Download OCT in Diabetic Macular Edema

Contents
5
27
Editorial
Photo Essay
55
Bilateral Anophthalmos in Two Brothers: A Case Report
Garima Agrawal MS, D.C. Mehta MS
Orthokeratology
Kirti Singh
Clinical Monthly Meeting
Retina
9
69
Case 2- Posterior Sympathetic Ophthalmia in a Child
Cornea & External Disease
Clinical Talk - Future of Retinal Imaging, Lasers and Therapy
Cyrus M. Shroff
Benign Intracranial Hypertension
Ronak P. Bhandari, Nayana A. Potdar, Chhaya A. Shinde,
Pallavi G. Sarate
35
65
Ritesh Narula, Manisha Agarwal, S.P. Chaudhary, Priyata Seth
Outcome of Endophthalmitis Cases in North East India
Harsha Bhattacharjee, Satyen Deka, Hemlata Deka, Manav Jyoti Barman,
Lokesh Jain, Kruto Kalita
21
Case 1- Management Dilemma in a case of Traumatic Glaucoma
Julie Pegu, Suneeta Dubey, Manisha Agarwal
OCT in Diabetic Macular Edema
Jatin Ashar, Pradeep Venkatesh, Bhavin Shah
15
61
77
81
Abstract
Membership Form
Goldenhar Syndrome: A case report & review of literature
Uday Gajiwala, Rajesh Patel, Manoj Gangwal
41
Pseudophakic Bullous Keratopathy
Shreekant A. Damgude, B.P. Guliani
85
Tearsheet
Target IOP
Neuro-Ophthalmology
49
Shibal Bhartiya, Subha Bansal
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
Sign-up facility for new DOS members
Online DOS TIMES at www.dostimes.org
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
Jatin Ashar MBBS, Pradeep Venkatesh MD, Bhavin Shah MBBS
M
acular edema is the most common cause of moderate visual
loss in patients with diabetes.
Macular edema is defined as a focal or diffuse area of retinal
thickening which may or may not be associated with hard exudates.
Depending on its location and size it can either a clinically significant
or non-significant.
A number of clinical methods and investigations have been used
for long for the assessment of macular edema in diabetic patients.
The slitlamp biomicroscopy has been the gold standard clinical
method for looking for macular edema. Investigative modality
like the fluorescein angiography have been used to quantify the
intraretinal leakage in these patients, though FA is useful only as a
guide for focal or grid treatment of thickened areas1. It is the retinal
thickening and not the amount of leakage that is important in the
determination of final visual outcome of patients with diabetic
macular edema.
OCT is a non contact, non invasive, micron resolution crosssectional study of retina which correlates very well with the retinal
histology.
Based on OCT DME can be classified into different pattern such
as cystoid macular edema, spongy swelling of the retina, hard
exudates, serous detachment, macular traction, taut posterior
hyaloid membrane.
Sponge like Thickening
•
most common change (Figure 1)
•
mostly in outer retinal layers while internal layers maintain
their normal reflectivity
•
cross-sectional scans show swelling of the retina giving it a
spongy appearance with increased retinal thickness, from a
normal value of 174 ± 18µm in an area 500microns in diameter
centered on fovea and a central foveal thickness of 152 ± 21
µm 2
•
expanded areas of low reflectivity
•
backscattering seen from intraretinal fluid accumulation
•
ring of hard exudates with shadowing effect
Cystoid Spaces
Role of OCT in DME
•
second most common pattern (Figure 2)
Diagnosis and Quantification of retinal thickening, extension of
retinal thickness, macular volume, retinal morphology and vitreoretinal relationship.
•
intraretinal cystoid spaces
•
involves variable depth of retina
•
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).
Retina
OCT in Diabetic Macular Edema
Retinal Map Analysis
Colour Map
It is a topographic representation of the retinal thickness calculated
from the linear scans.
Figure 1: OCT showing sponge like
diabetic macular edema
Mean Thickness Map
It provides an average thickness of each of 9 different sectors of
macula and has a high reliability and reproducibility.
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
Figure 2: OCT showing cystoid
diabetic macular edema
9
•
has intervening septa in between
•
progresses gradually to involve the whole of retina
Besides the above mentioned patterns other features such as hard
exudates, epiretinal tractional membranes may also be found in
patients with DME
Serous Detachment
Hard Exudates
•
increased macular thickness
•
high reflectivity spots with backscattering
•
hyporeflective areas corresponding to cysts in retina
•
•
may cause subfoveal retinal detachment
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
(Figure 3)
•
•
sometimes satellite retinal detachment may also be found
•
laser can worsen this condition
ERMs exerting an anteroposterior or tangential traction on
the macula can be noted on the OCT scan.
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
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)
Figure 3: OCT showing vitreomacular traction
Summary of OCT based classification of DME8
Retinal Thickness
Volume
Morphology
Epiretinal Traction
1. Fixation point:
Normal: 150 + 20m
Borderline: 170210 m Edema:
> 210 m
Meaningful only for
diffuse edema involving
at least the center and
the 1st ring, and not
for focal edema
well-defined and continuous
hyper-reflecting line over the
inner retinal surface with at
least one point of adhesion to
the retina in at least one of the
retinal map.
2. Central Zone:
Normal: 170 + 20 m
Borderline: 190
-230 m
Edema: > 230 m
Normal: 6.5 mm 3 + 1
Borderline: up to
8.0 mm 3 abnormal:
> 8.0 mm3
Abnormal: > 8.0 mm 3
E1: simple thickening-Compact
retinal thicknening, no clinically
visible cystoid spaces
E2: cystoid thickening - RT with
cysts
E2a: mild 2-4 central small
cysts (horizontal diameter 150200m, vertical diameter 400 m)
E2b: intermediate-cysts with
petaloid configuration or with
central cysts (horizontal diameter
less than 300m, vertical diameter
less than 600 m).
E2c: severe-with coalescence of
cysts with retinoschisis appearance
E3: neuroepithelial detacment
presence of subretinal
liquid (non-reflecting space)
above the hyperrflecting line of
the pigmented epithelium
isolated or associated with simple
or cystoid retinal thickening.
3. Perifoveal and
peripheral areas:
Normal: 230 + 20m
Boarderline: 250290m Edema: more
than 290m
10
T0: absence of epiretinal
hyper-reflectivity.
T1: presence of a continuous
line of flat hyper-reflectivity and
adherent to the retina without
significant retinal distortion.
T2: presence of continuous line
of hyper-reflectivity with multiple
points of adhesion to the retina
distortion.
T3: antero-posterior traction
with “gull wings” configuration.
DOS Times - Vol. 14, No. 8, February 2009
Correlation of OCT with Histopathologic Changes in DME
Histopathology
•
Intracytoplasmic swelling of Muller cells
•
Marked swelling of outer plexiform layer or Henle fiber layer
•
Liquefaction necrosis of the Muller cells
(a)
OCT
•
Sponge-like swellings
•
Prominent space with low reflectivity
•
Cystoid cavity formation
(b)
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
outer retinal layer (30.3%)
Figure 4 (a-b): Pre and post triamcinolone injection OCT
showing significant decrease in macular edema
Type 3: Foveolar detachment without traction (11.7%)
Type 4: Foveolar detachment with apparent vitreofoveal traction
(2.8%)
CSME corresponds to a RT more than 300 microns involving the
central zone and / or RT more than 320 micron in the 1st ring.
Edema confined to second ring is not considered clinically
significant.
•
Tractional detachments
•
Superficial folds with ERM
These patterns can be used in determining the treatment protocols
for macular edema (Figure 4)
•
Not visible if completely attached or completely detached
and too far
•
•
can present with perifoveolar detachment, double convexity
on cross sectional scan
•
can thickken as a sequale of fibrovascular proliferation
Sponge like swelling
•
•
cystoid swelling
•
•
IVTA
Serous detachment
•
•
focal grid / laser
laser & ppv
tractional retinal detachment and taut posterior hyaloid
•
ppv
Changes on OCT in DME
Intraretinal changes
Diffuse swelling: retinal swelling, no cystoid space, outerplexiform
and outer nuclear layers thicken and show hyporeflectivity
Posterior Hyaloid Membrane
Preretinal Hemorrhage
Mask the features of edema on OCT
Summary of OCT based classification of DME 8
Ultra Resolution (UR) OCT4
Advantages over Stratus OCT
•
Ultrahigh resolution make the small distortion in retinal
contour are more evident.
•
Axial resolution of 2 to 3 µm making the visualization of intra
retinal layers simpler.
•
Better visualization of hard exudates on UR-OCT
Cystoid spaces: hyporeflective spaces, in Henie’s and outer
plexiform layer
•
Small cystic changes in the ganglion cell and nuclear cell layers
is evident better than stratus OCT
Foveolar detachment: foveal or extrafoveal cystic spaces may lead
to detachment of foveola.
•
Thin epiretinal membrane not evident on Stratus OCT can
be visualized using UR-OCT.
Hard Exudates: hyperrective intraretinal deposits, outer plexiform
layer
•
Small disruption in the photoreceptor inner segment outer
segment region is also evident on UR OCT.
Inner Retinal Border
Role of OCT vs FA in DME
•
Foveolar edema-flattening of foveal pit
•
Dome shaped elevation of inner retinal boundary
•
Concave slopes due to rise of macular center
FA assess the vascular leakage and quantifies it in macular edema,
however, it is the actual thickness of the retina that correlates with
the final visual outcome of the patient3.
www.dosonline.org
11
Correlation of OCT and FA
Morphologic
9
According to the modified Otani scheme , OCT findings of macular
edema are characterized into four types:
DRT
0.69 + 0.41
20/97
Type 2: thickening of the fovea with markedly decreased optical
reflectivity in outer layers
CME
0.79 + 0.39
20/123
SRD without PHT
0.82 + 0.34
20/132
Type 3: thickening of the fovea with subfoveal fluid accumulation
and distinct outer border of detached retina
PHR with TRD
1.0 + 0.24
20/200
PHT without TRD
0.77 + 0.44
20/118
Type 1: thickening of the fovea with homogenous optical reflectivity
Type 3A: without foveal traction
DRT: diffuse retinal thickening; CME: diabetic cystoid macular
edema; SRD: serous retinal detachment; PHT: posterior hyaloidal
traction; TRD: traction retinal detachment.
Type 3B: with apparent vitreofoveal traction
Fluorescein angiography findings can be categorized into three
types:
Type 1: focal leakage with well defined focal areas of leakage
References
Type 2: diffuse leakage which is widespread and ill defined
1.
Giacomo Panozzo, Elena Gusson, Barbara Parolini and Andrea
Mercanti.. Role of OCT in the diagnosis and follow up of diabetic
macular edema. Seminars in Ophthalmology 2003, Vol. 18, No. 2,
pp. 74–81
2.
The eyes with previous macular focal or grid photocoagulation
tend to show an increased proportion of OCT type 2 and OCT
type 3A compared with eyes without prior photocoagulation.
Bandello, D. Roman Pognuz,, A. Polito, A. Pirracchio, F. Menchini,
M. Ambesi,. Diabetic macular edema: classification, medical and
laser therapy. Seminars in Ophthalmology2003, Vol. 18, No. 4, pp.
251–258
3.
Focal leakage on FA is closely related with type 1 or homogenous
foveal thickening on OCT, while diffuse or cystoid leakage is related
to either type 2 or type 3A or fluid collection predominantly in
outer retinal layers or subretinal space.
Ozdek SC, Erdinc MA, Gurelik G, Aydin B, Bahceci U, Hasanreisoglu
B. Optical coherence tomographic assessment of diabetic macular
edema: comparison with fluorescein angiographic and clinical
findings.. Ophthalmologica. 2005 Mar-Apr;219(2):86-92.
4.
Srinivasan VJ, Wojtkowski M, Witkin AJ, Duker JS, Ko TH, Carvalho
M, Schuman JS, Kowalczyk A, Fujimoto JG. High-definition and
3-dimensional imaging of macular pathologies with high-speed
ultrahigh-resolution optical coherence tomography.
Ophthalmology.2006 Nov;113(11):2054.e1-14.
Type 3: diffuse cystoid leakage with pooling in cystic spaces of
macula
Focal leakage on FA is mostly associated with non proliferative
diabetic retinopathy while diffuse leakage with proliferative diabetic
retinopathy.
OCT vs RTA5
•
OCT has excellent sensitivity and considerable specificity
making it an excellent general screening tool for diabetic
macular edema.
5.
Goebel W, Franke R. Retinal thickness in diabetic retinopathy:
comparison of optical coherence tomography, the retinal thickness
analyzer, and fundus photography. Retina. 2006;26:49–57.
•
RTA has a higher specificity making it the tool to confirm the
indication for laser therapy.
6.
The correlation between fluorescein angiographic and optical
coherence tomographic features in clinically significant diabetic
macular edema. Kang SW, Park CY, Ham D-I. Am J Ophthalmol
137:313–322
7.
Kim BY, Smith SD, Kaiser PK. Optical coherence tomographic
patterns of diabetic macular edema. Am J Ophthalmol. 2006
Sep;142(3):405-12
8.
Panozzo G, Parolini B, Gusson E, Mercanti A, Pinackatt S, Bertoldo
G, Pignatto S. Diabetic macular edema: an OCT-based classification..
Semin Ophthalmol. 2004 Mar-Jun;19(1-2):13-20.
9.
Otani T, Kishi S, Maruyama Y: Patterns of diabetic macular edema
with optical coherence tomography. Am J Ophthalmol 1999;127:688693.
Problems in Performing OCT in Diabetic Patients
•
Poor papillary dilatation
•
Media opacities in form of cataract, vitreous hemorrhage
may affect the scan quality
Mean Visual Acuity Association with Morphological
Subtypes of DME7
V/A for patients without CME increased by 0.16 log MAR units/
100 micron increase in retinal thickness. This reflects on the Snellen
acuity from 20/20 to 20/30
In the presence of CME, the mean increase is of 0.40U on logMAR
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
Mean Visual Acuity Mean Visual Acuity
(logMAR)
(Snellen)
First Author
Jatin Ashar MBBS
DOS Times - Vol. 14, No. 8, February 2009
Retina
Outcome of Endophthalmitis Cases in
North East India
*Harsha Bhattacharjee MS, *Satyen Deka MS, DNB, *Hemlata Deka MS, *Manav Jyoti Barman DO, DNB,
**Lokesh Jain MS, *Kruto Kalita MS
I
n the study 178 patients were evaluated and treated by retina
specialists at our centre between 2003-2008. The diagnosis of
endophthalmitis was based on presence of pain, inflammation
including anterior chamber cellular reaction and flare and vitreous
cells,corneal involvement, decrease in visual acuity and loss of
media clarity with or without the presence of hypopyon. At all
visits, the ocular examinations included best corrected visual acuity,
slitlamp biomicroscopy (anterior segment examination and 90 D
examination of posterior segment) and indirect ophthalmoscopy
for posterior segment examination. Posterior segment evaluation
with B scan USG was carried out where ever possible.Information
collected include history of the precipitating cause,visual status at
presentation,interval between development of symptoms and
treatment at our centre,microbiological profile of organisms
cultured , interventions undertaken and final visual outcome and
ocular status. The primary treatment was always as per the
recommendation of the Endophthalmitis Vitrectomy Study (EVS).
Briefly, this included injection of intravitreal antibiotics to all
patients where the presenting visual acuity was hand movement
close to face (HMCF) or better and eyes with vision less than
HMCF but with corneal involvement severe enough to preclude
vitreous surgery. In vitrectomy cases the primary goal of surgery
was to remove the core vitreous along with vitreous debris. Before
vitrectomy was started 0.5 to 0.1 ml of undiluted vitreous sample
was collected from the mid vitreous by manual aspiration with a 2
ml syringe for microbiological study. After that the infusion fluid
was switched on and vitrectomy started. Intravitreal antibiotics
were injected at the end of the procedure.
Immediate inoculation of the undiluted vitreous samples were
done onto blood agar, chocolate agar, non-nutrient agar,
Sabouraud’s dextrose agar, thioglycolate and brain heart infusion
broth. Sabouraud’s agar plates were incubated at 25 deg celcius to
enhance growth of fungi while rest of the plates were incubated at
37 deg celcius. Blood agar plates were incubated under aerobic
and anaerobic conditions, and chocolate agar was incubated with
5% carbon dioxide. Gram’s stain, Giemsa stain and KOH stain
with calcoflour white were done. A culture was considered positive
when there was growth of the same organism on two or more
media, confluent growth at the site of inoculation on one solid
medium, or growth in one medium with consistent direct
microscopy findings. The final diagnosis was made as bacterial,
fungal, polymicrobial or negative based on the above criteria.
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
BeltolaGuwahati – 78102 (Assam)
**Jain Eye Hospital
204-A GT Road,Shahdara, Delhi
www.dosonline.org
along with a positive fungal culture from a single or subsequent
sample from the same eye.
Intravitreal and topical antibiotics were given to all patients
following standard protocol. For empiric gram negative organism
coverage intravitreal amikacin (0.4 mg in 0.1 ml) or ceftazidime
(2.25 mg in 0.1 ml) were given. For empiric gram positive organism
coverage Vancomycin (1mg in 0.1 ml) was given. In the case of
fungal micro organism being isolated intravitreal amphotericin B
(0.005 mg in 0.1 ml) was given.
Results
Out of 178 endophthalmitis cases 104 (58% ) cases were post
operative, 53 (30% ) post traumatic, 11 (6%) endogenous and 10(6%)
post corneal ulcer perforation cases. Out of 104 postoperative
endophthalmitis cases 9 cases were diagnosed as fibrinous uveitis.
There were 3 post operative cases from our centre.
Postoperative endophthalmitis
The predisposing surgeries included phacoemulsification surgery
(21 cases, 22%), ECCE (31 cases, 33%), SICS (29 cases,31%), phaco
with trabeculectomy (1 case,1%), trabeculectomy with MMC (11
cases,12%) and VR procedures (2 cases, 2%) (Table 1). The duration
between the precipitating surgery and presentation at the institute
was less than 24 hours in 4 (4.2%), between 1 and 7 days in 33
(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
movement in 25 (26.3%) and better than hand movement in 25
(26.3%) of cases (Table 3). Vitreous biopsy culture was positive in
52 (54.7%) cases, with bacteria cultured in 50 (52.6%) and fungi in
8 (8.4%) of cases (sum is greater than the total of 54.7% because of
polymicrobial cultures). The microbiological profile is presented
Table 1: Types of Surgery
Type of surgery
No: of cases
Phaco IOL
21
ECCE IOL
31
SICS IOL
29
Phaco + Trab
1
Trab + MMC
11
VR procedures
2
Total
95
15
Table 2: Duration of presentation of cases
(Postoperative endophthamitis cases)
Table 4: Microbiological spectrum of post operative
endophthalmitis cases
Type of micro organism
Table 3: Presenting visual acuity and final visual acuity
(Post operative endophthalmitis cases)
in Table 4. Out of 104 cases 53 cases received intravitreal antibiotics
only while 42 cases received both intravitreal antibiotics and
vitrectomy.
No: cultured
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
Table 6: Duration of presentation and final outcome
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
endophthalmitis cases in the study is 53. The presenting vision
was light perception or worse in 36 (67.9%), hand movement in 15
(28.3%) and better than hand movement in 2 (3.7%) of cases (Table
5). The duration between the precipitating trauma and
presentation at the centre was less than 24 hours in 5 (9.4%),
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
39 (73.5%) cases, with bacteria cultured in 36 (67.9%) and fungi in
3 (5.7%) of cases (sum is greater than the total of 73.5% because of
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
At followup 18 (34.9%) of 53 patients had improvement in vision
while in 35 (65.1%) cases the vision remained same or deteriorated.
The anatomical outcome was poor (phthisis bulbi, evisceration or
enucleation) in 14 (26.4%) of cases.
Endogenous endophthalmitis
The number of endogenous endophthalmitis cases in the study is
11. The presenting vision was light perception or worse in 7 (63.6%),
hand movement in 3 (27.2%) and better than hand movement in
DOS Times - Vol. 14, No. 8, February 2009
Table 7: Microbiological spectrum (Post traumatic
endophthalmitis cases)
perception or worse in 6 (60%), hand movement in 2 (20%) and
better than hand movement in 2 (20%) of cases (Table 11). The
duration between the development of symptoms and presentation
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
was positive in only 5 (50%) cases, with bacteria cultured in 4 (40%)
and fungi in 1 (10%) of cases. Out of 10 cases 8 cases received
intravitreal antibiotics only while 2 cases received both intravitreal
antibiotics and vitrectomy. Table 13 shows the microbiological
profile of organisms cultured from endophthalmitis cases following
corneal ulcer perforation.
Type of micro organism
No: cultured
Gram pos. Cocci
16
Gram neg. cocci
5
Gram pos. Bacilli
12
Gram neg. bacilli
3
Fungi
3
Negative
12 (30.8%)
At followup 3 (30%) of 10 patients had improvement in vision
while in 7 (70%) cases the vision remained same or deteriorated.
The anatomical outcome was poor in 3 (30%) cases.
Polymicrobial
9.5%
Discussion
1 (9%) of cases (Table 8). The duration between the precipitating
surgery and presentation at the institute was between 1 and 7 days
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
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).
Out of 11 cases 7 cases received intravitreal antibiotics only while
4 cases received both intravitreal antibiotics and vitrectomy. Table
10 shows the microbiological profile of organisms cultured from
post traumatic endophthalmitis cases.
At followup 5 (45.4%) of 11 patients had improvement in vision
while in 6 (54.5%) cases the vision remained same or deteriorated.
The anatomical outcome was poor in 3 (27.2%) cases.
Endophthalmitis following corneal ulcer perforation
In this study the number of cases of endophthalmitis following
corneal ulcer perforation is 10. The presenting vision was light
The main objective was to study the profile of endophthalmitis
cases presenting at the institute and to observe any clinical
presentation features associated with poor visual outcome. The
microbiological profile of organisms cultured from the involved
eyes was also studied. Among the clinical features at presentation,
importance was laid on visual acuity at presentation and duration
between onset of symptoms and presentation at the institute and
to observe whether these two factors had any bearing on the final
outcome. For the purpose of analysis the presenting visual acuity
was divided into three groups. They constituted vision less than or
equal to light perception, hand movement and better than hand
movement. The duration of presentation was also divided into
three categories, which were a duration of less than 24 hours,
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
> HM
Total cases
1 (9%)
11
1
5 (45.4%)
6 (54.5%)
3 (27.2%)
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
1 – 7 days
5 (45.5%)
3
2
> 7 days
6 (54.5%)
2
4
Total cases
www.dosonline.org
11
5 (45.4%)
6 (54.5%)
3
3 (27.2%)
17
Table 10: Microbiological spectrum (Endogenous
endophthalmitis)
Type of micro organism
No: cultured
Gram pos. Cocci
2
Gram neg. Bacteria
presenting between 1 and 7 days, 19 (73%) of the cases had a poor
outcome. Among the culture positive cases (39) there was a poor
outcome in 27(69.2%) cases while improvement occurred in12
(30.8%) of the cases. As such poor presenting visual acuity and a
culture positivity for any organism are indicative of an unfavourable
outcome in post traumatic cases. This is in agreement with
numerous studies conducted in different parts of the world.
In the endogenous and post corneal ulcer perforation cases the
study groups were too small to detect any specific trends.
Gram pos. Bacilli
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
Gram neg. Bacilli
Fungi
1
Negative
9 (81.8%)
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
> HM
2 (20%)
1
1
10
3 (30%)
7 (70%)
Total cases
3 (30%)
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
18
1 – 7 days
5
2
3
> 7 days
5
1
4
3
Total cases
10
3 (30%)
7 (70%)
3 (30%)
initial visual acuity d” LP there was visual improvement in only 5
(11.1%) cases while in 40 (88.8 %) cases the vision remained the
same or deteriorated. There was poor anatomical outcome in 7
(15.5%) cases. Majority of the cases 58 (61 %) presented at the
institute after 7 days and among them the outcome was poor in 41
(70.6 %) cases. Among culture positive cases 67% had a poor
outcome while improvement was present in 33% cases. Therefore
three factors indicative for an unfavourable outcome in
postoperative cases in this study were a poor presenting visual
acuity, delayed presentation and culture positivity for any organism.
This has been established in a large number of studies.
Table 13: Microbiological spectrum (endophthalmitis
following corneal ulcer perforation)
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.
The anatomical outcome was poor in 11 (30.5 %) cases. The
number of cases presenting late (> 7 days) were 22 (41.5%) out of
which 15 (68.5%) cases had no improvement of vision. The
anatomical outcome was poor in 8 (36.3%) cases. The outcome
was also poor in cases presenting within 7 days. Out of 26 cases
Type of micro organism
No: cultured
Gram pos. Cocci
3
Gram neg. cocci
1
Gram pos. Bacilli
1
Gram neg. bacilli
1
Fungi
1
Sterile culture
5 (50%)
At followup 3 (30%) of 10 patients had improvement in vision
while in 7 (70%) cases the vision remained same or deteriorated.
The anatomical outcome was poor in 3 (30%) cases.
DOS Times - Vol. 14, No. 8, February 2009
as regards post operative Endophthalmitis one can go a long way
in saving the eye by early recognition of the problem and timely
intervention.
7.
Gupta A, Gupta V, Gupta A, Dogra MR, Pandav SS. Spectrum and
clinical profile of post cataract surgery endophthalmitis in North
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
following in-patient surgery. Arch Ophthalmol 1991;109:1085-89.
9.
TThompson WS, Rubsamen PE, Flynn HW, Schiffman J, Cousins
SW. Endophthalmitis after penetrating trauma: risk factors and
visual acuity outcomes. Ophthalmology 1995;102:1696-701.
Jalali S, Das T, Gupta S. Presumed noninfectious endophthalmitis
after cataract surgery. J Cataract Refract Surg 1996;22:1492-97.
2.
Speaker MJ, Menikoff JA. Prophylaxis of endophthalmitis with
topical povidone- iodine. Ophthalmology 1992;98:1769-75.
3.
Endophthalmitis Vitrectomy Study Group. Results of the
endophthalmitis study group. A randomized trial of immediate
vitrectomy and of intraocular antibiotics for the treatment of postoperative bacterial endophthalmitis. Arch Ophthalmol
1995;113:1479-96.
Wisniewski SR, Hammer ME, Grizzard WS, Kelsey SF, Everett D,
Packo KH, et al. An investigation of the hospital charges reltaed to
the treatment of endophthalmitis in the endophthalmitis vitrectomy
study. Ophthalmology 1997;104:739-45.
5.
Kunimoto DY, Das T, Sharma S, Jalai S, Majji AB, Gopinathan U, et
al. Microbial spectrum and susceptibility of isolates. Part I. Postoperative endophthalmitis. Am J Ophthalmol 1999;128:240-42.
6.
Anand AR, Therese LK, Madhavan HN. Spectrum of aetiological
agents of postoperative endophtalmitis and antibiotic susceptibility
of bacterial isolates. Indian J Ophthalmol 2000;48:123-28.
11. Thompson JT, Parver LM, Enger CL, Mieler WF, Liggett PE.
Infectious endophthalmitis after penetrating injuries with retained
intraocular foreign bodies. Ophthalmology 1993;100:1468-74.
12. Alfaro DV, Roth D, Liggett PE. Post-traumatic endophthalmitis:
Causative organisms, treatment, and prevention. Retina
1994;14:206-11.
13. Jalali S, Das T, Majji AB. Hypodermic needles: a new source of
penetrating ocular trauma in Indian children. Retina 1999;19:21317
14. Das T,Kunimoto DY,Sharma S,Jalali S, Majji AB,Gopinathan U,Rao
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
Extra Word: SYNDROME
6.
2.
DUANE
1.
MOBIUS
BROWN
7.
3.
CENTURION
HORNER
WAARDENBURG
8.
TURNER
4.
www.dosonline.org
REITER
4.
10. Williams DF, Mieler WF, Abrams GW, Lewis H. Results and
prognostic factors in penetrating ocular injuries with retained
intraocular foreign bodies. Ophthalmology 1988;95:911-16.
5.
1.
19
Ronak P. Bhandari MS, Nayana A. Potdar MS, Chhaya A. Shinde MS, Pallavi G. Sarate MS
T
he presentation of a patient with increased intracranial
pressure and papilloedema is an emergency. Although this
scenario may be the harbinger of a brain mass, other clinical entities
should be considered. A significant number of patients presenting
in the above fashion are diagnosed with the syndrome of
pseudotumor cerebri. Although idiopathic intracranial
hypertension, pseudotumor cerebri, and benign intracranial
hypertension are synonymous with this diagnosis, the preferred
term is idiopathic intracranial hypertension.
abduction. It was bilateral sixth nerve palsy (Figure 2, 3). Anterior
segment was within normal limits except pupils were bilaterally
equal, 3 mm in size and sluggishly reacting to light. On fundus
examination both eyes showed that the disc margins were blurred,
discs were hyperemic (Figure 4). Retinal veins were dilated,
tortuous. Spontaneous venous pulsations were absent. There were
flame shaped retinal hemorrhages near the disc. There was bilateral
severe papilloedema. Visual fields was not possible due to
uncooperative patient.
The diagnostic criteria include the following: symptoms and signs
restricted to those of elevated intracranial pressure; normal findings
on neuroimaging studies, excluding nonspecific findings of raised
intracranial pressure; and increased cerebrospinal fluid pressure
with a normal composition.
A CT scan brain was done which was within normal limits
(Figure 5). Routine blood investigations like complete blood count,
blood sugars, liver, renal functions and serum electrolytes were
within normal limits. X-ray chest was within normal limits. Patient
Retina
Benign Intracranial Hypertension
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
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 2: Right Lateral Rectus Palsy
Figure 1: Pre-Op Photo on Presentation
Figure 3: Left Lateral Rectus Palsy
Lokmanya Tilak Memorial General Hospital,
Sion Mumbai
www.dosonline.org
21
Figure 4: Papilloedema
Figure 5: Normal CT Scan
was transferred to neuromedicine. CSF cytology and biochemical
studies done were within normal limits. CSF culture was negative
and meningitis was ruled out. However CSF manometry showed
pressures greater than 45 cm H2O. (Normal is between 15-25 cm
H2O). The patient was started on intravenous mannitol and oral
glycerol as measures to reduce the intra cranial pressure. The
ventricular system was normal on MRI brain with orbit. A
diagnosis of pseudotumor cerebri was established and patient
was taken for Right sided theco-peritoneal shunt surgery.
(Figure 8) On post op day 2 the CSF pressure was recorded as 22
cm H2O and the symptoms of the patient improved dramatically
(Figure 6). The vision on day 7 was both eyes 6/24. On fundus
examination the disc edema was less in both eyes (Figure 7). Retinal
hemorrhages were getting resolved. Disc hyperemia was reduced.
Papilloedema was resolving in both eyes.
brain tumor without a brain tumor being present (pseudo meaning
false). The space around the brain is filled with water-like fluid.
This space containing the fluid cannot expand. It is high pressure
in this space that produces the symptoms of idiopathic intracranial
hypertension (IIH).
The vision after 6 weeks was 6/9 uncorrected in both eyes. The
patient was advised regular follow up. His vision and field changes
were monitored.
Discussion
Idiopathic Intracranial Hypertension is a condition of high pressure
in the fluid around the brain. It is also known as pseudotumor
cerebri because there are some of the signs and symptoms of a
Incidence
Studies of American-based populations have estimated that the
incidence of pseudotumor cerebri ranges from 0.9-1.0 per 100,000
in the general population. This incidence rate increases to 1.6-3.5
per 100,000 in women, and 7.9-19 per 100,000 in women who are
overweight.1
A strong predilection of this disease exists for women. In the
literature, the female-to-male ratio ranges from 2:1 to 10:1.
Although idiopathic intracranial hypertension may affect
individuals of any age, most patients with this disease present in
the third decade of life. Idiopathic intracranial hypertension does
occur in the pediatric population; these younger patients are often
not obese.
Symptoms and Signs
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 8: Scar of Surgery
Figure 7: Post-Op Fundus
Headache (94%): Headache is present in nearly all patients with
IIH and is the usual symptom for which patients seek medical
attention. The headaches of the IIH patient are usually severe
throbbing and daily. They are different from previous headaches,
may awaken the patient and usually last for hours. Nausea and
vomitting less common. The headache is often the worst head
pain ever experienced. Although uncommon, the presence of pain
behind the eyeball that is worsened with the movements of the
eyes can occur. With Valsalva maneuver the intra cranial pressure
increases and hence the symptoms.
Transient visual obscurations: Visual obscurations are episodes of
transient blurred vision that usually last less than 30 seconds and
are followed by full recovery of vision. Visual obscurations occur
in about 3/4 of IIH patients. The attacks may be involving one or
both eyes. They are not correlated with the degree of intracranial
hypertension or with the extent of optic nerve swelling. Visual
obscurations do not appear to be associated with poor visual
outcome.
Table 1: Modified Dandy Criteria2
•
Signs & symptoms of increased ICP - CSF pressure
>25 cmH2O
•
No localizing signs with the exception of abducens
nerve palsy
•
Normal CSF composition
•
Normal to small (slit) ventricles on imaging with no
intracranial mass
to subretinal hemorrhages are poor visual prognostic signs. In
some instances, the disc swelling is asymmetric, or, rarely, the
appearance of the optic nerve may be relatively normal.
Pulsatile intracranial noises: Pulsatile intracranial noises or pulsesynchronous tinnitus is common in IIH. The sound is often
unilateral. In patients with intracranial hypertension, compression
of the jugular vein on the side of sound abolishes it. The periodic
compressions were thought to convert the laminar blood flow to
turbulent.
If left untreated, chronic disc swelling eventually leads to clinically
significant visual loss. Although all patients present with enlarged
blind spots during their initial perimetry, uncontrolled papilledema
results in progressive peripheral visual field constriction or nerve
fiber bundle defects (eg, nasal depression, nasal steps, arcuate
scotomas). The central visual field is affected in end-stage chronic
papilledema.
Visual loss: The most serious problem patients have is vision loss.
About 5% of patients go blind in at least one eye. These are usually
patients who do not return for follow-up evaluation.
Sudden loss of central vision may result from an associated anterior
ischemic optic neuropathy, a vascular occlusion, or an associated
subretinal neovascular membrane.
Diplopia: Patients who present with double vision most frequently
complain of horizontal displacement of the images. Vertical diplopia
is rare, but it has been reported
The diplopia noted in patients with idiopathic intracranial
hypertension is invariably due to unilateral or bilateral sixth nerve
palsy. These cranial nerve palsies diminish with the lowering of
the intracranial pressure. Occasionally, patients with diplopia
present with oculomotor or trochlear nerve palsy. In rare instances,
vertical diplopia is due to a skew deviation.
Physical
The most significant finding in patients with this disease is bilateral
disc edema secondary to the increased intracranial pressure. This
papilledema varies from patient to patient and is indistinguishable
from optic nerve swelling caused by intracranial space-occupying
lesions. In more pronounced cases of disc swelling, macular
involvement with subsequent edema and diminished central vision
may be present. High-grade and atrophic papilledema in addition
www.dosonline.org
Causes
Most cases of pseudotumor cerebri occur in young women who
are obese and, less frequently, in men who are otherwise healthy.
If this disorder presents in an individual who is not overweight,
ruling out associated risk factors is necessary.
23
Risk factors
•
Drusen of the optic nerve heads
Exogenous substances
•
Malignant hypertension
•
The list of exogenous substances associated with idiopathic
intracranial hypertension is extensive. Although an association
exists between these substances and this disorder, the exact
causal relationship is somewhat lacking in the literature.
•
Bilateral infiltrative/infectious/inflammatory optic neuropathy
•
Bilateral anterior ischemic optic neuropathy
•
Bilateral optic nerve papillitis
Exogenous substances associated with idiopathic intracranial
hypertension include amiodarone, antibiotics (eg, nalidixic
acid, penicillin, tetracycline), carbidopa, levodopa,
chlordecone, corticosteroids (eg, topical, systemic),
cyclosporine, danazol, growth hormone, indomethacin,
ketoprofen, lead, leuprolide acetate, levonorgestrel implants,
lithium, oral contraceptives, oxytocin, perhexiline, phenytoin,
and vitamin A (>100,000 U/d)/retinoic acid.
•
Bilateral optic nerve tumors (eg, glioma, meningioma).
•
Systemic diseases
The main complication of this disorder is progressive optic
neuropathy. Despite timely treatment, some patients develop an
optic neuropathy related to the optic nerve edema. Generally, this
dysfunction presents in a progressive fashion with constriction of
the peripheral visual field; worsening nerve fiber bundle visual
field defects; a decrease of color vision; and, in end-stage disease,
a drop in the central visual function. Occasionally, a patient may
develop an acute loss of vision due to ischemic optic neuropathy
associated with papilledema.
•
Pathophysiology
•
Ironically, withdrawal from corticosteroids may result in
idiopathic intracranial hypertension.
The following diseases have been associated with idiopathic
intracranial hypertension: anemia, chronic respiratory
insufficiency, familial Mediterranean fever, hypertension,
multiple sclerosis, polyangiitis overlap syndrome, psittacosis,
renal disease, Reye syndrome, sarcoidosis, systemic lupus
erythematosus, and thrombocytopenic purpura.
Disorders of cerebral venous drainage
•
Cerebral venous compression by extravascular tumors or
secondary thrombosis.
•
Radical neck dissection, even if completed only on the right
side (predominant drainage from the head is via the right
jugular vein). Spontaneous recanalization usually occurs, but,
if delayed, chronic papilledema may result.
Endocrine disturbances
•
Pregnancy is occasionally associated with idiopathic
intracranial hypertension. This disorder can present at any
stage of pregnancy.
Differential Diagnosis
Bilateral optic nerve head edema is presumably due to increased
intracranial pressure secondary to an intracranial tumor until
proven otherwise. Ruling out malignant hypertension prior to
pursuing the diagnostic trail of pseudotumor cerebri is always
worthwhile.
If the patient's blood pressure is normal or minimally to moderately
elevated, obtaining urgent neuroimaging studies to rule out a
space-occupying lesion or a dural sinus thrombosis is essential. If
the neuroimaging study does not demonstrate any pathology and
if a lumbar puncture demonstrates a raised opening pressure,
then one of the above-mentioned causes of increased intracranial
pressure must be considered.
In the absence of a specific offending agent, the diagnosis of the
patient is presumably idiopathic intracranial hypertension.
•
24
Complications
Pseudopapilledema
The pathophysiology of this disorder is unclear. A relative
resistance to the absorption of cerebrospinal fluid across the
arachnoid villi is widely presumed to be present. Other theories
support an abnormality in the cerebral circulation with a resulting
increase in the brain's water content. The subsequent increase in
the intracranial pressure is transmitted to the structures within
the intracranial cavity, including the optic nerves. The disease
commonly occurs in women who are overweight. The role of
obesity in this disorder is unclear. Obesity has been proposed to
increase intra-abdominal pressure, which, in turn, raises cardiac
filling pressures. This rise in pressure leads to impeded venous
return from the brain (due to the valveless venous system that
exists from the brain to the heart) with a subsequent elevation in
intracranial venous pressure. If not treated appropriately, chronic
interruption of the axoplasmic flow of the optic nerves with ensuing
papilledema due to this pressure may lead to irreversible optic
neuropathy.3
Mortality and Morbidity
The morbidity of this disorder is mainly related to the effects of
papilledema. If left untreated, long-standing disc edema results in
an irreversible optic neuropathy with accompanying constriction
of the visual field and loss of color vision. In end-stage papilledema,
central visual acuity is also involved.4
Conclusion
The diagnosis of IIH is made by identifying the typical symptoms
of the disease along with documentation of a high spinal fluid
pressure (done during a gaurded spinal tap). The neurologic
examination is normal except for the presence of papilledema.
Sometimes double vision occurs, caused by limitation of lateral
eye movement. Lastly, neuroimaging procedures such as CT scans
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
be followed frequently with tests of vision until the doctor is
confident that there is no vision loss occurring. Vision testing
should then be done once or twice a year or whenever new
DOS Times - Vol. 14, No. 8, February 2009
symptoms occur. Unfortunately, IIH is a life-long disease and tends
to occur during periods of weight gain. The symptoms though are
very treatable and, if treatment is started early enough, the vision
loss is reversible.
Although rare a strong suspicion of IIH in chronic severe headaches
and immediate investigations followed by proper surgery can
salvage the vision of the patient even in established papilloedema.
Besides the vetriculo peritoneal shunt another surgical option
would have been optic nerve sheathotomy.
References
1.
Procedures for Idiopathic Intracranial Hypertension in the United
States, 1988-2002". Neurosurgery 57 (1): 97-108. PubMed. Retrieved
on 2006-11-08.
2.
Friedman, Deborah I.; Daniel M. Jacobson (November 26, 2002).
"Diagnostic criteria for idiopathic intracranial hypertension".
Neurology 59 (10): 1492-1495. PubMed. Retrieved on 2006-11-08.
3.
Brazis PW, Lee AG: Elevated intracranial pressure and pseudotumor
cerebri. Curr Opin Ophthalmol 1998 Dec; 9(6): 27-32
4.
Friedman DI, Jacobson DM: Idiopathic intracranial hypertension. J
Neuroophthalmol 2004 Jun; 24(2): 138-45.
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
Kirti Singh MD, DNB, FRCS(E)
I
t is the temporary reduction or modfification of myopia achieved
by the programmed application of specially designed contact
lenses which reshape the cornea. A reversible technique it involves
use of an overnight lens-wearing protocol. Orthokeratology has
been around since 1960 when George Jessen created the first
orthokeratology design from PMMA material, These early lenses
used were conventional flat-fitting, rigid contact lenses and induced
significant with-the-rule corneal toricity due to decentration. 1, 2
With the advent of reverse-geometry lens designs made of high
Dk material in late 1980 this undesirable effect has decreased
drastically3,4,5,6
Reverse-geometry lenses, propagated by Wlodyga and Stoyan,
are fitted with a base curve flatter than the central corneal curvature.
The flat base curve applies pressure to the central treatment zone,
achieving sphericalization of the prolate cornea and resulting in
reduction of myopic refractive error. To aid centration a secondary
curve is ground which is steeper than the central base curve. 7
Cornea
Orthokeratology
How does it work?
Tear film meniscus behind the contact lens hydraulically
redistributes epithelial cells under the lens from the centre towards
periphery.
The central flat base curve exerts a positive pressure on the central
corneal epithelium. As a consequence of this central corneal
epithelium is thinned out. The steep secondary curve , creates an
annular tear pool which by virtue of negative pressure leads to
mid peripheral corneal epithelial and stromal thickening (Figure
2)8, 9,10, 11 Reduction in corneal epithelial thickness by 20 microm
or more has been documentedover a 3 month period of ortho K
wear.12
The end result is a reduction in corneal convexity and sagittal
corneal height, which effectively reduces myopic error. Age plays
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.
Reverse curve 2nd Curve
Figure1: 5 Curve Reverse geometry Lens
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
Guru Nanak Eye Centre,
Maulana Azad Medical College,
New Delhi
www.dosonline.org
Figure 3: 5 Curve Reverse geometry
Lens bulls eye fluorescein pattern
27
Peripheral curve
Select initial lens from Trial Kit
This aids lens lift, ensures adequate tear exchange and helps in
removal of lens.
This initial lens is based on the adjusted flat K. Flat k is adjusted
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)
is compatible with allowing sufficient oxygen transmission to
prevent corneal hypoxia like Boston XO
Centration of the shaping lens is evaluated on the
fluorescein pattern
It can be used for which types of patients:
•
Age - juvenile to adult myopes
•
Range : -1.00D to -6.00D
•
Cylindrical error -2.00D or less with-the-rule corneal
astigmatism OR < -0.75D against-the-rule astigmatism.
•
Flat K range - 40.00D(8.44mm)~45.50D(7.42mm)
Refractive endpoint is typically reached with these lenses after 7 to
10 days of wear with use of an overnight 7-8 hour lens-wearing
protocol.3,4
28
Centration is critical to achieve the ortho-k effect. Decentered
lenses do not produce the desired myopia reduction, in addition
they distort the cornea. A good fluorescein pattern is a bull’s eye
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
reservoir’, under the steeper secondary curve and a peripheral
circular band of alignment tapering to the edge lift. (Figure 3)
A well fitting lens should move approximately 1mm with blinking
Vision Examination
Remove lenses after 1 hour of wearing. Evaluate patient’s visual
acuity before and after lens wearing.
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.
•
Flat-K value: Radius of curvature for centre. Radius of curvature
for periphery also needs to be assessed by adapting corneal
eccentricity with flat-k value.
Higher the “e” value, more rapidly does the cornea flatten
towards the periphery, thus the potential to flatten central
corneal curvature is greater.
•
Corneal Eccentricity “e “: defined as the rate at which the cornea
flattens from the central area to the peripheral area.
In low eccentricity, oblate corneas the effect of Ortho K is
reduced.
Some examples of ortho K trial fits;
Corneal size: this determines which part of cornea comes in contact
with the alignment curve. Normal corneal size of over 11.6 mm
requires a 10.6 mm diagnostic/ inventory lens.
Good Centration- fluorescein fit and topography picture (Figure 4a
& 4b)
Figure 4a: Good Centration
Figure 4b: Fluorescein fit and
topography picture
•
Lens movement is quick and proper as natural. (1-2mm)
DOS Times - Vol. 14, No. 8, February 2009
Figure 5b: Fluorescein fit and
topography picture
Figure 5a: Poor
Centration (Steep Fit)
•
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
(Figure 5a & 5b)
•
Corneal topography: Cornea size - 12.3mm.
•
Eccentricity - 0.49(0.46-0.54)
•
Lens movement is slight (1mm“!) and rides low
Thus trail lens selected would be :
•
Base curve zone Touch: Touch zone narrow. ( < 4mm)
•
•
Reverse Curve Zone: Air bubble/ thick ring
Eccentricity as less than 0.5 therefore No need to need to
adjust flat-K value.
•
Alignment Zone: Very black part
•
Initial trial lens selected = 7.61
•
Initial lens selected
•
10.6mm/7.63/-3.50D
Poor Centration ( Flat Fit) fluorescein fit and topography picture
(Figure 6a & 6b)
OR
10.6mm/7.58/-3.50D
•
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
•
Alignment Zone: Not black, with fluorescein
If lens adheres, air bubbles would be noted in the reverse curve
(reservoir) zone then - change the central bearing (make the fit
steeper)
Eccentricity
Eccentricity
Trial set - Flat K
Flat K Adjustment
Power Adjust
0.30 - 0.39
0.50-0.75D steep
43.50(7.76)-43.75(7.72)
-3.50~-3.75D
0.40 - 0.45
0.00-0.25D steep
43.00(7.85)-43.25(7.80)
-3.00~-3.25D
0.46 - 0.54
Flat K
43.00D(7.85mm)
-3.00D
0.55 - 0.60
0.25-0.50D flat
42.75(7.90)-42.50(7.94)
-2.75~-2.50D
0.61 - 0.65
0.50-0.75D flat
42.50(7.94)-42.25(7.99)
-2.50~-2.25D
0.66 - 0.70
0.75-1.00D flat
42.25(7.99)-42.00(8.04)
-2.25~-2.00D
www.dosonline.org
29
Figure 6b: Fluorescein fit and
topography picture
Figure 6a: Poor
Centration (Flat Fit)
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.
•
Check unaided visual acuity, Slit Lamp examination and
corneal topography.
Finally the lens with Corneal Diameter 10.6mm/7.58/-3.50D has
been ordered and was dispensed to the patient.
•
Perform Over-refraction to determine lens’ target power
It is observed that her visual acuity before lens wearing was 6/36)
and after lens wearing is 6/6 .
Topography Image after One Hour
We can see that here red rounded part (corresponds to the
alignment curve) is centered after 1hour of wear (Figure 7a & 7b).
After 3 days, this red rounded part becomes more evident.
(Figure 8)
30
Does this technology work?
Unaided vision improvement and reduction of the myopic
refractive error have been documented now by many authors in
different ethnicities.13,14,15,16
The average percentage reductions in spherical equivalent have
been noted to be in the range of 84 - 92% and errors of till -5D
have been corrected.15,16
A multicentre study recently found evidence to state that overnight
orthokeratology is efficacious and safe for young myopic
patients.17,18
DOS Times - Vol. 14, No. 8, February 2009
Figure 8: After 3 Days
This has been confirmed by a large cohort studies evaluated over
a long term period of at least 12 months.14,19
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.
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):1815; discussion 205-6.
9.
Alharbi A, Swarbrick HA. The effects of overnight orthokeratology
lens wear on corneal thickness. Invest Ophthalmol Vis Sci. 2003
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
to apply ocular lubricants.20
11. Jayakumar J, Swarbrick HA. The effect of age on short-term
orthokeratology. Optom Vis Sci. 2005 Jun;82(6):505-11.
Another optical problem noted is decreased corneal optical quality
in terms of increased higher-order aberrations and decreased
contrast sensitivity. This correlates with the magnitude of myopic
correction.21, 22,23 However, these induced optical higher order
aberrations along with the improvement in uncorrected visual
acuity are fully reversible after discontinuation of the Ortho K
lens even after 1 year of use.23
Lens decentration during overnight wear can lead to diplopia,
glare and reduction in the orthokeratology effect. In a study
involving 270 eyes over a 6 month period of OrthokK lens wear
decentration was more in patients with greater initial astigmatism
and use of smaller lenses. 24
Microbial keratitis is the most severe, potentially vision-threatening
adverse response associated with orthokeratology contact lens
wear. The organisms identified have been Pseudomonas
aeruginosa and Acanthamoeba species. 25, 26 It has been associated
with the use of tap water in care regimens for overnight
orthokeratology and the patient must be forewarned against using
tap water.
Conclusions
Ortho-K lenses are effective for myopia correction by overnight
wearing about 7 hours. Refractive endpoint is typically reached
after 7 to 10 days of wear with the use of an overnight lens-wearing
protocol. Under close monitoring, overnight ortho-k is effective
and safe for reducing low to moderate myopia and the treatment
is well accepted by the children.
References
1.
Kerns RL. Research in orthokeratology. Part VIII: results, conclusions
and discussion of techniques. J Am Optom Assoc 1978;49:308-14.
www.dosonline.org
12. Soni PS, Nguyen TT, Bonanno JA. Overnight orthokeratology:
visual and corneal changes. Eye Contact Lens. 2003 Jul;29(3):13745
13. Johnson KL, Carney LG, Mountford JA, Collins MJ, Cluff S, Collins
PK.Visual performance after overnight orthokeratology. Cont Lens
Anterior Eye. 2007 Mar;30(1):29-36. Epub 2007 Jan 9.
14. Soni PS, Nguyen TT; XO Overnight Orthokeratology Study Group.
Overnight orthokeratology experience with XO material. Eye
Contact Lens. 2006 Jan;32(1):39-45.
15. Cheung SW, Cho P, Chui WS, Woo GC. Refractive error and visual
acuity changes in orthokeratology patients. Optom Vis Sci. 2007
May;84(5):410-6.
16. 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.
17. Walline JJ, Rah MJ, Jones LA The Children’s Overnight
Orthokeratology Investigation (COOKI) pilot study. Optom Vis
Sci.2004 Jun;81(6):407-13.
18. Rah MJ, Jackson JM, Jones LA, Marsden HJ, Bailey MD, Barr JT
Overnight orthokeratology: preliminary results of the Lenses and
Overnight Orthokeratology (LOOK) study. Optom Vis Sci. 2002
Sep;79(9):598-605.
19. Van Meter WS, Musch DC, Jacobs DS, Kaufman SC, Reinhart WJ,
Udell IJ.Safety of overnight orthokeratology for myopia: a report
by the American Academy of Ophthalmology. Ophthalmology.
2008 Dec;115(12):2301-2313.e1. Epub 2008 Sep 20.
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
31
sensitivity after overnight orthokeratology. Invest Ophthalmol Vis
Sci. 2008 Oct;49(10):4314-20. Epub 2008 May 23.
22. Hiraoka T, Okamoto F, Kaji Y, Oshika T. Optical quality of the
cornea after overnight orthokeratology.Cornea. 2006 Dec;25(10
Suppl 1):S59-63.
23. Hiraoka T, Okamoto F, Kaji Y, Oshika T…Hiraoka T, Okamoto C,
Ishii Y, Okamoto F, Oshika T. Recovery of corneal irregular
astigmatism, ocular higher-order aberrations, and contrast sensitivity
after discontinuation of overnight orthokeratology. Br J Ophthalmol.
2008 Nov 19. [Epub ahead of print]
24. Yang X, Zhong X, Gong X, Zeng J. Topographical evaluation of the
decentration of orthokeratology lenses. Yan Ke Xue Bao. 2005
Sep;21(3):132-5, 195.
25. Watt KG, Swarbrick HA. Trends in microbial keratitis associated
with orthokeratology. Eye Contact Lens. 2007 Nov;33(6 Pt 2):3737; discussion 382.
26. Shehadeh-Masha’our R, Segev F, Barequet IS, Ton Y, Garzozi HJ.
Orthokeratology associated microbial keratitis. Eur J Ophthalmol.
2009 Jan-Feb;19(1):133-6.
Author
Kirti Singh MD, DNB, FRCS(E)
32
DOS Times - Vol. 14, No. 8, February 2009
Cornea
Goldenhar Syndrome: A case report &
review of literature
Uday Gajiwala MS, Rajesh Patel MS, Manoj Gangwal DO, FCPS
G
oldenhar syndrome also called oculo auriculo vertebral
dysplasia or first & second branchial arch syndrome, is mostly
unilateral, occurs most commonly in men and involves anomalies
of face, ears, eyes and vertebrae in most cases. When
malformations primarily involves the jaw, mouth and ear of one
side, the disorder is referred as Hemifacial microsomia, but if
abnormalities of vertebrae and the eyes are also present, the
disorder is often called Goldenhar syndrome.
Case History
A fifty year female patient with negative family history presented
with dimness of vision in left eye and painless swelling in left eye
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
with refraction. There was against the rule astigmatism of 3 dioptre
due to inferolateral limbal dermoid of about 5X5 mm size. She
had 15 degrees concomitant divergent squint with full eyeball
movement. There was no other ocular abnormality. Fundus
examination was normal in both eyes.
Photographs
On ENT examination, patient had pre auricular skin tags which
were lying in line joining tragus & angle of mouth. Patient had no
complaint of deafness in both the ears. X-ray PNS & Schullers
views were also normal with normal external auditory meatus.
CVS & CNS examination was normal.
X-ray cervical spine was not showing occipitalisation of atlas or
hemi vertebrae which is usually associated with Limabal Dermoid
in Goldenhar syndrome.
CT scan was not showing any abnormality.
Patient was not ready for its excision as prognosis for visual
improvement was poor due to meridional amblyopia and she was
not worried about cosmetic problem in this age.
Thus, the patient was having following findings:
•
Limbal dermoid
•
Abnormalities of ear (pre auricular skin tag)
Discussion
The incidence of this syndrome is 1:3000 to 1:26,500 with onset at
birth. It occurs in family &/or as sporadic cases. Familial cases are
consistent with autosomal dominant, autosomal recessive and
multifactoral patterns of inheritance. Chromosomal abnormality
or defective gene disorder is common in men (70%). It can be
unilateral or bilateral but mostly it is unilateral.
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,
Jhagadia, Bharuch, Gujarat
www.dosonline.org
35
Normal X Ray Cervical spine
the same affected family. It may be present in monozygotic twins
with only one twin being affected.
Dermoid Showing Hair
Ocular Manifestations
Epibulbar Tumors
Review of Literature
First identifies by M.Goldenhar in 1952
First called oculo auriculo vertebral dysplasia by R.J. Gorlin.
Clinical Features
Facial asymmetry
Dermoid or lipodermoids.
•
Found in 35% of cases.
•
Yellow or pink, solid ovoid masses up to 10 mm in diameter.
•
Unilateral or bilateral.
•
May impair vision.
65-70% cases have some facial asymmetry & in 20% of cases the
asymmetry is marked.
Others
The right side of the face is more severely affected in 60% of cases.
•
Strabismus
10-33% has bilateral facial involvement and/or mandibular regions
on the affected side.
•
Microphthalmia or anophthalmia.
•
Blepharo-phimosis in 10% of cases.
There also may be unilateral hypoplasia of the facial musculature
with facial muscle weakness and depressor anguli oris hypoplasia
on the affected side.
•
Narrowing of palpebral fissure
•
Retinal anomalies.
Macrostomia
•
Upper lid colobomas.
Greatly exaggerated width of the mouth due to lateral cleft like
extension of the corner of the mouth.
Other manifestations
External Ear
May range from mildly dystrophic ear to microtia to anotia.
Pre-auricular skin tags, pits and/or sinuses and occur between the
tragus and the angle of the mouth.
Usually unilateral but can be bilateral in 33% of cases.
Middle Ear
Narrow or atretic
Inner Ear
Conductive &/or sensorineural hearing loss.
36
•
Neurological
•
Microcephaly or hydrocephaly.
•
Occipital encephalocele.
•
Arnold chiari malformation.
•
Mental retardation in 5-15% of patients.
•
Spina bifida.
Congenital Heart disease
•
VSD, PDA, Tetralogy of Fallot, Coarctation of Aorta.
•
Transposition of Great arteries.
DOS Times - Vol. 14, No. 8, February 2009
Renal
Management
•
Ectopia &/or fused kidneys.
Supportive
•
Renal agenesis.
Multidisciplinary approach
•
Multicystic dysplastic kidneys.
•
Pediatrics, plastic, orthopaedic, ophthalmic and ENT surgeons
•
Hydronephrosis, hydro-ureter, ureteral duplication.
•
Early and regular hearing tests.
•
Uretero-pelvic junction obstruction.
•
Genetic counselling.
•
Vesico-ureteral reflux.
Prognosis
Musculoskeletal
•
Scoliosis
Normal life span and intelligence in a majority of those patients
without significant complications.
•
Talipes equino varus (club feet)
References
•
Bifid or digitalized thumb
•
Hypoplastic Thumb
1.
Baraitser M. & R. M. Winter, color atlas of congenital malformation
syndrome (1st edition) P: 40-41 (1996).
2.
Baum J. L. & Feingold M., ocular aspects of Goldenhar syndrome.
AJO, 75:250, 1973.
3.
Bergsma D. (Ed) Birth defects, atlas and compendium. Baltimore:
Williams & Wilkins.
4.
Benjamin S. N. & Allen H. F. classification for limbal dermoids,
choriostomas & branchial arch anomalies: presentation of an unusual
case. Arch Ophthalmology, 87:305, 1972.
5.
Geeraets W. J. ocular syndromes (3rdedi) Philadelphia: Lea & Febiger,
1976. Jones K. L., Smith's Recognizable patterns of human
malformations (5th edi), P: 642-645 (1997).
Vertebral
6.
Journal of Genetics, Human 1:243 (l952).
•
Cervical fusion in 20-25% of cases.
7.
Journal of Pediatrics 63:991-999 (1963).
•
Platybasia & occipitalisation of the atlas in 30% of cases.
8.
•
Klippel-Feil anomaly.
Mansour A. M. et. al, ocular findings in facio auriculo vertebral
sequence (Goldenhar Gorlin syndrome) AJO 100:555, 1985.
•
Hemi vertebrae
9.
•
Hypoplasia of vertebrae
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
Investigations
Skeletal X-Rays
Skull
•
Hypoplasia of the maxillary, temporal & malar bones.
•
Hypoplasia or absent mandibular ramus & condyles.
•
Reduced pneumatization of mastoid region.
CT/MRI
•
Agenesis of or lipoma in corpus callosum
•
Calcification of falx cerebri
•
Hypoplastic septum pellucidum.
•
Intra-cranial dermoid cyst.
10. Das A, Mazumdar S, Chatterjee SS.Atypical case of Oculo-Facioauriculo-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
Shreekant A Damgude MBBS, B.P. Guliani MS
P
seudophakic bullous keratopathy refers to the development
of irreversible corneal oedema following cataract surgery which
initially develops in stroma progressing towards intercellular
epithelial region with characteristic formation of bullae.
Though with newer surgical techniques and IOL designs, incidence
of this complication has decreased dramatically, it still represents
an important cause of visual disability following routine and
complicated cataract surgery.
Pathophysiology
Corneal transparency depends on ability of cornea to remain in
dehydrated state. Corneal epithelium and endothelium are both
semipermeable membranes that create a barrier to the flow of
water and other electrolytes.
The major factor which prevents corneal hydration is Na+K+ AT
Pase pump which lies within lateral cell membrane in endothelial
layer of cornea. Endothelium is incapable of mitotic activity. At
birth endothelial cell count is approximately 7500 cells /mm2. In
older adults it is approx. 2500-2700 cells / mm2.
Normal rate of endothelial cell loss after age of 20 is approx. 0.5 %
per year. Surgical trauma, inflammation and corneal dystrophies
(Fuch's endothelial dystrophies, posterior polymorphous
dystrophies) can accelerate this normal aging loss.
The final common pathway in development of PBK is damage to
corneal endothelium when cell density reaches to critically low
level of about 300-500 cells / mm2. It leads to rearrangement of
remaining cells to cover posterior corneal surface in the form of
polymegathism (greater than normal variation of corneal
endothelial cell size resulting in irregular large and small cells) and
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.
Cornea
Pseudophakic Bullous Keratopathy
The lack at adhesive proteins in epithelial basement membrane
(fibronectin, laminin and collagen type IV) and accumulation of
antiadhesive proteins, such as tenacin C and thrombospondin -1,
leads to loss of contact of epithelial cells with each other and with
underlying subepithelial tissue, resulting in subepithelial bullae
and fibrosis3.
Incidence: Current incidence is about 0.1% FDA report on
intraocular lens (1978-1982)4 is shown in Table 1.
Table 1: Association of PBK with of intraocular lens
Incidence of PBK
Type of lens
0.06%
PC IOL
1.2 %
AC IOL
1.5 %
Iris fixated lens
Long term incidence of PBK has been found to be 15%.
It is also correlated with endothelial cell loss5 in cataract surgery.
(Table 2)
Table 2: Endothelial cell loss in cataract surgery
ECCE
SICS
PE
4.72 %
4.21%
5.41%
Clinical features
Poor visual acuity: It is due to inability of stroma to maintain
corneal transparency.
Epithelial oedema can be responsible for great changes in visual
activity due to irregularity in corneal refractive surface. Mild stromal
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
Safdarjung Hospital,
New Delhi
www.dosonline.org
41
Etiology
Preoperative risk factors
Specular microscopy: Though there is no direct correlation between
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.
Figure 2: Formation of Bulla between epithelial
basement membrane and Bowman’s membrane
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
oedema doesn't cause severe visual loss. However epithelial
oedema can cause a significant drop in vision.
Pain / discomfort: Pain occurs due to exposure of corneal nerves
to noxious environment.6 As the oedema progresses, bullae are
formed their rupture results in pain, photophobia & epiphora.
Slit lamp examination reveals folds in descemet's membrane and
obvious overall thickening of central and peripheral cornea. In
more advanced cases, vesicles & bullae may be seen on corneal
surface.
In patients with predisposing conditions (like fuch's dystrophy &
Posterior Polymorphous dystrophy), cornea guttata may be seen.
Guttate excrescences appear as "golden brown" confluent
endothelial lesions & give posterior surface a "beaten metal
appearance"7,8.
42
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.
It has been found that HPMC is not as protective as sodium
hyaluronate during phacoemulsification. Protective benefit of
sodium hyaluronate is improved when used in combination with
chondroitin sulphate.16
Toxic substances used to disinfect instruments may inadvertently
be introduced into the eye. Water, not saline, should be used to
rinse the instruments.
Intracular irrigation must be just adequate enough. Excess may
cause endothelial injury.
For topical and intracameral anaesthesia only 0.5 ml of 1%
preservative free lidocaine should be used17,18. Other preparations
may cause corneal toxicity.
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.
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.
•
(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.
•
Serial measurements are helpful to note progression; and
also to assess effectiveness of therapeutic regimens.
Descemet's detachment is more common with clear corneal
incisions which may lead to postoperative corneal oedema.
•
Ultrasonic pachymetry is more reproducible and requires
less skill than optical.
Postoperative causes
Optical pachymetry is especially useful in measuring the depth of
corneal pathology, when fall thickness of corneal stroma is not
involved and for therapeutic reasons to estimate the depth of this
pathology. It is preoperative investigation for excimer laser
phototherapeutic keratectomy.
Figure 4: Late painful bullous keratopathy
Routine uncomplicated phacoemulsification results in 9%
endothelial cell loss at 1 year postoperatively. In one series, it has
been found to be 11.9%22
Regardless of type of surgery and type of IOL implanted,
continuing endothelia cell loss more than usual 1% per year occurs
in patients who have undergone cataract extraction.
Treatment
Medical Care
Type of lens implanted
Topical hyperosmotic agent
Anterior chamber IOLs contribute to endothelial damage by
"Intermittent touch" between IOL & corneal.23
2% and 5% sodium chloride solution and ointment.
IOL haptics & footplates can cause chronic irritation with low
grade inflammation
IOLs are also known to disrupt the normal flow of aqueous in
anterior chamber which is the nutrient flow to endothelial cells.24
Iris supported lenses may cause greater endothelial loss as they
can have contact with endothelium during ocular saccades25.
Closed loop ACIOL have been responsible for large amount of
corneal pathology.
Following ICCE, incidence at PBK has been found to be 2.3% in
retrospective study of 1041 patients.26
Mechanism: These agents create hypertonic tear film, drawing
water out of cornea.
Because evaporation of tear film is minimal at night with eyes
closed, corneal oedema tends to be worse in morning. Hence 5%
sodium chloride ointment should be applied at nigh to conjunctival
cul-de-sac.
Typical regimen includes application of Muro 128, 2-5% drop
hourly in affected eye until noon. As the day progresses,
evaporation of tear film begins to create relative hypertonicity of
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
oedema & thickness in postoperative setting, even if
intraocular pressure is normal or only mildly elevated.
•
β-blockers & -agonists are first line agents for this purpose.
•
Prostaglandins analogs & miotics should be avoided because
both drugs may adversely affect intraocular inflammation.
•
Utility of topical CA (-) is under question because they may
cause endothelial toxicity in compromised corneas.
This is photographic method of assessment of endothelium in
vivo. Light is projected onto cornea and reflected images from
optical interface (endothelium and aqueous) can be visualized.
High magnifications photographs allows quantification of cell
density. Corneas with cell densities less than 1000 cells / mm2 are
at moderate to high risk of developing corneal oedema following
intraocular surgery.
www.dosonline.org
43
Topical steroids
These should be used in cases of uveitis provided infectious
keratitis & epithelial defects are absent.
Hydrophillic contact lenses
This fashioned membrane was then secured to the edge of defect
by 10-0 nylon sutures which were either continuously running
interrupted or a combination of both.
These on extended wear basis, can be used to decrease pain
associated with epithelial bullae.
Attention was given to make the membrane flattened tightly anto
corneal surface and approximate to the epithelial edge.
These lenses as such do not reduce the amount of oedema. These
can improve visual activity to the extent that they mask surface
irregularity.
These contact lenses can be used in association with 5% hypertonic
saline (as cornea) and thus they can improve visual acuity by
decreasing epithelial & stromal oedema.
Pain associated with PBK can be due to rupture of bullae with
exposure of corneal nerves endings or swelling of epithelium
leading to stretching of nerve endings. These lenses alleviate pain
as long as the lens remains in place.
Acts as effective precorneal protective layer. Shields abnormal
epithelium from environment and prevents bullae from bursting.
The lens doesn't prevent formation of bullae but perhaps when
new bullae do occur, the corneal nerves' endings are not exposed
to drying & other noxious stimul, when the lens cover them.
Fitting of lens is an important consideration. Lenses with excessive
movement can irritate epithelium & be uncomfortable.
Lenses that are too tight can act as suction cap and result in
inflammation and even anterior uveitis (Tight lens syndrome).
Surgical Care
Conjunctival flap
In 1958, Gundersen introduced the technique of using conjunctiva
alone without use of tenon's capsule to cover the cornea. It is onlay
flap of thin conjunctiva without use of tenon's capsule. The
exclusion of tenon's capsule increases flap longevity.
A Gundersen-type flap undermines the superior bulbar
conjunctiva & moves in & down to cover the cornea with intact
"bridges" nasally and temporally.
The flap is thought to increase the local blood supply, promote
healing and replace damaged or defective corneal epithelium27.
Amniotic membrane transplantation
In some cases BCL was applied.
Results: This turned immensely successful for ocular pain relief in
43 of 48 eyes with painful bullous keratopathy.
This is technically easier to perform avoid potential complications
of conjunctival flap such as ptosis, it gives better cosmetic
appearance. Hence it is superior to conjunctival flap.
It provides additional advantage in that resultant cornea doesn't
manifest surgically induced limbal stem cell deficiency, which is
invariably created by conjunctival flap. As a result such corneas
are amenable for corneal transplantation if necessary.
Amniotic transplantation also restores corneal epithelial integrity.
All except 4 eyes (92%) maintained healed and intact epithelium
during the entire follow up period.
Bullae resolved in 90% cases. Even with recurrent epithelial
oedema, pain did not occur.
Amniotic basement membrane prevents epithelial apoptosis,
enhances epithelial migration, differentiation. It also reinforces
adhesion of basal epithelial cells29,30,31.
It also prolongs life span of corneal and conjunctival progenitor
cells32.
Stromal matrix inhibits TGF-β, hence causing less scars
postoperatively32.
Amniotic membrane transplantation proxides new and
nonantigenic human basement membrane for renewed expansion
of epithelial cells33.
Cautenisation of Bowman's membrane
It can be performed for pain relief. This procedure is thought to
produce dense fibrous barrier between corneal stroma and
epithelium so that fluid can not permeate into epithelial cells and
produce ballous changes.
Study of Amniotic membrane transplantation performed in 50
eyes28
Anterior stromal pundure in bullous kercetopathy
Important steps of procedure
28 patients of bullous keratopathy (1996-99) were studied with
minimum follow up of 1 month. In all these patients approximately
200 punctures were given with bent 26 G needle sparing peripheral
cornea.
Loose epithelium was first removed with sponge, 0.12 forceps or
a blade to where epithelium became quite adherent. In general
this would create large corneal epithelial defect upto 1 or 2 mm
from limbus.
Preserved amniotic membrane was removed from storage
medium and cut to same size as debrided corneal surface, with
basement membrane surface up in all cases.
The side of BM could be distinguished from stromal side by touch
44
with sponge. The former was not sticky, while the later was &
could be caught by the sponge.
A clinicopathologic study34
•
Symptromatic relief was noted in all.
•
20 patients had complete relief.
•
8 patients experienced mild symptroms such as tearing and
occasional pain.
DOS Times - Vol. 14, No. 8, February 2009
•
Complete regression of bullae in 10 patients.
•
Partial regression of bullae in 18 patients.
•
Visual activity decreased in 12 patients.
•
Objective evidence of subepithelial fibrosis and scarring was
seen in all.
Anterior stromal puncture is an effective, simple and low cost
alternative for treating patients with symptomatic bullous
keratopathy35.
Annular keratotomy
It is used to treat pain associated with bullous keratopathy in eyes
with poor visual potential. A partical thickness corneal incision is
made with a trephine and relieves pain by severing branches of
corneal ciliary nerves to decrease corneal sensation36.
Diamond burr polishing of basement membrane
•
One patient had persistent epithelial defect that required
botulinum toxin ptosis.
Corneal transplantation
Penetrating keratoplasty for PBK:
•
Review study of 81 causes40 of PK done for PBK during
1987-93.
•
IOL explant was done in 24 (29.6%) cases.
•
IOL was retained in 12 (14.9%) cases.
•
IOL exchange was done in 45 (55.5%) cases.
•
Minimum follow up period was 6 months.
•
Graft remained clear in 54 (66.6%) patients.
•
Commonest cause of graft failure was "graft rejection"(17.2%).
Following epithelial debridement, 4.5 mm to 5 mm diamond burr
can be used gently to polish basement membrane throughout
area of epithelial debridement37.
They recommended explantation of all closed loop AC IOLs and
iris claw IOLS and that AC recomstraction during PK with IOL
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)
for PBK38.
Failure rate
Lens
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
In this study from LVPEI, they suggested,
Thomas et al39 proposed that PTK is effective procedure and bullae
resolve and pain is abolished in large number of patients with
superficial ablation.
Anterior segment reconstruction during PK →Better outcome
Anterior, +Gonioplasty, +Iridoplasty, Vitrectomy
But in this study, it was proposed that preterminal neural plexus
of cornea is located just deep to Bowman's membrane. Hence
moderately deep ablation would have superior effect on decreasing
the pain; by ablation of neural plexus.
Advantage of Anterior Vitrectomy
•
It will lead to decreased swelling of corneal stroma by
decreasing the quantity of mucopoly sacharides and hence
decreasing osmotic load.
Advantage of gonioplasty & Irioplasty
•
To restore pupil to normal round configuration
Increased scarring associated with deeper ablation may result
in increased stability of epithelium.
•
Prevents development of glaucoma and allograft rejection
related to formation of anterior synechiae due to centripetal
tension exerted on iris.
•
Give additional support to AC lens.
•
Improves optical function of iris.
•
Superficial PTK showed improvement in 62% cases whereas 38%
required penetrating keratoplasty.
Intermediate PTK showed improvement in 40% cases whereas
60% required penetrating keratoplasty.
•
↓ CME
•
Prevents decentration of IOL by vitreous strands
Deep PTK: Mean ablation performed was of 206 um thickness.
To perform IOL exchange either with PCIOL or an open loop
kelman type ACIOL.
•
66% (16 out at 24) showed improvement.
Explantation of all iris fixated lens / closed loop IOLS.
•
One required PK.
•
To avoid delay in corneal surgery once PBK is diagnosed.
•
One required amniotic membrane transplantation.
•
Anterior segment reconstruction and monitoring of I.O.P.,
both pre & postoperatively.
•
Two patients required retreatment.
•
Three patients continued having discomfort
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
improvement in visual acuity.
intraocular lens implantation, Jpn J Ophthalmol 37:414, 1993.
2.
Champion R, Green WR : Intraocular lenses: a histopathologic study
of eyes, ocular tissues and intraocular lenses obtained surgically,
Ophthalmology 92:1628,1985.
3.
Maseruka H, Ataullah SM, Zardi L et al : Tenascin - cytotactin (TNC) variants in pseudophakic / aphakic bullous keratopathy corneas,
Eye 12:729, 1998.
4.
FDA report on intraocular lens (1978-1952) stark WJ, Worthen
DM, Holladay JT Ophthalmology 90:311-317, 1983.
5.
George R, Rupauliha P, Sripriya AV, Rajesh PS, Vahan PV, Praveen S.
Ophthalmic Epidemiol. 2005 Oct; 12(5): 293-7.
6.
Ljubimov AV, Burgeson RE, Butkowski RJ, Couchman JR, WuRR,
Ninomiya Y, et al. Extracellular matrix alterations in human corneas
with bullous kercetopathy. Invest Opthalmol Vis Sci 1996;37:9971007.
7.
Waring GO III, Rodrigues MM, Laibson PR. Corneal dystrophies.
II. Endothelial dystrophies. Surv. Ophthalmol 1978;23:147-168.
8.
Cibis GW, Krachmer JH, Phelps CD, et al. The clinical spectrum of
posterior polymorphous dystrophy. Arch ophthalmol 1977; 95: 14291537.
9.
Lugo M, Cohen EJ, Eagle RC, Parker AV Ophthalmic Surg. 1988
Jan; 19(1):16-19.
In general, options for IOL exchange are
•
Modern flexible loop ACIOL.
•
PCIOL in ciliary sulcus.
•
Iris fixation of PCIOL.
•
Suturing of PCIOL in sulcus.
Flexible ACIOL should be reserved for
•
Eyes with minimal anterior segment pathology.
•
Less than 900 angle synechiae.
•
Well controlled I.O.P.
Generally width of IOL chosen should correspond to a
measurement of horizontal white to white corneal diameter plus
1 mm.
Sutured intraocular lenses should be reserved for
•
Extensive anterior segment pathology.
•
Lack of iris support for ACIOL.
•
Patients with glaucoma.
No study till date has clearly pointed to advantage of one technique
or style of intraocular lens replacement in terms of graft survival,
vision or development of secondary complications. Hence it
remains the choice of particular surgeon.
Newer techniques
11. Wirbelauer C, Anders N, Pham DT et al : Corneal endothelial cell
changes in pseudoexpoliation syndrome after cataract surgery, Arch
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
modality, with following distinct advantages:24
13. Kaufman HE, Katz JC : Endothelial damage from intraocular
insertion, Invest Ophthalmol 15:996, 1976.
•
This preserves healthy portions of patients' native cornea.
•
It avoids additional risks and postoperative complications.
•
Additional benefit is that postoperative astigmatism is very
minimal.
Flap endokeratoplasty
Free papers accepted in XXV congress of ESCRS in Sept. 2006
compares "Flap endokeratoplasty versus penetrating keratoplasty
for PBK: 5 yrs. follow up results"42.
5 yrs follow up shows all clear grafts, no significant difference
between BCVA, endothelial cell count or mean postoperative
refraction. Visual recovery and postoperative astigmatism were
better in patients who have undergone Flap EKP.
Preliminary results show that flap EKP could be a successful
alterative to penetrating keratoplasty for treatment of PBK. This
might be the future as far as treatment of PBK is concerned.
References
1.
46
10. Wirbelauer C, Anders N, Pham DT et al: Early postoperative
endothelial cell loss after corneoscleral tunnel incision and
phacoenulsification in pseudoexfoliation syndrome, Ophthalmologe
94 (5) : 332,1997.
Liu G et al: Histopathological study of pseudophakic bullous
keratopathy developing after anterior chamber or iris-supported
14. Edelhauser HF et al : Intraocular irrigating solutions : their effect on
the corneal endothelium, Arch Ophthalmol 93: 648, 1975.
15. Li J et al : Effects of BSS and BSS + irrigation solutions an rabbit
corneal transendothelial electrical potential differences, Cornea 12:
199, 1993.
16. Glasser DB et al : Endothelial protection and viscoelastic retention
during phaconulsification and intraocular lens implantation, Arch
Ophthalmol 109: 1438, 1991.
17. Martin RG, Miller JD, Cox CC 3rd et al : Safety and efficacy of
intracameral injections of unpreserved lidocaine to reduce intraocular
sensation, J Cataract Retract Surg 24 : 961, 1998.
18. Elvira JC, Hueso JR, Martinez-Toldos J et al : Induced endothelial
cell loss in Phacoemulsification using topical anaesthesia plus
intracameral lidocaine, 1 J cataract Retract Surg 25: 640,1999.
19. Edelhauser HF et al : Corneal oedema and intraocular use of
epinephrine, Am J Ophthalmol 93: 327, 1982.
20. Sandboe FD, Medin W, Bjerknes R : Toxicity of vancomycin on
corneal endothelium in rabbits; Acta Ophthalmologica scand 76 :
675, 1998.
21. Alp B N, Elibol O, Sargon MF et al : The effect of povidone iodine
on the corneal endothelium, Cornea 19 : 546,2000.
DOS Times - Vol. 14, No. 8, February 2009
22. Zhongua Yan Ke Za Zhi 2000 Jul; (36) 4 : 282-4. He W, Lu P.
23. Sugar J, Mitchelson J, Kraff M : Endothelial trauma and cell loss
from intraocular lens insertion, Arch Ophthalmol 96 : 449, 1978.
24. Paula Wynn. E medicine article, University of Iowa 2005 Nov.
25. Drews RC : Intermittent touch syndrome, Arch Ophthalmol 100 :
1440, 1982.
26. Kirsten Baggesen, E. B. Knudsen, K. Naeser. Acta Ophthalmologica
scandinavia 1999.
27. Gundersen T. Surgical treatment of bullous keratopathy, Arch
Ophthalmol 1960; 64 : 260 - 67.
28. Renato T.F. Pires, Scheffer C.G. Tseng. Arch Ophthalmol. 1999;
117 : 1291-97.
29. Khodadoust AA, Silverstein AM, Kenyon KR, Dowling JE. Adhesion
of regenerating corneal epithelium : the role of basement membrane.
Am J Ophthalmol 1968; 65 : 39-48.
30. Guo M, Grinnell F. Basement membrane and human epidermal
differentiation in vitro. J Invest Dermatol 1989; 93 : 372-8.
33. Fukada K, Chikama T, Makamura M, Nishida T. Differential
distribution of subchains of basement membrane components type
IV collagen and laminin among the amniotic membrane, cornea
and conjunctiva. Cornea 1999; 18 : 73-9.
34. Anterior stromal puncture in bullous keratopathy; a
clinicopathologic study Sridhar MS Rao GN. Cornea 2001 Aug 20
(6) : 573-9.
35. Anterior stromal puncture in the treatment of bullous keratopathy:
6 months follow up. Gomes JA, Haraquchi DK, Zambrano DU,
Izquierdo Junior L, Cuhna MC, de Freitas D. Cornea 2001 Aug
20(6) : 570-2.
36. Annular Keratotomy for the treatment of painful bullous
keratopathy.Koeniq SB. Am J Ophthalmol. 1996 Jan; 121 (1) : 934.
37. David Verdier. E medicine article on Map dot fingerprint dystrophy,
2005 Dec.
38. Raj Maini, Laurence Sullivan, Grant R Snibson, Hugh R Taylor,
Michael S Laughnan. Br J Ophthalmol 2001; 85 : 912 - 915.
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
withdrawal from cell cycle. Proc Natl Acad Sci USA 1996; 93 :
3500-13.
40. Agrawal Vinay, Vagh Mustali, Sangwan Virender, Rao Gullapalli N.
Indian J Ophthalmol 1994; 42 (2) : 75-80.
32. Tseng SCG, Li D-Q, Ma X. Suppression of TGF-? receptor type II
and myofibroblast differentiation in cultured human corneal and
limbal fibroblasts by amniotic membrane matrix. J cell Physiol
1999; 179 : 325 - 35.
42. M. Alfayez. Flap EKP vs Penetrating keratoplasty for PBK : 5 yr.
Follow up results. XXX congress of ESCRS. Sept. 2006.
41. Sugar. Analysis of corneal endothelial and graft survival in PBK.
Trans Am Ophthalmol Soc. 87 : 762-801, 1989.
First Author
Shreekant A. Damgude MBBS
www.dosonline.org
47
Sumita Sethi MS, Mridula Mehta MS, DNB, Shibal Bhartiya MS, Sonia Bhargav MS, DNB
T
he Relative Afferent Pupillary Defect (RAPD), or MarcusGunn Pupil, named after British ophthalmologist Robert
Marcus Gunn, is an extremely significant and highly objective clinical
finding in the examination of the visual system1,2. There are many
different conditions leading to RAPD, from severe glaucoma to an
optic nerve tumor. Also significantly, there are many conditions
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
that should be used properly and not extrapolated to the point of
irrelevance.
Normally the usual pupillary response to direct light is that both
pupils contract equally. While moving the light quickly from one
eye to the other, both pupils should hold their level of contraction.
When light is shown in the good eye, both pupils constrict. Then
shifting it to the other eye, an injured optic nerve will also transmit
(a)
light but to a lesser and slower degree. As a result, when the light
is moved from the good to the bad eye the brain interprets this as
a decrease of light being presented. The brain’s response is to
dilate both pupils to let in more light. This dilation response is in
both eyes, despite only one eye being affected. This is the essence
of the RAPD3,4,5 (Figure 1a & b).
Neuro-Ophthalmology
Relative Afferent Pupillary Defect
Neuroanatomy and physiology of the pupillary light reflex
pathway
Pupillary size is governed by the balance of actions of two opposing
muscle groups of the iris: the dilator and sphincter pupillae.
Regulation of the pupillary size is predominantly achieved by reflex
mechanisms in response to the amount of ambient light.
(Figure 2 )
Constriction: The majority of retinal axons provide afferent
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 EdingerWestphal nucleus, tertiary parasympathetic neurons travel in the
superficial dorsomedial aspect of the ipsilateral occulomotor nerve
(b)
Figure 1: Schematic diagram showing
(a) : normal pupillary response to a bright source of light
(b): pupillary response in case of relative
afferent pupillary defect
Dr. Rajendra Prasad Centre for Ophthalmic Sciences,
All India Institute of Medical Sciences,
New Delhi-110029
www.dosonline.org
Figure 2: The pupillary light reflex pathway.
(ON-optic nerve, OT-optic tract, PT-pretectal
nucleus, EW-Edinger Westphal nucleus,
CG-ciliary ganglion, SCN-short ciliary nerves
49
to reach the ciliary ganglion. The ganglion then gives rise to 8 to 10
short ciliary nerves , which subdivide into 16 to 20 branches.
Approximately 3% of these fibers ultimately converge upon and
innervate the pupillary sphincter muscles (promoting constriction),
thereby contributing to the regulation of pupil size.
Dilation: Pupillary dilation is mediated through three-neuron
sympathetic (adrenergic) pathways that originate in the
hypothalamus and descends caudally to the first synapse, located
in the cervical spinal cord (levels C8-T2). The second-order neuron
then travels from the sympathetic trunk, through the brachial
plexus, over the lung apex. It then ascends to the superior cervical
ganglion .The third-order neuron ascends within the adventitia of
the internal carotid artery, through the cavernous sinus, where it
is in close relation to the sixth cranial nerve. The oculosympathetic
pathway then joins the ophthalmic (V1) division of the trigeminal
nerve, and innervate the iris dilator muscle.
Clinical examination and quantification of Rapid afferent
papillary defect
The evaluation of the pupils is best performed in a dimly
illuminated room. This allows for some pupillary dilation, while
also allowing for the observation of the consensual pupillary
response. It is reasonable to test the individual reaction of each
pupil initially. In some cases one pupil may not be reactive due to
a variety of conditions. In cases where neither pupil reacts to light,
no further testing can be done, except for testing the pupillary
response to accommodation (focusing at near).
The “swinging flashlight test” is a popular and an established way
for identifying an RAPD6-10. In this test, a strong, steady light is
used. The light is shone into one eye, quickly switched to the other
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
give a “relative” indication of the functioning of each eye and optic
nerve. If both eyes are equally dysfunctional, no “relative” defect
would be found. The results of the test can be graded as follows
(as per the I-V system of Bell et al)9.
Grade I, initial weak constriction with greater redilatation;
Grade II, initial stall and greater redilatation;
Grade III, immediate pupillary dilatation;
Grade IV, immediate pupillary dilatation following 6 seconds of
illumination;
Grade V, immediate pupillary dilatation with no secondary
constriction at all.
Certain standard conditions have been identified for applying this
grading. The patient fixes on a 6/60 Snellen letter at 6 metres in a
darkened room. An indirect ophthalmoscope head set
(standardised as for the luminance measurements) is used as the
light source. A swinging flashlight test is than carried out with each
pupil illuminated for 3 seconds.
However subjective grading leads to discrepancies between
clinicians, is difficult to quantify, and limits its use in diagnosis. By
accurately quantifying the defect additional information can be
provided, which may aid management—for example, assessing
retinal ischaemia following central retinal vein occlusion.13
Thompson et al describe a method of quantification of RAPD
using neutral density filters7.The swinging flashlight test is
50
performed with increasing density of filter before the healthy eye
until the sign disappears or is reversed. Neutral density filters (NDF)
are available in the form of a rack or bar containing 10 filters of
increasing density. The density of the filters is measured in log
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
CRVO had an RAPD of 0.3 log unit or less and none greater than
0.9. Ischaemic CRVO had RAPD of 1.2 or greater in 91% and none
less than 0.6.
Ramsay et al have shown that crossed polarised filters (CPF) produce
exponential attenuation of light and can be used in the same way
as NDF to assess RAPD in CRVO.11
However the limitation with the use of NDF and CPF is their
scarce availability in the clinical setting.
The Sbisa or Bagolini filter bar, is similar to NDF, and is used
commonly by orthoptists to assess density of suppression in
strabismus. It consists of 17 sequential red filters. While Sbisa bar
filters are red and NDF neutral, it is the apparent brightness not
colour that determines pupil response (Figure 4 a & b). Sbisa bars
are generally much easier to obtain than NDFs in most
departments, McCormick et al, have described their use in the
same way as the NDF, to neutralise and therefore quantify a relative
afferent pupillary defect14.
Quantifying RAPD by these methods depends upon clearly
identifying an end point. The end point can be taken as either the
elimination of the sign (brisk constriction with the same redilatation
in both eyes) or reversal (looking at the eye with the RAPD-brisk
constriction by direct illumination and dilatation by indirect
illumination).
Step vise approach to a patient of RAPD
Knowing whether the patient is aware of changes in vision. If so, are
these changes acute or gradual? An acute optic neuropathy or
retinal detachment would be associated with sudden vision loss,
while gradual vision loss might support the existence of a
compressive lesion.15-17
Eliciting significant ocular and medical history. Does the patient
have a history of vascular disease, cancer, auto-immune disease,
recent infections or trauma?
Carefully examining the patient while paying special attention to the
retina and optic nerve. Optic nerve findings can be subtle or nonexistent. For instance, a relatively normal appearing nerve might
be present in a retrobulbar Optic Neuritis.
The need to refer for further testing depends on the case. If the
patient’s history and retinal examination do not offer an obvious
explanation for the RAPD, one must assume that a condition
affecting the optic nerve or optic tract is causing it. An immediate
visual field and color vision test should be performed.
Depending on the condition, the patient may need to be referred
for emergent neuro-imaging and laboratory testing . For example,
an individual who presents with an RAPD as a result of swollen
optic nerve and a history of leukemia, would require emergent
imaging and radiation treatment if the swollen nerve represented
a leukemic infiltrative optic neuropathy.
Computed tomography (CT Scan) and Magnetic resonance
imaging (MRI) are valuable tools for diagnosis of optic nerve
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
neuropathy) and in orbital diseases causing compressive damage
to optic nerve (Figure 3 a,b,c).
•
Orbital disease – Including compressive damage to the optic
nerve from thyroid related orbitopathy (compression from
enlarged extraocular muscles in the orbit), orbital tumors, or
vascular malformations.
A RAPD is an index of significant optic nerve or retinal disease,
when there is a difference in the disease process between the
two eyes. If both eyes have severe but equal disease, there
will be no RAPD. Thus, a “bilateral” RAPD does not exist.
•
Radiation optic nerve damage
•
Miscellaneous optic neuropathies, such as Leber’s optic
neuropathy (usually eventually bilateral) and other inheritable
optic neuropathies.
•
Severe disease in one eye,causing a RAPD ,will not result in
anisocoria. The pupil of the diseased eye will be equal in size
to that of the other eye due to the consensual light reaction
(unless the iris itself is diseased or unreactive).
•
Optic nerve infections or inflammations - Cryptococcus can
cause a severe optic nerve infection in the immunocompromised. Sarcoidosis and Lyme disease can cause
inflammation of the optic nerve.
•
One functioning pupil is sufficient to determine the presence
of an RAPD, (due to the presence of consensual light reflex).
•
•
The visual acuity does not necessarily correlate with an RAPD.
Some conditions lead to a marked reduction of visual acuity
with an RAPD, while others spare the central vision. Usually,
extensive loss of peripheral vision is associated with an RAPD.
Surgical damage to the optic nerve - This could include damage
following retrobulbar anesthesia; damage following orbital
hemorrhage related to eye, orbital, sinus, or plastic surgery;
damage following neurosurgical procedures such as pituitary
tumor resection; and damage related to migration of an orbital
plate after surgery to correct a blow-out fracture.
Characteristics of the Relative Afferent Pupillary Defect
•
Conditions leading to a Relative Afferent Pupillary Defect
Retinal Causes of a Relative Afferent Pupillary Defect
Optic nerve disorders
Usually retinal disease has to be quite severe for an RAPD to be
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 optic neuropathies - These include arteritic (Giant
Cell Arteritis) and non-arteritic causes. Usually associated
are a loss of vision or a step deformity in the visual field.
•
Glaucoma – Though glaucoma normally is a bilateral disease,
asymmetric optic nerve involvement can result in an RAPD.
•
•
Traumatic optic neuropathy - This includes direct ocular
trauma, orbital trauma, and remote head injuries which can
damage the optic nerve as it passes through the optic canal
into the cranial vault.
Optic nerve tumor - This is a rare cause, and includes primary
tumors of the optic nerve (glioma, meningioma) or tumors
compressing the optic nerve (sphenoid wing meningioma,
pituitary lesions, etc.)
www.dosonline.org
•
Ischemic retinal disease - including ischemic central retinal
vein occlusion, central retinal artery occlusion, severe ischemic
branch retinal or arterial occlusions, severe ischemic diabetic
or sickle-cell retinopathy.
•
Ischemic ocular disease (Ocular ischemic syndrome) – due to
obstruction/compression of the ophthalmic or carotid artery
on one side.
•
Retinal detachment - An RAPD may be seen if the macula is
detached, or if at least two quadrants of retina are detached.
•
Severe macular degeneration - If unilateral and severe
•
Intraocular tumor - Retinal and choroidal tumors if severe
(including melanoma, retinoblastoma, and metastatic lesions)
51
(a)
Figure 4 (a): Neutral density filter
(b): Sbisa bar. Note that Sbisa bar filters are
red and NDF neutral
Retinal infection – Extensive retinitis (Cytomegalovirus, herpes
simplex,).
Amblyopia
If severe, can lead to a relative afferent pupillary defect. Usually
the visual acuity would be 20/400, or worse.
Cerebral Vascular Disease
Usually, it is an optic nerve disorder that leads to an RAPD, rather
than an optic tract or visual cortex disorder. However, there tends
to be a higher percentage of crossed vs. uncrossed nerve fibers at
the optic chiasm. Thus, in a patient with a homonymous
hemianopia from an optic tract disorder, an RAPD maybe noted
in the eye with the temporal visual field defect. The nasal retina
serves the temporal visual field, and these are the fibers that would
cross at the chiasm.
Conditions not leading to a Relative Afferent Pupillary
Defect
•
Bilateral nutritional or metabolic optic neuropathies .
1.
Wikipedia contributors, ‘Marcus Gunn pupil’, Wikipedia, The Free
Encyclopedia,17July2008,21:11UTC,<http://en.wikipedia.org/w/
index.php?title=Marcus_Gunn_pupil&oldid=226320762>.
2.
Thompson HS. Pupillary signs in the diagnosis of optic nerve
disease.Trans Ophthalmol Soc UK 1976;96:377-81.
3.
Kardon, R. Anatomy and physiology of the autonomic nervous
system. In: Walsh and Hoyt Clinical Neuro-ophthalmology, 6th ed,
Miller, NR, Newman, NJ, Biousse, V, Kerrison, JB (Eds), Williams &
Wilkins, Baltimore 2005. p.649.
4.
Johnston, JA, Parkinson, D. Intracranial sympathetic pathways
associated with the sixth cranial nerve. J Neurosurg 1974; 40:236.
5.
Digre, KB. Principlesand techniques of examination of the pupils,
accommodation and lacrimation. In: Walsh and Hoyt Clinical Neuroophthalmology, 6th ed, Miller, NR, Newman, NJ, Biousse, V, Kerrison,
JB (Eds), Williams & Wilkins, Baltimore 2005. p.715.
6.
Enyedi, L.B. et al. A Comparison of the Marcus Gunn and
Alternating
Light
Tests
for
Afferent
Pupillary
Defects.Ophthalmology 5/1998; 105:871-873.
7.
Thompson HS, Corbett JJ, Cox TA. How to measure the relative
afferent pupillary defect.Diagnostic and Surgical Techniques
1981;26:39–42.
8.
Browning DJ, Tiedeman JS. The test light affects quantitation of
the afferent pupillary defect.Ophthalmology 1987;94:53–5.
9.
Bell RA, Waggoner PM, Boyd WM, et al. Clinical grading of relative
afferent pupillary defects.Arch Ophthalmol 1993;111:938–42.
10. Sun F, Tauchi P, Stark L. Binocular alternating pulse stimuli:
experimental and modelling studies of the pupil reflex to light.Math
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.
•
Refractive Error
13. Servais GE, Thompson HS, Hayreh SS. Relative afferent pupillary
defect in central retinal vein occlusion.Ophthalmology 1986;93:301–
3.
•
Media Opacity including Cataract, Corneal scar, Hyphema,
vitreous hemorrhage. Previous eye surgery
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.
•
Strabismus
•
Conditions with an Efferent Pupillary Defect
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.
•
•
52
Bilateral retinitis pigmentosa
References
(b)
•
•
•
Third Cranial Nerve Palsy
•
Adie’s Pupil
•
Horner’s Syndrome
Mild retinal diseases
•
Mild background diabetic retinopathy
•
Central serous choroidopathy
•
Non-ischemic vein occlusions
•
Mild macular degeneration
Conditions which are typically bilaterally symmetrical will not
show an RAPD:
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;116:15441545.
First Author
Sumita Sethi MS
DOS Times - Vol. 14, No. 8, February 2009
Photo Essay
Bilateral Anophthalmos in Two Brothers:
A Case Report
Garima Agrawal MS, D.C. Mehta MS
A
rare case of bilateral congenital anophthalmos in two brothers
both having the same congenital anomaly is reported.
Anophthalmos is a rare congenital anomaly and the same anomaly
in two siblings is even rarer.
Anophthalmos is a condition in which there is absence of one or
both eyes. It is a congenital anomaly. Here we report a case of two
siblings with bilateral congenital anophthalmos.
Case Report
Two children one 6 months old and the other 2 years old were
brought to our OPD by their parents for ocular examination.
On examination the babies appeared healthy and smiling
(Figures 1-4) except for the eyes. There was no systemic
abnormality.
On ocular examination eyelids were present but curved in.
Eyelashes were present. Lacrimal puncta were present. Palpebral
aperture was almost absent. After the eyelids were retracted no
ocular tissue was visible clinically except for the conjunctiva in
both the eyes of both the children. (Figures 1-6). Both the orbits
were small. Ultrasonography revealed no normal ocular tissue.
The 2 children had no other siblings. They were both full term
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
of the eyelids in child in figure 1
Figure 4: 6 month old sibling with
bilateral anophthalmos
M&J Institute of Ophthalmolgy
B J Medical College, Ahmedabad.
www.dosonline.org
55
Figure 5: Right eyeball not seen on retraction
of eyelid of child in Figure 4
Discussion
The first case of anophthalmos was reported by Thomas Bartholin
in 1657. His case had a bilateral anophthalmos associated with
abnormalities of nose, mouth, lips and extremities. Von Hippel
collected 87 cases in literature out of which 64 were bilateral and
23 unilateral1.
Primary clinical anophthalmos as described by Duke Elder consists
of a small but well formed orbit. The lid are curved in with small
orbital aperture. The orbital cavity is lined with conjunctiva. The
lacrimal puncta and the eyelashes are usually present or either
may be absent. Certain other associated ocular abnormalities
described are ankyloblepharon, epicanthus, absent lacrimal gland,
absent optic nerve, narrow optic canal, an abnormal development
of optic chiasma and lateral geniculate body1.
True anophthalmos is a histopathological diagnosis where there
are no recognizable ocular structures present in the orbit. The
term clinical anophthalmos is used when there is no evidence of a
globe or tissue on clinical examination but without any
histopathological confirmation (Duke Elder 1964)1.
A number of cases of anophthalmos have been reported. Those
in siblings are very rare.
Ashley (1947) has reported a case of bilateral anophthalmos in a
brother and sister2.
Sensi A et al (1987) reported monolateral or bilateral anophthalmos
recurring in the absence of other associated defects in 6 members
of a family. The malformation appears to be inherited as a
dominant(autosomal/X linked) trait with incomplete penetrance3.
Ben Ezra D, Sela M, Peer J reported a case of four uneventful
pregnancies and deliveries in one family. 2nd sibling suffered from
bilateral anophthalmia while 4th presented with a unilateral
microphthalmia. The microphthalmia was due to in utero globe
perforation as evident by a central corneal scar with cataractous
lens4.
Other than this there have been reports of isolated bilateral
56
Figure 6: Left eyeball not seen on retraction
of eyelid of child in Figure 4.
anophthalmos in one child. Few associated with other
abnormalities and few not.
Rosenbaum(1917) reports a case of congenital bilateral
anophthalmos of a male child who presented with absence of
both eyes at birth. In other aspects the child was healthy and well
developed. On the third day however the child died in convulsions.
No autopsy was done5.
Maria DL et al (1967) reported a case of bilateral congenital
anophthalmos in a premature male child6.
Chapu BA (1975) reported a case of congenital bilateral primary
anophthalmos in a full term otherwise healthy child at birth1.
Brunquell PJ et al (1984) report a 27 year old man with X linked
true anophthalmos. No evidence of optic globe, nerves or chiasma
was found. Rudimentary structures suggesting optic tracts were
present. Lateral geniculate nuclei were present but gliotic calcrine
cortex was thinner but had usual lamination. The normal patches
of cytochrome oxidase activity in layers II and III of visual cortex
were absent7.
Albernaz VS et al reported the imaging findings in patients with
clinical anophthalmos. They found that patients with bilateral
anophthalmos represented a distinct group fom those with
unilateral anophthalmos. Bilateral anophthalmos was associated
with absence of optic chiasma, reduced size of posterior optic
pathways and agenesisn or dysgenesis of corpus callosum. Patients
with unilateral anophthalmos had severe cranio-facial anomalies.
Imaging of the face is helpful in patients with unilateral
anophthalmos8.
The etiology of this disorder is complex Genetic and environmental
causes have been postulated. Studies that can specifically identify
the causative factors for this disorder are warranted.
Conclusion
A case of bilateral anophthalmos in two brothers is reported. This
is a very rare case where the same anomaly of bilateral
anophthalmos has been seen in two brothers. The author could
not come across a similar article in literature.
DOS Times - Vol. 14, No. 8, February 2009
References
1.
Chapu BA. Bilateral primary anophthalmos. Indian J
Ophthalmol.1975;23(4):36-37.
2.
Ashley. Bilateral Anophthalmos in a brother and sister.J
Hered.1947;38:174-176.
3.
Sensi A, Incorvaia C, Sebastian A, Calzolari E.Clinical Anophthalmos
in a family. Clinical Genetics.1987;32(3):156-159.
4.
Ben Ezra D, Sela M, Peer J. Ophthalmologica 1989;198(3):140-4.
5.
Rosenbaum.Congenital bilateral anophthalmos. Can Med Assoc
J.1919 March;9(3):254-256.
6.
MariaDL, Srivastava SK, Sankholkar PS. Bilateral congenital
anophthalmos. Indian J Ophthalmol 1967;15:241-2.
7.
Brunquell PJ, Papale JH, Horton JC, Williams RS, Zgrabik MJ, Albert
DM, Hedley-Whyle CT. Sex linked hereditary bilateral
anophthalmos. Pathologic and radiologic correlation. Arch
Ophthalmol.1984;102(1):108-113.
8.
Albernaz VS, Castillo M, Hudgins PA, Mukherji SK. Imaging
findings in patients with clinical anophthalmos.Am J
Neuroradiology.1997;18(3):555-561.
First Author
Garima Agrawal MS
www.dosonline.org
57
Julie Pegu MS, Suneeta Dubey MS, Manisha Agarwal MS
S
erious elevation of intraocular pressure (IOP) after trauma is
the result of functional impairment of the aqueous outflow
pathways. With blunt injury, the typical sequence of events is an
initial tearing of the angle structures (contusion angle injury), which
is frequently associated with intraocular bleeding and inflammation
and results in scarring and permanent reduction in the facility of
outflow. Traumatic glaucoma is often refractory to conventional
treatment and most cases may require surgical intervention.
However, there is no set protocol for the surgical management as
the results are unpredictable because of the complex nature of
trauma. The treatment therefore, should be individualized
depending on the extent of injury and the visual prognosis. This
case represents the dilemma a clinician can encounter while
managing a case of traumatic glaucoma.
perception of light, and IOP was 50 mm Hg. There was presence
of ciliary congestion, diffuse corneal epithelial edema and diffuse
hyphema. The pupil was irregular, semi dilated and sluggishly
reacting to light. There were multiple sphincter tears present
superiorly. The rest of the anterior segment (AS) view was hazy on
day one. B scan USG showed signs of vitreous hemorrhage. Routine
anti glaucoma treatment was initiated with tab acetazolamide (half
tab TID) and timolol maleate, 0.5%, (BID), along with topical
corticosteroids and cycloplegics. On day four, the hyphema had
cleared sufficiently and the AS view was clearer which showed the
presence of mild inferior iridodonesis (Figure 1). The inferior
iridodonesis made us to suspect the presence of some amount of
Clinical Meeting: Clinical Case 1
Management Dilemma in 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
on the fourth day
Figure 3: Pulling of the vitreous tag with an
end gripping forcep after anterior vitrectomy
Dr. Shroff's Charity Eye Hospital,
5027 Kedarnath Road, Daryaganj, New Delhi
www.dosonline.org
61
Figure 4: After vitrectomy the
tube was cleared of the vitreous
Few vitreous strands were seen inferiorly in
anterior chamber but not blocking the tube
zonular dehiscence inferiorly but there was no obvious phacodonesis
or vitreous prolapse. The IOP was still high at 46 mm Hg.
Consequently, anti glaucoma treatment was stepped up to maximal
medical treatment (glycerol syrup and brimonidine eye drop were
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
high at early forties. Gonioscopy done on the third week, revealed
angle recession extending 120 to 180 degrees with a resolving blood
clot present inferiorly. Rest of the angle was wide open. The left
eye had wide open angles. The vitreous hemorrhage had cleared
by the third week and the fundus was found to be within normal
limits with a cup disc ratio of 0.4 : 1. The LE had a C:D ratio of 0.3:1.
At this stage we decided to intervene surgically. We were weighing
two options, either to do trabeculectomy with MMC or a tube
implant. In this case, we decided to choose the later as the patient
was young and the presence of inferior iridodonesis could have
complicated the trabeculectomy surgery, as sudden decompression
of the anterior chamber (AC) might lead to vitreous prolapse.
With valved implants the results tend to be more predictable as
the AC is maintained throughout, and complications like
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
Figure 5: At 6 months
follow up
Figure 6: Well formed bleb at
6 months follow up
Discussion
In severe trauma with associated findings like hyphema, angle
recession and vitreous hemorrhage, the outlook for
trabeculectomy and other drainage procedures is relatively
unfavourable. Trabeculectomy is reported to be more frequently
successful in patients over the age of 40 years1 than in younger
patients. In one of the study2, the success rate of trabeculectomy
was only 38% (17/45) in patients aged 10 to 29 years whereas it was
65% (43/65) in patients aged 30 to 49 years. As the young people
are more susceptible to trauma, the surgical prognosis becomes
poorer because of their relative youth. The young age of the
patients produces a strong tendency for blebs to scar down and
fail. The adjunctive use of cytostatic agents have shown favourable
results when applied but its use can have the serious disadvantage
of tending to produce dangerously thin blebs in patients who have
a long expectancy of life3. Also, the use of mitomycin C can lead to
DOS Times - Vol. 14, No. 8, February 2009
serious ocular complications like late endophthalmitis and ocular
hypotony that may result in permanent visual loss4,5. There are
limited studies on the long term surgical outcome in traumatic
glaucoma. In one of the largest series of 87 cases of traumatic
glaucoma by Mermoud et al6 with a mean follow up of 23 months
(range, 3 to 72 months), the success rate in trabeculectomy group
(47 cases) was 53%, in trabeculectomy combined with antimetabolite group (20 cases) it was found to be 70% and in the
molteno implant group (20 cases) it was 60%. Of concern were
three cases of late bleb infection in the group that received
postoperative antimetabolite therapy, one of whom lost all sight.
In another long term series 3, insertion of molteno implants in
traumatic glaucoma produced IOP control at 10.9 years in 76% of
the cases (40 cases). There were no implant related infections.
However, there were eleven IOP related surgical interventions,
most related to blockage or malposition of the internal end of the
tube, but the IOP was controlled after clearance of the vitreous
with vitrectomy. In traumatic glaucoma, the success rate of the
surgical complications is expected to be higher because of the
complex trauma, but if treated with vigilance and on time, most of
it can be managed with a favourable prognosis.
with anti metabolites or the use of an artificial draining device,
with frequent follow ups3. Finally, patients having filtration surgery
should be carefully instructed on the early signs and symptoms of
bleb-related endophthalmitis and told to report immediately if
these occur, as early treatment appears to be associated with an
improved prognosis for visual recovery and bleb function.
References
1.
Stewart RH, Kimbrough RL, Bacch H, Allbright M. Trabeculectomy
and modifications of trabeculectomy. Ophthalmic Surg 1979; 10:7680.
2.
Gressel MG, Heuer DK, Parrish RK II. Trabeculectomy in young
patients. Ophthalmology 1984; 91:1242- 6.
3.
Fuller JR, Bevin TH, Molteno AC. Long term follow up of traumatic
glaucoma treated with Molteno implants. Ophthalmology 2001;
108:1796-1800.
4.
Greenfield DS, Suner IJ, Miller MP, et al. Endophthalmitis after
filtration surgery with mitomycin. Arch Ophthalmol 1996; 114:9439.
5.
Higginbotham EJ, Stevens RK, Musch DC, et al. Bleb related
endophthalmitis after trabeculectomy with mitomycin C.
Ophthalmology 1996;103:650-6.
6.
Mermoud A, Salmon JF, Barron A, et al. Surgical management of
post-traumatic angle recession glaucoma. Ophthalmology 1993;
100:634-42.
Conclusion
Traumatic glaucoma encompasses a spectrum of disease. Because
of the greater chance of failure of primary trabeculectomy in
traumatic glaucoma, it has been suggested that this type of
glaucoma would be best managed by trabeculectomy combined
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
Ritesh Narula MS, Manisha Agarwal MS, S.P. Chaudhary MS, Priyata Seth DO
W
e report a case with an atypical presentation of sympathetic
ophthalmia (SO) in a child caused by multiple surgical
interventions in the exciting eye. Fundus fluorecein angiography
and Optical coherence tomography findings were noted. Early
diagnosis and management with intravenous methyl-prednisolone
and immunosuppressants helped in salvaging the only seeing eye
of the child.
Case report
A nine- year- old boy presented with decrease of vision in the left
eye for 3 days duration. He had a history of cataract surgery with
intraocular lens implantation done at the age of nine months
elsewhere followed by membranectomy under general anesthesia.
He had a history of blunt trauma with a finger to the right eye
causing retinal detachment for which he underwent pars plana
vitrectomy with IOL repositioning and was subsequently diagnosed
to have recurrent total retinal detachment for which no
intervention was advised.
On examination the best corrected visual acuity in the right eye
was inaccurate projection of rays and in the left eye 6/24, N24.
Applanation tonometry recorded an intraocular pressure of 6 mm
of Hg in the right eye and 14 mm of Hg in the left eye. Slit lamp
examination of the right eye showed organized blood clot in the
anterior chamber with vascular in-growth (Figure 1) and the left
eye showed minimal retrolental flare. There was no view of the
fundus in the right eye and the fundus examination of the left eye
showed pocket of sub-retinal fluid (SRF) at the macula. 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 2a-e).Optical coherence
tomography of the left eye showed multiple pockets of subretinal
fluid with hyperreflective echoes underneath the serous retinal
detachment. (Figure 3)
Clinical Meeting: Clinical Case 2
Posterior Sympathetic Ophthalmia in a Child
The child was diagnosed as sympathetic ophthalmia and treated
with intravenous methylprednisolone for 3 days followed by a
tapering dose of oral prednisolone along with topical prednisolone
acetate 1% six times a day and atropine-1% two times a day in the
left eye. Enucleation of the right eye was advised which was refused
by the father of the child.
Follow-up at one week the BCVA in the left eye improved to 6/9,
< N36 (pharmacologically dilated pupils under atropine-1%).
Applanation tonometry recorded an IOP of 16mm of Hg in the
left eye. Fundus examination of the left eye showed a residual
pocket of shallow SRF.
At one month follow-up the BCVA in the left eye was 6/6, N9P.
Applanation tonometer recorded an IOP of 38mm of Hg. Fundus
examination and OCT of the left eye showed no evidence of SRF
at the macula. The child was started on oral and topical
antiglaucoma drugs. Topical prednisolone-1% was stopped and
oral prednisolone was tapered and stopped.
At two months the child presented with distortion of images in the
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
organized blood clot
with vascular ingrowth
Retina and Oculoplasty Services
Dr. Shroff’s Charity Eye Hospital,
Daryaganj, New Delhi
www.dosonline.org
Sympathetic ophthalmia, also known as sympathetic uveitis, is a
rare bilateral granulomatous panuveitis that occurs after a
penetrating injury to an eye. Following injury to an eye-a result of
either surgery or accident-a variable period of time passes before
a sight threatening inflammation develops in both eyes. The injured
eye is known as the exciting eye and the fellow eye as the
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
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
left eye showed multiple pockets of subretinal
fluid with hyperreflective echoes underneath
the serous retinal detachment
of SO increases when these surgical procedures are accompanied
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
Figure 4: Fundus examination and
OCT of the left eye showed SRF
at the macula suggestive of
recurrent inflammation in the left eye
DOS Times - Vol. 14, No. 8, February 2009
include bilateral low grade persistent uveitis associated with
granulomatous (“mutton-fat”) or small, white keratic precipitates,
diffuse thickening of the iris or iris nodules and Dalen Fuchs nodules
on fundus examination though not pathognomic of SO1. However
our case differed in the following ways- the child developed SO
several years after the last surgical intervention in the exciting eye,
he presented with a sudden drop of vision there was only mild
retrolental cells and flare in the sympathizing eye with no signs of
anterior segment inflammation in either eye. Lastly on fundus
examination there was serous retinal detachment at the macula.
All these factors made the diagnosis of SO difficult in our case but
based on clinical suspicion we started the child on treatment and
were able to salvage the eye. Enucleation of the exciting eye having
no visual potential helps in preventing the recurrence of SO by
decreasing the antigen load causing inflammation repeatedly.
Histopathology of the enucleated eye also helped in confirming
the diagnosis of SO in our case.
The presentation in this case closely resembles an entity described
in literarture as “ Posterior sympathetic ophthalmia”. It was first
described by Mcpherson et al 5 as a variant of sympathetic
ophthalmia with predominantly posterior segment signs and
minimal AC reaction. In a long term study of 40 patients from
North India by Gupta et al6; 36 of these patients presented with
evidence of predominantly posterior segment involvement with
22 eyes of these having no anterior segment inflammation.
Whether these cases represent an atypical form of sympathetic
ophthalmia or just the early stage of classical presentation is
debatable.
We report this case to highlight that SO may have an atypical
clinical presentation and a high clinical suspicion maybe required
for a timely diagnosis and management to salvage the vision in the
sympathesizing eye which at many a times is the only seeing eye of
the patient as in this case.
References
1.
Duke-Elder S, Perkins ES. Diseases of the Uveal Tract. Vol 9. In:
Duke-Elder S, ed. System of Ophthalmology. Vol 9. London, England:
Henry Kimpton; 1966: 558–559.
2.
Lubin JR, Albert DM, Weinstein M. Sixty-five years of sympathetic
ophthalmia. A clinicopathologic review of 105 cases (1913–
1978).Ophthalmology 1980; 87:109–21.
3.
Goto H, Rao NA. Sympathetic ophthalmia and Vogt-KoyanagiHarada syndrome. Int Ophthalmol Clin 1990; 30:279–85.
4.
Gass JD. Sympathetic ophthalmia following vitrectomy. Am J
Ophthalmol1982; 93:552–8.
5.
McPherson SD Jr, Dalton HT. Posterior form 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
Cyrus M. Shroff MD
T
he practise of vitreo-retinal medicine and surgery has changed
dramatically in the past few years. Gone are the days when
retinologists were the butt of jokes like "They always carry their
vision charts on them - their hands!" The past year has been a
satisfying one for us, a year primarily of consolidation of some of
the recent advances in diagnostics, drugs (the anti-VEGF
revolution!) and lasers. We will review the advances made in the
retinal scenario along with exciting future possibilities under the
following headings:
•
Retinal Imaging
•
Lasers
•
Drugs and their applications -with special reference to AMD
and Diabetic Retinopathy. Expanding indications of anti-VEGF
agents.
•
Pushing the frontiers
•
Gene Therapy
•
Nano Medicine
•
Artificial Retina- bionic eye
Retinal Imaging
Spectral and ultra-high resolution OCT are now quite firmly
ensconced in the armamentarium of retinal diagnostics and these
have the advantage of 3 Dimensinal imaging. Also 100% of the
mapped area is measured in Spectral domain compared to only
5% in time domain, so there are less chances of missing a small
lesion with the newer modalities. (Figure 1) One of the
manufacturers of SD-OCT (Bioptigen) provides a novel feature
of a hand-held probe opening up new capabilities to researchers
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.
that may occur with disease, in the RPE cell monolayer in outer
retina and subneurosensory retinal space. FAF gives information
over and above that obtained by fundus photography, fundus
fluorescein angiography and optical coherence tomography. It
would be useful in understanding pathophysiologic mechanisms,
phenotype-genotype correlation, identification of predictive
markers for disease progression and monitoring of new therapies.
Examples of diseases where this holds promise are AMD especially
the dry type, macular and diffuse retinal dystrophies, central serous
retinopathy, chorio-retinal inflammatory disease and Angioid
streaks. It's clinical value coupled with its simple, efficient and noninvasive nature is increasingly appreciated.
Clinical Meeting: Clinical Talk
Future of Retinal Imaging, Lasers and Therapy
Retinal lasers
The pattern scan laser (PASCAL) has been an interesting
development. The PASCAL laser's capabilities can be maximally
exploited in fresh cases for pan retinal photocoagulation where it
is indeed very quick. (Figure 3) Pan retinal photocoagulation can
also be achieved with lower delivery of energy to the retina.
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
or hemorrhage. High cost and lack of indirect ophthalmoscopic
delivery have also been limiting factors.
Nonthermal Retina Regeneration Therapy (2RT; Elex, Adelaide,
Australia), is a short-pulsed laser treatment (pioneered by John
Marshall and Peter Hamilton of UK) that is intended to treat
diabetic maculopathy and macular edema at an earlier stage of
the disease. The therapy may also have application in the treatment
of early-stage age-related macular degeneration.
The laser used for 2RT is a Q-switched, green Nd:YAG laser with
a wavelength of 532 nm. Its pulse duration is 3 nanoseconds with
energy of 1 J per pulse which causes the RPE cells to release matrix
metalloproteinase enzymes, which are believed to 'clean up' the
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 &
Shroff Eye Centre,
New Delhi
www.dosonline.org
Figure 1: Time Domain versus
Spectral Domain OCT
69
Pro-re-nata (PRN) basis with visual acuity, OCT and FA criteria.
In the 'treat and extend' strategy it is recommended to treat till the
macula is dry and continue to treat with extended time span
between injections, even when dry.
Figure 2: Combination OCT ICG
"Combination treatment" combining PDT, and anti-VEGF agents,
with or without intra-vitreal steroids, also has made the treatment
more finite. Reduced fluence PDT has improved the safety profile.
The results of two multicenter trials, SUMMIT trial and PDEX II
trial comparing combination therapy to ranibizumab monotherapy
are eagerly awaited. The SUMMIT trial has 3 arms; Standard
fluence PDT with intra-vitreal ranibizumab, reduced fluence PDT
with intra-vitreal ranibizumab and monthly intra-vitreal
ranibizumab. In the PDEX II trial another effective modality, intravitreal steroids has been added. It compares reduced fluence PDT
with 0.5 mg ranibizumab and 500mg dexamethasone to monthly
ranibizumab.
Two agents of promise in phase III trials are VEGF trap and
Epiretinal Brachytherapy with Lucentis. VEGF trap is a recombinant
protein which binds and inactivates circulating VEGF. Phase II
studies with VEGF trap showed mean best corrected visual acuity
improvement of 5.7 letters and reduction in CMT in all groups at
12 weeks compared to baseline.
Figure 3: Pascal laser
bruch's membrane. The laser thus triggers what is described as
photoregeneration of the retinal pigment epithelium and Bruch's
membrane.
With 2RT, there is potential to have a laser treatment that affects
only the RPE without damaging the photoreceptor cells, meaning
that we can get closer to the fovea without damaging central vision.
For the first time, there is the possibility of very early intervention
in patients with diabetic macular edema to preserve a greater
degree of functional vision for a longer time.
Figure 4: Neovista
Drugs and their Applications
Age related macular degeneration
In AMD management we have entered a 'consolidation phase".
The two major challenges are early detection and dosing regimes.
Early detection of AMD progression is the single most important
factor for minimising the risk of permanent vision loss. Educating
at risk population and general practitioners, good referral network
and use of telemedicine should help towards this goal.
The challenge of reducing the number of intra-vitreal anti-VEGF
agents while striving to maintain the benchmark visual gain in
trials with monthly injections (MARINA & ANCHOR) is a daunting
one. The most commonly used strategy is to give a 'loading dose'
of three, 4-6 weekly injections followed by further treatment on a
70
Figure 5: Neurotech NT 501
DOS Times - Vol. 14, No. 8, February 2009
Phase III study (View 2) is recruiting approx. 1200 subjects in over
200 centres worldwide. Primary objective is to assess the efficacy
of intra-vitreal VEGF Trap compared to Ranibizumab in preventing
vision loss in subjects with all subtypes of neovascular AMD.
Epiretinal Brachytherapy (NEOVISTA) Beta radiation is applied
to the macula after a vitrectomy, using a strontium 90 isotope
source (Figure 4). A multicenter feasibility study enrolled 34
participants with any CNV type. These patients received a single
24 Gy treatment of epiretinal brachytherapy with two injections of
intra-vitreal Avastin - one dose prior to or at the time of radiation
delivery and another, one month later. Analysis of 18 month follow
up on 25 patients showed mean improvement in visual acuity of
10.7 letters. 44% gained 15 or more letters and 8 % gained 30 or
more letters. This is better than the visual results with current
standard of care. CABARNET (CNV secondary to AMD treated
with Beta Radiation Epiretinal Therapy) is a large phase 3
multicentre ongoing trial comparing combination treatment
(Radiation plus Ranibizumab) to Ranibizumab monotherapy.
The future therefore definitely lies in a combined attack on multiple
sites of the angiogenesis cycle.
Bevasiranib (CAND5) is a siRNA which switches off VEGF
production, but clinical effect is not seen till the preexisting VEGF
is cleared. It has a potentially longer duration of effect than currently
available anti-VEGF agents. It is well tolerated at multiple doses.
Phase 3 trial comparing initial neutralization of VEGF - 3 doses of
Ranibizumab followed by Bevasiranib every 8 or 12 weeks
(maintenance) versus Ranibizumab alone is currently underway.
Fenretinide is a Retinoic acid derivative It is an orally administered
drug being studied for use in geographic atrophy associated with
dry AMD and it acts by reducing accumulation of lipofuscin in
RPE cells.
The Lucentis Vs Avastin debate rages on. The retinal world eagerly
awaits the results of the Comparison of AMD Treatments, Lucentis
Vs Avastin Trial (CATT). This is a phase III trial currently recruiting
patients (target 1200 patients) at 57 locations. Objective is to evaluate
relative efficacy and safety of treatment of neovascular AMD with
Lucentis and Avastin with fixed and variable dosing schedules.
Preliminary results are expected in 2010 and study would be
completed by 2011. However Avastin continues to be used
vigorously all over the world. Available data suggests good efficacy
and safety profile though it lacks the level of evidence of a
randomized, controlled clinical trial. The sheer economics of it
makes it available to almost any patient who requires it.
Diabetic Retinopathy
The publication that grabbed headlines this year was" A
Randomised Trial Comparing intra-vitreal Triamcinolone
Acetonide and Focal/Grid Photocoagulation for Diabetic Macular
Edema - a trial conducted by the Diabetic Retinopathy Clinical
Research Network" - DRCR. net.
Their conclusions were that over a 2 year period, focal/grid
photocoagulation is more effective and has fewer side effects than
1 mg or 4 mg doses of preservative free triamcinolone for most
patients with DME who have characteristics similar to the cohort
in this trial. They recommended that focal/grid photocoagulation
should currently be the benchmark against which other treatments
are compared in clinical trials for DME.
www.dosonline.org
Figure 6: Bionic Eye
Important limitations of this study are that focal and diffuse edema
have not been separated in terms of treatment requirement and
metabolic status of the patient has also not been a criteria.
The fact that the 4-mg intravitreal triamcinolone group had a
greater positive treatment response on visual acuity and retinal
thickening at 4 months, whereas the photocoagulation group had
a greater positive response later, raises the possibility that
combining focal/grid photocoagulation with intravitreal
triamcinolone may produce greater benefit for DME than either
focal/grid photocoagulation or intravitreal triamcinolone alone. A
DRCR.net study currently is evaluating a combination of
intravitreal triamcinolone and focal/grid photocoagulation, a
combination of ranibizumab and photocoagulation, and
ranibizumab alone in eyes with characteristics similar to those
included in the current study (protocol available at www.drcr.net).
Pushing the frontiers
Gene Therapy: According to Paul A. Silving , MD, Phd, director of
NEI, the eye is an especially good target for gene therapy. It is a
"wonderful place to test therapy ideas", since the eye is a "Separate
Compartment", we only need to administer a microscopic quantity
of gene vector (instead of circulating it in the body) and the results
are obviously measurable.
Michael Redmond's pioneering work showed that the gene RPE65
was critical for metabolism of Vitamin A for proper photoreceptor
functioning. A mutation in RPE 65 accounts for 10% of all Leber's
Congenital Amaurosis ( LCA) patients.
Recent Phase I Studies of gene therapy for young adults with a
mutation in the RPE65 genes and a severe form of recessive retinitis
pigmentosa have used adeno-virus mediated delivery to introduce
a human RPE 65 gene construct to the RPE. Results from two trials
for LCA have suggested moderate visual improvement in some
adults as monitored by psychophysical tests and studies are in
progress to see if greater visual improvement can be achieved
safely in younger patients. A Canadian & American research group
from Montreal Children's hospital hopes to begin a human gene
therapy trial in Canada within 5 years for LCA, Stargardt macular
dystrophy & retinitis pigmentosa.
71
Nano Medicine: This is a highly specific medical intervention at
nano scale (1-100nm) for curing diseases or repairing damaged
tissues. Applications in Retinal Diseases include:
•
Drugs & Delivery (Neurotech NT-150)
•
Retinal Tissue Engineering (RTE)
Neurotech (Figure 5) NT- 501 intra-vitreal implant is a Ciliary
Neurotrophic Factor (CNTF) secreting encapsulated cell device.
CNTF is a cytokine, which is a survival factor for various neuronal
cells and seems to prevent neurodegeneration. A semi-permeable
membrane encapsulates genetically engineered human RPE cells
that secrete CNTF. It prevents host antibodies and immune cells
from entering the device but allows nutrients to diffuse in, to
nourish the cells within and CNTF to diffuse out. The purpose is
to support and rescue degenerating retinal tissue using neurotophic
factors. This holds promise for retinitis pigmentosa and dry age
related macular degeneration. Phase I trial is completed. Phase II
& III patients have been recruited and results are expected by
2009/2010.
RTE: Replacement of damaged retinal cells using retinal progenitor
cells delivered on bioresorbable polymer scaffold and transplanted
into sub-retinal space is a promising therapeutic strategy. Likewise,
using tissue engineering strategies, sheets of retinal pigment
epithelium (RPE) are grown on synthetic biodegradable polymers
for subsequent transplantation.
Optoelectric retinal prosthesis "Bionic Eye":
Argus II, 60 electrode epiretinal prosthesis has been developed by
researchers at.
Doheny Eye Institute, Los Angeles. 16 subjects with advanced
retinitis pigmentosa.
have been implanted with this device at 8 centres. Orientation and
mobility with device 'on' were better than with device 'off ' with
door and line tests.
Author
Cyrus M. Shroff MD
S.B. Eye Hospital
Mainpuri (UP) – 205001
Applications are invited for the following posts:(a) Eye Surgeon:- MS/DNB/DOMS having experience in
phaco/S.I.C.S./IOL.
(b) O.T. Assistant:- Trained with experience Contact with
full Bio-Data Immediately to:-
Director
Dr. J.K. Purang (Eye Surgeon)
Mob.: 09811021192, 011-25446622, 25163444
Email: [email protected]
72
DOS Times - Vol. 14, No. 8, February 2009
J E Moore1,2, J E Graham1, E A Goodall1, D A Dartt3, A Leccisotti1,4, V E McGilligan1, T C B Moore1
British Journal of Ophthalmology 2009;93:66-72
1
3
Abstracts
Concordance between Common Dry Eye Diagnostic Tests
Centre for Molecular Biosciences, University of Ulster, Northern Ireland, UK, 2 Royal Group Hospitals, Belfast, Northern Ireland, UK
Schepens Eye Research Institute, Boston, Massachusetts, USA, 4 Casa di Cura Rugani, Siena, Italy
AIM
Large variations in results of diagnostic tests for mild to moderate dry eye are widely recognised. The purpose of this study was to assess
if there was concordance between common dry eye diagnostic tests.
METHODS
A total of 91 subjects were recruited to the study. The tear film and ocular surface were evaluated using the phenol red thread test (PRT),
tear film break-up time (TBUT), biomicroscopic examination and impression cytological assessment of conjunctival goblet cells. Dry
eye symptoms were assessed using McMonnies’ dry eye questionnaire (MQ) and statistical correlations between all tests were assessed.
RESULTS
This study cohort did not include severe aqueous deficient dry eye patients as determined by the PRT. A statistically significant
difference was noted between PRT results and all other tests (p<0.001). Only Meibomian gland pathology, MQ, reduced goblet cell
density and TBUT (<7s) demonstrated correlation determined by McNemar’s test.
CONCLUSION
A correlation was found only between tests assessing lipid/mucous deficiency (Meibomian gland evaluation, goblet cell density, TBUT
and MQ).
Prevalence of Dry Eye at High Altitude: A Case Controlled
Comparative Study
Gupta N, Prasad I, Himashree G, D'Souza P.
High Alt Med Biol. 2008 Winter;9(4):327-34
Department of Ophthalmology, Lady Hardinge Medical College and Associated Smt. Sucheta Kriplani Hospital and Kalawati Saran
Children Hospital, New Delhi, India.
High altitude is associated with physiological as well as pathological changes in the eye related to adverse environmental conditions that
result in increased tear evaporation and contribute to a higher incidence of dry eye in these regions. We aimed to study the difference
in prevalence of dry eye at high altitude and at low altitude. The prevalence of dry eye among the natives and the army soldiers who
were recently posted at high altitude was also studied and compared. 200 adults above 20 years of age were enrolled. 100 subjects were
recruited at a high altitude region (study group), of which 50 were native Ladakhis and 50 were soldiers recently posted at Leh, Ladakh,
India (height; 3300 m above sea level; temperature: 18 degrees C to 24 degrees C). 100 subjects, age and sex matched, were screened at
a low altitude region, New Delhi, India (218 m above sea level; temperature: 19 degrees C to 24 degrees C) to serve as the control group.
Prevalence of dry eye was assessed through standard questionnaires (McMonnies' Questionnaire (MMI), Ocular Surface Disease
Index Questionnaire (OSDI), and Schirmer's basic secretion test. On the basis of the parameters studied (symptoms, MMI, OSDI and
Schirmer's test), dry eye was diagnosed in 20% of subjects screened at high altitude and in 9% of subjects in the control group screened
at low altitude. In the study group, the prevalence of dry eye was significantly higher amongst the native population (54%) than in the
army soldiers who were recently posted at that region (26%). The difference was statistically significant (p < 0.005). In conclusion, dry
eye is more common at high altitude, particularly in the native population. Awareness among people residing at high altitude and the
treating medical personnel needs to be created for early detection and treatment of dry eye to prevent vision-threatening complications.
www.dosonline.org
77
Plasmin enzyme-assisted
vitrectomy for primary and
reoperated eyes with stage 5
retinopathy of prematurity
Wu WC, Drenser KA, Lai M, Capone A, Trese MT
Retina. 2008 Mar;28(3 Suppl):S75-80
Antiangiogenic therapy with
intravitreal bevacizumab for
retinopathy of prematurity
Retina. 2008 Mar;28(3 Suppl):S19-25
Quiroz-Mercado H, Martinez-Castellanos MA, HernandezRojas ML, Salazar-Teran N, Chan RV
Associated Retinal Consultants, PC, Royal Oak, Michigan, USA.
Retina Service, Asociacion Para Evitar La Ceguera en México
PURPOSE
(APEC), Mexico City, Mexico. [email protected]
To review the surgical outcome of plasmin enzyme-assisted
vitreoretinal surgery in managing stage 5 retinopathy of
prematurity (ROP).
PURPOSE
METHODS
To evaluate the role of antiangiogenic therapy with intravitreal
bevacizumab for retinopathy of prematurity (ROP).
A retrospective, consecutive interventional cases series of 80 eyes
(68 patients) with stage 5 ROP, treated with autologous or maternal
plasmin enzyme-assisted vitreoretinal surgery, were reviewed. All
study patients underwent surgery between 1995 and 2004 with
plasmin enzyme-assisted vitreoretinal surgery. Thirty-eight eyes
had previous vitreous surgery without retinal breaks (Group 1).
Fifteen eyes had previous vitreous surgery with retinal breaks
(Group 2). Twenty eyes received previous laser, and/or cryotherapy,
and/or scleral buckling, but no vitrectomy (Group 3). Seven eyes
did not receive any treatment previously (Group 4).
METHODS
RESULTS
RESULTS
Postoperative anatomic outcome, functional results, and surgical
complications in each group of eyes at an average follow-up of 49
months were recorded. Following surgery of all 80 eyes, anatomic
success was achieved in 68.8%. Six eyes (7.5%) achieved 20/60 to
20/600 vision. Fifty-nine eyes (73.8%) achieved vision worse than
20/600 to light perception. Eleven eyes (13.8%) achieved no light
perception. Visual results were uncertain in 4 eyes (5%) because of
the inability to measure reliably.
Thirteen patients (18 eyes; mean age +/- SD, 4 +/- 3 months; mean
follow-up, 6 months) were included in the study. We found
neovascular regression in 17 eyes. One patient with stage IVa ROP
had spontaneous retinal reattachment after an intravitreal
injection of bevacizumab. There were no serious ocular or systemic
adverse events.
CONCLUSION
Plasmin enzyme-assisted vitrectomy in eyes with and without
previous vitrectomy surgery can achieve visual improvement in
stage 5 ROP. Early intervention with vitreous surgery for stage 4A
ROP will achieve better anatomic and visual results and reduce
the number of children with stage 5 ROP.
In this noncomparative, prospective, interventional case series,
bevacizumab was injected into the vitreous of patients with ROP
in three different groups: group I, patients with stage IVa or IVb
ROP who had no response to conventional treatment; group II,
patients with threshold ROP who were difficult to treat with
conventional therapy because of poor visualization of the retina;
and group III, patients with high-risk prethreshold or threshold
ROP.
CONCLUSION
The use of bevacizumab may be promising in the treatment of
patients with ROP. Further studies need to be performed to
determine the safety and long-term efficacy of intravitreal injection
of bevacizumab, either as first-line therapy or after failure of
conventional therapy.
DOS Correspondent
Noopur Gupta MS, DNB
78
DOS Times - Vol. 14, No. 8, February 2009
Forthcoming Events : National
February 2009
25-26 CHENNAI
July 2009
20-22 CHENNAI
Neuro-Ophthalmology Update 2009
Sankara Nethralaya, 18, College Road,
Chennai, Tamil Nadu
Contact Person & Address
S. Ambika, Conference Secretariat
E-mail: [email protected], [email protected]
March 2009
20-22 NEW DELHI
Annual Conference of
Delhi Ophthalmological Society
Contact Person & Address
Dr. Namrata Sharma
Room No. 474, 4th Floor,
Dr. Rajendra Prasad Centre for Ophthalmic Sciences,
All India Institute of Medical Sciences,
Ansari Nagar, New Delhi – 110029
Ph.: 011-65705229, Fax: 26588919,
E-mail: [email protected], Website: www.dosonline.org
Indian Intraocular Implant & Refractive
Surgery Convention
Hotel Taj Coromandel, Chennai
Tel : +91-44-2811 2811 Fax : +91-44-2811 5871
Email : [email protected]
Website : http://www.iirsi.com
October 2009
2-4
BHAVNAGAR, GUJARAT
37th Annual Gujarat Ophthalmological Conference
Vision-2009
Contact Person & Address
Dr. Nilesh Parekh
22, ‘VINAY’ Behind Central Salt, Opp. New Filter Tank,
Bhavnagar-364002
Mobile : +09428408788, Fax : +91-0278-2566388
Email : [email protected]
Website : http://www.iirsi.com
Forthcoming Events : International
February, 2009
6-8
ROME, ITALY
May, 2009
3-7
FLORIDA
13th ESCRS Winter Refractive Surgery Meeting
Rome, Italy
Phone: +3535 1209 1100 / Fax: +353 1209 1112
Email: [email protected]
Web Site: http://www.escrs.org
March, 2009
17-22 CHICAGO
Illinois Eye Review
Chicago, Illinois, United States
Contact Name: Cindy
Phone: 312.996.6590 Fax: 312.996.7770
Email: [email protected]
Web Site: http://www.IllinoisEyeReview.org
April, 2009
4-8
SAN FRANCISCO, CA, USA
ASCRS/ASOA Symposium and Congress
Francisco, USA
Phone: 701 591 2220 / Fax: 1703 591 0614
Web Site: http://www.ascrs.org
16-18
GENEVA, SWITZERLAND
4th International Congress on Glaucoma Surgery
Organising Secretariat
O.I.C. Srl - Organizzazione Internazionale Congressi
Viale Matteotti, 7 - 50121 Firenze, Italy
Phone: 39/055/50351, Fax: 39/055/5001912
E-mail: [email protected]
www.dosonline.org
Greater Fort Lauderdale/Broward County
Convention Center
1950, Eisenhower Blvd.,
Fort Lauderdale, Florida - 33316
Phone: 1.240.221.2900, Email: [email protected]
16-19
INDONESIA
Asia Pacific Academy of Ophthalmology (APAO)
Indonesian Ophthalmologists Association (IOA)
Department of Ophthalmology
Faculty of Medicine, Universitas Indonesia,
Jalan Salemba Raya No. 6, Jakarta - 10430, Indonesia
Phone : (62-21) 3190 7282, Fax : (62-21) 392 7516
E-mail : [email protected]
June, 2009
13-16 NETHERLANDS
SOE 2009, 17th Congress of the European Society
of Ophthalmology,
Contact: Congrex Sweden AB
Attn: SOE 2009, P.O. Box 5619
SE-114 86 Stockholm, Sweden
Tel: +46 8 459 66 00, Fax: +46 8 661 91 25
E-mail: [email protected]
September, 2009
12-16 BARCELONA, SPAIN
XXVII Congress of the ESCRS
Phone: +35312091100, Fax: 35312091112
Email: [email protected], Web Site: http://www.escrs.org
79
Delhi Ophthalmological Society
(LIFE MEMBERSHIP FORM)
Name (In Block Letters) __________________________________________________________________________
S/D/W/o _____________________________________________________________ Date of Birth _____________
Qualifications _________________________________________________________ Registration No. __________
Sub Speciality (if any) ___________________________________________________________________________
ADDRESS
Clinic/Hospital/Practice ______________________________________________________________________
_______________________________________________________________ Phone __________________
Residence ________________________________________________________________________________
_______________________________________________________________ Phone __________________
Correspondence ___________________________________________________________________________
_______________________________________________________________ Phone __________________
Email ___________________________________________________________ Fax No. ________________
Proposed by
Dr. ____________________________________ Membership No. _________ Signature __________________
Seconded by
Dr. ____________________________________ Membership No. _________ Signature __________________
[Must submit a photocopy of the MBBS/MD/DO & State Medical Council / MCI Certificate for our records.]
I agree to become a life member of the Delhi Ophthalmological Society and shall abide by the Rules and
Regulations of the Society.
(Please Note : Life membership fee Rs. 3100/- payable by DD for outstation members. Local Cheques acceptable, payable
to Delhi Ophthalmological Society)
Please find enclosed Rs.___________in words ____________________________________________________ by Cash
Cheque/DD No.____________________ Dated_____________ Drawn on______________________________________
Three specimen signatures for I.D. Card.
Signature of Applicant
with Date
FOR OFFICIAL USE ONLY
Dr._______________________________________________________________has been admitted as Life Member of
the Delhi Ophthalmological Society by the General Body in their meeting held on________________________________
His/her membership No. is _______________. Fee received by Cash/Cheque/DD No._______________ dated_________
drawn on __________________________________________________________________.
(Secretary DOS)
www.dosonline.org
81
INSTRUCTIONS
82
1.
The Society reserve all rights to accepts or reject the application.
2.
No reasons shall be given for any application rejected by the Society.
3.
No application for membership will be accepted unless it is complete in all respects and accompanied by a Demand Draft of Rs.
3100/- in favour of “Delhi Ophthalmological Society” payable at New Delhi.
4.
Every new member is entitled to receive Society’s Bulletin (DOS Times) and Annual proceedings of the Society free.
5.
Every new member will initially be admitted provisionally and shall be deemed to have become a full member only after formal
ratification by the General Body and issue of Ratification order by the Society. Only then he or she will be eligible to vote, or apply
for any Fellowship/Award, propose or contest for any election of the Society.
6.
Application for the membership along with the Bank Draft for the membership fee should be addressed to Dr. Namrata Sharma,
Secretary, Delhi Ophthalmological Society, R.No. 474, 4th Floor, Dr. R.P. Centre for Ophthalmic Sciences, AIIMS, Ansari Nagar,
New Delhi - 110 029.
7.
Licence Size Coloured Photograph is to be pasted on the form in the space provided and two Stamp/ Licence Size Coloured
photographs are required to be sent along with this form for issue of Laminated Photo Identity Card (to be issued only after the
Membership ratification).
8.
Applications for ‘Delhi Life Member’ should either reside or practice in Delhi. The proof of residence may be in the form Passport/
Licence/Voters Identity Card/Ration Card/Electyricity Bill/MTNL (Landline) Telephone Bill.
DOS Times - Vol. 14, No. 8, February 2009
Anagram Time
Columns
DOS Quiz
Each of the following words is a jumbled ophthalmic or related term. There is, however, an extra letter in every set of letters. These
extra letters will also form a eight letter ophthalmic word when unjumbled.
So get cracking.
1. UNAMED
___ ___ ___ ___ ___
____
2. NOWROB
___ ___ ___ ___ ___
____
3. SHORREN
___ ___ ___ ___ ___ ___
____
4. RETURNY
___ ___ ___ ___ ___ ___
____
5. RETERNI
___ ___ ___ ___ ___ ___
____
6. BIRSUMO
___ ___ ___ ___ ___ ___
____
7. CURENETION
___ ___ ___ ___ ___ ___ ___ ___ ___
____
8. BARWUDGARDEN
___ ___ ___ ___ ___ ___ ___ ___ ___ ___ ___
____
Answers on page number 43
Missed DOS Times Copy
If you have missed your copy of DOS Times.
Please Contact:
Secretary DOS : Dr. Namrata Sharma
Room No. 474, 4th Floor,
Dr. Rajendra Prasad Centre for Ophthalmic Sciences ,
All India Institute of Medical Sciences,
Ansari Nagar, New Delhi – 110029
Ph.: 91-11-65705229, Fax: 91-11-26588919,
E-mail: [email protected],
Website: www.dosonline.org
Saurabh Sawhney DO, DNB Ashima Aggarwal MS, DNB
Insight Eye Clinic, New Delhi
Sharma Eye Hospital
Arya Chowk, Ambala City
Requires
1. ONE Oculoplasty (Part Time)
2. ONE Squint Surgeon (Part Time)
3. ONE Ophthalmic Surgeon proficient in SICS/
Phaco surgeries (Fulltime)
4. TWO Optometrists (preferably from AIIMS)
Raj Eye Hospital
Naseerabad, Gorakhpur
Requires ONE Fulltime Retina Surgeon
with preferably fellowship experience
Contact: Dr. J.S. Guja
Sai Retina Foundation
Ph.: 2673688, 26279867, 9350102339
www.dosonline.org
83
Determinants of Target IOP
Glaucoma Suspect at Moderate Risk for Visual Loss
•
IOP level at which optic nerve damage occurred
•
•
Extent and rate of progression of glaucomatous damage, if
known
Fellow of eye with established GON (excluding secondary
unilateral glaucoma). OHT with multiple risk factors (thin
CCT, high lOP, suspicious disc)
•
GLC gene mutations associated with severe glaucoma
•
Presence of other risk factors
•
Recurrent optic disc hemorrhages
•
Patient’s age
•
PXF syndrome
•
Expected life span
•
Younger age
•
Medical history
Emperical Formula for Target IOP
Glaucoma Suspect or Other Condition with Low Risk for
Glaucomatous Visual Loss
Target IOP = “Maximum IOP – Maximum IOP% - Z”
More important
•
Z is an optic nerve damage severity factor.
•
OHT(Ocular hypertension)
•
Z Optic Nerve damage
•
Older age
•
0→Normal disc and Normal visual field
•
PACS (anatomically narrow angle with no PAC signs or
raised lOP)
•
1→Abnormal Disc and Normal visual field
•
Pigment dispersion syndrome with normal lOP
•
2→Visual field loss not threatening fixation
•
Glaucoma suspect disc, including disc asymmetry
•
3→Visual field loss threatening or involving fixation
•
Family history of glaucoma
Risk Categories
Less important
Glaucoma with High 5-year Risk for Progressive Visual Loss or
High 5-year Risk for Visual Disability
•
Steroid responder, steroid user
•
Myopia
Moderate to advanced Glaucomatous optic neuropathy (GON)
with correlating visual field(VF) loss and
•
Zone β peripapillary atrophy
•
Demonstrated progression over a short time
•
Diabetes mellitus
•
Higher lOPs
•
Uveitis
•
Bilateral VF loss
•
Systemic hypertension
•
Pigmentary and (Pseudoexfoliative)PXF glaucoma
•
Very advanced VF loss, fixation threat, or glaucoma-related
visual disability
•
Young age with advanced disease
•
Secondary glaucoma
•
ACG (angle closure glaucoma)
Glaucoma with Moderate 5-year Risk for Visual Loss or
Glaucoma Suspect with High Risk for Visual Loss
•
Mild GON with correlating early VF loss and higher lOP
•
Mild-to-moderate glaucoma with low IOP2
•
PAC with high lOP and PAS
•
Younger age
www.dosonline.org
Tearsheet
Target IOP
American Academy of Ophthalmology Guidelines
•
Glaucoma patients with mild damage (optic disc cupping
but no visual field loss)
→ Reduction of 20-30% from baseline
•
Glaucoma patients with advance damage
→ Reduction of 40% or more from baseline
•
Normal pressure glaucoma
→ Reduction of 30% from baseline
•
Ocular hypertension
→ Reduction of 20% from baseline
•
Open angle glaucoma with IOP in the mid to high 20s →
Target IOP range 14-18 mmHg
85
•
Advanced Glaucoma → Target IOP < 15 mmHg
Glaucoma Suspect at Moderate Risk for Visual Loss:
•
OHT whose IOP > 30 mmHg with no sign of optic
nerve damage → Target IOP < 20 mmHg
•
Monitor closely for change or treat depending on risk
and patient preferences
Asia Pacific Glaucoma Guidelines
•
Glaucoma with High Risk for Progressive Visual Loss or Visual
Disability: Pressure reduction of 40% or 1 to 2 SD below the
population mean (9 to 12 mmHg), if achievable safely.
Treat if risk(s) increase(s) with pressure reduction of
20% or 1 SD above the population mean, whichever is
lower
•
Glaucoma with Moderate Risk for Visual Loss or Glaucoma
Suspect with High Risk for Visual Loss: Pressure reduction of
>30% or population mean, whichever is lower.
The fellow eye of unilateral glaucoma may require the
same target as the affected eye depending on risk and
state.
Glaucoma Suspect with Low Risk for VisualLoss
Monitor, no treatment.
Shibal Bhartiya MS, Shubha Bansal DNB
Dr. Rajendra Prasad Centre for Ophthalmic Sciences,
All India Institute of Medical Sciences, New Delhi-110029
86
DOS Times - Vol. 14, No. 8, February 2009