. Watt KG, Swarbrick HA. Trends in microbial keratitis associated
Suppl 1):S59-63. with orthokeratology. Eye Contact Lens. 2007 Nov;33(6 Pt 2):373-
7; discussion 382.
23. Hiraoka T, Okamoto F, Kaji Y, Oshika T…Hiraoka T, Okamoto C,
Ishii Y, Okamoto F, Oshika T. Recovery of corneal irregular 26. Shehadeh-Masha’our R, Segev F, Barequet IS, Ton Y, Garzozi HJ.
astigmatism, ocular higher-order aberrations, and contrast sensitivity Orthokeratology associated microbial keratitis. Eur J Ophthalmol.
after discontinuation of overnight orthokeratology. Br J Ophthalmol. 2009 Jan-Feb;19(1):133-6.
2008 Nov 19. [Epub ahead of print]

Author
Kirti Singh MD, DNB, FRCS(E)

32 DOS Times - Vol. 14, No. 8, February 2009

Goldenhar Syndrome: A case report & Cornea
review of literature

Uday Gajiwala MS, Rajesh Patel MS, Manoj Gangwal DO, FCPS

Goldenhar syndrome also called oculo auriculo vertebral CVS & CNS examination was normal.
dysplasia or first & second branchial arch syndrome, is mostly
unilateral, occurs most commonly in men and involves anomalies X-ray cervical spine was not showing occipitalisation of atlas or
of face, ears, eyes and vertebrae in most cases. When hemi vertebrae which is usually associated with Limabal Dermoid
malformations primarily involves the jaw, mouth and ear of one in Goldenhar syndrome.
side, the disorder is referred as Hemifacial microsomia, but if
abnormalities of vertebrae and the eyes are also present, the CT scan was not showing any abnormality.
disorder is often called Goldenhar syndrome.
Patient was not ready for its excision as prognosis for visual
Case History improvement was poor due to meridional amblyopia and she was
not worried about cosmetic problem in this age.
A fifty year female patient with negative family history presented
with dimness of vision in left eye and painless swelling in left eye Thus, the patient was having following findings:
since birth. On ophthalmic examination, Best Corrected Visual
Acuity in right eye was 6/6 but in left eye it was 6/36 not improving • Limbal dermoid
with refraction. There was against the rule astigmatism of 3 dioptre
due to inferolateral limbal dermoid of about 5X5 mm size. She • Abnormalities of ear (pre auricular skin tag)
had 15 degrees concomitant divergent squint with full eyeball
movement. There was no other ocular abnormality. Fundus Discussion
examination was normal in both eyes.
The incidence of this syndrome is 1:3000 to 1:26,500 with onset at
Photographs birth. It occurs in family &/or as sporadic cases. Familial cases are
consistent with autosomal dominant, autosomal recessive and
On ENT examination, patient had pre auricular skin tags which multifactoral patterns of inheritance. Chromosomal abnormality
were lying in line joining tragus & angle of mouth. Patient had no or defective gene disorder is common in men (70%). It can be
complaint of deafness in both the ears. X-ray PNS & Schullers unilateral or bilateral but mostly it is unilateral.
views were also normal with normal external auditory meatus.
Pathogenesis

Genetic defect: abnormal neural crest morphology-malformation
of the derivatives of the first & second branchial arches-facial,
auricular, ocular, vertebrae & other anomalies. Variable phenotypic
expression is characteristic with this syndrome & the spectrum of
phenotypic anomalies can range from mild to severe even within

Limbal Dermoid Pre-Auricular Skin Tag

Sewa Rural, 35
Jhagadia, Bharuch, Gujarat

www.dosonline.org

Normal X Ray Cervical spine Dermoid Showing Hair

the same affected family. It may be present in monozygotic twins Ocular Manifestations
with only one twin being affected. Epibulbar Tumors
• Dermoid or lipodermoids.
Review of Literature • Found in 35% of cases.
• Yellow or pink, solid ovoid masses up to 10 mm in diameter.
First identifies by M.Goldenhar in 1952 • Unilateral or bilateral.
• May impair vision.
First called oculo auriculo vertebral dysplasia by R.J. Gorlin. Others
• Strabismus
Clinical Features • Microphthalmia or anophthalmia.
• Blepharo-phimosis in 10% of cases.
Facial asymmetry

65-70% cases have some facial asymmetry & in 20% of cases the
asymmetry is marked.

The right side of the face is more severely affected in 60% of cases.

10-33% has bilateral facial involvement and/or mandibular regions
on the affected side.

There also may be unilateral hypoplasia of the facial musculature • Narrowing of palpebral fissure
with facial muscle weakness and depressor anguli oris hypoplasia Retinal anomalies.

on the affected side. •

Macrostomia • Upper lid colobomas.

Greatly exaggerated width of the mouth due to lateral cleft like Other manifestations
extension of the corner of the mouth. Neurological
External Ear • Microcephaly or hydrocephaly.
• Occipital encephalocele.
May range from mildly dystrophic ear to microtia to anotia. • Arnold chiari malformation.
Pre-auricular skin tags, pits and/or sinuses and occur between the • Mental retardation in 5-15% of patients.
tragus and the angle of the mouth. • Spina bifida.
Usually unilateral but can be bilateral in 33% of cases. Congenital Heart disease
Middle Ear • VSD, PDA, Tetralogy of Fallot, Coarctation of Aorta.
• Transposition of Great arteries.
Narrow or atretic
Inner Ear

Conductive &/or sensorineural hearing loss.

36 DOS Times - Vol. 14, No. 8, February 2009

Renal Management

• Ectopia &/or fused kidneys. Supportive
• Renal agenesis.
• Multicystic dysplastic kidneys. Multidisciplinary approach
• Hydronephrosis, hydro-ureter, ureteral duplication.
• Uretero-pelvic junction obstruction. • Pediatrics, plastic, orthopaedic, ophthalmic and ENT surgeons
• Vesico-ureteral reflux.
• Early and regular hearing tests.
Musculoskeletal
• Genetic counselling.
• Scoliosis
• Talipes equino varus (club feet) Prognosis
• Bifid or digitalized thumb
• Hypoplastic Thumb Normal life span and intelligence in a majority of those patients
Investigations without significant complications.
Skeletal X-Rays
References
Skull
1. Baraitser M. & R. M. Winter, color atlas of congenital malformation
• Hypoplasia of the maxillary, temporal & malar bones. syndrome (1st edition) P: 40-41 (1996).
• Hypoplasia or absent mandibular ramus & condyles.
• Reduced pneumatization of mastoid region. 2. Baum J. L. & Feingold M., ocular aspects of Goldenhar syndrome.
AJO, 75:250, 1973.
Vertebral
3. Bergsma D. (Ed) Birth defects, atlas and compendium. Baltimore:
• Cervical fusion in 20-25% of cases. Williams & Wilkins.
• Platybasia & occipitalisation of the atlas in 30% of cases.
• Klippel-Feil anomaly. 4. Benjamin S. N. & Allen H. F. classification for limbal dermoids,
• Hemi vertebrae choriostomas & branchial arch anomalies: presentation of an unusual
• Hypoplasia of vertebrae case. Arch Ophthalmology, 87:305, 1972.

CT/MRI 5. Geeraets W. J. ocular syndromes (3rdedi) Philadelphia: Lea & Febiger,
1976. Jones K. L., Smith's Recognizable patterns of human
• Agenesis of or lipoma in corpus callosum malformations (5th edi), P: 642-645 (1997).
• Calcification of falx cerebri
• Hypoplastic septum pellucidum. 6. Journal of Genetics, Human 1:243 (l952).
• Intra-cranial dermoid cyst.
7. Journal of Pediatrics 63:991-999 (1963).

8. Mansour A. M. et. al, ocular findings in facio auriculo vertebral
sequence (Goldenhar Gorlin syndrome) AJO 100:555, 1985.

9. Gorlin RJ, Cohen MM Jr, Levin LS.Oculoauriculovertebral
Spectrum.In: Syndromes of Head and Neck.3rd ed.oxford University
Press: New York; 1990.p.641-9

10. Das A, Mazumdar S, Chatterjee SS.Atypical case of Oculo-Facio-
auriculo-vertebral Dysplasia (Goldenhar Gorlin Syndrome).IJO
1999; 47:131-3

11. A. Das, B.Ray, D.Das, S.Das.A Case of Goldenhar Gorlin syndrome
with unusual association of Hypoplastic thumb.IJO 2008; 56:150-2

First Author
Uday Gajiwala MS

www.dosonline.org 37

Pseudophakic Bullous Keratopathy Cornea

Shreekant A Damgude MBBS, B.P. Guliani MS

Pseudophakic bullous keratopathy refers to the development The lack at adhesive proteins in epithelial basement membrane
of irreversible corneal oedema following cataract surgery which (fibronectin, laminin and collagen type IV) and accumulation of
initially develops in stroma progressing towards intercellular antiadhesive proteins, such as tenacin C and thrombospondin -1,
epithelial region with characteristic formation of bullae. leads to loss of contact of epithelial cells with each other and with
underlying subepithelial tissue, resulting in subepithelial bullae
Though with newer surgical techniques and IOL designs, incidence and fibrosis3.
of this complication has decreased dramatically, it still represents
an important cause of visual disability following routine and Incidence: Current incidence is about 0.1% FDA report on
complicated cataract surgery. intraocular lens (1978-1982)4 is shown in Table 1.

Pathophysiology Table 1: Association of PBK with of intraocular lens

Corneal transparency depends on ability of cornea to remain in Incidence of PBK Type of lens
dehydrated state. Corneal epithelium and endothelium are both 0.06% PC IOL
semipermeable membranes that create a barrier to the flow of 1.2 % AC IOL
water and other electrolytes. 1.5 % Iris fixated lens

The major factor which prevents corneal hydration is Na+K+ AT Long term incidence of PBK has been found to be 15%.
Pase pump which lies within lateral cell membrane in endothelial
layer of cornea. Endothelium is incapable of mitotic activity. At It is also correlated with endothelial cell loss5 in cataract surgery.
birth endothelial cell count is approximately 7500 cells /mm2. In (Table 2)
older adults it is approx. 2500-2700 cells / mm2.
Table 2: Endothelial cell loss in cataract surgery
Normal rate of endothelial cell loss after age of 20 is approx. 0.5 %
per year. Surgical trauma, inflammation and corneal dystrophies ECCE SICS PE
(Fuch's endothelial dystrophies, posterior polymorphous 4.72 % 4.21% 5.41%
dystrophies) can accelerate this normal aging loss.
Clinical features
The final common pathway in development of PBK is damage to
corneal endothelium when cell density reaches to critically low Poor visual acuity: It is due to inability of stroma to maintain
level of about 300-500 cells / mm2. It leads to rearrangement of corneal transparency.
remaining cells to cover posterior corneal surface in the form of
polymegathism (greater than normal variation of corneal Epithelial oedema can be responsible for great changes in visual
endothelial cell size resulting in irregular large and small cells) and activity due to irregularity in corneal refractive surface. Mild stromal
pleomorphism(variation in cell shape; increased proportion of
non-hexagonal cells). Descemetl's membrane is produced in excess
amount.Endothelial pump fails and stromal oedema develops.

Oedema may fluctuate in response to changing I.O.P. ( Imbibition
pressure = I.O.P.- Swelling pressure). Later on epithelial oedema
ensues because of anterior movement of aqueous and fluid in
stroma leading to formation of blisters and bullae. Condition
typically worsens during night because of lack of evaporation and
lack of hypertonic environment.

Histopathology of PBK

Prime feature is attenuation and loss of corneal endothelial cells
with associated epithelial bullae and stromal oedema1.

There is thickening of the posterior collagenous layer of descemet's
membrane and decrease in stromal keratocytes2

Figure 1: Bullous Keratopathy

Department of Ophthalmology 41
Safdarjung Hospital,
New Delhi

www.dosonline.org

Etiology

Preoperative risk factors

Figure 2: Formation of Bulla between epithelial Specular microscopy: Though there is no direct correlation between
basement membrane and Bowman’s membrane endothelial cell density and oedema, significant correlation has
been found between variation in cell shape (Plesmorphism) and
size (Polymegathism) and development of postoperative corneal
oedema.

Pleomorphic cells react adversely to intraocular surgery.
Polymegathic cells may not fit together leaving gaps and
compromising the endothelial structural barrier.

Incidence of preoperative endothelial dystrophy in PBK has been
found in 67% of patients (Retrospective analysis).9

Pseudoexfoliation syndrome has been associated with an increased
incidence of PBK, due to higher rate of vitreous loss but also due
to clinically & histopathologically distinct keratopathy (Irregular
thickening of Descemet's membrane and focal accumulation of
locally produced pseudoexfoliation material. A reduced
preoperative endothelial cell count of 10.5 to 11.1%. is
demonstrated in patients with pseudoexfoliation syndrome
undergoing cataract surgery10,11.

Endothelial cell density less than 2000 cells/mm2 was found in
patients with previous history of corneal diseases, angle closure
glaucoma, pseudoexfoliation syndrome and a history of trauma12.

Intraoperative risk factors

Surgical trauma to endothelium

Type of cataract surgery

Lenses made of methacylate adhere instantaneously to endothelial
surface when contact upon lens insertion occurs.13

Figure 3: Aphakic bullous keratopathy Reusable cannulas with viscoelastic can result in toxic residues
being introduced into the eye. Hence disoposable annuals should
be used. During intraocular irrigation, physiologic saline causes
corneal swelling and endothelial damage. Ringers solution does
so to a lesser degree. Balanced salt solution enriched with
bicarbonate, dextrose and glutathione (BSS plus) has been shown
to protect the endothelium better than BSS alone14,15.

oedema doesn't cause severe visual loss. However epithelial It has been found that HPMC is not as protective as sodium
oedema can cause a significant drop in vision. hyaluronate during phacoemulsification. Protective benefit of
sodium hyaluronate is improved when used in combination with
Pain / discomfort: Pain occurs due to exposure of corneal nerves chondroitin sulphate.16
to noxious environment.6 As the oedema progresses, bullae are
formed their rupture results in pain, photophobia & epiphora. Toxic substances used to disinfect instruments may inadvertently
be introduced into the eye. Water, not saline, should be used to
Slit lamp examination reveals folds in descemet's membrane and rinse the instruments.
obvious overall thickening of central and peripheral cornea. In
more advanced cases, vesicles & bullae may be seen on corneal Intracular irrigation must be just adequate enough. Excess may
surface. cause endothelial injury.

In patients with predisposing conditions (like fuch's dystrophy & For topical and intracameral anaesthesia only 0.5 ml of 1%
Posterior Polymorphous dystrophy), cornea guttata may be seen. preservative free lidocaine should be used17,18. Other preparations
Guttate excrescences appear as "golden brown" confluent may cause corneal toxicity.
endothelial lesions & give posterior surface a "beaten metal
appearance"7,8. Intraocular epinephrine19, benzal konium chloride preserved
viscoelastic, vancomycin (> 1 mg / ml)20 and inadvertent exposure
of endothelium to 5% povidone-iodine21 can cause endothelial
toxicity.

42 DOS Times - Vol. 14, No. 8, February 2009

Digital analysis of photographs can be used to assess parameters
such as coefficient of variation (for polymegathism) and percentage
of hexagonal cells present (for pleomorphism). Endothelial cells
that show great variability in size and shape are considered to be
under physiologic stress and are abnormal.

Specular photomicrographs can be used to assess corneal diseases
such as Fuch's dystrophy & posterior polymorphous dystrophy.
Former shows guttate excrescences while later shows patchy areas
of normal endothelium adjacent to abnormal endothelium with
vesicles and plaques.

Ultrasound pachymetry and optical pachymetry

Both are used to measure corneal thickness.

Figure 4: Late painful bullous keratopathy • (N) central corneal thickness is 550 um centrally increasing
upto 770um peripherally. Corneal thickness above 0.6 mm
(600 um) is suspect for corneal oedema.

Descemet's detachment is more common with clear corneal • Serial measurements are helpful to note progression; and
incisions which may lead to postoperative corneal oedema. also to assess effectiveness of therapeutic regimens.

Postoperative causes • Ultrasonic pachymetry is more reproducible and requires
less skill than optical.
Routine uncomplicated phacoemulsification results in 9%
endothelial cell loss at 1 year postoperatively. In one series, it has Optical pachymetry is especially useful in measuring the depth of
been found to be 11.9%22 corneal pathology, when fall thickness of corneal stroma is not
involved and for therapeutic reasons to estimate the depth of this
Regardless of type of surgery and type of IOL implanted, pathology. It is preoperative investigation for excimer laser
continuing endothelia cell loss more than usual 1% per year occurs phototherapeutic keratectomy.
in patients who have undergone cataract extraction.
Treatment
Type of lens implanted
Medical Care
Anterior chamber IOLs contribute to endothelial damage by
"Intermittent touch" between IOL & corneal.23 Topical hyperosmotic agent

IOL haptics & footplates can cause chronic irritation with low 2% and 5% sodium chloride solution and ointment.
grade inflammation
Mechanism: These agents create hypertonic tear film, drawing
IOLs are also known to disrupt the normal flow of aqueous in water out of cornea.
anterior chamber which is the nutrient flow to endothelial cells.24
Because evaporation of tear film is minimal at night with eyes
Iris supported lenses may cause greater endothelial loss as they closed, corneal oedema tends to be worse in morning. Hence 5%
can have contact with endothelium during ocular saccades25. sodium chloride ointment should be applied at nigh to conjunctival
cul-de-sac.
Closed loop ACIOL have been responsible for large amount of
corneal pathology. Typical regimen includes application of Muro 128, 2-5% drop
hourly in affected eye until noon. As the day progresses,
Following ICCE, incidence at PBK has been found to be 2.3% in evaporation of tear film begins to create relative hypertonicity of
retrospective study of 1041 patients.26 tears drawing fluid out of cornea. This accounts for typical history
of improving vision towards end of the day.
Imaging Studies
Pressure lowering agents
Specular microscopy
• Lowering of intraocular pressure can decrease corneal
This is photographic method of assessment of endothelium in oedema & thickness in postoperative setting, even if
vivo. Light is projected onto cornea and reflected images from intraocular pressure is normal or only mildly elevated.
optical interface (endothelium and aqueous) can be visualized.
• β-blockers & -agonists are first line agents for this purpose.
High magnifications photographs allows quantification of cell
density. Corneas with cell densities less than 1000 cells / mm2 are • Prostaglandins analogs & miotics should be avoided because
at moderate to high risk of developing corneal oedema following both drugs may adversely affect intraocular inflammation.
intraocular surgery.
• Utility of topical CA (-) is under question because they may
cause endothelial toxicity in compromised corneas.

www.dosonline.org 43

Topical steroids with sponge. The former was not sticky, while the later was &
could be caught by the sponge.
These should be used in cases of uveitis provided infectious
keratitis & epithelial defects are absent. This fashioned membrane was then secured to the edge of defect
by 10-0 nylon sutures which were either continuously running
Hydrophillic contact lenses interrupted or a combination of both.

These on extended wear basis, can be used to decrease pain Attention was given to make the membrane flattened tightly anto
associated with epithelial bullae. corneal surface and approximate to the epithelial edge.

These lenses as such do not reduce the amount of oedema. These In some cases BCL was applied.
can improve visual activity to the extent that they mask surface
irregularity. Results: This turned immensely successful for ocular pain relief in
43 of 48 eyes with painful bullous keratopathy.
These contact lenses can be used in association with 5% hypertonic
saline (as cornea) and thus they can improve visual acuity by This is technically easier to perform avoid potential complications
decreasing epithelial & stromal oedema. of conjunctival flap such as ptosis, it gives better cosmetic
appearance. Hence it is superior to conjunctival flap.
Pain associated with PBK can be due to rupture of bullae with
exposure of corneal nerves endings or swelling of epithelium It provides additional advantage in that resultant cornea doesn't
leading to stretching of nerve endings. These lenses alleviate pain manifest surgically induced limbal stem cell deficiency, which is
as long as the lens remains in place. invariably created by conjunctival flap. As a result such corneas
are amenable for corneal transplantation if necessary.
Acts as effective precorneal protective layer. Shields abnormal
epithelium from environment and prevents bullae from bursting. Amniotic transplantation also restores corneal epithelial integrity.
All except 4 eyes (92%) maintained healed and intact epithelium
The lens doesn't prevent formation of bullae but perhaps when during the entire follow up period.
new bullae do occur, the corneal nerves' endings are not exposed
to drying & other noxious stimul, when the lens cover them. Bullae resolved in 90% cases. Even with recurrent epithelial
oedema, pain did not occur.
Fitting of lens is an important consideration. Lenses with excessive
movement can irritate epithelium & be uncomfortable. Amniotic basement membrane prevents epithelial apoptosis,
enhances epithelial migration, differentiation. It also reinforces
Lenses that are too tight can act as suction cap and result in adhesion of basal epithelial cells29,30,31.
inflammation and even anterior uveitis (Tight lens syndrome).
It also prolongs life span of corneal and conjunctival progenitor
Surgical Care cells32.

Conjunctival flap Stromal matrix inhibits TGF-β, hence causing less scars
postoperatively32.
In 1958, Gundersen introduced the technique of using conjunctiva
alone without use of tenon's capsule to cover the cornea. It is onlay Amniotic membrane transplantation proxides new and
flap of thin conjunctiva without use of tenon's capsule. The nonantigenic human basement membrane for renewed expansion
exclusion of tenon's capsule increases flap longevity. of epithelial cells33.

A Gundersen-type flap undermines the superior bulbar Cautenisation of Bowman's membrane
conjunctiva & moves in & down to cover the cornea with intact
"bridges" nasally and temporally. It can be performed for pain relief. This procedure is thought to
produce dense fibrous barrier between corneal stroma and
The flap is thought to increase the local blood supply, promote epithelium so that fluid can not permeate into epithelial cells and
healing and replace damaged or defective corneal epithelium27. produce ballous changes.

Amniotic membrane transplantation Anterior stromal pundure in bullous kercetopathy

Study of Amniotic membrane transplantation performed in 50 A clinicopathologic study34
eyes28
28 patients of bullous keratopathy (1996-99) were studied with
Important steps of procedure minimum follow up of 1 month. In all these patients approximately
200 punctures were given with bent 26 G needle sparing peripheral
Loose epithelium was first removed with sponge, 0.12 forceps or cornea.
a blade to where epithelium became quite adherent. In general
this would create large corneal epithelial defect upto 1 or 2 mm • Symptromatic relief was noted in all.
from limbus.
• 20 patients had complete relief.
Preserved amniotic membrane was removed from storage
medium and cut to same size as debrided corneal surface, with • 8 patients experienced mild symptroms such as tearing and
basement membrane surface up in all cases. occasional pain.

The side of BM could be distinguished from stromal side by touch

44 DOS Times - Vol. 14, No. 8, February 2009

• Complete regression of bullae in 10 patients. • One patient had persistent epithelial defect that required
botulinum toxin ptosis.
• Partial regression of bullae in 18 patients.
Corneal transplantation
• Visual activity decreased in 12 patients.
Penetrating keratoplasty for PBK:
• Objective evidence of subepithelial fibrosis and scarring was
seen in all. • Review study of 81 causes40 of PK done for PBK during
1987-93.
Anterior stromal puncture is an effective, simple and low cost
alternative for treating patients with symptomatic bullous • IOL explant was done in 24 (29.6%) cases.
keratopathy35.
• IOL was retained in 12 (14.9%) cases.
Annular keratotomy
• IOL exchange was done in 45 (55.5%) cases.
It is used to treat pain associated with bullous keratopathy in eyes
with poor visual potential. A partical thickness corneal incision is • Minimum follow up period was 6 months.
made with a trephine and relieves pain by severing branches of
corneal ciliary nerves to decrease corneal sensation36. • Graft remained clear in 54 (66.6%) patients.

Diamond burr polishing of basement membrane • Commonest cause of graft failure was "graft rejection"(17.2%).

Following epithelial debridement, 4.5 mm to 5 mm diamond burr They recommended explantation of all closed loop AC IOLs and
can be used gently to polish basement membrane throughout iris claw IOLS and that AC recomstraction during PK with IOL
area of epithelial debridement37. exchanged should be done using PCIOL or open loop kelman
type ACIOL.

Phototherapeutic keratectomy for bullous keratopathy Failure of retained lens in PK for PBK41

Nidek EC 5000 excimer laser used for performing PTK (3 types) Failure rate Lens
for PBK38.
34% ACIOL
Superficial PTK 8 to 25 um ablation 29% Iris supported lens
Intermediate PTK 50 to 100 um ablation 6% PCIOL
Deep PTK 25% of stromal thickness ablation

Thomas et al39 proposed that PTK is effective procedure and bullae In this study from LVPEI, they suggested,
resolve and pain is abolished in large number of patients with Anterior segment reconstruction during PK →Better outcome
superficial ablation. Anterior, +Gonioplasty, +Iridoplasty, Vitrectomy
Advantage of Anterior Vitrectomy
But in this study, it was proposed that preterminal neural plexus • ↓ CME
of cornea is located just deep to Bowman's membrane. Hence • Prevents decentration of IOL by vitreous strands
moderately deep ablation would have superior effect on decreasing Advantage of gonioplasty & Irioplasty
the pain; by ablation of neural plexus. • To restore pupil to normal round configuration

• It will lead to decreased swelling of corneal stroma by
decreasing the quantity of mucopoly sacharides and hence
decreasing osmotic load.

• Increased scarring associated with deeper ablation may result • Prevents development of glaucoma and allograft rejection
in increased stability of epithelium. related to formation of anterior synechiae due to centripetal
tension exerted on iris.
Superficial PTK showed improvement in 62% cases whereas 38%
Give additional support to AC lens.
required penetrating keratoplasty. •

Intermediate PTK showed improvement in 40% cases whereas • Improves optical function of iris.
60% required penetrating keratoplasty.
To perform IOL exchange either with PCIOL or an open loop
Deep PTK: Mean ablation performed was of 206 um thickness. kelman type ACIOL.

• 66% (16 out at 24) showed improvement. Explantation of all iris fixated lens / closed loop IOLS.
• One required PK.
• One required amniotic membrane transplantation. • To avoid delay in corneal surgery once PBK is diagnosed.
• Two patients required retreatment.
• Three patients continued having discomfort • Anterior segment reconstruction and monitoring of I.O.P.,
both pre & postoperatively.
www.dosonline.org
CME is important cause for poor visual recovery in such patients.
Despite risk of infection, secondary glaucoma and graft rejection,

45

penetrating keratoplasty still remains treatment to get maximum intraocular lens implantation, Jpn J Ophthalmol 37:414, 1993.
improvement in visual acuity.
2. Champion R, Green WR : Intraocular lenses: a histopathologic study
In general, options for IOL exchange are of eyes, ocular tissues and intraocular lenses obtained surgically,
Ophthalmology 92:1628,1985.
• Modern flexible loop ACIOL.
3. Maseruka H, Ataullah SM, Zardi L et al : Tenascin - cytotactin (TN-
• PCIOL in ciliary sulcus. C) variants in pseudophakic / aphakic bullous keratopathy corneas,
Eye 12:729, 1998.
• Iris fixation of PCIOL.
4. FDA report on intraocular lens (1978-1952) stark WJ, Worthen
• Suturing of PCIOL in sulcus. DM, Holladay JT Ophthalmology 90:311-317, 1983.

Flexible ACIOL should be reserved for 5. George R, Rupauliha P, Sripriya AV, Rajesh PS, Vahan PV, Praveen S.
Ophthalmic Epidemiol. 2005 Oct; 12(5): 293-7.
• Eyes with minimal anterior segment pathology.
6. Ljubimov AV, Burgeson RE, Butkowski RJ, Couchman JR, WuRR,
• Less than 900 angle synechiae. Ninomiya Y, et al. Extracellular matrix alterations in human corneas
with bullous kercetopathy. Invest Opthalmol Vis Sci 1996;37:997-
• Well controlled I.O.P. 1007.

Generally width of IOL chosen should correspond to a 7. Waring GO III, Rodrigues MM, Laibson PR. Corneal dystrophies.
measurement of horizontal white to white corneal diameter plus II. Endothelial dystrophies. Surv. Ophthalmol 1978;23:147-168.
1 mm.
8. Cibis GW, Krachmer JH, Phelps CD, et al. The clinical spectrum of
Sutured intraocular lenses should be reserved for posterior polymorphous dystrophy. Arch ophthalmol 1977; 95: 1429-
1537.
• Extensive anterior segment pathology.
9. Lugo M, Cohen EJ, Eagle RC, Parker AV Ophthalmic Surg. 1988
• Lack of iris support for ACIOL. Jan; 19(1):16-19.

• Patients with glaucoma. 10. Wirbelauer C, Anders N, Pham DT et al: Early postoperative
endothelial cell loss after corneoscleral tunnel incision and
No study till date has clearly pointed to advantage of one technique phacoenulsification in pseudoexfoliation syndrome, Ophthalmologe
or style of intraocular lens replacement in terms of graft survival, 94 (5) : 332,1997.
vision or development of secondary complications. Hence it
remains the choice of particular surgeon. 11. Wirbelauer C, Anders N, Pham DT et al : Corneal endothelial cell
changes in pseudoexpoliation syndrome after cataract surgery, Arch
Newer techniques Ophthalmol 116 (2) : 145, 1998.

DLEK (Deep lamellar endothelial Keratoplasty) 12. Ishikawa : Risk factors for reduced corneal endothelial cell density
before cataract surgery, J Cataract Refract Surg 28 : 1982, 2002.
In patients with normal stroma and superficial cornea, where only
endothelium is damaged; this procedure is a very good treatment 13. Kaufman HE, Katz JC : Endothelial damage from intraocular
modality, with following distinct advantages:24 insertion, Invest Ophthalmol 15:996, 1976.

• This preserves healthy portions of patients' native cornea. 14. Edelhauser HF et al : Intraocular irrigating solutions : their effect on
the corneal endothelium, Arch Ophthalmol 93: 648, 1975.
• It avoids additional risks and postoperative complications.
15. Li J et al : Effects of BSS and BSS + irrigation solutions an rabbit
• Additional benefit is that postoperative astigmatism is very corneal transendothelial electrical potential differences, Cornea 12:
minimal. 199, 1993.

Flap endokeratoplasty 16. Glasser DB et al : Endothelial protection and viscoelastic retention
during phaconulsification and intraocular lens implantation, Arch
Free papers accepted in XXV congress of ESCRS in Sept. 2006 Ophthalmol 109: 1438, 1991.
compares "Flap endokeratoplasty versus penetrating keratoplasty
for PBK: 5 yrs. follow up results"42. 17. Martin RG, Miller JD, Cox CC 3rd et al : Safety and efficacy of
intracameral injections of unpreserved lidocaine to reduce intraocular
5 yrs follow up shows all clear grafts, no significant difference sensation, J Cataract Retract Surg 24 : 961, 1998.
between BCVA, endothelial cell count or mean postoperative
refraction. Visual recovery and postoperative astigmatism were 18. Elvira JC, Hueso JR, Martinez-Toldos J et al : Induced endothelial
better in patients who have undergone Flap EKP. cell loss in Phacoemulsification using topical anaesthesia plus
intracameral lidocaine, 1 J cataract Retract Surg 25: 640,1999.
Preliminary results show that flap EKP could be a successful
alterative to penetrating keratoplasty for treatment of PBK. This 19. Edelhauser HF et al : Corneal oedema and intraocular use of
might be the future as far as treatment of PBK is concerned. epinephrine, Am J Ophthalmol 93: 327, 1982.

References 20. Sandboe FD, Medin W, Bjerknes R : Toxicity of vancomycin on
corneal endothelium in rabbits; Acta Ophthalmologica scand 76 :
1. Liu G et al: Histopathological study of pseudophakic bullous 675, 1998.
keratopathy developing after anterior chamber or iris-supported
21. Alp B N, Elibol O, Sargon MF et al : The effect of povidone iodine
on the corneal endothelium, Cornea 19 : 546,2000.

46 DOS Times - Vol. 14, No. 8, February 2009

22. Zhongua Yan Ke Za Zhi 2000 Jul; (36) 4 : 282-4. He W, Lu P. 33. Fukada K, Chikama T, Makamura M, Nishida T. Differential
distribution of subchains of basement membrane components type
23. Sugar J, Mitchelson J, Kraff M : Endothelial trauma and cell loss IV collagen and laminin among the amniotic membrane, cornea
from intraocular lens insertion, Arch Ophthalmol 96 : 449, 1978. and conjunctiva. Cornea 1999; 18 : 73-9.

24. Paula Wynn. E medicine article, University of Iowa 2005 Nov. 34. Anterior stromal puncture in bullous keratopathy; a
clinicopathologic study Sridhar MS Rao GN. Cornea 2001 Aug 20
25. Drews RC : Intermittent touch syndrome, Arch Ophthalmol 100 : (6) : 573-9.
1440, 1982.
35. Anterior stromal puncture in the treatment of bullous keratopathy:
26. Kirsten Baggesen, E. B. Knudsen, K. Naeser. Acta Ophthalmologica 6 months follow up. Gomes JA, Haraquchi DK, Zambrano DU,
scandinavia 1999. Izquierdo Junior L, Cuhna MC, de Freitas D. Cornea 2001 Aug
20(6) : 570-2.
27. Gundersen T. Surgical treatment of bullous keratopathy, Arch
Ophthalmol 1960; 64 : 260 - 67. 36. Annular Keratotomy for the treatment of painful bullous
keratopathy.Koeniq SB. Am J Ophthalmol. 1996 Jan; 121 (1) : 93-
28. Renato T.F. Pires, Scheffer C.G. Tseng. Arch Ophthalmol. 1999; 4.
117 : 1291-97.

29. Khodadoust AA, Silverstein AM, Kenyon KR, Dowling JE. Adhesion 37. David Verdier. E medicine article on Map dot fingerprint dystrophy,
of regenerating corneal epithelium : the role of basement membrane. 2005 Dec.
Am J Ophthalmol 1968; 65 : 39-48.
38. Raj Maini, Laurence Sullivan, Grant R Snibson, Hugh R Taylor,
30. Guo M, Grinnell F. Basement membrane and human epidermal Michael S Laughnan. Br J Ophthalmol 2001; 85 : 912 - 915.
differentiation in vitro. J Invest Dermatol 1989; 93 : 372-8.
39. Thoman et al. Br J Ophthalmol 1995; 79: 335-338.
31. Boudreau N Werb Z, Bissell MJ. Suppression of apoptosis by basement
membrane requires three dimensional tissue organisation and 40. Agrawal Vinay, Vagh Mustali, Sangwan Virender, Rao Gullapalli N.
withdrawal from cell cycle. Proc Natl Acad Sci USA 1996; 93 : Indian J Ophthalmol 1994; 42 (2) : 75-80.
3500-13.
41. Sugar. Analysis of corneal endothelial and graft survival in PBK.
32. Tseng SCG, Li D-Q, Ma X. Suppression of TGF-? receptor type II Trans Am Ophthalmol Soc. 87 : 762-801, 1989.
and myofibroblast differentiation in cultured human corneal and
limbal fibroblasts by amniotic membrane matrix. J cell Physiol 42. M. Alfayez. Flap EKP vs Penetrating keratoplasty for PBK : 5 yr.
1999; 179 : 325 - 35. Follow up results. XXX congress of ESCRS. Sept. 2006.

First Author
Shreekant A. Damgude MBBS

www.dosonline.org 47

Relative Afferent Pupillary Defect Neuro-Ophthalmology

Sumita Sethi MS, Mridula Mehta MS, DNB, Shibal Bhartiya MS, Sonia Bhargav MS, DNB

The Relative Afferent Pupillary Defect (RAPD), or Marcus- light but to a lesser and slower degree. As a result, when the light
Gunn Pupil, named after British ophthalmologist Robert is moved from the good to the bad eye the brain interprets this as
Marcus Gunn, is an extremely significant and highly objective clinical a decrease of light being presented. The brain’s response is to
finding in the examination of the visual system1,2. There are many dilate both pupils to let in more light. This dilation response is in
different conditions leading to RAPD, from severe glaucoma to an both eyes, despite only one eye being affected. This is the essence
optic nerve tumor. Also significantly, there are many conditions of the RAPD3,4,5 (Figure 1a & b).
which lead to a profound loss of vision (such as a complete vitreous
hemorrhage), which will not give RAPD. It is a valuable clinical sign Neuroanatomy and physiology of the pupillary light reflex
that should be used properly and not extrapolated to the point of pathway
irrelevance.
Pupillary size is governed by the balance of actions of two opposing
Normally the usual pupillary response to direct light is that both muscle groups of the iris: the dilator and sphincter pupillae.
pupils contract equally. While moving the light quickly from one Regulation of the pupillary size is predominantly achieved by reflex
eye to the other, both pupils should hold their level of contraction. mechanisms in response to the amount of ambient light.
When light is shown in the good eye, both pupils constrict. Then (Figure 2 )
shifting it to the other eye, an injured optic nerve will also transmit
Constriction: The majority of retinal axons provide afferent
(a) information to the lateral geniculate nucleus en route to the primary
visual areas on the calcarine cortex. However, approximately 10%
of the fibers bypass the lateral geniculate nucleus and are relayed
to the pretectal area of the rostral midbrain. These fibers travel
through the brachium to synapse at the level of the superior
colliculus. Second-order neurons subsequently relay pupillary light
information to Edinger-Westphal nuclei bilaterally. This dual and
near simultaneous innervation provides the anatomical basis for
both the direct and the consensual light reflexes. From the Edinger-
Westphal nucleus, tertiary parasympathetic neurons travel in the
superficial dorsomedial aspect of the ipsilateral occulomotor nerve

(b)

Figure 1: Schematic diagram showing Figure 2: The pupillary light reflex pathway.
(a) : normal pupillary response to a bright source of light (ON-optic nerve, OT-optic tract, PT-pretectal

(b): pupillary response in case of relative nucleus, EW-Edinger Westphal nucleus,
afferent pupillary defect CG-ciliary ganglion, SCN-short ciliary nerves

Dr. Rajendra Prasad Centre for Ophthalmic Sciences, 49
All India Institute of Medical Sciences,
New Delhi-110029

www.dosonline.org

to reach the ciliary ganglion. The ganglion then gives rise to 8 to 10 performed with increasing density of filter before the healthy eye
short ciliary nerves , which subdivide into 16 to 20 branches. until the sign disappears or is reversed. Neutral density filters (NDF)
Approximately 3% of these fibers ultimately converge upon and are available in the form of a rack or bar containing 10 filters of
innervate the pupillary sphincter muscles (promoting constriction), increasing density. The density of the filters is measured in log
thereby contributing to the regulation of pupil size. units ranging from 0.3 to 3.0 in steps of 0.3. Servais et al quantified
RAPD using NDF in patients with CRVO13; 90% of non-ischaemic
Dilation: Pupillary dilation is mediated through three-neuron CRVO had an RAPD of 0.3 log unit or less and none greater than
sympathetic (adrenergic) pathways that originate in the 0.9. Ischaemic CRVO had RAPD of 1.2 or greater in 91% and none
hypothalamus and descends caudally to the first synapse, located less than 0.6.
in the cervical spinal cord (levels C8-T2). The second-order neuron
then travels from the sympathetic trunk, through the brachial Ramsay et al have shown that crossed polarised filters (CPF) produce
plexus, over the lung apex. It then ascends to the superior cervical exponential attenuation of light and can be used in the same way
ganglion .The third-order neuron ascends within the adventitia of as NDF to assess RAPD in CRVO.11
the internal carotid artery, through the cavernous sinus, where it
is in close relation to the sixth cranial nerve. The oculosympathetic However the limitation with the use of NDF and CPF is their
pathway then joins the ophthalmic (V1) division of the trigeminal scarce availability in the clinical setting.
nerve, and innervate the iris dilator muscle.
The Sbisa or Bagolini filter bar, is similar to NDF, and is used
Clinical examination and quantification of Rapid afferent commonly by orthoptists to assess density of suppression in
papillary defect strabismus. It consists of 17 sequential red filters. While Sbisa bar
filters are red and NDF neutral, it is the apparent brightness not
The evaluation of the pupils is best performed in a dimly colour that determines pupil response (Figure 4 a & b). Sbisa bars
illuminated room. This allows for some pupillary dilation, while are generally much easier to obtain than NDFs in most
also allowing for the observation of the consensual pupillary departments, McCormick et al, have described their use in the
response. It is reasonable to test the individual reaction of each same way as the NDF, to neutralise and therefore quantify a relative
pupil initially. In some cases one pupil may not be reactive due to afferent pupillary defect14.
a variety of conditions. In cases where neither pupil reacts to light,
no further testing can be done, except for testing the pupillary Quantifying RAPD by these methods depends upon clearly
response to accommodation (focusing at near). identifying an end point. The end point can be taken as either the
elimination of the sign (brisk constriction with the same redilatation
The “swinging flashlight test” is a popular and an established way in both eyes) or reversal (looking at the eye with the RAPD-brisk
for identifying an RAPD6-10. In this test, a strong, steady light is constriction by direct illumination and dilatation by indirect
used. The light is shone into one eye, quickly switched to the other illumination).
and is repeated back and forth. Since light in one pupil causes both
pupils to constrict, quickly switching from one eye to the other will Step vise approach to a patient of RAPD
give a “relative” indication of the functioning of each eye and optic
nerve. If both eyes are equally dysfunctional, no “relative” defect Knowing whether the patient is aware of changes in vision. If so, are
would be found. The results of the test can be graded as follows these changes acute or gradual? An acute optic neuropathy or
(as per the I-V system of Bell et al)9. retinal detachment would be associated with sudden vision loss,
while gradual vision loss might support the existence of a
Grade I, initial weak constriction with greater redilatation; compressive lesion.15-17

Grade II, initial stall and greater redilatation; Eliciting significant ocular and medical history. Does the patient
have a history of vascular disease, cancer, auto-immune disease,
Grade III, immediate pupillary dilatation; recent infections or trauma?

Grade IV, immediate pupillary dilatation following 6 seconds of Carefully examining the patient while paying special attention to the
illumination; retina and optic nerve. Optic nerve findings can be subtle or non-
existent. For instance, a relatively normal appearing nerve might
Grade V, immediate pupillary dilatation with no secondary be present in a retrobulbar Optic Neuritis.
constriction at all.
The need to refer for further testing depends on the case. If the
Certain standard conditions have been identified for applying this patient’s history and retinal examination do not offer an obvious
grading. The patient fixes on a 6/60 Snellen letter at 6 metres in a explanation for the RAPD, one must assume that a condition
darkened room. An indirect ophthalmoscope head set affecting the optic nerve or optic tract is causing it. An immediate
(standardised as for the luminance measurements) is used as the visual field and color vision test should be performed.
light source. A swinging flashlight test is than carried out with each
pupil illuminated for 3 seconds. Depending on the condition, the patient may need to be referred
for emergent neuro-imaging and laboratory testing . For example,
However subjective grading leads to discrepancies between an individual who presents with an RAPD as a result of swollen
clinicians, is difficult to quantify, and limits its use in diagnosis. By optic nerve and a history of leukemia, would require emergent
accurately quantifying the defect additional information can be imaging and radiation treatment if the swollen nerve represented
provided, which may aid management—for example, assessing a leukemic infiltrative optic neuropathy.
retinal ischaemia following central retinal vein occlusion.13
Computed tomography (CT Scan) and Magnetic resonance
Thompson et al describe a method of quantification of RAPD imaging (MRI) are valuable tools for diagnosis of optic nerve
using neutral density filters7.The swinging flashlight test is

50 DOS Times - Vol. 14, No. 8, February 2009

Figure 3: CT scan findings in a few patients with RAPD:
(a): Sclerosis of greater wing of sphenoid bone with enhancing soft tissue components in the

middle cranial fossa and orbit on right side, s/o Sphenoid wing meningioma,
extending to the orbit on right side

(b): Oblong mass in the right orbit – retrobulbar region, s/o probable diagnosis of optic nerve glioma
(c): Well defined intensely enhancing intraconal lesion in the right orbit, s/o probable
diagnosis of cavernous hemangioma

disorders (especially optic nerve tumours and traumatic optic • Orbital disease – Including compressive damage to the optic
neuropathy) and in orbital diseases causing compressive damage nerve from thyroid related orbitopathy (compression from
to optic nerve (Figure 3 a,b,c). enlarged extraocular muscles in the orbit), orbital tumors, or
vascular malformations.
Characteristics of the Relative Afferent Pupillary Defect
• Radiation optic nerve damage
• A RAPD is an index of significant optic nerve or retinal disease,
when there is a difference in the disease process between the • Miscellaneous optic neuropathies, such as Leber’s optic
two eyes. If both eyes have severe but equal disease, there neuropathy (usually eventually bilateral) and other inheritable
will be no RAPD. Thus, a “bilateral” RAPD does not exist. optic neuropathies.

• Severe disease in one eye,causing a RAPD ,will not result in • Optic nerve infections or inflammations - Cryptococcus can
anisocoria. The pupil of the diseased eye will be equal in size cause a severe optic nerve infection in the immuno-
to that of the other eye due to the consensual light reaction compromised. Sarcoidosis and Lyme disease can cause
(unless the iris itself is diseased or unreactive). inflammation of the optic nerve.

• One functioning pupil is sufficient to determine the presence • Surgical damage to the optic nerve - This could include damage
of an RAPD, (due to the presence of consensual light reflex). following retrobulbar anesthesia; damage following orbital
hemorrhage related to eye, orbital, sinus, or plastic surgery;
• The visual acuity does not necessarily correlate with an RAPD. damage following neurosurgical procedures such as pituitary
Some conditions lead to a marked reduction of visual acuity tumor resection; and damage related to migration of an orbital
with an RAPD, while others spare the central vision. Usually, plate after surgery to correct a blow-out fracture.
extensive loss of peripheral vision is associated with an RAPD.
Retinal Causes of a Relative Afferent Pupillary Defect
Conditions leading to a Relative Afferent Pupillary Defect
Usually retinal disease has to be quite severe for an RAPD to be
Optic nerve disorders clinically evident. Symmetrical bilateral retinal disease will not show
an RAPD.
• Optic neuritis - Even very mild optic neuritis with a minimal
loss of vision can lead to a very strong RAPD. • Ischemic retinal disease - including ischemic central retinal
vein occlusion, central retinal artery occlusion, severe ischemic
• Ischemic optic neuropathies - These include arteritic (Giant branch retinal or arterial occlusions, severe ischemic diabetic
Cell Arteritis) and non-arteritic causes. Usually associated or sickle-cell retinopathy.
are a loss of vision or a step deformity in the visual field.
• Ischemic ocular disease (Ocular ischemic syndrome) – due to
• Glaucoma – Though glaucoma normally is a bilateral disease, obstruction/compression of the ophthalmic or carotid artery
asymmetric optic nerve involvement can result in an RAPD. on one side.

• Traumatic optic neuropathy - This includes direct ocular • Retinal detachment - An RAPD may be seen if the macula is
trauma, orbital trauma, and remote head injuries which can detached, or if at least two quadrants of retina are detached.
damage the optic nerve as it passes through the optic canal
into the cranial vault. • Severe macular degeneration - If unilateral and severe

• Optic nerve tumor - This is a rare cause, and includes primary • Intraocular tumor - Retinal and choroidal tumors if severe
tumors of the optic nerve (glioma, meningioma) or tumors (including melanoma, retinoblastoma, and metastatic lesions)
compressing the optic nerve (sphenoid wing meningioma,
pituitary lesions, etc.)

www.dosonline.org 51

(a) • Bilateral retinitis pigmentosa

(b) • Bilateral nutritional or metabolic optic neuropathies .

Figure 4 (a): Neutral density filter References
(b): Sbisa bar. Note that Sbisa bar filters are
1. Wikipedia contributors, ‘Marcus Gunn pupil’, Wikipedia, The Free
red and NDF neutral Encyclopedia,17July2008,21:11UTC,<http://en.wikipedia.org/w/
index.php?title=Marcus_Gunn_pupil&oldid=226320762>.
• Retinal infection – Extensive retinitis (Cytomegalovirus, herpes
simplex,). 2. Thompson HS. Pupillary signs in the diagnosis of optic nerve
disease.Trans Ophthalmol Soc UK 1976;96:377-81.
Amblyopia
If severe, can lead to a relative afferent pupillary defect. Usually 3. Kardon, R. Anatomy and physiology of the autonomic nervous
the visual acuity would be 20/400, or worse. system. In: Walsh and Hoyt Clinical Neuro-ophthalmology, 6th ed,
Cerebral Vascular Disease Miller, NR, Newman, NJ, Biousse, V, Kerrison, JB (Eds), Williams &
Usually, it is an optic nerve disorder that leads to an RAPD, rather Wilkins, Baltimore 2005. p.649.
than an optic tract or visual cortex disorder. However, there tends
to be a higher percentage of crossed vs. uncrossed nerve fibers at 4. Johnston, JA, Parkinson, D. Intracranial sympathetic pathways
the optic chiasm. Thus, in a patient with a homonymous associated with the sixth cranial nerve. J Neurosurg 1974; 40:236.
hemianopia from an optic tract disorder, an RAPD maybe noted
in the eye with the temporal visual field defect. The nasal retina 5. Digre, KB. Principlesand techniques of examination of the pupils,
serves the temporal visual field, and these are the fibers that would accommodation and lacrimation. In: Walsh and Hoyt Clinical Neuro-
cross at the chiasm. ophthalmology, 6th ed, Miller, NR, Newman, NJ, Biousse, V, Kerrison,
Conditions not leading to a Relative Afferent Pupillary JB (Eds), Williams & Wilkins, Baltimore 2005. p.715.
Defect
• Refractive Error 6. Enyedi, L.B. et al. A Comparison of the Marcus Gunn and
• Media Opacity including Cataract, Corneal scar, Hyphema, Alternating Light Tests for Afferent Pupillary
Defects.Ophthalmology 5/1998; 105:871-873.
vitreous hemorrhage. Previous eye surgery
• Strabismus 7. Thompson HS, Corbett JJ, Cox TA. How to measure the relative
• Conditions with an Efferent Pupillary Defect afferent pupillary defect.Diagnostic and Surgical Techniques
1981;26:39–42.
• Third Cranial Nerve Palsy
• Adie’s Pupil 8. Browning DJ, Tiedeman JS. The test light affects quantitation of
• Horner’s Syndrome the afferent pupillary defect.Ophthalmology 1987;94:53–5.
• Mild retinal diseases
• Mild background diabetic retinopathy 9. Bell RA, Waggoner PM, Boyd WM, et al. Clinical grading of relative
• Central serous choroidopathy afferent pupillary defects.Arch Ophthalmol 1993;111:938–42.
• Non-ischemic vein occlusions
• Mild macular degeneration 10. Sun F, Tauchi P, Stark L. Binocular alternating pulse stimuli:
• Conditions which are typically bilaterally symmetrical will not experimental and modelling studies of the pupil reflex to light.Math
show an RAPD: Biosci 1983;67:225–45.

11. Ramsay A, Williamson TH, Parks S, et al. Crossed polarising filters
to measure relative afferent pupillary defects: reproducibility,
correlation with neutral density filters and use in central retinal
vein occlusion.Eye 1995;9:624–8.

12. Thompson HS. Pupillary signs in the diagnosis of optic nerve
disease.Trans Ophthalmol Soc UK 1976;96:377–81.

13. Servais GE, Thompson HS, Hayreh SS. Relative afferent pupillary
defect in central retinal vein occlusion.Ophthalmology 1986;93:301–
3.

14. A McCormick, R Bhola, L Brown, D Squirrel, J Giles, I Pepper.
Quantifying relative afferent pupillary defects using a Sbisa bar. Br
J Ophthalmol. 2002 September; 86(9): 985–987.

15. Johnson R, Bell R. Relative afferent pupillary defect in a lesion of
the pretectal afferent pupillary pathway. Can J Ophthalmol.
1987;22:282-284.

16. Forman S, Behrens M, Odel J, Spector R, Hilal S. Relative afferent
pupillary defect with normal visual function. ArchOphthalmol. 1990;
108:1074-1075.

17. Girkin C, Perry J, Miller N. A relative afferent pupillary defect
without any visual sensory defect. Arch Ophthalmol. 1998;116:1544-
1545.

First Author
Sumita Sethi MS

52 DOS Times - Vol. 14, No. 8, February 2009

Bilateral Anophthalmos in Two Brothers: Photo Essay
A Case Report

Garima Agrawal MS, D.C. Mehta MS

A rare case of bilateral congenital anophthalmos in two brothers On examination the babies appeared healthy and smiling
both having the same congenital anomaly is reported. (Figures 1-4) except for the eyes. There was no systemic
Anophthalmos is a rare congenital anomaly and the same anomaly abnormality.
in two siblings is even rarer.
On ocular examination eyelids were present but curved in.
Anophthalmos is a condition in which there is absence of one or Eyelashes were present. Lacrimal puncta were present. Palpebral
both eyes. It is a congenital anomaly. Here we report a case of two aperture was almost absent. After the eyelids were retracted no
siblings with bilateral congenital anophthalmos. ocular tissue was visible clinically except for the conjunctiva in
both the eyes of both the children. (Figures 1-6). Both the orbits
Case Report were small. Ultrasonography revealed no normal ocular tissue.

Two children one 6 months old and the other 2 years old were The 2 children had no other siblings. They were both full term
brought to our OPD by their parents for ocular examination. normal deliveries. The parents were both normal. Mother’s
antenatal history was normal and she had not taken any drug
during the 2 pregnancies.

Figure 1: 2 year sibling with bilateral
anophthalmos

Figure 3: Left eyeball not seen on retraction
of the eyelids in child in figure 1.

Figure 2: Right eye ball not seen on retraction Figure 4: 6 month old sibling with
of the eyelids in child in figure 1 bilateral anophthalmos

M&J Institute of Ophthalmolgy 55
B J Medical College, Ahmedabad.

www.dosonline.org

Figure 5: Right eyeball not seen on retraction Figure 6: Left eyeball not seen on retraction
of eyelid of child in Figure 4 of eyelid of child in Figure 4.

Discussion anophthalmos in one child. Few associated with other
abnormalities and few not.
The first case of anophthalmos was reported by Thomas Bartholin
in 1657. His case had a bilateral anophthalmos associated with Rosenbaum(1917) reports a case of congenital bilateral
abnormalities of nose, mouth, lips and extremities. Von Hippel anophthalmos of a male child who presented with absence of
collected 87 cases in literature out of which 64 were bilateral and both eyes at birth. In other aspects the child was healthy and well
23 unilateral1. developed. On the third day however the child died in convulsions.
No autopsy was done5.
Primary clinical anophthalmos as described by Duke Elder consists
of a small but well formed orbit. The lid are curved in with small Maria DL et al (1967) reported a case of bilateral congenital
orbital aperture. The orbital cavity is lined with conjunctiva. The anophthalmos in a premature male child6.
lacrimal puncta and the eyelashes are usually present or either
may be absent. Certain other associated ocular abnormalities Chapu BA (1975) reported a case of congenital bilateral primary
described are ankyloblepharon, epicanthus, absent lacrimal gland, anophthalmos in a full term otherwise healthy child at birth1.
absent optic nerve, narrow optic canal, an abnormal development
of optic chiasma and lateral geniculate body1. Brunquell PJ et al (1984) report a 27 year old man with X linked
true anophthalmos. No evidence of optic globe, nerves or chiasma
True anophthalmos is a histopathological diagnosis where there was found. Rudimentary structures suggesting optic tracts were
are no recognizable ocular structures present in the orbit. The present. Lateral geniculate nuclei were present but gliotic calcrine
term clinical anophthalmos is used when there is no evidence of a cortex was thinner but had usual lamination. The normal patches
globe or tissue on clinical examination but without any of cytochrome oxidase activity in layers II and III of visual cortex
histopathological confirmation (Duke Elder 1964)1. were absent7.

A number of cases of anophthalmos have been reported. Those Albernaz VS et al reported the imaging findings in patients with
in siblings are very rare. clinical anophthalmos. They found that patients with bilateral
anophthalmos represented a distinct group fom those with
Ashley (1947) has reported a case of bilateral anophthalmos in a unilateral anophthalmos. Bilateral anophthalmos was associated
brother and sister2. with absence of optic chiasma, reduced size of posterior optic
pathways and agenesisn or dysgenesis of corpus callosum. Patients
Sensi A et al (1987) reported monolateral or bilateral anophthalmos with unilateral anophthalmos had severe cranio-facial anomalies.
recurring in the absence of other associated defects in 6 members Imaging of the face is helpful in patients with unilateral
of a family. The malformation appears to be inherited as a anophthalmos8.
dominant(autosomal/X linked) trait with incomplete penetrance3.
The etiology of this disorder is complex Genetic and environmental
Ben Ezra D, Sela M, Peer J reported a case of four uneventful causes have been postulated. Studies that can specifically identify
pregnancies and deliveries in one family. 2nd sibling suffered from the causative factors for this disorder are warranted.
bilateral anophthalmia while 4th presented with a unilateral
microphthalmia. The microphthalmia was due to in utero globe Conclusion
perforation as evident by a central corneal scar with cataractous
lens4. A case of bilateral anophthalmos in two brothers is reported. This
is a very rare case where the same anomaly of bilateral
Other than this there have been reports of isolated bilateral anophthalmos has been seen in two brothers. The author could
not come across a similar article in literature.

56 DOS Times - Vol. 14, No. 8, February 2009

References 6. MariaDL, Srivastava SK, Sankholkar PS. Bilateral congenital

anophthalmos. Indian J Ophthalmol 1967;15:241-2.

1. Chapu BA. Bilateral primary anophthalmos. Indian J 7. Brunquell PJ, Papale JH, Horton JC, Williams RS, Zgrabik MJ, Albert

Ophthalmol.1975;23(4):36-37. DM, Hedley-Whyle CT. Sex linked hereditary bilateral

2. Ashley. Bilateral Anophthalmos in a brother and sister.J anophthalmos. Pathologic and radiologic correlation. Arch

Hered.1947;38:174-176. Ophthalmol.1984;102(1):108-113.

3. Sensi A, Incorvaia C, Sebastian A, Calzolari E.Clinical Anophthalmos 8. Albernaz VS, Castillo M, Hudgins PA, Mukherji SK. Imaging

in a family. Clinical Genetics.1987;32(3):156-159. findings in patients with clinical anophthalmos.Am J

4. Ben Ezra D, Sela M, Peer J. Ophthalmologica 1989;198(3):140-4. Neuroradiology.1997;18(3):555-561.

5. Rosenbaum.Congenital bilateral anophthalmos. Can Med Assoc
J.1919 March;9(3):254-256.

First Author
Garima Agrawal MS

www.dosonline.org 57

Management Dilemma in a case of Clinical Meeting: Clinical Case 1
Traumatic Glaucoma

Julie Pegu MS, Suneeta Dubey MS, Manisha Agarwal MS

Serious elevation of intraocular pressure (IOP) after trauma is perception of light, and IOP was 50 mm Hg. There was presence
the result of functional impairment of the aqueous outflow of ciliary congestion, diffuse corneal epithelial edema and diffuse
pathways. With blunt injury, the typical sequence of events is an hyphema. The pupil was irregular, semi dilated and sluggishly
initial tearing of the angle structures (contusion angle injury), which reacting to light. There were multiple sphincter tears present
is frequently associated with intraocular bleeding and inflammation superiorly. The rest of the anterior segment (AS) view was hazy on
and results in scarring and permanent reduction in the facility of day one. B scan USG showed signs of vitreous hemorrhage. Routine
outflow. Traumatic glaucoma is often refractory to conventional anti glaucoma treatment was initiated with tab acetazolamide (half
treatment and most cases may require surgical intervention. tab TID) and timolol maleate, 0.5%, (BID), along with topical
However, there is no set protocol for the surgical management as corticosteroids and cycloplegics. On day four, the hyphema had
the results are unpredictable because of the complex nature of cleared sufficiently and the AS view was clearer which showed the
trauma. The treatment therefore, should be individualized presence of mild inferior iridodonesis (Figure 1). The inferior
depending on the extent of injury and the visual prognosis. This iridodonesis made us to suspect the presence of some amount of
case represents the dilemma a clinician can encounter while
managing a case of traumatic glaucoma.

Case

A 14 year male presented to us on 15th may 2008 with the history
of blunt trauma to the right eye with a cricket ball, following which
he complained of severe pain, redness and marked diminution of
vision in the right eye. There was no history of presence of any
systemic disease or blood disorders. There was no family history
of glaucoma.

On slit lamp examination, the left (LE) was essentially within
normal limits. The vision in the right eye was decreased to

Figure 2: Vitreous from behind the lens
blocking the lumen of the tube

Figure 1: Slit lamp picture of the right eye Figure 3: Pulling of the vitreous tag with an
on the fourth day end gripping forcep after anterior vitrectomy

Dr. Shroff's Charity Eye Hospital, 61
5027 Kedarnath Road, Daryaganj, New Delhi

www.dosonline.org

Figure 4: After vitrectomy the Figure 5: At 6 months
tube was cleared of the vitreous follow up
Few vitreous strands were seen inferiorly in
anterior chamber but not blocking the tube

zonular dehiscence inferiorly but there was no obvious phacodonesis Figure 6: Well formed bleb at
or vitreous prolapse. The IOP was still high at 46 mm Hg. 6 months follow up
Consequently, anti glaucoma treatment was stepped up to maximal
medical treatment (glycerol syrup and brimonidine eye drop were Discussion
added). From day six today 24, the vision had improved from
counting finger at one meter to 6/36, but the IOP was still very In severe trauma with associated findings like hyphema, angle
high at early forties. Gonioscopy done on the third week, revealed recession and vitreous hemorrhage, the outlook for
angle recession extending 120 to 180 degrees with a resolving blood trabeculectomy and other drainage procedures is relatively
clot present inferiorly. Rest of the angle was wide open. The left unfavourable. Trabeculectomy is reported to be more frequently
eye had wide open angles. The vitreous hemorrhage had cleared successful in patients over the age of 40 years1 than in younger
by the third week and the fundus was found to be within normal patients. In one of the study2, the success rate of trabeculectomy
limits with a cup disc ratio of 0.4 : 1. The LE had a C:D ratio of 0.3:1. was only 38% (17/45) in patients aged 10 to 29 years whereas it was
At this stage we decided to intervene surgically. We were weighing 65% (43/65) in patients aged 30 to 49 years. As the young people
two options, either to do trabeculectomy with MMC or a tube are more susceptible to trauma, the surgical prognosis becomes
implant. In this case, we decided to choose the later as the patient poorer because of their relative youth. The young age of the
was young and the presence of inferior iridodonesis could have patients produces a strong tendency for blebs to scar down and
complicated the trabeculectomy surgery, as sudden decompression fail. The adjunctive use of cytostatic agents have shown favourable
of the anterior chamber (AC) might lead to vitreous prolapse. results when applied but its use can have the serious disadvantage
With valved implants the results tend to be more predictable as of tending to produce dangerously thin blebs in patients who have
the AC is maintained throughout, and complications like a long expectancy of life3. Also, the use of mitomycin C can lead to
postoperative hypotony are uncommon. So, right eye Ahmed
valve implantation was done on day 28. The surgery was uneventful.
On first postoperative day the IOP came down to 10 mm Hg but
on the fourth day, the IOP again rose to 52 mm Hg and we noticed
a band of vitreous coming from behind the inferior part of the
lens and blocking the tube (Figure 2). It is important to mention
here that there was no vitreous in the AC pre-operatively but due
to the negative pressure in the AC, vitreous got pulled from the
site where mild inferior iridodonesis was noted. Anti glaucoma
drugs were started but the IOP was uncontrolled and on day 10
surgical removal of the vitreous tag was done. The procedure
involved cutting the vitreous tag midway with vitrectomy cutter
and then pulling the tag from the tube lumen with an end gripping
forcep (Figure 3). Postoperatively, the IOP was controlled and the
tube was free of vitreous block (Figure 4). In the last follow up (at
6 months), the IOP was maintained at low teens, vision was 6/9,
the tube was free of any blockage (Figure 5) with a well formed
bleb (Figure 6).

62 DOS Times - Vol. 14, No. 8, February 2009

serious ocular complications like late endophthalmitis and ocular with anti metabolites or the use of an artificial draining device,
hypotony that may result in permanent visual loss4,5. There are with frequent follow ups3. Finally, patients having filtration surgery
limited studies on the long term surgical outcome in traumatic should be carefully instructed on the early signs and symptoms of
glaucoma. In one of the largest series of 87 cases of traumatic bleb-related endophthalmitis and told to report immediately if
glaucoma by Mermoud et al6 with a mean follow up of 23 months these occur, as early treatment appears to be associated with an
(range, 3 to 72 months), the success rate in trabeculectomy group improved prognosis for visual recovery and bleb function.
(47 cases) was 53%, in trabeculectomy combined with anti-
metabolite group (20 cases) it was found to be 70% and in the References
molteno implant group (20 cases) it was 60%. Of concern were
three cases of late bleb infection in the group that received 1. Stewart RH, Kimbrough RL, Bacch H, Allbright M. Trabeculectomy
postoperative antimetabolite therapy, one of whom lost all sight. and modifications of trabeculectomy. Ophthalmic Surg 1979; 10:76-
In another long term series 3, insertion of molteno implants in 80.
traumatic glaucoma produced IOP control at 10.9 years in 76% of
the cases (40 cases). There were no implant related infections. 2. Gressel MG, Heuer DK, Parrish RK II. Trabeculectomy in young
However, there were eleven IOP related surgical interventions, patients. Ophthalmology 1984; 91:1242- 6.
most related to blockage or malposition of the internal end of the
tube, but the IOP was controlled after clearance of the vitreous 3. Fuller JR, Bevin TH, Molteno AC. Long term follow up of traumatic
with vitrectomy. In traumatic glaucoma, the success rate of the glaucoma treated with Molteno implants. Ophthalmology 2001;
surgical complications is expected to be higher because of the 108:1796-1800.
complex trauma, but if treated with vigilance and on time, most of
it can be managed with a favourable prognosis. 4. Greenfield DS, Suner IJ, Miller MP, et al. Endophthalmitis after
filtration surgery with mitomycin. Arch Ophthalmol 1996; 114:943-
Conclusion 9.

Traumatic glaucoma encompasses a spectrum of disease. Because 5. Higginbotham EJ, Stevens RK, Musch DC, et al. Bleb related
of the greater chance of failure of primary trabeculectomy in endophthalmitis after trabeculectomy with mitomycin C.
traumatic glaucoma, it has been suggested that this type of Ophthalmology 1996;103:650-6.
glaucoma would be best managed by trabeculectomy combined
6. Mermoud A, Salmon JF, Barron A, et al. Surgical management of
post-traumatic angle recession glaucoma. Ophthalmology 1993;
100:634-42.

First Author
Julie Pegu MS

Monthly Clinical Meetings Calendar 2008-2009

Dr. R.P. Centre for Ophthalmic Sciences Centre for Sight
3rd August, 2008 (Sunday) 7th December, 2008 (Sunday)

Venu Eye Institute & Research Centre Mohan Eye Institute
7th September, 2008 (Sunday) 28th December, 2008 (Sunday)

Army Hospital (R&R) DOS Picnic
28th September, 2008 (Sunday) Sunday, 4th January, 2009

World Sight Day Shroff Charity Eye Hospital
8th October, 2008 (Wednesday) 1st February, 2009 (Sunday)

Sir Ganga Ram Hospital Guru Nanak Eye Centre
2nd November, 2008 (Sunday) 22nd February, 2009 (Sunday)

Midterm Conference of DOS Bharti Eye Foundation
22nd & 23rd November, 2008 (Saturday - Sunday) 8th March, 2009 (Sunday)

Annual Conference of DOS 20th-22nd March, 2009 (Friday, Saturday & Sunday)

www.dosonline.org 63

Posterior Sympathetic Ophthalmia in a Child Clinical Meeting: Clinical Case 2

Ritesh Narula MS, Manisha Agarwal MS, S.P. Chaudhary MS, Priyata Seth DO

We report a case with an atypical presentation of sympathetic fluorescein angiography (FFA) of the left eye showed multiple
ophthalmia (SO) in a child caused by multiple surgical sites of choroidal leakage with coalescence of dye under serous
interventions in the exciting eye. Fundus fluorecein angiography detachment of the retina. (Figure 2a-e).Optical coherence
and Optical coherence tomography findings were noted. Early tomography of the left eye showed multiple pockets of subretinal
diagnosis and management with intravenous methyl-prednisolone fluid with hyperreflective echoes underneath the serous retinal
and immunosuppressants helped in salvaging the only seeing eye detachment. (Figure 3)
of the child.
The child was diagnosed as sympathetic ophthalmia and treated
Case report with intravenous methylprednisolone for 3 days followed by a
tapering dose of oral prednisolone along with topical prednisolone
A nine- year- old boy presented with decrease of vision in the left acetate 1% six times a day and atropine-1% two times a day in the
eye for 3 days duration. He had a history of cataract surgery with left eye. Enucleation of the right eye was advised which was refused
intraocular lens implantation done at the age of nine months by the father of the child.
elsewhere followed by membranectomy under general anesthesia.
He had a history of blunt trauma with a finger to the right eye Follow-up at one week the BCVA in the left eye improved to 6/9,
causing retinal detachment for which he underwent pars plana < N36 (pharmacologically dilated pupils under atropine-1%).
vitrectomy with IOL repositioning and was subsequently diagnosed Applanation tonometry recorded an IOP of 16mm of Hg in the
to have recurrent total retinal detachment for which no left eye. Fundus examination of the left eye showed a residual
intervention was advised. pocket of shallow SRF.

On examination the best corrected visual acuity in the right eye At one month follow-up the BCVA in the left eye was 6/6, N9P.
was inaccurate projection of rays and in the left eye 6/24, N24. Applanation tonometer recorded an IOP of 38mm of Hg. Fundus
Applanation tonometry recorded an intraocular pressure of 6 mm examination and OCT of the left eye showed no evidence of SRF
of Hg in the right eye and 14 mm of Hg in the left eye. Slit lamp at the macula. The child was started on oral and topical
examination of the right eye showed organized blood clot in the antiglaucoma drugs. Topical prednisolone-1% was stopped and
anterior chamber with vascular in-growth (Figure 1) and the left oral prednisolone was tapered and stopped.
eye showed minimal retrolental flare. There was no view of the
fundus in the right eye and the fundus examination of the left eye At two months the child presented with distortion of images in the
showed pocket of sub-retinal fluid (SRF) at the macula. Fundus left eye. The BCVA in the left eye was 6/12P, N18P. Fundus
examination and OCT of the left eye showed SRF at the macula
suggestive of recurrent inflammation in the left eye. (Figure 4)

Diagnosing as recurrent sympathetic ophthalmia the child was
treated again with intravenous methylprednisolone followed by
oral and topical prednisolone. Oral Azothioprine - 1.5 mg/kg
body weight was started after a systemic evaluation and complete
blood counts. Enucleation of the right eye was done after an
informed consent. Histopathology of the enucleated eye showed
organized haemorrhage in the anterior and posterior chamber
with histiocytic & foreign body giant cell & granulomatous reaction.
The child showed aprompt recovery and is maintain a best
corrected visual acuity of 6/6, N6 after 6 months of follow up.

Discussion

Figure 1: Right eye showing Sympathetic ophthalmia, also known as sympathetic uveitis, is a
organized blood clot rare bilateral granulomatous panuveitis that occurs after a
with vascular ingrowth penetrating injury to an eye. Following injury to an eye-a result of
either surgery or accident-a variable period of time passes before
Retina and Oculoplasty Services a sight threatening inflammation develops in both eyes. The injured
Dr. Shroff’s Charity Eye Hospital, eye is known as the exciting eye and the fellow eye as the
Daryaganj, New Delhi sympathizing eye1.

The various surgical procedures implicated for sympathetic
ophthalmia are- cataract extraction (particularly when
complicated), iris surgery (iridectomy), retinal detachment repair,
vitrectomy, paracentesis, cyclodialysis and keratectomy2. The risk

www.dosonline.org 65

Figure 2 (A-E): Fundus fluorescein angiography (FFA) of the left eye showed multiple sites of
choroidal leakage with coalescence of dye under serous detachment of the retina

Figure 3: Optical coherence tomography of the Figure 4: Fundus examination and
left eye showed multiple pockets of subretinal OCT of the left eye showed SRF
fluid with hyperreflective echoes underneath at the macula suggestive of

the serous retinal detachment recurrent inflammation in the left eye

of SO increases when these surgical procedures are accompanied DOS Times - Vol. 14, No. 8, February 2009
or followed by additional operations, particularly in the posterior
segment of the eye3. Advances in eye care especially in surgical
techniques have dramatically decreased the incidence of SO in the
past century. The incidence of post-vitrectomy SO has been
estimated at 0.01%4.

On review of the literature SO is said to be extremely small after 3
months though it never reaches zero2, and the typical symptoms
are photophobia, loss of accommodation or watering and signs

66

include bilateral low grade persistent uveitis associated with ophthalmia or just the early stage of classical presentation is
granulomatous (“mutton-fat”) or small, white keratic precipitates, debatable.
diffuse thickening of the iris or iris nodules and Dalen Fuchs nodules
on fundus examination though not pathognomic of SO1. However We report this case to highlight that SO may have an atypical
our case differed in the following ways- the child developed SO clinical presentation and a high clinical suspicion maybe required
several years after the last surgical intervention in the exciting eye, for a timely diagnosis and management to salvage the vision in the
he presented with a sudden drop of vision there was only mild sympathesizing eye which at many a times is the only seeing eye of
retrolental cells and flare in the sympathizing eye with no signs of the patient as in this case.
anterior segment inflammation in either eye. Lastly on fundus
examination there was serous retinal detachment at the macula. References
All these factors made the diagnosis of SO difficult in our case but
based on clinical suspicion we started the child on treatment and 1. Duke-Elder S, Perkins ES. Diseases of the Uveal Tract. Vol 9. In:
were able to salvage the eye. Enucleation of the exciting eye having Duke-Elder S, ed. System of Ophthalmology. Vol 9. London, England:
no visual potential helps in preventing the recurrence of SO by Henry Kimpton; 1966: 558–559.
decreasing the antigen load causing inflammation repeatedly.
Histopathology of the enucleated eye also helped in confirming 2. Lubin JR, Albert DM, Weinstein M. Sixty-five years of sympathetic
the diagnosis of SO in our case. ophthalmia. A clinicopathologic review of 105 cases (1913–
1978).Ophthalmology 1980; 87:109–21.
The presentation in this case closely resembles an entity described
in literarture as “ Posterior sympathetic ophthalmia”. It was first 3. Goto H, Rao NA. Sympathetic ophthalmia and Vogt-Koyanagi-
described by Mcpherson et al5 as a variant of sympathetic Harada syndrome. Int Ophthalmol Clin 1990; 30:279–85.
ophthalmia with predominantly posterior segment signs and
minimal AC reaction. In a long term study of 40 patients from 4. Gass JD. Sympathetic ophthalmia following vitrectomy. Am J
North India by Gupta et al6; 36 of these patients presented with Ophthalmol1982; 93:552–8.
evidence of predominantly posterior segment involvement with
22 eyes of these having no anterior segment inflammation. 5. McPherson SD Jr, Dalton HT. Posterior form sympathetic
Whether these cases represent an atypical form of sympathetic ophthalmia.Trans Am Ophthalmol Soc. 1975;73:251-63.

6. Gupta V, Gupta A, Dogra MR. Posterior sympathetic ophthalmia: a
single centre long term study of 40 patients from north india. Eye.
2008 Dec;22(12):1459-64.

First Author
Ritesh Narula MS

www.dosonline.org 67

Future of Retinal Imaging, Lasers and Therapy Clinical Meeting: Clinical Talk

Cyrus M. Shroff MD

The practise of vitreo-retinal medicine and surgery has changed that may occur with disease, in the RPE cell monolayer in outer
dramatically in the past few years. Gone are the days when retina and subneurosensory retinal space. FAF gives information
retinologists were the butt of jokes like "They always carry their over and above that obtained by fundus photography, fundus
vision charts on them - their hands!" The past year has been a fluorescein angiography and optical coherence tomography. It
satisfying one for us, a year primarily of consolidation of some of would be useful in understanding pathophysiologic mechanisms,
the recent advances in diagnostics, drugs (the anti-VEGF phenotype-genotype correlation, identification of predictive
revolution!) and lasers. We will review the advances made in the markers for disease progression and monitoring of new therapies.
retinal scenario along with exciting future possibilities under the Examples of diseases where this holds promise are AMD especially
following headings: the dry type, macular and diffuse retinal dystrophies, central serous
retinopathy, chorio-retinal inflammatory disease and Angioid
• Retinal Imaging streaks. It's clinical value coupled with its simple, efficient and non-
invasive nature is increasingly appreciated.
• Lasers
Retinal lasers
• Drugs and their applications -with special reference to AMD
and Diabetic Retinopathy. Expanding indications of anti-VEGF The pattern scan laser (PASCAL) has been an interesting
agents. development. The PASCAL laser's capabilities can be maximally
exploited in fresh cases for pan retinal photocoagulation where it
• Pushing the frontiers is indeed very quick. (Figure 3) Pan retinal photocoagulation can
also be achieved with lower delivery of energy to the retina.
• Gene Therapy However when fill in treatment is required it often needs to be
used as a single spot laser, as also when there are media opacities
• Nano Medicine or hemorrhage. High cost and lack of indirect ophthalmoscopic
delivery have also been limiting factors.
• Artificial Retina- bionic eye
Nonthermal Retina Regeneration Therapy (2RT; Elex, Adelaide,
Retinal Imaging Australia), is a short-pulsed laser treatment (pioneered by John
Marshall and Peter Hamilton of UK) that is intended to treat
Spectral and ultra-high resolution OCT are now quite firmly diabetic maculopathy and macular edema at an earlier stage of
ensconced in the armamentarium of retinal diagnostics and these the disease. The therapy may also have application in the treatment
have the advantage of 3 Dimensinal imaging. Also 100% of the of early-stage age-related macular degeneration.
mapped area is measured in Spectral domain compared to only
5% in time domain, so there are less chances of missing a small The laser used for 2RT is a Q-switched, green Nd:YAG laser with
lesion with the newer modalities. (Figure 1) One of the a wavelength of 532 nm. Its pulse duration is 3 nanoseconds with
manufacturers of SD-OCT (Bioptigen) provides a novel feature energy of 1 J per pulse which causes the RPE cells to release matrix
of a hand-held probe opening up new capabilities to researchers metalloproteinase enzymes, which are believed to 'clean up' the
and clinicians - it's easily used on experimental animals, in small
children and immobile patients. Soon we may also have capability
of intra-operative OCT attached to the microscope.

In the future we may be using adaptive optics SLO for ultimate
high resolution OCT which can resolve a cone and could be used
for the early detection of retinal degeneration.

A combination of Spectral OCT and SLO which can perform
microperimetry of small specific areas of retina Functional results
can be overlaid on fundus image FA / ICG image and thickness
map hence it combines analysis of structure and function.
(Figure 2)

Fundus autofluorescence imaging (FAF) has been in the spotlight.
There was an excellent review in "Retina" of March 2008 by Schnitz
- Valckenburg, Holz, Bird & Spaide1. This is a novel imaging method
that allows mapping of lipofuscin (LF) and other fluorophores

Dr. Shroff's Charity Eye Hospital & Figure 1: Time Domain versus
Shroff Eye Centre, Spectral Domain OCT
New Delhi
69
www.dosonline.org