DOS_Sept_2008

Contents

E5 ditorial Pediatric Ophthalmology

Glaucoma 45 Optical Coherence Tomography in Children: A feasible Option

11 Progression of Glaucomatous Optic Neuropathy: An Update Monica Gandhi, Suneeta Dubey, Julie Pegu

Ramanjit Sihota, Geetha Srinivasan, Viney Gupta 50 Pediatric Orbital Tumours

Cornea Ramesh Murthy, Santosh G Honavar, Geeta K. Vemuganti

27 Conjunctival Grafting for Pterygium Surgery-Tips and Tricks A59 bstracts

Srinivas K. Rao F63 orthcoming Events

Retina M67 embership Form

31 Cluster Endophthalmitis Columns

Lalit Verma, Shaifali Gupta, H.K. Tewari, Dinesh Talwar, 71 DOS Quiz
Avnindra Gupta
Saurabh Sawhney, Ashima Agarwal
35 Diffuse Unilateral Subacute Neuroretinitis with Macular Hole
Tearsheet
Subrata Mandal, Pradeep B Reddy, Naginder Vashisht, Zahir Abbas,
Pradeep Venkatesh, Rajpal Insan, Satpal Garg 75 Metabolic Control of Diabetes: Guidelines

39 Choroidal Metastases Pankaj Lamba, Ashish Ahuja, Prem Avtar Lamba

Sandeep Saxena, Ramit A.K. Rastogi, Kanwaldeep Singh,
Kuldeep Vishwkarma, Sanjiv Hansraj, Dipak Kumar

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4 DOS Times - Vol. 14, No.3, September 2008

Editorial

Evidence Based Medicine

Dear Colleagues and friends,
Evidence Based Medicine is the current trend of today. It is the process of systematically reviewing, appraising & using clinical
research findings to help in the delivery of optimum clinical care to the patients. It forms a part of the multifaceted process for
production of evidence through research & scientific observations and implementation of evidence-based practice through edu-
cation.
Every clinician strives to provide the best possible care for patients, but it is not always possible to keep up with the current de-
velopments or to translate them into clinical practice. One has to rely on published papers, which may not be always tailored to
meet the clinician’s needs.
Evidence is presented in many forms. Value of evidence can be ranked in descending order of credibility.
I. Strong evidence from at least one systematic review of multiple well-designed randomized controlled trials.
II. Strong evidence from at least one properly designed randomized controlled trial of appropriate size.
III. Evidence from well-designed trials such as non-randomised trials, cohort studies, time series or matched case-controlled

studies.
IV. Evidence from well-designed non-experimental studies from more than one centre for research group.
V. Options of respected authorities, based on clinical evidence, descriptive studies or reports of expert committees.
Sometimes research findings may contradict each other and obscure the true picture which may occur with small trials. Such a
fallacy can be over come by a process called as meta-analysis. It is done by pooling together all the results of various research
studies, by which the sample size can, in effect, be increased. Although pooling together the results of a number of trials will
provide a greater weight of evidence, it is still important to examine meta-analyses critically.
In an endeavour to enhance practice of Evidence based medicine, Delhi Ophthalmological Society has subscribed to online access
of the 18 journals through OVID research engine. We are also in the process of adding many more journals in print.
We have also bought the books for the DOS library which include American Academy of Ophthalmology Basic and clinical
science course (2008-2009) & Albert & Jakobiec’s Principles & Practice of Ophthalmology (4 Vols.) 2008 apart from many others.
The list of these will be available very soon.
Looking forward to seeing you at the Mid-term DOS Conference on 22nd & 23rd November and wishing you a very happy
Diwali!

Thanking you,

Namrata Sharma
Secretary,
Delhi Ophthalmological Society

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6 DOS Times - Vol. 14, No.3, September 2008

www.dosonline.org 7

Online Journal Available
Many New Journals at DOS Library

Dear DOS Members,

We are pleased to announce that DOS has subscribed to online access of the following 18 journals. We are also in the
process of adding a few more journals. These journals can be accessed at the DOS library situated at 4th floor of Dr. R.P.
Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi-110029. The timings are from 9.30
A.M. to 6.00 P.M. on week days and 9.30 A.M. - 2.00 P.M. on Saturday. The Library will remain closed on Gazetted
Holidays. Members are requested to utilise the available facilities i.e. Computer with Video Editing & Conversion facility
VHS to VCD, Journals Viewing, Books and Journals etc. The DOS members can get the full text articles of the current
issues as well as many back issues of these subscribed journals.

• Archives of Ophthalmology • Acta Ophthalmologic Scandinavica
• British Journal of Ophthalmology • Clinical & Experimental Ophthalmology
• Contemporary Ophthalmology • Cornea
• Current Opinion in Ophthalmology • Evidence-Based Ophthalmology
• International Ophthalmology Clinics • Journal of Glaucoma
• Journal of Neuro-Ophthalmologica • Journal of Pediatric Ophthalmology & Strabismus
• Journal of Refractive Surgery • Ophthalmic Surgery, Lasers and Imaging
• Ophthalmology Management • Retina
• RETINAL Cases & Brief Reports • Techniques in Ophthalmology

You are welcome to give any more suggestions for the improvement of the library facility and making the process simpler
for us.

Looking forward to hearing from you and hope this facility would be of benefit to all of us.

Regards.

(Dr. Namrata Sharma) (Dr. Vinay Garodia)
Secretary, DOS Library Officer Incharge

Mob: 9811084552
Email: [email protected], [email protected]

8 DOS Times - Vol. 14, No.3, September 2008

Delhi Ophthalmological Society Website

www.dosonline.org

The DOS Website (www.dosonline.org), features a new look with a re– designed Members 9
Corner. You can now access the Updated Members Directory, Submit your Query and
communicate easily with other fellow members. You can also update your membership
details in real time! Forthcoming Events, Trade Directory, Details on the DOS Conferences
and the DOST Program are available and regularly updated. The latest DOS Times & Delhi
Journal of Ophthalmology are also available for download. Online registration was available
for the 2008 Annual Conference and will be regular feature in our forthcoming Annual
Conference.

To Test Drive the DOS Website, login with your Membership No. & Password today.

We are in the process of making this experience even more seamless and welcome your
comments and suggestions. Please mail your opinions to [email protected].

www.dosonline.org

Progression of Glaucomatous Optic Glaucoma
Neuropathy: An Update

Ramanjit Sihota MD, FRCS, Geetha Srinivasan MS, DNB, Viney Gupta MD

G laucoma is a progressive optic neuropathy, which is Genetics
characterized by progressive loss of the retinal ganglion cells.
This process is an irreversible one as of now and its timely detection With all external variables remaining the same, what is it that
at the earliest stages is of paramount importance in order to halt influences the rate of progression?
the process before functional loss occurs.
The importance of genetic susceptibility has been well documented.
With the prevalence of glaucoma being 66.8 million worldwide and
glaucoma a cause of bilateral blindness in about 6.7 million, 1 it is
important for us to address this issue, to prevent increasing ocular
morbidity.

According to the Ocular Hypertension Treatment Study (OHTS),
the cumulative probability of developing glaucoma after 5 years is
9.5% in eyes with untreated ocular hypertension.2,3. (Figure 1) In a
retrospective study 4 in Olmsted County it was found that treated
patients of ocular hypertension had a 4%, 20- year, cumulative
probability of bilateral blindness and a 14%, 20-year, cumulative
probability of unilateral blindness. The Early Manifest Glaucoma
Trial (EMGT) 5 determined that newly diagnosed cases of glaucoma,
that were treated, had one-half the risk of progression compared
with those who were untreated. A similar study was conducted in
St. Lucia, West Indies, wherein they followed the disease progression
in untreated patients with diagnosed or “suspected” glaucoma to
end stage disease. 6,7 for a mean period of 10 years. They reported
that the probability of blindness in 10 years is 16%. In this scenario,
the main thrust in glaucoma management is diagnosing progression
so as to halt it.

Though clinical detection of changes in the optic nerve head and
RNFL corroborated by visual field assessment still remain the
accepted methods to detect progression in glaucoma, they are
fraught with problems. The main drawback in clinical evaluation of
the optic disc and retinal nerve fiber layer is that it is subjective with
considerable intra and interobserver variation.

Though the visual field remains the gold standard to detect change
objectively, up to 50% of local ganglion cells are lost to glaucoma
before detection using conventional automated perimetry. 8,9
Further, there is empiric evidence to suggest that conventional
perimetry is not the most sensitive technique to monitor changes
in the visual field. 10-14

Another point to consider is that, although, elevated intraocular Figure 1: Progression rates in glaucoma
pressure (IOP) has definitively been proven as a major risk factor estimated by various studies. OHTS Ocular
for the development and progression of glaucoma, therapeutic Hypertension Treatment study. EMGT Early
reduction of the IOP does not cause stabilization of the disease
process in many patients.15,16 Further, normal or low-tension Manifest Glaucoma Trial
glaucoma is a well established entity. So other factors must
necessarily play a role in the development and progression of this
disease.17 (Table 1)

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

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Table1: Historical evidence of risk factors for glaucoma visual field scores than the non-optineurin/412A ones. They also
progression found the frequency of TNF-alpha/-863A and optineurin/603A
(or Lys98) carriers were significantly higher in patients with POAG
No of FU Risk factors (p = 0.008) or NTG (p 0.027) than in control subjects. Among the
patients with POAG who were carriers of TNF-alpha/-863A, the
eyes period ones with optineurin/603A (or Lys98) had significantly worse (p
0.026) visual field scores than did those with non-optineurin/603A
Quigley 647 12 yr NFL, CD, disc crescent, age (or Lys98).27 Another study in Japan reported that open-angle
glaucoma patients (n=18) with the amino acid preserving
Greece 345 7 yr Age, HT, myopia polymorphism, c.412G > A, may be at a higher risk for the
progression of open- angle glaucoma. 28
Kass 62 5 yr IOP, family H
The major prohibitive factor in using genetic analysis for clinical
OHTS 1636 6yr CCT, age, race, CD decision making is the cost. In future, the cost effectiveness of genetic
screening will have to be weighed against the cost of conventional
EMGT 129 6yr Age, IOP, PXF, Fds screening in the decision making process.

AGIS 591 8-13 Age, ed, Male, DM Clinical evaluation

CIGTS 607 5 yr Age, Black, DM Despite the advances in imaging techniques the importance of
clinical evaluation of the optic nerve head and the retinal nerve
CNTGS 160 6yr Migraine, disc hhage fibre layer in the assessment of glaucoma patients remains
unchanged. This is largely because of the wide variability in the
TIGR/MYOC has been recognized as an important gene in the appearance of the optic nerve head and the limitations of the various
pathogenesis of glaucoma since approximately a decade 18,19,20 While investigative modalities in detecting progression.
specific mutations in this gene have been shown to be responsible
for primary open angle glaucoma (including juvenile and adult Morphologic changes in the optic nerve head are closely related
forms), there has been growing evidence that some sequence with functional deficits in the visual field in patients with chronic
changes (e.g., Pro370Leu) 21 are associated with a more aggressive open angle glaucoma. Thinning of the neuroretinal rim,
and earlier form of glaucoma. Alward et al 22 have reported that development of notches in the rim, optic disc hemorrhages, and
besides this sequence, other mutations (396 Ins397, Tyr437-His, focal or diffuse loss of the retinal nerve fiber layer, enlargement of
and Ile477Asn) also may have a role in more aggressive glaucomas, beta zone of peripapillary atrophy have all been shown to be
which may not be as amenable to medical and laser therapy. associated with progression of glaucoma. (Figure 2) Since these
changes precede the development of visual field defects by several
It has been postulated that the main site of the variations in this years. 29-33, identifying them by a thorough direct ophthalmoscopy
gene are at the promoter site. A strong association has been seen or slit lamp biomicroscopy (+78D) is extremely important to
between the mt.1 (+) variant and more rapid glaucoma disease prevent further functional loss.
progression.23,24. Since this variant is inherited it may act not only as
an indicator of those patients in whom therapy needs to be initiated, Neuroretinal rim: Glaucoma is characterized by progressive thinning
but also those who need more aggressive therapy. In a study of the neuroretinal rim. The pattern of loss of the rim varies and
conducted at the Wills Eye Hospital 23, 147 patients were followed may take the form of diffuse thinning, localized notching, or both in
up for an average of 15 years. In this study they found that that the combination. Thinning of the rim, while occurring in all sectors, is
patients with the mt.1(+) variant had a 55% and 93% risk of generally greatest at the inferior and superior poles, leading to a
progression of glaucoma at 10 and 15 years respectively. As loss of the physiological rim shape so that the infero-temporal rim
compared to this, mt.1 (-) patients showed a risk of progression of is no longer the thickest. 34,35,36 Another significant point to be
6% and 18% at 10 and 15 years respectively. remembered on evaluation is that the area of the neuroretinal rim
at baseline determines the risk of progression. This was shown
An isolated case report of progressive optic nerve cupping and by Jonas et al 37 who conducted a prospective study in 763 eyes of
neural rim decrease in a patient with normal intraocular pressures 416 white patients over a period of about 6 ½ years and found that
and bilateral autosomal dominant optic nerve colobomas 25, the smaller the area of the neuroretinal rim, the greater the risk of
prompts us to think of genetic influences in normal tension glaucoma. progression.
This is further supported by another study in normal tension
glaucoma patients by Drance et al 26, who showed that Asians had Another way to look at the same concept is that the larger the cup-
a slower rate of progression, and the few black patients enrolled disc ratio, the greater the risk of progression. Or in other words
had a tendency for faster progression. the more advanced the glaucomatous damage, the more the
propensity to progress. Araie et al 38 saw this in patients with
There have been numerous studies on the Japanese population to normal-pressure glaucoma. Friedman et al 39, who did a meta-
assess the role of genetics in the pathogenesis of glaucoma. One analysis on the risk factors for glaucoma progression, found that
such study conducted there found that the frequency of TNF- the NRR area was one of the variable risk factors for progression.
alpha/-857T and optineurin/412A carriers was significantly higher But Quigley et al. found that the association between NRR area
(p 0.006) in patients with POAG than in control subjects. Further, and progression was not so strong when other factors were
among patients with POAG who were carriers of TNF-alpha/- adjusted for. 40
857T, the optineurin/412A carriers had significantly worse (p 0.02)

12 DOS Times - Vol. 14, No.3, September 2008

Figure 2: Clinical determinants of glaucoma progression
A. Optic disc haemorrhage B. Large cup disc ratio C. Optic nerve pit D. Widening RNFL

Parapapillary Beta zone: There is agreement among many authors that among the morphological factors associated with progression;
that the beta zone of parapapillary atrophy occurs more often in greater cup-disc ratio was associated with a strong risk for
eyes with glaucomatous optic atrophy than in normal eyes. .41,42,43 progression. But as far as ocular hypertension is concerned
Enlargement of this zone has been correlated with progression of whether the increased cup disc ratio in progressors is an actual risk
glaucoma by some.44,45,46 Tezel et al. in fact noted that there was a factor or just an indication of structural loss is an issue to be
concordance between the presence and extent of the peripapillary resolved.49
atrophy and the subsequent development of visual field
abnormalities in cases of ocular hypertension. 47 In a study Retinal Nerve Fibre Layer (RNFL): The thickness of the RNFL is
conducted by Budde et al. an enlargement of this zone was found in more in normal subjects than in patients with glaucoma.50,51,damage
only 1.6% of cases of glaucoma and ocular hypertension after a to this layer occurring before the development of visual field
follow up of 3.9+-2.6 years of follow up. So enlargement of the beta loss.52,53,54,55 Sommer even stated that RNFL defects precede visual
zone of parapapillary atrophy seems to be of doubtful value in the field defects by as many as 6 years 56
detection of progression on follow up.48 Also in myopic eyes it may
be difficult to differentiate the myopic scleral crescent and inferior RNFL damage in glaucoma is either focal or diffuse and while the
crescent from parapapillary atrophy clinically. With the advent of former is difficult to appreciate clinically in early stages, focal loss
imaging techniques whether the value of this zone in detecting takes the form of wedge shaped defects, which can be picked up by
glaucomatous change increases is to be seen. careful evaluation on slit lamp biomicroscopy. The widening of
these defects if picked up early is of immense benefit in preventing
Cup- Disc ratio: While a CD ratio (CDR) of >0.65 is found in less further functional loss by intervening appropriately
than 5% of the normal population, a difference in the cup/disc ratio
between eyes with equal overall optic disc size is highly suggestive The assessment of the RNFL can be done by SLB with the +78D
of acquired damage. lens or by fundus photography. RNFL photography may identify
RNFL injury years before visual field damage becomes apparent.57
The OHTS reported that besides other factors (pattern standard
deviation, age, IOP), baseline vertical and horizontal cup-to-disc In fact Quigley et al54 found that the RNFL is a better predictor of
ratio is a good predictor of the onset of primary open-angle progression than the optic nerve head criteria.
glaucoma.3Even in normal tension glaucoma patients, Ariae et al38
found that a high cup-to-disc diameter ratio significantly Disc Haemorrhage: Disc haemorrhage is a specific indicator of the
influences progression of visual field defects in normal tension progression of glaucomatous optic nerve damage. 58 In patients
glaucoma. An elegant study conducted by Friedman et al39 with primary open-angle glaucoma, NTG and ocular hypertension,
concerning the strength of risk factors for progression of glaucoma patients with disc hemorrhage show more progressive visual field
and ocular hypertension, found that there was evidence to suggest changes as compared to patients without disc haemorrhage.59,60,
61,62 Though the association of disc haemorrhage and NTG is

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known,30,63,64, other reports have shown that even in NTG, there is Physiological Factors
increasing field loss in the eye with the disc hemorrhage 65 as
compared to the fellow eye without a disc haemorrhage. While glaucoma management is generally confined to reduction of
IOP to a so-called “target pressure”, there are many cases in whom
One interesting study reported that patients with optic disc glaucoma continues to progress despite therapeutically lowered
haemorrhage tend to show visual field progression in the central 10 IOP. 77 One of the many factors to be kept in mind in these patients
degrees.66 So this may be the area to concentrate on while following is the role of the autonomic nervous system in the regulation of
up these cases. Another interesting feature that has been reported IOP through its influence on the blood flow, blood pressure and
is that optic disc haemorrhages are often associated with rim heart rate variation. 78,79 Gerghel et al 80 have written an elegant
notching at the site of bleeding 67,68,69 and also with RNFL defects.30,67 review in which they state that chronic imbalance of the ANS could
lead to important adverse cardiovascular events; some of these
Do the RNFL defects develop after the appearance of disc consequences are manifested during sleep or shortly after
haemorrhage or do the disc hemorrhages appear in preexisting awakening in the morning and could exacerbate glaucoma.
areas of RNFL thinning? While several researchers (30,59,62) have
shown the RNFL to be the initial site of visible damage after optic This was corroborated by Waldmann et al 81 demonstrated by 24
disc hemorrhage, others 70 have reported that only 17% of hour ECG monitoring, that nocturnal episodes of myocardial
haemorrhages occurred within an existing RNFL defect, and the ischemia are commoner in patients with glaucoma.
majority occurred in either apparently normal RNFL or in areas
adjacent to or at the border of an existing RNFL defect. It is an established fact that secretion of aqueous humor shows a
circadian rhythm and is influenced by the two hormones-
However, it is important to remember that the prevalence of disc epinephrine 82 and cortisol 83Anything that alters this rhythm affects
haemorrhages is low. While it has been reported as 2.99% cases of IOP
glaucoma in the Singapore Eye Study 71, another study 72 in an
Asian population reported a prevalence of 8.2% in 793 glaucoma An A.N.S assessment is therefore not a bad idea in a progressive
cases. More importantly, disc hemorrhages are transient in nature glaucoma, where the IOP is within the target range.
and resolve spontaneously with time. So, while the presence of a
disc haemorrhage warrants more aggressive therapy and a closer Evidence for the above is exemplified by patients suffering from
follow up, the absence does not rule out its occurrence in the past.. both glaucoma and obstructive sleep apnoea (OSA) another A.N.S
disorder. In this condition, additional therapy in the form of nasal
Optic nerve pit: As early as 1978 73 it was suggested that pit-like continuous positive airway pressure could potentially prevent the
changes in selected patients with glaucoma might not represent progression of glaucomatous optic neuropathy. 84
congenital lesions but rather local, progressive nerve head disease,
occurring particularly in response to raised intraocular pressure. As of now A.N.S assessments are not routinely done in glaucoma
patients, but could facilitate better understanding of the disease in
Among patients with glaucoma, patients with acquired pit of the selected cases.
optic nerve represent a subgroup who are said to be at increased
risk for progressive optic disk damage and visual field loss. In a Visual fields
study by Ugurlu et al.74, of 46 acquired pits of the optic nerve in 37
eyes of 25 patients they found progression of optic disk damage in The visual field is the so-called gold standard to assess progression
64% in the group with acquired pit and in 12.5% in the group without objectively in glaucoma. However, it would be prudent to keep in
acquired pit. Progression of visual field loss occurred in 56% in the mind that conventional perimetry is probably not the most sensitive
former group and 25% in the latter group. More recently, it has technique to monitor changes in the visual field. ;85,86 Despite various
been seen that acquired pits of the optic nerve (APONs) may be an statatistical methods that have been advocated to assess whether
indication of abnormal susceptibility of the optic nerve to the the field of a given patient is truly worsening or just showing long
damaging effects of IOP.75 In this particular study the authors found term fluctuation, the ultimate decision is still based on training,
an optic nerve pit in 74% cases of low tension glaucoma and 15% of experience and personal bias.
cases of high tension glaucoma. More longitudinal follow up would
tell us the clinical implications of this information. A lot of programs like modified Andersons criteria, overview
printout, Peridata and delta program of Octopus, and Glaucoma
Blood vessel changes: Changes in the vasculature of the retinal change Probability analysis in the Humphrey are all designed to
arterioles are normally considered as a soft sign in the assessment facilitate interpretation of changes in the visual field in the central
of glaucoma patients. 76 30-2 and central 24-2 threshold tests.

While baring of the circumlinear vessels occurs with enlargement Broadly, there are two types of analyses to find out if a field has
of the cup and may be a sign of progression, there has also been progressed or not.
speculation that the diameter of the retinal arterioles is a sign
predictive of progression in glaucoma. But this was refuted by One method called trend analysis analyzes sequential fields over
Jonas et al who studied 763 eyes over a mean period of 67.4 months time by linear regression A linear regression of various parameters
and found that though the diameter of the retinal arteries at the like total deviation (TD), mean deviation (MD), corrected pattern
border of the optic disc was less at baseline in the group that standard deviation (CPSD) or corrected loss variance (CLV) is
showed progression, it was not a predictive risk factor for calculated by the software to analyze change and compared to the
progression of glaucoma. 37 threshold value. If it is significantly negative, then it is said to have
progressed. Besides change in global indices, this method can also
be used to find out change in mean sensitivity in various sectors of

14 DOS Times - Vol. 14, No.3, September 2008

the field and also for individual test locations. While regression of The AGIS investigators 97 analyzed 12,756 visual fields of 752 eyes
visual field parameters over time is thought to be a reasonable way and concluded that a 2 dB MD change or 2 units of the AGIS score
in which to identify visual field deterioration, regression of clusters is sufficient to diagnose progression. They initially used a criteria
of locations or of individual locations probably provides more of > 4 units, or a score of 19 or 20, sustained for 3 consecutive 6-
information about the location of loss than could regression of month follow-up visits, but later reported that to confirm
global indices. 87 progression a single confirmatory test is nearly as good as two
confirmatory tests.
The second type of analyses is called the event type analysis. In this
method progression is determined when the threshold deteriorates While the AGIS method of analyzing progression is said to have a
beyond the previously prescribed level. This uses paired t tests, very high specificity, the methods of using the TD slope are said to
confidence intervals, analysis of variance on mean threshold be more sensitive.98
sensitivity, pointwise glaucoma change probability analysis or original
scoring systems based on the results of the C24-2 program of the What is the mean rate of change in the visual field? Most studies
HFA. have found the mean rate of change for the total visual field between
–0.029+0.048 db/mo99,100 Surprisingly, some reports indicated a
Overview printout: While the overview printout does offer a very more rapid deterioration of the visual field in cases where the initial
easy way to detect glaucoma change in a maximum of as many as threshold values were larger.101This means that progressive damage
16 serial follow ups of a particular patient, just by a glance we must is greater in the earlier stages of the disease, but this has been
remember that it does not give us the reliability indices. Of course disputed by others. 102
all the fields being compared should be of the same strategy.
Finally, for an individual patient, the most appropriate method to
Glaucoma change probability programme 88 is probably one of the detect change may be based on the stage of the disease as well as
simplest and reliable methods to analyze progression. This event the status of the other eye.
based analysis program, compares the average of two baseline
visual fields with each follow-up field on a point-wise basis. It Short- wave automated perimetry (SWAP) Because of the limitations
provides a point by point statistical analysis of how much change a of white-on-white perimetry in diagnosis and monitoring of
given field has undergone when compared to the baseline field of glaucoma, use of other apparently more sensitive techniques like
the patient and also when compared to the database of stable SWAP were developed. SWAP is said to selectively isolate the S-
glaucoma patients. In this particular method, confirmed progression cone responses in the central visual field. 103 In this method a blue
occurs when there is a complete overlap in the location of 4 or stimulus is presented against a yellow background. This background
more black triangles on the pattern deviation plot in 2 of 3 consecutive saturates the rods and suppresses the sensitivity of the long and
follow-up fields. (Figure 3) medium wavelength pathways. 104 The basic principle in SWAP is
that in the early stages of glaucoma it is the large diameter ganglion
While there are various algorithms used by various study groups- cells of the koniocellular pathway that are lost selectively 105. Further
like the Advanced Glaucoma Intervention Study (AGIS), since these large diameter cells are relatively fewer in number,
Collaborative Initial Glaucoma Therapeutic Study (CIGTS), and involvement of these cells by the disease process should ideally be
many others they are more of relevance for research. able to target glaucomatous damage earlier than conventional SAP.
While there are reports that SWAP can detect glaucomatous damage
Whatever the method, the major problem with detection of several years earlier than SAP ,106,107 Girkin et al found that, SWAP
progression is long term fluctuation. 89-93 A system of neural networks showed a higher sensitivity even in detecting progressive optic disc
have been studied to sort out this problem. A typical neural network changes 108 While assessing a SWAP printout, we must remember
is composed of processing elements arranged in layers and that SWAP deficits have been found to progress at a rate twice as
connected to each other by “synapses”. This network, which is trained fast as those seen with SAP. (Figure 4)
to assess change in the visual field, has been found to agree with the
opinion of expert observers most of the time. 94 However, as of Despite these obvious advantages over SAP, SWAP has not actually
now this has limited relevance clinically. established itself as an indispensable tool in routine glaucoma
evaluation, because, it does not identify all the eyes that have
Another important consideration when analyzing visual field progressed and it identifies almost one third of normal eyes as
progression is –what is the number of fields and the frequency at having progressed.109 Whether longer follow up will increase
which we must ask for them to detect progression. The accepted specificity is to be seen. It is also a more tedious test for the patient
practice as of now is to get a repeatable defect on at least 2 of 3 to perform.
fields using the same testing strategy and pattern of test locations
to identify and monitor progression. One study reported that Frequency doubling perimetry (FDP): Since visual field testing with
average change in threshold sensitivities of less than 1 dB/year SAP is not specific for any particular ganglion cell type, the FDP
could not be detected even with seven fields done over 6 years. 95 was proposed as an alternative for the early detection of
This problem of detecting change so late can probably be countered glaucomatous functional damage.110 Among the retinal ganglion
by concentrating our monitoring on previously damaged parts of cells, the M-cell pathway is responsible for low contrast, high
the visual field. Boden et al 96 suggested the above, after they temporal frequency stimulus detection. These magnocellular (M)
found that the most common pattern of progression was cell neurons that constitute approximately 15% of the total number
deepening of a preexisting scotomas rather than the development of ganglion cell numbers. 111,112 are the first to die in glaucoma, and
of new scotomas. this unique pathological characteristic established the basis for
FDP. The magnocellular line, having larger and less number of

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Figure 3: A contiguous cluster of 4 such solid triangles repeated on 2 of 3
consecutive fields indicates progression

cells, may still be subject to selective loss and reduced redundancy. 109 in which FDP identified progression in 51% of eyes as against
39% with SAP. Another interesting fact that was highlighted in their
In comparison to conventional perimetry, FDT differs in its use of study was that progression of defects in the SWAP and FDT were
a larger target that stimulates a much larger retinal area. In a study often seen in the same quadrant, despite both testing different
by Haymes et al.113 FDT identified progression before SAP in the subsets of ganglion cells.
majority of patients with glaucoma, when fewer locations were
required to classify progression. This was similar to another study One advantage of FDT is that variability does not increase as

16 DOS Times - Vol. 14, No.3, September 2008

Figure 4: More extensive field defects on SWAP as compared to SAP

much with defect severity as the test retest variability of SAP does. In trend analysis, 114 various parameters like rim area, rim volume,
Since the size of the target is larger, it probably takes care not only mean height contour, cup shape measure, mean cup depth, mean
of the defect depth, but also effects of eccentricity on variability. height inside contour line and a combination of these parameters
Further, despite the obvious advantage of earlier detection of are analyzed over time. The change in these parameters is analyzed
defects, a major limitation with this psychophysical test is the high from a specific reference plane. The change is represented on a
false-positive rate that would reduce specificity. Probably a longer scale of +1 which indicates maximum improvement to –1 which
follow up will improve these results. indicates maximum deterioration of clinical value is the fact that
the RNFL and rim area are said to be the most reliable parameters
Despite these advantages, the practical usage of this modality as a to detect change.
detector of progression is still limited for several reasons- lack of
standardization in the criteria for progression, too many false Tan et al 115 have defined another method to identify change in
positives and not enough data on role in progressing glaucomatous which they considered only the change in nrr over time. They
optic neuropathy. analyzed the nrr in 30 degree sectors of the rim area which they
defined by a new experimental plane. This sectoral analysis is said
Confocal Scanning laser ophthalmoscopy: CSLO (HRT II) was to be satisfactorily reproducible with the changes predictably
introduced primarily for quantitative imaging of the optic disc in occurring at the poles, but almost 20% of their patients showed
glaucoma. Presently the main clinical use of the CSLO is detection change only on CSLO and not on SAP. Whether this is because
of change over time. A topographic height map of the optic disc change in morphology precedes visual field defects would be clear
and peripapillary retina is produced with high spatial resolution. only with a longer perimetric follow up.
The advantages over conventional optic disc photography include
quick image acquisition, quantitative analysis, and the ability to Topographic change analysis is another method used to analyze
obtain good quality images in most subjects without pupil dilation. change over time that relies on significant change in values in
discrete areas of the image called superpixels. BC Chauhan et al
The detection of progression by the HRT II is either by a trend 116 used the 256*256 pixel array from the topographic image and
analysis or Topographic Change Ananlysis (TCA) divided each pixel into a 64*64 superpixel array, where each

www.dosonline.org 17

Figure 5: HRT Trend Analysis

superpixel contained 16 (4*4) pixels. This was used to study change sometimes, the GDx VCC (variable corneal compensation) is more
in these discrete areas in the form of a disc index. This method is objective and reproducible.117 The role of the SLP in detecting
said to detect change in discrete parts of the image studied. They glaucoma has been acknowledged by many, but the change in the
found that this method detected significant change in 65% of RNFL over time as a method of detecting glaucomatous change is
glaucoma cases out of which 42% showed visual field progression yet to be validated. The Nerve Fibre Indicator (NFI) a global
on SAP. But surprisingly, they also noted that there were almost measure based on the entire RNFL thickness map is probably a
41% who showed progression first on perimetry. The lack of reliance better parameter in the assessment of preperimetric glaucoma,
on a reference plane and contour line, which eliminate variability since the RNFL shows larger variations. 118
(which is highest at the edge of the cup and along blood vessels and
lowest in the peripapillary retina) and enhances reproducibility are Boehm et al119derived limits of change from a test retest study in 27
the main advantages of this method. normal eyes of 16 subjects. There are also reports120 about the role
of the GDX in the detection of preperimetric glaucoma. But the
Though there are statistical cut offs to determine change in the change in RNFL with age and race and only a moderate correlation
form of percentiles, as to how much change is clinically relevant is of this parameter with glaucomatous visual field loss makes it difficult
not clear. Also, in both the methods the change needs to be repeated to quantify the amount of change that can be interpreted as clinically
and confirmed by a repeat testing. Variability is an added issue. significant. Presently its role in the detection of progressing
(Figure 5) glaucomatous damage is still ill defined mainly because of lack of
standardized criteria defining progression (Figure 6A & B) and a
The role of the HRT as of now is not as an alternative, but as a large overlap between normal and glaucoma eyes.
complementary tool in analyzing glaucomatous change. The advent
of newer methods to detect change will probably help us in better Optical coherence tomography (OCT): While the OCT is said to
standardization and clinical application. distinguish normal from glaucomatous eyes in several studies ,121,122
as far as glaucoma progression is concerned more longitudinal
Scanning laser polarimetry (SLP): SLP gives a quantitative measure data , would give us clear cut criteria to define deterioration One
of the thickness of the peripapillary nerve fiber layer. It analyzes the study123 that evaluated 64 eyes (37 patients) of glaucoma/suspects
change in polarization in light backscattered from the fundus. Once defined RNFL thinning of 20um as progression on the OCT. In
the polarization of the anterior segment is neutralized, the change this prospective longitudinal study nearly double the number
in polarization is proportional to the RNFL thickness. While the of eyes showed a progression by OCT (22%) as compared to
older version of the instrument which had a fixed corneal SAP (9%).
compensation has shown spurious measurements of the RNFL

18 DOS Times - Vol. 14, No.3, September 2008

Figure 6A: Stable glaucoma on GDX VCC

So although it is a tool with potential to detect change by virtue of Further, it is important to have a uniform pupillary dilation at all
its high resolution of about 10 u as well as its ability to scan multiple visits, to obtain stereo-base pairs which will be comparable.
regions like the macula, optic nerve head and retinal nerve fibre
layer, future studies are awaited on this aspect. Simultaneous photography with its definite added advantage of
better reproducibility is also said to be 2 times more sensitive in
Stereophotography: Though the technique of stereophotography picking up subtle alterations in vessel position, than a simple fundus
dates back to 1850, it still remains the gold standard to assess photograph.
morphological change in the optic disc and retinal nerve fibre layer.
Sequential photography in which serial images are obtained is Stereochronoscopy is a modification of the simultaneous image
cheaper than simultaneous photography but less reproducible. acquisition process in which the observer wears 3D stereo goggles
which contain liquid crystal display (LCD) shutters synchronized

www.dosonline.org 19

Figure 6B: Progressive diffuse loss of NFL inferiorly, but with a normal
Nerve fiber indicator- Should we start treatment?

to the monitor. 124 The detection of changes in the neuroretinal rim • Laser Doppler Flowmetry and
is said to be more sensitive by this method.
• Fluorescein Angiography
Vascular factors: While ischemia of the optic nerve head is an accepted
patho-mechanism in glaucoma, in some patients in whom reduction Colour Doppler Imaging: Helps us to assess compromised blood
of IOP does not halt the disease process, it probably plays a much flow in the ophthalmic artery, short posterior ciliary artery and
more important role. In fact it has been seen that even glaucoma central retinal artery, by calculating the volume, velocity and
patients with normal IOP show signs of ocular and cerebral resistivity index. It has been seen that the ophthalmic artery diastolic
ischemia.125 Further a meta-analysis of studies carried out over the velocity was significantly lower in patients who progressed
past 07 decades about of the influence of IOP in glaucoma showed perimetrically, than in stable patients.127 The ophthalmic artery
that 57% of patients showed disease progression, with persons at resistivity index, which signifies the ease which with the blood flows
all levels of IOP equally likely to exhibit deterioration.126 in a particular region, has been found to be much higher in
deteriorating patients. The study by Gerghel at al 80 found that the
The influence of vascular factors on glaucoma has been studied by mean ocular perfusion pressure and end diastolic velocity in the
central retinal artery and ophthalmic artery were much more
• Colour Doppler imaging compromised in the patients in whom IOP stability did not prevent
perimetric deterioration. The value of these studies is in the fact

20 DOS Times - Vol. 14, No.3, September 2008

that the measurement of haemodynamic variables, though most risk factor. Not only is there a proven association between mean,
often for experimental than clinical application, are reproducible peak and baseline IOP with deterioration in numerous studies
and reliable 128. But it must be remembered that ophthalmic artery 142,143, , fluctuation in IOP as little as .3mm is also said to influence
variables are more reproducible than SPCA.129 disease stability.144 The OHTS study found that every 1 mm Hg
increase in mean IOP was associated with a10% increased risk of
Drance et al130 who used a transcranial Doppler USG method to progression from OHT to glaucoma. 3
analyze OA blood flow also confirmed that those patients with a
progressive visual field loss have lower ophthalmic artery blood In a large majority of patients just keeping this risk factor under
flow. check by attaining the target IOP appears to halt the disease process.
However, in the ultimate analysis the target IOP is an individualized
The main impediment in accurate interpretation is that CDI number for each eye. that would probably prevent further
measures blood velocity and not blood flow, though the two can functional; loss.
probably be correlated reasonably.131
A study 145 of chronic primary angle closure glaucoma (CPACG)
Laser Doppler flowmetry: is a technique that uses a diode laser eyes at our centre found that two thirds of these maintained stable
beam of 670 nm with a 20 mW intensity and approximately 200 um visual fields with an IOP <21mmHg.
is delivered through a fundus camera. This measures the relative
optic nerve blood volume, velocity and flow in the optic nerve A review of 97 eyes with advanced glaucoma involving the central
head. But though this technique has the advantage of low 10 degree fixation at our tertiary centre showed that an IOP >18
invasiveness, the contribution of the deeper and superficial layers mm Hg was responsible for progression, with all other systemic
of the optic nerve head are speculative.132 Topical antiglaucoma and ocular factors (Ocular disease, Pachymetry, baseline visual field
medication is said to alter heamodynamics133 of the optic nerve, defect)being comparable. PACG eyes progressed faster than POAG
though this is disputed by others134 Whatever the facts ONH eyes and HVF 10-2 detected the deterioration earlier than HVF30-
measurements have been found to be considerably lower in the 2.
inferotemporal rim, which is the region most prone to develop
glaucomatous damage. Miscellanous factors

Fluorescein Angiography: FFA reveals reduced retinal blood flow Central corneal thickness: While the relevance of central corneal
and dye leakage from the ONH capillaries, suggesting ischemia in thickness in measurement of IOP was described as early as 1975
the precapillary region. Another characteristic feature is delay in and later corroborated by many others 146, it was the Ocular
choroidal filling.135 But these have been seen in all cases of glaucoma Hypertension Treatment Study (OHTS) 3 that gave the crucial
without specific reference to the stability or progression of the association of CCT as a powerful predictor for the development of
disease process. POAG. They found that patients with a CCT of 555um or less had
a 3- fold greater risk of developing POAG as compared with patients
Electrophysiological tests: The Pattern ERG (PERG) is a noninvasive who had a corneal thickness of more than 588 um. This fact has
electrophysiological technique that is altered not only in glaucoma been disputed by the EMGT5, though other studies have also found
136 and OHT, 137 but has also been shown to be capable of detecting that those POAG eyes with a thinner mean CCT showed significant
disease progression. 138. The PERG results have been reported to deterioration. Kim et al. 147 reported a hazard Ratio of 1.44 per 40
be abnormal in at least one of the outcome measures (amplitude, um thinner CCT. The influence of age, race, gender, refractive
phase and interocular asymmetry in amplitude and phase) in 69% change, diagnosis of glaucoma, surgical procedures and mean IOP
of early glaucoma patients as compared to normals 139 on CCT though not indisputably proven should be kept in mind
when evaluating a glaucoma patient.
A few reports have demonstrated that this test is capable of detecting
glaucomatous change before it manifests on the SAP.3 While Trick Pseudoexfoliation: PXF glaucoma is a known risk factor for
140 reported that all eyes with ocular hypertension that developed progressive glaucoma.148 with the EMGT reporting a hazard ratio
perimetric defects on SAP had abnormal PERGs before the field of 3.15.
defect was manifest, others 113 found that a large percentage of
patients (almost one third)of OHT who showed no field defects Diabetes mellitus: While majority of the studies like AGIS, CIGTS,
also showed abnormal PERG. Primate glaucoma studies indicate CNGTS and EMGT found that diabetics had a greater likelihood to
that PERG amplitude reductions corroborate with the degree of progress, the OHTS surprisingly found that diabetes mellitus
cupping of the optic nerve head. But, since the PERG is better probably protects against glaucomatous damage. But this could be
equipped to detect generalized dysfunction of the retinal ganglion attributed to sample bias.
cells rather than focal defects for which SAP is definitely superior, a
combination of the two may probably aid in detection of progression Age: The AGIS, CIGTS, EMGT as well as a number of other studies
better than either test alone. Further the influence of age on the found that age was an independent risk factor for progression of
PERG parameters should be taken into consideration during analysis. both OHT as well as glaucoma.
Whether race as an independent entity has a bearing on the PERG
needs to be ascertained. Bayer et al 113 found that the amplitude of Blood Pressure: Glaucoma is said to be associated with systemic
a specific PIN2 wave showed a reduction in 64.8% of POAG before hypotension as well as systemic hypertension. In NTG patients
progressive standard visual field defects. who showed progression of the disease, hypertension has been
reported as an association. 149 However, low blood pressure has
Intraocular Pressure: Of the factors that have been conclusively also been implicated. Kaiser et al reported the association of low
shown to cause glaucoma progression, this is the only modifiable blood pressure even in POAG patients who progressed, 150 despite
normalized IOP. While the systolic and diastolic dip in blood

www.dosonline.org 21

pressure has beem implicated as a factor responsible for progression predict the development of glaucomatous visual field loss. Arch
in POAG as well as NTG, Georgopoulos et al. 151 have postulated Ophthalmol. 1993;111:645-650.
that even in OHT converting to glaucoma systemic hypertension is 15. Rosetti L, Marchetti I, Orzalesi N, et al. Randomized clinical trials on medical
a risk factor.The role of systemic antihypertensive agents leading to treatment of glaucoma. Are they appropriate to guide clinical practice?
a nocturnal fall in blood pressure in glaucoma progressors has also Arch Ophthalmol 1993;111:96-103.
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risk factor for glaucomatous damage? J Glaucoma 1999 8:212-219.
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90. Birch MK, Wishhart PK, O’Donnell NP. Determining progressive visual
field loss in serial Humphrey visual fields. Ophthalmology 1995;102:1227-
34;discussion 1234-216.
91. Wild JM, Searle AET, Dengler-harles M et al. Long term follow-up of
baseline learning and fatigue effects in automated perimetry of glaucoma
and ocular hypertensive patients. Acta Ophthalmol 1991;69:210-216.
92. Werner EB, Petrig B, Krupin T et al. Variability of automated visual fields

23

in clinically stable glaucoma patients. Invest Ophthalmol Vis Sci. 117. Lleo-Perez A, Ortuno-Soto A, Rahhal MS, et al. Interobserver
1989;30:1083-1089. reproducibility of retinal nerve fiber layer measurements using scanning
93. Boeglin RJ, Caprioli J, Zalauf M. Long term fluctuation of the visual field laser polarimetry and optical coherence tomography in normal and ocular
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94. Caudill M, Butler C Naturally Intelligent Systems.Cambridge, Mass: MIT
Press,1990. 118. Medeiros FA, Zangwill LM, Bowd C et al. Use of progressive glaucomatous
95. Brigatti L, Nouri-Mahdavi K, Weitzman M. et al. Automatic detection of optic disc change as the reference standard for evaluation of diagnostic tests
Glaucomatous visual field progression with neural networks. Arch in glaucoma. Am J Ophthalmol 2005;139:1010-1018.
Ophthalmol. 1997;115:725-728.
96. Boden C, Blumenthal EZ, Pascual J et al. Patterns of glaucomatous visual 119. Boehm MD, Nedrud C, Greenfield DS, Chen PP. Scanning laser polarimetry
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2004;138:1029-1036. glaucoma. Arch Ophthalmol 2003:121:189-194.
97. AGIS Investigators. Distinguishing progression of glaucoma from visual
field fluctuation. Ophthalmology 2004;111:2109-2116. 120. Mohammadi K, Bowd C, Weinreb RN, et al. Retinal nerve fiber layer
98. Mayama C, Araie M, Suzuki Y, et al. Statistical evaluation of the diagnostic thickness measurements with scanning laser polarimetry predict
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in glaucoma. Ophthalmology 2004;111:2117-2125.
99. O’Brien C, Schwartz B. The visual field in chronic open angle glaucoma.: 121. Greaney MJ, Hoffman DC, Garway Heath DF, et al. Comparison of optic
The rate of change in different regions of the field. Eye. 1990;557-562. nerve imaging methods to distinguish normal eyes from those with
100. O’Brien C, Schwartz B, Takamoto T, et al. Intraocular pressure and the rate glaucoma. Invest Ophthalmol Vis Sci 2002;43:140-45.
of visual field loss in chronic open-angle glaucoma. Am J Ophthalmol
1991;11:491-500. 122. Medeiros FA, Zangwill LM, Bowd C. Comparison of GDxVCC Scanning
101. Schwartz B, TakamotoT Martin J Increased rate of visual field loss associated laser polarimeter, HRT II confocal scanning laser ophthalmoscope and
with larger initial visual field threshold values on follow-up of open-angle stratus optical coherence tomography for the detection of glaucoma. Arch
glaucoma. J Glaucoma 2004;13:120-129. Ophthalmol 2004;122:827-837.
102. Rasker MTE, van den Enden A, Bakker D, et al. Rate of visual field loss in
progressive glaucoma. Arch Ophthalmol 2000;118:481-88. 123. Woolstein G, Schuman JS, Price LL, et al. Optical coherence tomography
103. Martin PR, White AJ, Goodchild AK, et al. Evidence that blue-on cells are longitudinal evaluation of retinal nerve fiber layer thickness in glaucoma.
part of the third geniculocortical pathway in primates. Eur J Neurosci Arch Ophthalmol 2005;123:464-470.
1997;9:1536-1541.
104. SamplePA, Weinreb RN. Color perimetry for assessment of primary open 124. Barry CJ, Eikelboom R, Kanagasingam Y et al. Comparison of optic disc
angle glaucoma. Invest Ophthalmol Vis Sci. 1990;1869-1875. image assessment methods when examining serial photographs for
105. de Monasterio FM. Asymmetry of on- and off-pathways of blue-sensitive glaucomatous progression. Br J Ophthalmol. 2000 Jan;84(1):28-30.
cones of the retina of macaques. Brain Res. 1979;166:39-48.
106. Sample PA, Weinreb RN. Progressive color visual field loss in glaucoma. 124a.Ophthalmol 2000;84;28-30.Stereochronoscopy
Invest Ophthalmol Vis Sci. 1992;33:2068-2071. 125. Alon Harris, Janescu-Cuypers C, Martus B et al. Simultaneous measurement
107. Johnson CA, Adams AJ, Casson EJ, et al. Blue-on-yellow perimetry can
predict the development of glaucomatous visual field loss. Arch of blood flow and intraocular pressure in glaucoma. Acta Ophthalmol.
Ophthalmol 1993,111:645-650. Scand. 2001;79:336-341.
108. Girkin CA, Endadi A, Sample PA, et al. Short-wavelength automated 126. Chauhan BC. The relationship between intraocular pressure and visual
perimetry and standard perimetry in the detection of progressive optic disc field progression in glaucoma, in Drance SM (ed): Update to glaucoma,
cupping. Arch Ophthalmol 2000;118:1231-1236. blood flow and drug treatment. Amsterdam. Kugler,1995,pp1-6.
109. Bayer AU, Erb C. Short wavelength automated perimetry, Frequency 127. Galassi F, Sodi A, Ucci F et al. Ocular haemodynamics and glaucoma
doubling Technology Perimetry, and Pattern electroretinography for prognosis. Arch Ophthalmol 2003;121:1711-1715.
prediction of progressive glaucomatous standard visual field defects. 128. Harris A, Williamson TH, Martin B et al. Test re-test reproducibility of
Ophthalmology 2002;109:1009-1017. Colour Doppler Imaging assessment of blood velocity in orbital vessels. J
110. Glovinsky Y, Quigley HA, Dunkelberger GR. Retinal ganglion cell loss is Glaucoma. 1995;4:281-286.
size dependent in experimental glaucoma. Invest Ophthalmol Vis Sci. 129. Kaiser JH, Schoetzan A, Flammer J. The frequency distribution of blood
1991;32:484-91. flow velocities in the extraocular vessels. Graefes Arch Clin Exp Ophthalmol.
111. Chaturvedi N, Hedley-Whyte ET, Dreyer EB. Lateral geniculate nucleus in 1996;234:537-541.
glaucoma. Am J Ophthalmol. 1993;116:182-88. 130. Drance SM, Rojanapangpun P. The ophthalmic artery velocity in open angle
112. Landers J, Goldberg I, Graham S. A comparison of short wavelength glaucoma. In: Drance SM, ed. Update to Glaucoma, Ocular blood flow
automated perimetry with frequency doubling perimetry for the early and Drug treatment. New York, NY: Kugler Publications 1995:55-56.
detection of visual field loss in ocular hypertension. Clin Experiment 131. Zink JM, Grunwald JE, Piltz-Seymour J, et al. Association between lower
Ophthalmol.2000;28:248-252. optic nerve laser Doppler blood volume measurements and glaucomatous
113. Bayer AU, Erb C. Short wavelength automated perimetry, frequency visual field progression. Br J Ophthalmol 2003;87:1487-1491.
doubling technology perimetry, and pattern electroretinography for 132. Petrig BL, Riva CE, Hayreh SS. Laser Doppler flowmetry and optic nerve
prediction of progressive glaucomatous standard visual field defects. head blood flow. Am J Ophthalmol 1999;127:413-25.
Ophthalmology 2002;109:1009-1017. 133. Grunweld JE. Effect of topical timolol in the humal retinal circulation. Invest
114. Patterson AJ, Garway-Heath DF, Strouthidis NG, Crabb DP. A new statistical Ophthalmol Vis Sci. 1996:27;1713-19.
approach for quantifying change in series of retinal and optic nerve head 134. Findl O, Rainer G, Dallinger S, et al. Assessment of optic disc blood flow in
topography images. Invest Ophthalmol Vis Sci 2005;46:1659-1667. patients with open angle glaucoma. Am J Ophthalmol 2000;130:589-96.
115. Tan JC, Poinoosawmy D, Hitchings RA. Tomographic identification of 135. Yin ZQ, vargan A, Millar TJ, et al. widespread choroidal insufficiency in
neuroretinal rim loss in high-pressure, normal- pressure, and suspected primary open angle glaucoma. J Glaucoma 6:23-32;1997.
glaucoma. Invest Ophthalmol Vis Sci 2004;45;2279-2285. 136. Price MJ, drance SM, Price M, et al. The pattern Electroretinogram and
116. Chauhan BC, McCormick TA, Nicolela MT, LeBlanc RP. Optic disc and visual evoked potential in glaucoma. Graefes Arch Clin Exp Ophthalmol
visual field changes in a prospective longitudinal study of patients with 1988;226:542-7.
glaucoma: comparison of scanning laser tomography with conventional 137. Ambrosio G, Arienzo G, Aurilia P, et al. Pattern electroretinographs in
perimetry and optic disc photography. Arch Ophthalmol 2001;119:1492- ocular hypertension. Doc Ophthalmol 1988;69:161-5.
1499. 138. Graham SL, Drance SM, Chauhan BC, et al. Comparison of psychophysical
and electrophysiological testing in early glaucoma. Invest Ophthalmol Vis
Sci 1996;37:2651-62.
139. Ventura LM, Porciatti V, Ishida K, et al. Pattern electroretinogram
abnormality and glaucoma. Ophthalmology 2005;112:10-19.
140. Trick GL. Pattern Electroretinography: an electrophysiological technique
applicable to primary open angle glaucoma and ocular hypertension. J
Glaucoma 1992;1:271-9.
141. Porciatti V, Falsini B. Inner retina contribution to the flicker
Electroretinogram: a comparison with the Pattern Electroretinogram. Clin
Vis Sci 1993;8:435-47.

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142. Nouri Mahdavi K, Hoffman D, Coleman AC, et al. Predictive factors for 147. Kim JW, Chen PP. Central corneal pachymetry and visual field progression
glaucomatous visual field progression in the AGIS study:ophthalmology in patients with open angle glaucoma. Ophthalmology 2004;111:2126-2132.
2004;11:1627-1635.
148. Leske MC, Heijl A, Hussein M, et al. for the early Manifest Glaucoma Group.
143. Advanced Glaucoma Intervention study. AGIS study:7 The relationship Factors for glaucoma progression and the effect of treatment. The Early
between control of intraocular pressure and visual field deterioration. Am Manifest Glaucoma Trial. Arch Ophthalmol 2003;121:48-56.
J Ophthalmol 2000;130:429-440.
149. Rouhiainen HJ, Terasvirta ME. Haemodynamic variables in progressive
144. Oliver JE, Hattenhauer MG, Herman D,et al. Blindness and glaucoma. A and non progressive low tension glaucoma. Acta Ophthalmol 68:34-36, 1990.
comparison of patients progressing to blindness from glaucoma with
patients maintaining vision. Am J Ophthalmol 2002;133:764-772. 150. Kaiser HJ, Flammer J, Graf T et al. Systemic blood pressure in glaucoma
patients. Graefes Arch Clin Exp Ophthalmol. 231:677-680.
145. Sihota R, Sood A, Gupta V, et al. A prospective longterm study of primary
chronic angle closure glaucoma. Acta Ophthalmol. Scand.2004;82:209-213. 151. Georgopoulos G, Andreanos D, Liokos N, et al. Risk factors in ocular
hypertension. Arch Ophthalmol 1994;112:644-649.
146. Medeiros FA, sample PA, Weinreb RN. Corneal thickness measurements
and frequency doubling technology perimetry abnormalities in ocular 152. Leske MC, Heijl A, Hyman L, et al. Predictors of long term progression in
hypertensive eyes. Ophthalmology 2003;110:1903-8. the Early Manifest Glaucoma Trial. Ophthalmology 2007;114:1965-1972.

First Author
Ramanjit Sihota MD, FRCS

Answer Quiz No. 3

4. GLAUCOMA 3. PTOSIS 2. ARGON 1. GRID
8. MICROANEURYSM 7. RETINOPATHY 6. CATARACT 5. RUBEOSIS
Extra Word: DIABETES

www.dosonline.org 25

Conjunctival Grafting for Pterygium Cornea
Surgery - Tips and Tricks

Srinivas K Rao DO DNB FRCSEd

A pterygium is a fibrovascular, wing-shaped encroachment of pterygium, leaving only the cosmetically unacceptable, altered
the conjunctiva onto the cornea. Ultraviolet light-induced portions on the head of the lesion. Tent up the conjunctiva and
damage to the limbal stem cell barrier with subsequent dissect it off the body using Westcott scissors. Take care not to
conjunctivalization of the cornea is the currently accepted etiology buttonhole the conjunctiva. Making small snips with the scissors
of this condition. Indications for surgery include visual impairment, will help avoid this problem, and it is important to try and dissect a
cosmetic disfigurement, motility restriction, recurrent thin layer of conjunctiva for optimal cosmesis. When working on
inflammation, interference with contact lens wear and rarely, nasal pterygia, it is important to stop dissection before the caruncle
changes suggestive of neoplasia. Surgical techniques include simple is reached, to reduce bleeding. The most important caveat is to
excision, with or without adjunctive measures like postoperative ensure that the upper and lower borders of the pterygium are
beta-irradiation, thiotepa drops, intraoperative and postoperative identified and the healthy sclera visualized. Failure to do this can
mitomycin-C and various techniques of conjunctival grafting. The promote the occurrence of “around-the-graft” recurrence.
reported recurrence rates of these techniques vary widely from 5%
for mitomycin C use and conjunctival autografting to 89% for Removing the pterygium head
simple excision. This article highlights the key surgical caveats for
the appropriate management of this common condition. The corneal epithelium 2 mm ahead of the head of the pterygium
is scraped off with a hockey-stick knife. This exposes the altered
Obtain adequate exposure epithelium just adjacent to the head of the pterygium, which is

Use peribulbar anesthesia, although the procedure can be done
with topical gel or drops, supplemented if necessary with
subconjunctival anesthetic injection. Use a good speculum to expose
the eye and a superior rectus bridle suture to help position the eye
appropriately, during surgery. The bridle suture can be pulled
temporally to better expose nasal pterygia.

Preserve conjunctiva

Leave the pterygium attached to the sclera – to serve as a “third
hand” during surgery. Incise the conjunctiva at the head of the

Figure 2a: Expose borders of pterygium body

Figure 1: Good exposure Figure 2b: Preserve conjunctiva over
the pterygium
Darshan Eye Clinic
Block T 80 (New No 24), Vth Main Road 27

Chennai 600040

www.dosonline.org

Figure 3: Removing the head of the pterygium Figure 6: Excise a thin graft

Figure 4: Excising the pterygium Figure 7: Placing the graft into position

the corneal surface with multiple cuts, which tend to heal with
scarring.

Excising the pterygium

Figure 5: Measuring the scleral bed In primary pterygia, the body of the pterygium can easily be removed
from the scleral surface using blunt dissection. In recurrent pterygia,
thickened and more firmly attached to the underlying cornea. The a combination of blunt and sharp dissection is required to remove
hockey stick knife is used to elevate this thickened epithelium off the adherent fibrovascular tissue from the scleral surface. Especially
the underlying cornea. Once this plane is defined, the pterygium in recurrent pterygia, extreme care should be taken to avoid damage
head can be easily avulsed using a combination of blunt dissection to the medial rectus muscle. If required, tagging the muscle will
and traction. It is important to use blunt dissection to avoid violating help identify tissues and avoid inadvertent damage to the muscle.
The pterygium tissue must be aggressively excised, by pulling on
the lesion to ensure that a good area of clearance is obtained.
However, it is important to avoid cutting the underlying rectus
muscle or the caruncle. The limbal region must be smoothed out
by using gentle scraping with the edge of the hockey stick knife, or
by using a burr. If this region is irregular, the adherence of the graft
can be compromised.

Planning the graft size

After the pterygium is excised, gentle hemostasis is achieved using
a wet-field cautery. Usually, the bleeders are from the cut edges of
the Tenon’s and pterygium tissue. The scleral bed itself must be

28 DOS Times - Vol. 14, No.3, September 2008

cauterized gently, if at all, as excessive cautery can interfere with the Without lifting the tissue off the cornea, it is rotated and moved
“take” of the conjunctival graft. It is also useful to cauterize carefully into its scleral bed with fine non-toothed forceps. A limbus-limbus
the four corners of the bed, as these are the areas where sutures orientation is maintained. This helps avoid inadvertent scrolling of
will be taken, and it is important that there is no bleeding from the graft with resultant inversion of the surfaces. It is important
these sites, which can pool under the graft. The scleral bed that the graft fits adequately into the bed. If it is too small, it tends
dimensions are measured using calipers – the limbal and forniceal to retract and potentates a recurrence. On the other hand, if it is
edges, and the width of the bed from the limbus to the forniceal excessively large, it tends to bunch and fold, which can result in
edge. poor cosmesis, and can affect the adherence of the graft to the
underlying sclera.
Harvesting the graft
Suturing the graft
The bridle suture is used to rotate the eye downwards to expose
the superior bulbar sclera. The measured dimensions are marked It is important that the 4 corners of the graft be secured using
onto the superotemporal conjunctiva using several cautery spots. episcleral sutures. These help fix the graft in position and avoid
Using a non-toothed forceps and Westcott scissors, the graft is displacement of the graft, which can occur as the tissue around the
excised starting at the forniceal end. Care must be taken to obtain scleral beds contracts and retracts in the postoperative period. Once
as thin a graft as possible without button-holing. Once the limbus the graft is secured with the 4 cardinal sutures at the 4 corners, the
is reached, the graft is flipped over onto the cornea and the Tenon’s forniceal edge can then be sutured with further interrupted sutures,
attachments at the limbus are meticulously dissected. The flap is which need not include the episclera. The suture can be 10-0 nylon
then excised using a Vannas scissors, taking care to include the or vicryl – both work equally well. If is important to suture the graft
limbal tissue. carefully, as suture placement in the fornices can be difficult, especially
if bleeding obscures visualization, resulting in inadvertent globe
Graft placement perforation.

After excision, the conjunctival-limbal graft is slid onto the cornea.

Figure 8: Cardinal sutures must be episcleral Figure 10: Place graft adjacent to bed

Figure 9: Closing the donor site Figure 11: Place fibrinogen on graft

www.dosonline.org 29

Figure 12: Flip graft into position Figure 13: Smooth graft into place and co-apt edges

Closing the donor site scleral bed. In such situations, the intraoperative use of
mitomycin C can also be considered. If there is significant
The superior rectus bridle suture is removed and the forniceal corneal scarring involving the visual axis, then a lamellar
conjunctiva is pulled forwards and anchored to the limbal episcleral keratoplasty may have to be combined with pterygium surgery
tissue with 2 interrupted 10-0 nylon sutures. This prevents exposure to help restore visual function
of the Tenon’s and reduces the proliferative stimulus to the • With the advent of fibrin glue, the need to suture the graft is
conjunctiva. reduced, saving time and effort. The glue is reconstituted in
two parts – the fluid thrombin and the more viscous fibrinogen.
Postoperative care The graft is excised and placed on the cornea – with the stromal
side facing the surgeon and the limbal edge of the graft adjacent
At the conclusion of surgery, 0.5cc dexamethasone is injected to the limbus of the scleral bed. The thrombin is placed in the
subconjunctivally and the eye is firmly patched with antibiotic eye scleral bed and the fibrinogen on the graft. The graft is then
ointment. Starting the next day, topical steroids are used at 2 hourly flipped into the bed, bringing into contact, the two components.
intervals and then tapered gradually over the next 6 weeks. Antibiotic The ensuing reaction results in the formation of sticky fibrin,
eye ointment is also used frequently in the postoperative period – which anchors the graft to the scleral bed. The graft is smoothed
both to reduce the risk of infection and to decrease the irritation into position and the edges are co-apted to bring about a secure
caused by the suture ends. adhesion of the graft to the bed and the conjunctival edges.
The process of adhesion usually takes about 45 to 60 seconds,
While the above paradigms can be used to successfully perform which is time enough for the surgical manipulations. The rest
pterygium surgery with conjunctival-limbal autografting, there are of the procedure is as described before.
a few variations to this approach that need mentioning. In conclusion, the pterygium is a common condition in India, which
is part of the “pterygium belt” described by Cameron. While the
• If there is limbal disturbance, apart from the pterygium and traditional bare sclera method of excision is associated with a high
the surgeon is reluctant to disturb the superior limbus, a free recurrence rate and should not be performed today, conjunctival
conjunctival graft can be obtained and used grafting, with its variations, can help treat this condition successfully
in the majority of patients. In this article, tips and tricks that help
• If there is a risk of glaucoma at a later date and the surgeon increase the success rate are described.
does not want to disturb the superior bulbar conjunctiva, then
an inferior conjunctival-limbal graft can be used. Author
Srinivas K. Rao DO, DNB, FRCSEd
• For two-headed pterygia, a conjunctival graft can be performed
for the larger head, and a rotational graft for the smaller head.
In this technique, the conjunctiva over the body of the
pterygium with the smaller head is excised, taking care to
include normal tissue above ad below the body, so that a large
rectangular piece of tissue is obtained. This is placed in saline
and pterygium excision is performed as before. After
hemostasis, the excised conjunctiva is placed, epithelial side
up, with the orientation rotated 180 degrees. The limbal side
of the graft is placed facing the forniceal border of the bed and
vice versa. It is then sutured in position as described previously.

• In the event of extensive recurrence of pterygium, with
multiple previous attempts, and tissue shortage, one can
consider the use of amniotic membrane to help secure the

30 DOS Times - Vol. 14, No.3, September 2008

Cluster Endophthalmitis Retina

Lalit Verma MD, Shaifali Gupta MS, HK Tewari MD, Dinesh Talwar MD, Avnindra Gupta MS

Post-operative endophthalmitis is catastrophic complication of • Immediate treatment
intraocular surgery. Although its reported incidence has
decreased significantly in the present era from 1% to 0.05%, it still • Send samples of conjunctival swabs and if needed of vitreous
remains a dreaded complication for all eye specialists. and anterior chamber also.

Cluster endophthalmitis is a term defined as the occurrence of two • Inform microbiologist to fully subtype the organisms grown.
or more than two infections at a time, or the occurrence of repeated
post- operative infections under similar circumstances, i.e with the • Revaluate operation theatre.
one surgeon, same staff, same operation theatre, same equipments,
etc. These infections usually occur as a result of a breach in standard • Fluids and tubings should also be sent for microbiological
protocol of pre-operative, intraoperative and postoperative care. assessment.
Cluster post-operative endophthalmitis is generally exogenous in
origin. It can be either bacterial or fungal. Red Alert: 2 cases in < or equal to 200 cases, 3 cases in < or equal to
600 cases, 4 cases in < or equal to 800 cases
Risk Factors
• Treat promptly and vigorously all endophthalmitis cases.
• Contamination of water.
• Involve the hospital consultant microbiologist and hospital
• Multiple dose fluids and drugs. infection team at an early stage.

• Defects in sterilization of instruments. • Alert the lead clinician, clinical director and the medical director
and submit a patient safety incident form in line with local
• Contaminated irrigating solutions. reporting procedures. In due course this should trigger a report
to the National Patient Safety Agency (NPSA) and
• Contaminated intra-ocular lenses. the Commission for Healthcare Audit and Inspection
(CHAI) (8).
• Contaminated viscoelastics.
• Consider reporting to the hospital clinical governance team.
• Hospital personal construction activity.
• Give serious consideration to cessation of all intraocular surgery
• Poor operation theatre hygiene. in the interests of patient safety whilst investigating the cause.

Measures to be taken in the event of Cluster endophthalmitis • Ensure colleagues are aware to ensure identification and
reporting of further cases.
Depending on the number of cases,Green / Amber / or Red Alert is
Keep detailed records of all action taken.
sounded and measures suggested include: •

Green Alert: One case of endophthalmitis is noted, one in > or • Document patient/surgical risk factors such as vitreous loss,
equal to 100 cases, or two in > or equal to 600 cases. blepharitis, nasolacrimal disease, immunosuppression,
duration of surgery.
• Review the case. •
• Discuss with colleague(s) Try to identify common hospital factors such as draping and
surgical technique, antibacterial prophylaxis, surgeon, nursing
• Start immediate treatment. staff and other personnel, theatres, solutions, viscoelastics,
intraocular lenses, disposable and non-disposable equipment,
• Make sure operation theatre and intraoperative tubing and which autoclave used, and any changes in procedure or
fluids maintain sterility. environment which may coincide with the outbreak.
Microbiology may reveal a common organism or subtype.
• Proper preoperative and postoperative care is taken. Track batch numbers of solutions, disposables and lenses.
Consider microbiological culture of solutions or viscoelastics.
• Continue operation theatre. Where appropriate, consider taking nasal and skin swabs from
theatre personnel including surgeons. Note on which day of
Amber Alert: One case in 75 cases, 2 cases in 300-500 cases, 3 cases the week, which position on the list and at what time of day
in 700-800 cases. patients were operated.

• Examine the cases • Assess efficacy of cleaning and sterilization processes. Arrange
• Discuss with colleague. for professional assessment of the hospital sterilizing service.
Be alert to signs of failure of this process such as damaged or
Retina Management Group debris laden instruments, and blocked lumens.
Centre for Sight, Safdarjung Enclave, New Delhi

www.dosonline.org 31

(Top Row): Three patients after uneventful intraocular surgery develop Endophthalmitis
(Middle Row): Post Intra vitreal Antibiotic Injection (Day 1)
(Bottom Row): 4 weeks Post Intravitreal Injection

• Confirm that the theatre environment is to a sufficiently high outbreak. Furthermore, an analytical technique new to

standard with regard to cleanliness, air-flow and ergonomics. medicine but widely used in the food industry known as Hazard

Repeat plate tests and airborne particulate matter test as Analysis Critical Control Points (HACCP) and which was

appropriate in consultation with the infection control team. originally developed by a group including NASA to ensure

• Check theatre records to identify cases from other specialties sterility of food used on space missions, may be used to identify
or potentially ‘dirty’ cases which may have been operated in key points in the healthcare process to target corrective action
adjacent sessions. and monitoring.

Carefully screen patients who present with ophthalmic complaints,
• Assess that all equipment and disposables are functional and especially postoperatively, and to educate them about which
used according to manufacturers instructions. Confirm single symptoms to report. Each of these identified risks is squarely
use where instruments are so designated. Ensure correct within physician control and thus can be modified. A Witty (WIT-
procedures are followed for cleaning and sterilization especially D) Approach to Avoiding Mistakes” proposes an easy-to-
of phaco hand pieces.
remember and effective strategy for improving the diagnostic

• Check that reasonable preventive measures are utilized with process. Establish a prioritized differential diagnosis in order to

consideration of the above list. rule out the worst case scenario; determine the information you

need to obtain during the history and examination, or through

• A statistical approach may be necessary, comparing precise studies, to rule that in or out; tell the patient and other health care

procedures, solutions, disposables, lenses and involved providers to ensure that you are notified of all signs and symptoms

personnel between endophthalmitis and non-endophthalmitis that could help establish the diagnosis and determine the treatment

cases in order to pinpoint a possible cause. Other statistical plan; and document your decision-making process and follow-up

methods have been utilized to confirm and investigate an plan.

32 DOS Times - Vol. 14, No.3, September 2008

Deciding when to Postpone or Resume Surgery to find an alternative facility. If you do not have privileges at other
facilities, you will need to refer the patient to an ophthalmologist
Faced with a cluster of either endophthalmitis, the surgical facility who does. Contact the affected patients: “The _____ surgical facility
and the individual surgeon will need to decide whether or not it is is evaluating a potential safety issue. For your protection, your
safe to proceed with other scheduled ophthalmic cases at that surgery will be postponed OR your surgery will need to be done at
location. Patient safety should be the driving factor, and all patients ___________ surgical facility. Since I do not operate at that facility,
must feel confident that the causative factors have been identified would you like for me to refer you or do you have another
and addressed. At times, the surgery center may need the assistance ophthalmologist you would like to see for your surgery?” When
of outside consultants in order to conduct the investigation and cause has been found out and proper sterilization has been ensured
make the decision to cancel or resume procedures. Most elective you can slowly start operating with few cases a day and if everything
cases can be postponed. Patients may be inconvenienced but will goes well you can resume surgeries as were before the outbreak
appreciate that you are working to ensure the best outcome for but with full precautions.
their eye condition. For urgent and emergent ones, you will need

First Author
Lalit Verma MD

www.dosonline.org 33

Diffuse Unilateral Subacute Neuroretintis with Retina
Macular Hole

Subrata Mandal MD, FRCS, Pradeep B Reddy, Naginder Vashisht MD, Zahir Abbas,
Pradeep Venkatesh MD, Rajpal Insan MD, Satpal Garg MD

Diffuse unilateral subacute neuroretinitis (DUSN) is a clinical Figure 2: FFA demonstrates hyperflorescence area
syndrome characterized by visual loss, vitritis, papillitis, retinal corresponding to those clinical
vasculitis, and recurrent crops of evanescent gray-white outer retinal
lesions in early stage and later by progressive visual loss, optic
atrophy, retinal vessel narrowing, and diffuse retinal pigment
epithelium (RPE) degeneration occurring in one eye of otherwise
healthy patients.1-3 Early diagnosis of the disease is important
because destruction of the worm with laser photocoagulation
prevents further loss of visual function and occasionally can be
followed by improvement in vision.4 Herein we report a case where
the larva survived even after laser photocoagulation with high
power and was removed surgically.

Case report

A 12-year-old male presented with complaints of decreased vision
in the right eye for two weeks. The patient also complained of
abdominal discomfort and sore throat followed by vomiting 3 days
before loss of vision and some skin lesion over shoulder region.
The patient had history of worm infestation one year back for
which he was treated by a local physician for deworming.

On ophthalmic examination, best corrected visual acuity (BCVA)
was 3/60 in the affected right eye and 6/6 in the left eye. Anterior
segment evaluation was unremarkable except relative afferent
pupillary defect in the right eye. The intraocular pressures were
normal in both the eyes. There was no heterochromia of iris.
Fundus examination revealed normal disc, and normal retinal
vessels in right eye. No vitritis was noted. A macular hole of more

Figure 1: Showing nematode coming Figure 3: OCT showing macular hole with surrounding
through the macular hole retina detachment

Dr. Rajendra Prasad Centre for Ophthalmic Sciences, than 400ì in size was present associated with surrounding macular
All India Institute of Medical Sciences, New Delhi detachment and subretinal blood. A small nematode of
approximately 3 disc diameters length was found in the macular
www.dosonline.org region with half its body in subretinal space and half in the vitreous
coming through macular hole. (Figure 1) The worm was live and
was moving vigorously. Rest of the retina shows multiple hypo-
pigmented subretinal linear tracts in both centrally and peripherally
suggestive of toxic inflammatory reaction to material left in the
wake of the nematode moving in the subretinal space. Fundus
fluorescein angiography demonstrated these tracts very well as
hyperflurescence area corresponding to those seen clinically.
(Figure 2) Optical coherence tomography was performed and
showed macular hole with surrounding retinal detachment. The

35

Figure 4: Post laser photocoagulation

Figure 5: Post surgery with siliconoil temponade

cross section of larva was seen as hyperreflective area measuring 90 the end of the surgery. Postoperatively BCVA was 3/60 at day one.

microns in size in the centre of the hole. (Figure 3) Media was clear and macular hole was closed. (Figure 5)

On the next day, the larva was found to migrate to the inferonasal In the systemic examination patient was found to have a skin lesion
peripheral subretinal space. Immediately laser photocoagulation for which dermatological consultation was sought for. (Figure 6)
of the larva and surrounding retina was done with 600 mW power He was diagnosed to have cutaneous larva migrans for which he
by laser indirect ophthalmoscope. (Figure 4) However two hours was prescribed ivermectin and albendazole.
after laser, the larva was seen to penetrate the retina and come
partially into the vitreous cavity. The larva was viable and moving Discussion
aggressively. On the next day he was posted for surgical removal
of worm under general anesthesia. Pars plana vitrectomy was In 1950, Wilder observed nematode larvae in the enucleated eyes
done and an attempt was made to remove the worm with active of children, thus establishing ocular larva migrans as an entity;
suction via green tip connected with aspiration tube of vitrectomy these larvae were later identified as Toxocara canis. In 1952, Parsons
probe. However the worm could not be retrieved because photographed and reported intraretinal worm in a 25-year-old
posterior vitreous detachment (PVD) did not take place. Tricort man who had suffered unilateral loss of visual acuity. For eleven
assisted PVD induction was done and during the process of years before 1977 the it was named as ‘unilateral wipe out
completing the vitrectomy the worm was caught in the cutter and syndrome’. It was in 1977, Gass and associates named the syndrome
was vitrectomised. After that thorough examination was done to as diffuse unilateral subacute neuroretinitis (DUSN).The majority
note any iatrogenic breaks or dialysis. A superonasal small dialysis of DUSN have reported to occur in south eastern united states,
was found and cryoed. Silicon oil endotamponade was given at the carribean islands and Brazil. Pathogenic organisms for the
DUSN are Toxocara canis and Ancylostoma caninum in case of

36 DOS Times - Vol. 14, No.3, September 2008

Observation and destruction of the nematode is the only methods
of accurately reaching a diagnosis and treating the patients with
DUSN. Laser photocoagulation is an effective means of treatment.
However as the nematode is detected in only 25% of cases, when
the nematode cannot be found oral thiabendazole and ivermectin
can be given according to Gass and his associates. Medical treatment
may be useful when there is abundant inflammatory reaction which
obscures the retina and makes the detection of the organism
difficult, but allows ready entry of the drugs. Gass extracted one
nematode transclerally after killing with cryotherapy. Eduardo cunha
de Souza and Yoshitaka Nakashima reported a case where the
organism travitreally. Larva escaping the laser may also be an
indication for surgery

Figure 6: Skin lesion showing cutaneous References
larva migrans
1. Gass JDM, Gilbert WR Jr, Guerry RK, Scelfo R. Diffuse unilateral
subacute neuroretinitis. Ophthalmology. 1978;85:521-545.

2. Gass JDM, Braunstein RA. Further observations concerning the diffuse

unilateral subacute neuroretinitis syndrome. Arch Ophthalmol.

smaller nematodes and Baylisascaris procyonis in case of larger 1983;101:1689-1697.

nematode. Reports of DUSN from India are sparse. 3. Cunha de Souza E, Lustosa da Cunha S, Gass JDM. Diffuse unilateral

Clinical features of this syndrome include insidious, usually severe subacute neuroretinitis in South America. Arch Ophthalmol.
loss of peripheral and central vision, vitritis, diffuse and focal pigment 1992;110:1261-1263.

epithelial derangement with relative sparing of the macula, 4. Raymond LA, Gutierrez Y, Strang LE, et al. Living retinal nematode

narrowing of retinal vessels, optic atrophy, increases retinal (filarial-like) destroyed with photocoagulation. Ophthalmology.

circulation time and subnormal electroretinographic findings. 1978;85:944-949.

Macular hole has not been reported in DUSN till now.

First Author
Subrata Mandal MD, FRCS

www.dosonline.org 37

Choroidal Metastases Retina

Sandeep Saxena MS, MAMS, Ramit A.K. Rastogi MBBS, Kanwaldeep Singh MBBS,
Kuldeep Vishwkarma MBBS, *Sanjiv Hansraj MS, Dipak Kumar MS

Historically, metastatic carcinoma to the eye, particularly to metastasis, can precede the diagnosis of the primary malignancy.
the choroid, was considered a rare event. Perls1 reported the In a study by Ferry and Font9 46% of the patients had tumor-
first case of choroidal metastasis in 1872, and Lemoine and McLeod2 related symptoms that preceded the detection of the primary
reported only 230 cases in the literature in their 1936 review. In a neoplasm. In a study by Shields et al11 34% of patients had no
survey of 8,712 patients with malignancies, Godtfredsen3 reported history of cancer at the time of ocular diagnosis. In both the Ferry
only six patients (0.07%) with choroidal metastasis. Over the last 30 study9 and the Shields study,11 lung cancer was the most common
years, however, a number of reports have appeared noting a much primary tumor detected in patients with no neoplasm at the time
higher incidence of metastatic disease to the eye.4-8 Bloch and of ocular diagnosis (35% and 41%, respectively, in these studies).
Gartner4 performed postmortem examinations on 230 patients There was also a significant percentage of patients in whom no
who died of systemic carcinoma and found 28 patients (12%) with primary tumor was ever detected (51% and 39%, respectively, of
metastatic tumor in the eye and/or orbit4 Given the incidence and the patient. As seen in Table 2, breast and lung cancers are the most
consequence (ie, blindness) associated with an untreated ocular commonly detected malignancies to metastasize to the eye. Mewis
metastasis, all ophthalmologists and oncologists should be familiar and Young12 analyzed 250 patients with known breast carcinoma.
with this entity. At the time of evaluation, 152 patients were referred secondary to
ocular symptoms, and 98 were asymptomatic. In the asymptomatic
Epidemiology group, nine patients (9.2%) were found to have metastatic disease.
Taken as a whole, ocular metastasis is a major clinical oncologic
The frequency of choroidal metastasis in patients with cancer is problem. Eliassi-Rad et al16 estimated that in 1993, 66,262 (12.6%)
estimated to be approximately 2% to 7%.4-7 If all intraocular of 526,000 patients who died of their cancer would have ocular
metastases are considered, this number rises to approximately 12%.4- metastasis.
7 Intraocular metastasis is now considered the most common
malignancy of the eye8 Ocular metastasis, and particularly choroidal Pathogenesis/Site of Intraocular Metastasis

Table 1: Prevalence of Ocular Metastasis in Patients Dying of While any portion of the eye can be involved by metastatic disease,
Cancer4-6 or Patients with Generalized Malignancy7 the most common tissue involved is the highly vascular choroid.4-12
There is no good explanation as to why the eye, particularly the
Primary Site Bloch and Nelson Eliassi- Albert7 choroid, is a common site for metastasis. Ferry and Font9
speculated that the distribution of tumors within the choroid may
Gartner4* et al5 Rad et al6 be related to its vascularity characteristics. Also, as previously noted,
there are significant differences between primary tumors and their
Breast 37% 9.7% 8.3% 13.5% incidence of ocular metastasis. The reasons for these differences

Lung 6% 6.7% 6.1% 4.0%

Colorectal 3% 4.2% 0.0% -

Prostate 11% 0.0% 4.2% 12.5% Table 2: Patients who Present with Ocular Metastases:
Primary Sites
Uterus/cervix 25% - 5.0% -

Skin - 2.0% 14.3% - Ferry Freedman Shields

and Font9 and Folk10 et al11

Thyroid 11% - - - Breast 40.0% 49.0% 47%

Kidney 11% - - - Lung 30.0% 14.0% 21%

Leukemia - 28.8% 34.8% - Gastrointestinal - - 4%

Lymphoma - 6.7% 23.3% -

Multiple - - 29.0% - Kidney 4.0% - 2%
myeloma
Skin -- 2%

Sarcoma - 0.0% 16.0% - Prostate 1.3% 3.6% 2%

- = not stated Unknown 18.3% 8.0% 17%
* = includes four cases of orbital metastasis

Cutaneous melanoma - 4.5% -

Department of Ophthalmology Other 6.4% 20.9% 5%
C.S.M. Medical University, Lucknow (U.P.) - = not stated

*Mansarovar Eye Hospital, Lucknow

www.dosonline.org 39

Figure 1: Colour fundus photograph Figure 2: Colour fundus photograph shows
shows choroidal mass with choroidal mass with associated serous retinal
detachment and retinal pigment epithelium
associated serous retinal detachment
in choroidal metastasis alterations in choroidal metastasis from
from carcinoma breast. carcinoma breast

Figure 3: Colour fundus photograph shows a Figure 4: Colour fundus photograph
large choroidal mass with associated leopard shows a large elevated choroidal
mass with associated retinal
skin pigmentation and retinal pigment
epithelium alterations in choroidal metastasis hemorrhage in choroidal metastasis
from carcinoma lung
from carcinoma lung

are also unexplained, but Ferry and Font suggest that the high Presentation, Diagnosis, and Workup
incidence of breast cancer metastasis may be related to the longer
life expectancy of breast cancer patients with metastasis, thus Majority of symptomatic patients note a decreased visual acuity at
providing a longer time for intraocular metastasis to develop. the time of presentation.9,11 Other presenting signs or symptoms
include diplopia, photophobia, ptosis, blepharitis, metamorphopsia,
Shields et al11 surveyed 420 consecutive patients with uveal pain, flashes and floaters, mass lesion, uveitis, exophthalmos,
metastases. The tumors were unilateral in 320 patients and bilateral secondary glaucoma, and detached retina.9,11 Most choroidal
in 100 patients. This proportion of bilateral cases is considerably metastases occur posterior to the equator of the fundus in the
more than the 4.4% noted by Ferry and Font. The study by Mewis macular or paramacular regions.
and Young12 of breast cancer patients noted a 31% incidence of
bilaterality. In both the Ferry study and the Mewis study, the Presence of metastatic disease to the eye is obviously high in the
incidence of subsequent bilaterality was notable (17.6% and 15%, differential diagnosis of ocular lesions when a primary cancer is
respectively). There seems to be no predilection for metastasis to present elsewhere. Other conditions can be mistaken for metastatic
preferentially affect the right or left eye.6,11 In each affected eye, disease13; therefore, a careful evaluation is necessary for a correct
more than one metastasis may be noted. Shields et al11 reported diagnosis. The differential diagnosis includes amelanotic nevus,
multiple foci in 20% of patients with choroidal metastasis. The mean amelanotic melanoma, choroidal hemangioma, posterior scleritis,
number of uveal metastasis noted per eye was 2.0, and the choroidal osteoma, retinitis, hemorrhage, choroiditis,
maximum number noted was 13. rhegmatogenous retinal detachment, reactive lymphoid

40 DOS Times - Vol. 14, No.3, September 2008

Figure 5a: Fluorescein fundus angiography of Figure 5b: Fluorescein fundus angiography
choroidal metastasis from carcinoma breast shows increased fluorescence and staining of
choroidal metastasis from carcinoma breast
shows fluorescence

Figure 6a: Fluorescein fundus angiography of Figure 6b: Fluorescein fundus angiography
choroidal metastasis from carcinoma lung shows shows staining of choroidal metastasis
from carcinoma lung
early fluorescence
Figure 7: B scan ultrasound of
hyperplasia, lymphoma, Harada’s disease, uveal effusion syndrome, choroidal metastasis shows
and central serous chorioretinopathy.8.13
acoustically solid convex mass.
Diagnosis of ocular metastases is based primarily on clinical findings
supplemented by imaging studies. 41

Fundus Examination: The choroidal metastases usually appear as
solid, flat, plaque-like, mottled, yellow-brown lesions with associated
serous retinal detachment.14 (Figure 1&2) show choroidal
metastases from Carcinoma Breast, whereas, (Figure 3&4) show
choroidal metastases from Carcinoma Lung. Retinal pigment
epithelial hyperplasia overlying the metastatic tumor (leopard skin
pigmentation), choroidal detachment and rarely a mushroom shape
may also occur with choroidal metastases. A number of ancillary
ophthalmologic procedures which include ultrasonography,
fluorescein angiography, computed tomography/magnetic
resonance imaging, fine-needle aspiration, or wedge biopsy.8 can
assist in the diagnosis of metastatic tumors

Fluorescein Angiography: Fluorescein angiography of metastatic

www.dosonline.org

Figure 8a: Spectral – Domain Optical Figure 8b: Spectral – Domain Figure 8c: Spectral – Domain Optical
coherence tomography on raster scan Optical coherence tomography coherence tomography retinal pigment
shows serous retinal detachment with
retinal pigment epithelium excrescences retinal thickness map shows epithelium deformation map shows
elevated retinal layers elevated retinal pigment epithelium

choroidal tumors has diagnostic value. The most common thickness and retinal pigment epithelium deformation(Figure 8 &
angiographic finding in metastatic choroidal tumors noted by David 9). Resolution of subretinal fluid can be documented following
and Robertson15 was fluorescence that appeared in the early therapy.
arteriolar or arteriovenous phase with progressive and more intense
staining in the late phase (Figure 5 & 6) Occasionally, biopsies of intraocular lesions are needed to ascertain
the diagnosis.17Fine-needle aspiration or, more rarely, a wedge
Ultrasonography: On B-scan ultrasound, metastatic tumors tend biopsy can be obtained.8,17
to be acoustically solid convex masses with a lower silhouette, ie,
lower height-to-base ratios than malignant melanoma(Figure 7).16 Computed tomography and magnetic resonance imaging have a
A-scan ultrasound shows moderate internal reflectivity compared limited role in the diagnosis of ocular metastasis8 . Nevertheless,
with melanoma, which is usually low. brain imaging is useful before initiation of radiotherapy to assist in
treatment planning. This is especially important when treating
Spectral - Domain Optical Coherence Tomography: Optical patients with breast cancer. Mewis and Young12 reported that 22%
coherence tomography can depict overlying subretinal fluid , retinal of patients diagnosed with choroidal metastasis had a concurrent
pigment epithelial hyperplasia and retinal pigment epithelium diagnosis of central nervous system metastasis. An additional 19%
detachment . Optical coherence tomography with 3D imaging shows of patients had a subsequent diagnosis. When concurrent brain
a poorly imaged/ well defined lesion with altered retinal metastasis is diagnosed, the radiation technique is usually altered
to include the entire cranial contents.

Figure 9a: Spectral – Domain Optical Figure 9b: Spectral – Domain Optical coherence
coherence tomography retinal thickness tomography retinal pigment epithelium deformation

map shows elevated retinal layers. map shows marked retinal pigment epithelium
deformation due to underlying choroidal mass.
42
DOS Times - Vol. 14, No.3, September 2008

Management 8. Shields JA, Shields CL. Intraocular Tumors: A Text and Atlas. 4th ed.
Philadelphia, Pa: WB Saunders Co; 1992:208-238.
Numerous options are available for the therapy of ocular
metastasis, including observation, chemotherapy, photocoagulation, 9. Ferry AP, Font RL. Carcinoma metastatic to the eye and orbit. I: A
cryosurgery, surgical resection, or radiotherapy. The specific therapy clinicopathologic study of 227 cases. Arch Ophthalmol. 1974;92:276-
chosen for a patient is an individualized process that considers the 286.
clinical condition of the patient. For example, a patient with an
asymptomatic metastasis who is near death probably does not 10. Freedman MI, Folk JC. Metastatic tumors to the eye and orbit: patient
require therapy. On the other hand, a symptomatic patient with survival and clinical characteristics. Arch Ophthalmol. 1987; 105:1215-
controlled systemic disease should receive therapy to prevent further 1219.
deterioration in vision. The most commonly applied treatment is
external beam radiotherapy. 11. Shields CL, Shields JA, Gross NE, et al. Survey of 520 eyes with uveal
metastases. Ophthalmology. 1997;104:1265-1276.
References
12. Mewis L, Young SE. Breast carcinoma metastatic to the choroid:
1. Perls M. Contributions to pathology of tumors. Virchows Arch Pathol analysis of 67 patients. Ophthalmology. 1982;89:147-151.
Anat. 1872;56:437.
13. Michelson JB, Stephens RF, Shields JA. Clinical conditions mistaken
2. Lemoine AN, McLeod J. Bilateral metastatic carcinoma of the choroid. for metastatic cancer to the choroid. Ann Ophthalmol. 1979;11:149-
Arch Ophthalmol. 1936;15:804-821. 153.

3. Godtfredsen E. On the frequency of secondary carcinomas in the 14. Merrill CF, Kaufman DI, Dimitrov NV. Breast cancer metastatic to
choroid. Acta Ophthalmol. 1944;22:394-400. the eye is a common entity. Cancer. 1991;68:623-627.

4. Bloch RS, Gartner S. The incidence of ocular metastatic carcinoma. 15. David DL, Robertson DM. Fluorescein angiography of metastatic
Arch Ophthalmol. 1971;85:673-675. choroidal tumors. Arch Ophthalmol. 1973;89:97-99

5. Nelson CC, Hertzberg BS, Klintworth GD. A histopathologic study 16. Coleman DJ, Abramson DH, Jack RL, et al. Ultrasonic diagnosis of
of 716 unselected eyes in patients with cancer at the time of death. tumors of the choroid. Arch Ophthalmol. 1974;91:344-354. .
Am J Ophthalmol. 1983;95:788-793.
17. Foulds WS, Lee UL, Roxburgh ST. Can chorio-retinal biopsy be
6. Eliassi-Rad B, Albert DM, Green WR. Frequency of ocular metastases justified? Trans Ophthalmol Soc U K. 1985;104:864-868.
in patients dying of cancer in eye bank populations. Br J Ophthalmol.
1996;80:125-128. 18. Brady LW, Shields JA, Augsburger JJ, et al. Malignant intraocular
tumors. Cancer. 1982;49:578-585.
7. Albert DM. Tumor metastasis to the eye: tumor incidence in 213
adult patients with generalized malignancy. Am J Ophthalmol. 19. Chu FCH, Huh SH, Nisce LZ, et al. Radiation therapy of choroid
1967;63:723-726. metastasis from breast cancer. Int J Radiat Oncol Biol Phys. 1977;2:
273-279.

20. Dobrowsky W. Treatment of choroid metastases. Br J Radiol.
1988;61:140-142.

First Author
Sandeep Saxena MS, MAMS

www.dosonline.org 43

Optical Coherence Tomography in Children: Pediatric Ophthalmology
A Feasible Option

Monica Gandhi MS, Suneeta Dubey MS, Julie Pegu MS

It is not uncommon to see children in our clinics in whom we The limitations are that there is not much information available in
suspect glaucoma. It could be because they are disc suspects or age group less than 18. In Stratus OCT the normative database is
they have a family history of glaucoma. To further evaluate the available for age more than 18 years. (Figure 1)
optic nerve changes in glaucoma can be challenging, as visual field
analysis in children is usually unreliable.1 Here in is the role of The RNFL thickness varies with race also. 12
optical coherence tomography (OCT) because it is not subjective
and only requires a little concentration from the children. Does RNFL thickness change with pathology?

The principles of OCT have been published in the literature.2 The There are various optic neuropathies, which can alter the RNFL
Stratus OCT uses low-coherence interferometry to assess thickness. RNFL thickness and macular thickness in children has
peripapillary RNFL thickness. This instrument quantifies RNFL been evaluated and it has been found that there is a correlation
thickness by measuring the difference in temporal delay of back- with glaucoma, 23.3% decrease in RNFL thickness and 6.6% decrease
scattered light from the RNFL and a reference mirror. RNFL is in macular thickness. The RNFL thickness had been shown to have
differentiated from other retinal layers with an algorithm that a greater diagnostic capacity compared to macular thickness. 13
detects the anterior edge of retinal pigment epithelium and
determines the photoreceptor layer position. What is the normal RNFL thickness in children?

The Fast RNFL thickness protocol combines 3 RNFL thickness (3.4) There is considerable variation in global RNFL thickness among
circle scans into one scan. This protocol acquires three 3.4 mm children. Salchow et al (14) reported average global RNFL thickness
diameter circle scans in 1.92 seconds. Each resulting image consists ranging from 69.6 to 144.5 um, with a mean of 107 +/- 11.1 um. In
of RNFL thickness measurements (in microns) along a 360° circular a study by Bourne et al RNFL thickness varied from 44 to 124 um,
(ring) path (one thickness value per 3.6°) around the optic disc. although it included subjects who were normal and also with
glaucoma. Other investigators found a range of 42 to 157 um in
Three circular scans of 3.4 mm diameter centered on the optic disc normal subjects, but this study also included subject’s upto 79 years
judged to be of acceptable quality are obtained for each test eye. of age and it was done using the earlier models of OCT.
Mean RNFL thickness for quadrant is calculated from the three
images obtained. We studied the RNFL thickness in 100 normal participants between
8-17 years. These children had intraocular pressure below 22mmHg
Why RNFL? and normal optic discs. They had no family history of glaucoma.

The RNFL thickness is known to decrease in various optic nerve The refractive status ranged from -5 D sph to + 5 D sph.
pathologies3, 4, 5 and can be potentially documented earlier than
achromatic standard perimetry.6, 7 We found that the mean Global RNFL thickness was 103.11 ± 9.72
ìm (range 80.4-126.3 ìm). The RNFL was thickest superiorly, followed
OCT has been documented in normal adults and has been used as by inferior quadrant, thinner nasally and thinnest in the temporal
an adjunct in diagnosis and follow up of glaucoma in adults. 3,4,8,9,10 quadrant. The difference between the superior and the inferior
In a study by R. Parikh et al (10) the OCT in early glaucoma in Asian quadrant was statistically significant (P = 0.024). (Figure 2)
eyes was studied and suggested that OCT can have a role in screening
of glaucoma. The comparisons of RNFL thickness in varying We also documented the time taken and the technical feasibility of
severities of glaucoma have been studied and there is a decrease in obtaining good quality scans. It was noted that most of the children
the thickness as the pathology advances.3,4 Several investigators were cooperative for the scans and the average time taken was 2.5
have provided estimates of the sensitivityand specificity for detecting min with a range of 1-6mins. This is much less time compared to
glaucoma with imaging instruments including OCT. 11 the other tests like visual fields examination. Also the feasibility was
97%. The scan could be obtained with ease in 76% and with some
Is RNFL thickness different in children? difficulty in 21%. The scan was not possible in 3%. These subjects
were apprehensive or were inattentive to fixate at the internal
There is documentation that the RNFL thickness varies with the fixation.
age with 2 um decrease per decade. The studies show inverse relation
with age. 12 The data in this study can help identify variation in the RNFL
thickness in the other children in an attempt to help as an adjunct to
Department of Ophthalmology diagnosis of glaucoma and other optic neuropathies and their
Dr Shroff’s Charity Eye Hospital, follow up.
Darya Ganj, New Delhi
OCT-3 may prove to be a valuable tool in diagnosing glaucoma in
the pediatric population, and may be adjunctive to optic nerve

www.dosonline.org 45

Figure 1: Fast RNFL thickness average in a 14 year old. Note that normative data for less than
18 years is not available so comparison with age matched normals not possible

Figure 2: RNFL thickness in normal children as a function examination and photography. We hope that this technology will
of peripapillary location (n = 97 eyes). Peripapillary be additionally useful in evaluating children who are glaucoma
location is given in clock hours (3 - nasal, 6- inferior, suspects, based either upon their optic nerve or other clinical
9- temporal, 12 - Superior). OE, mean; error bars, 1 parameters.
standard deviation above and below mean
References

1. Natalie C. Kerr, David W. Litchford Jr, Qunming Dong
Peter A. Netland. Reliability and artifact in automated visual field
testing of children. Annuls of ophthalmology. Sept 2005 Vol 37(3)

2. Huang D, Swanson EA, Lin CP, et al. Optical coherence tomography.
Science. 1991; 254:1178-1181

3. Sihota R, Sony P, Gupta V, Dada T, Singh R. Diagnostic capability of
optical coherence tomography in evaluating the degree of
glaucomatous retinal nerve fiber damage. Invest. Ophthalmol Vis Sci
2006 May 47(5): 2006-10

4. Christopher Bowd; Robert N. Weinreb; Julia M. Williams; Linda M.

46 DOS Times - Vol. 14, No.3, September 2008

Zangwill. The Retinal Nerve Fiber Layer Thickness in Ocular J. Ganesh Babu, Ravi Thomas. Normal Age-Related Decay of Retinal
Hypertensive, Normal, and Glaucomatous Eyes with Optical Nerve Fiber Layer Thickness.Ophthalmology. May 2007; Vol. 114(5):
Coherence Tomography. Arch Ophthalmol, Jan 2000; 118: 22 - 26. 921-926

5. Barboni P, Savini G, Valentino ML, et al. Retinal nerve fiber layer 11. 14. Shan C. Lin, Kuldev Singh, Henry D. Jampel, Elizabeth
evaluation by optical coherence tomography in Leber’s hereditary A. Hodapp. Optic Nerve Head and Retinal Nerve Fiber Layer Analysis:
optic neuropathy. Ophthalmology 2005; 112:120–6 A Report by the American Academy of Ophthalmology.
Ophthalmology October 2007; Vol. 114 (10): 1937-1949
6. Quigley HA, Addicks EM, Green WR. Optic nerve damage in human
glaucoma. III. Quantitative correlation of nerve fiber loss and visual 12. Poinoosawmy D, Fontana L, Wu JX, Fitzke FW, Hitchings RA.
field defects in glaucoma, ischemic optic neuropathy, papilledema, Variation of nerve fiber layer thickness measurements with age and
and toxic optic neuropathy. Arch Ophthalmol.1982; 100:135-146. ethnicity by scanning laser polarimetry. Br J Ophthalmol 1997;
81:350-54
7. Quigley HA, Dunkelberger GR, Green WR. Retinal ganglion cell
atrophy correlated with automated perimetry in human eyes with 13. Hess DB, Asrani SG, Bhide MG, et al. Macular and retinal nerve fiber
glaucoma. Am J Ophthalmol 1989; 107:453– 64. layer analysis of normal and glaucomatous eyes in children using
optical coherence tomography. Am J Ophthalmol 2005; 139:509 –
8. Sony P, Sihota R, Tewari HK, Venkatesh P, Singh R. Quantification of 17.
the retinal nerve fibre layer thickness in normal Indian eyes with
optical coherence tomography. Indian J Ophthalmol 2004; 52:303-9 14. Daniel J. Salchow, Yuri S. Oleynikov, Michael F. Chiang, Shana
E. Kennedy-Salchow, Kevin Langton, James C. Tsai, Lama A. Al-
9. Ramakrishnan R, Mittal S, Ambatkar S, Kader MA. Retinal nerve Aswad. Retinal Nerve Fiber Layer Thickness in Normal Children
fibre layer thickness measurements in normal Indian population by Measured with Optical Coherence Tomography. Ophthalmology.May
optical coherence tomography. Indian J Ophthalmol 2006; 54:11-5 2006 Vol. 113, Issue 5, Pages 786-791

10. Rajul S. Parikh, Shefali R. Parikh, G. Chandra Sekhar, S. Prabakaran,

First Author
Monica Gandhi MS

www.dosonline.org 47

Peadiatric Ophthalmology Pediatric Orbital Tumours

Ramesh Murthy MD FRCS, Santosh G Honavar MD FACS, Geeta K Vemuganti MD DNB

Orbital tumours are uncommon when compared to tumors Lacrimal gland tumours
involving other parts of the body. The management is often • Adenoid cystic carcinoma
complex and includes a wide range of diagnostic and therapeutic • Pleomorphic adenoma
options. There have been a number of studies of the incidence of • Pleomorphic adenocarcinoma
various orbital tumours in children.1-3 Most reports have shown a Histiocytic and Fibrocytic tumours
high incidence of cystic and vasculogenic tumours.1-3 The incidence • Benign fibrous histiocytoma
of malignant tumours has been reported to range from 24% to
46%,3 which is however higher in our population (53%) due to
orbital spread of retinoblastoma.4

Classification of Common Orbital tumours in children • Histiocytosis X

Cystic tumours Osseous tumours
• Dermoid cysts • Aneurysmal bone cyst
• Microphthalmos with cyst • Ossifying fibroma
• Meningocoele and encephalocoeles • Fibrous dysplasia
• Dacryocystocoele Metastatic tumours
• Dacryops • Neuroblastoma
• Teratomas • Wilms’ tumour
• Parasitic cysts • Ewing’s sarcoma
Vasculogenic tumours Miscellaneous tumours
• Capillary hemangioma

• Lymphangioma • Primitive neuroectodermal tumours

• Cavernous hemangioma • Ewing’s sarcoma

• Vascular malformations • Alveolar soft part sarcoma

Myogenic tumours • Dermolipoma
• Rhabdomyosarcoma
Optic nerve and Meningeal tumours The more commonly encountered benign lesions include dermoids,
• Optic nerve glioma dermolipomas, neurofibromas, vascular lesions like capillary
• Meningioma hemangiomas, cavernous hemangiomas, lymphangioma, histiocytic
Peripheral nerve tumours lesions like Langerhans cells histiocytosis, fibroosseous lesions like
• Neurofibroma aneurysmal bone cyst, fibrous dysplasia, ossifying fibroma, optic
• Neurilemmoma nerve gliomas, neural tumours like neurofibroma, encephalocoele
Lymphoid and leukemic tumours and cystic lesions like microphthalmos with cyst. Common
• Burkitts lymphoma malignant tumours include rhabdomyosarcoma, Burkitt’s
• Leukemia lymphoma, leukemia and adenoid cystic carcinoma of the lacrimal
Secondary tumours gland.
• Retinoblastoma
• Squamous cell carcinoma Cystic tumours

L.V. Prasad Eye Institute Dermoid cysts
Hyderabad, India
Dermoids (Figure 1a) account for 25% of all lesions in the orbit.5
Orbital dermoids are cystic lesions, while conjunctival or corneal
dermoids are solid. These result from the entrapment of epithelial
structures at the site of closure of fetal fissures. They can be
superficial or deep. Superficial dermoids lie anterior to the orbital
septum or in the anterior orbit. Deep dermoids are rounded,
encapsulated tumours with keratin, fatty materials and dermal
structures such as hair. These are unilateral without any sex

50 DOS Times - Vol. 14, No.3, September 2008

rupture of the cyst wall. The cyst enlarges due to increase in
secretions in the lumen, which causes distension of the cyst wall
and focal thinning with chances of rupture and leak leading to orbital
inflammation. In addition as the child grows the dermoid is prone
(1a) to traumatic rupture. Superficial dermoids can be excised by a lid
crease approach with blunt dissection. Deep dermoids need removal
if they are causing any functional deficit. Every effort should be
made to remove the tumour with an intact capsule, however if
closely related to vital structures, the wall may be excised and the
contents aspirated without spillage, following which removal is
easier.

Microphthalmos with cyst

This condition is associated with neuronal migration syndromes
like Walker-Warburg syndrome, Aicardi syndrome and other
(1b) craniofacial malformations like agenesis of the corpus callosum.
The orbit may not develop fully due to the abnormal eye. These
are usually unilateral with superior globe displacement (Figure
2a&2b). The eyeball is usually microphthamic or anophthalmic. CT
scan is essential to identify any associated bony abnormalities. The
cysts can be treated by ethanolamine oleate sclerotherapy or
excision.9

Meningocoeles and Encephalocoeles

(1c) These result from the herniation of maldeveloped tissues of the
central nervous system including the meninges (meningocoele),

Figure 1a: Dermoid cyst in a 30 year old lady who Figure 2a: Orbitopalpebral cyst in a 1 year old
complained of a small mass over the temporal girl. There was a large cyst in the inferior orbit
with a microphthalmic right eye. Recurrence
portion of the right brow present since childhood, occurred following cyst aspiration, complete
which had been gradually increasing in size over collapse of the cyst occurred after injection of
the last 1 year. Figure 1b: CT scan coronal view,
revealed the presence of a large cystic lesion in the ethanolamine oleate – a sclerosing agent.

superotemporal orbit of the right eye. Figure 2b: Ultrasound Bscan shows the
Figure 1c: CT scan axial view, showed this large presence of the large anterior cyst with

cystic lesion in the right superior orbit with a microphthalmic eye behind it.
corresponding fossa formation of the bone.
51
predilection. These are present from birth, but are innocuous and
are accidentally discovered. 6 They can present with orbital
inflammation due to foreign body inflammation following leakage
of the keratin in the cyst. Deep dermoids present in the second or
third decade of life usually with slowly progressive proptosis. They
may cause globe displacement and consequent diplopia. CT scan
(Figure 1b&1c) shows a well circumscribed cystic mass with thin
walls and hypodensity within, due to the presence of lipid within
the lumen. MRI shows a well circumscribed homogeneous lesion,
hypointense to extraocular muscles in T1-weighted images; the
fatty contents are best seen by diffusion-weighted imaging. Small
dermoids can be observed till the second or third decade of life.
Surgical excision is done before the cyst causes pressure on the
eyelid and orbital structures. During excision care is taken to avoid

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Figure 3a: Capillary hemangioma of the Figure 3b: Regression of capillary hemangioma
left upper lid and the orbit in an infant of the orbit and upper lid following injection of

at presentation, age 4 months intralesional steroids, age 7 months.

Figure 3c: Further regression following Figure 3d: Near total regression
the second dose of intralesional triamcinolone, of the lesion at

age 10 months the age of 14 months

brain tissue (encephalocoele) or both (meningoencephalocoele). excision can be difficult in massive tumours and with extension into
Common sites of development include the suture lines of orbital the cranium, craniotomy is indicated. Recurrence can occur with
bones or the orbital fissures and optic canal. These are more malignant transformation in incompletely excised lesions.
common in children with congenital craniofacial cleft syndromes.
Anterior meningoencephalocoele commonly presents as a bluish Parasitic cysts
lesion in the medial canthal area due to herniation between the
frontal and lacrimal bones.8 Posterior meningoencephalocoeles These are rare in the orbit, though cysticercosis is a fairly common
present with slowly progressive proptosis and globe displacement. condition seen in our population. Cysticercosis is caused by the
Pulsating proptosis may also be present. CT scan demonstrates a larval form of the cestode, Taenia solium. This usually presents
well-circumscribed cystic mass with a bony defect. Surgical excision with acute inflammation or motility disturbances. Ultrasound B
needs to be performed in conjunction with a neurosurgeon. scan shows a cystic lesion with a high reflective spot, scolex within.
Management is usually medical with a combination of albendazole
Dacryops and systemic steroids. 11 Hydatid cyst is caused by the larvae of the
tapeworm of the genus Echinococcus. The definitive host is the dog
These are bluish transilluminant cysts visible through the and humans are the accidental hosts. Presentation is usually with
conjunctiva. These form due to obstruction of the ducts of the proptosis, oculomotor palsies and optic disc edema. Management
lacrimal glands, affecting the palpebral lobe more often than the is surgical, with a lateral orbitotomy for deeply located lesions.12
orbital lobe. Complete surgical excision of the cyst is curative. 10
Vasculogenic tumours
Teratomas
Capillary hemangioma
This germ cell tumour contains tissues derived from the endoderm,
ectoderm and mesoderm. These lesions contain skin, bowel, lung, This is the most common vascular orbital tumour in children. This
brain, thyroid, cartilage and bone tissue. They are usually unilateral accounts for nearly 0.7 to 2.2 % of biopsy proven tumours in the
and have a female predilection. Mostly benign, malignant orbit. Presentation is usually at birth or in the first 8 weeks (Figure
transformation is also known to occur. Growth occurs in the first 3a). Females are more commonly affected. They are seen as red,
6 -12 months due to collection of secretions from the different multilobulated cutaneous lesions in most cases, as they are more
tissues into the partially cystic spaces of the tumour.7 Surgical common in the anterior orbit and usually cause astigmatism and
myopic refractive error. For the first few months the tumour

52 DOS Times - Vol. 14, No.3, September 2008

enlarges, then slowly regression starts which is completed by 7 Figure 4a: Lymphangioma in a 12 year old girl,
years of age in nearly 70% of cases. Visceral hemagiomas can also with right eye proptosis and lateral globe
coexist and entrapment of platelets in the tumour can lead to displacement
thrombocytopenia with extensive hemorrhage, Kasabach-Merritt
syndrome.13 CT reveals a well-circumscribed lesion with a
heterogeneous internal structure. On MRI it is isointense to
hyperintense on T1 and hyperintense on T2-weighted images with
respect to extraocular muscle with marked enhancement
after contrast injection. Signal void areas are characteristic of this
tumour. Treatment consists of observation, intralesional
corticosteroids, systemic corticosteroids, systemic interferon and
surgical resection. Intralesional corticosteroids consist of a
combination of a long acting and a short acting steroid,
triamcinolone and betamethasone. Injection should be performed
with care to avoid the risk of intraarterial embolization. Involution
occurs rapidly within 6 to 8 weeks with the tumour shrinking to less
than 20 % the original volume (Figure 3b,3c&3d). Complications
include subcutaneous fat atrophy, eyelid necrosis and retinal artery
occlusion. Oral prednisolone can be given in a dose of 2 to 3 mg/Kg/
day for 4-6 weeks with a slow taper. Interferon alfa 2a and 2b can be
given in a dose of 1 to 3 million U/day. Surgical resection can be
used for anterior lesions unresponsive to intralesional
corticosteroids. Embolization can be performed alone or preceding
surgical excision. Intralesional Nd: YAG laser photocoagulation has
also been tried.

Lymphangioma

This tumour is most commonly seen in children less than 10 years Figure 4b: CT scan axial view revealed the
of age. Subcutaneous involvement results in a bluish hue of presence of a diffuse soft tissue mass lesion filling
the skin. The tumor is ill-defined and composed of ectatic lymph
channels. These do not regress with time but become the entire orbit pushing the globe anteriorly
more encapsulated. Clinically this presents with slowly progressive
proptosis, ptosis and motility restriction (Figure 4a). Figure 4c: CT scan coronal view revealed the
Spontaneous hemorrhage can occur and lead to ecchymosis, presence of a heterogeneous soft tissue mass filling
subconjunctival hemorrhage and acute proptosis. Proptosis can
increase following trauma or upper respiratory tract infection. the entire orbit
Hemorrhage can lead to the formation of chocolate cysts in the
tumour channels. Similar lesions may be present in the skin and CT scan shows a well-circumscribed mass usually in the muscle
soft palate.14 These can also be associated with arteriovenous cone with minimal contrast enhancement. MRI shows an oval mass
malformation and orbital cellulitis. Ultrasonography reveals large, with hypointensity to orbital fat on T1-weighted images. Small
compressible irregular cystic spaces. CT shows an irregular, tumours can be kept under observation. When cosmetically
multicystic lesion (Figure 4b&4c). MRI shows hypointensity on T1 unacceptable proptosis is present or the optic nerve is compromised,
and T2- weighted images (due to deoxyhemoglobin) which complete surgical removal is performed aided by a cryoprobe.
gradually changes to hyperintensity due to lysis of red blood cells Orbital varix
with release of methhemoglobin. T2 weighted images may show Orbital varix develops due to weakening of the vein wall followed
fluid levels. Surgical intervention is avoided for small, asymptomatic by stagnation of blood flow. This results in thrombosis and
lesions. Surgery is required for cosmesis, corneal exposure or optic
nerve dysfunction. Debulking or partial anterior excision is 53
performed in most cases. Large blood cysts can be managed by
aspiration. Recurrences are more common when motility restriction
is present at the initial examination.

Cavernous Hemangioma

While this is the most common tumour of the orbit in adults, this
may be seen in children as well. This unilateral, solitary tumour
presents with slowly progressive axial proptosis because of its
intraconal location.16 Visual acuity can be slightly impaired due to
a relative hypermetropia. Occasionally it may be anterior and
present as a subcutaneous bluish mass. Intraosseous cavernous
hemangiomas are rare and can simulate a fibroosseous tumour.

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(5a) (5b) (5c)

(5d) (5e) (5f)

Figure 5a: Rhabdomyosarcoma of the right orbit in a 14 year old girl. This girl was advised to undergo treatment; however she did not turn
up until 2 months later. Figure 5b: CT scan coronal view, revealed the presence of a soft tissue mass in the left orbit involving the ethmoid
and maxillary sinuses and most of the medial portion of the orbit. In addition there was an area of bone destruction in the superomedial

wall of the orbit with intracranial extension. Figure 5c: CT scan axial view, showing the presence of this large soft tissue lesion with
destruction of the medial wall of the orbit and extension into the ethmoid sinuses. Figure 5d: 2 months later, the patient returned with a
massive proptosis and lateral globe displacement due to an inflamed soft tissue mass and gross right nasal deviation. Figure 5e: CT scan
coronal view, showed the presence of a large soft tissue mass involving the ethmoidal sinuses bilaterally and the left orbit with extensive
bone destruction. Figure 5f: CT scan axial view, showed the large mass involving the ethmoidal sinuses and left orbit pushing the globe to

the periphery. The right orbit showed involvement of the medial portion of the orbit.

proximal dilation leading to a varix. Orbital varix usually presents which may involve the orbital bones or sinuses (Figure
between 10-30 years of age and involves the superior ophthalmic 5b,5c,5d,5e,5f). Amongst the four histologic types, pleomorphic,
vein. This lesion is dark red in colour. Positional proptosis occurs embryonal, alveolar and botryoid, the embryonal variant is the
because of connection to the systemic venous circulation. Thrombosis most common. These tumours develop from undifferentiated
or hemorrhages of the affected vein can cause painful proptosis or mesenchymal cells that possess the capacity to differentiate into
compressive optic neuropathy. Varix can be primary or secondary. striated muscle. Management is by incision biopsy to establish the
A secondary varix is associated with an intracranial arterio venous diagnosis, followed by chemotherapy with multiple drugs and
malformation. X-ray shows calcification (phleboliths) in the varix. adjuvant radiation. With early recognition and proper treatment,
Colour Doppler flow imaging demonstrates increase in blood flow these tumours have a good prognosis. Chance of local tumour
during the Valsalva maneuver. CT and MRI demonstrate an recurrence is about 20 % and metastasis 6%.
irregular mass in the posterior orbit; CT can reveal phleboliths.
Surgical excision can be performed for the anterior portion of the Optic nerve and meningeal tumours
lesion. Jugular compression, Trendelenburg position and increasing
the intra thoracic pressure prevent collapse and facilitate surgical Optic nerve glioma
excision. Embolization before surgical excision has also been
attempted. Juvenile pilocytic astrocytoma is a primary tumour of the glial cells
and occurs primarily in children. Usually seen in the first decade of
Myogenic tumours life, this tumour has a slight female preponderance. Presentation is
with unilateral, painless, progressive visual loss and axial
Rhabdomyosarcoma proptosis (Figure 6a). Bilateral tumours may be seen in
neurofibromatosis. Sudden exacerbation may occur due to bleeding
This highly malignant tumour of striated muscles occurs at a mean or mucinous degeneration within the tumour (Figure 6b). More
age of 7 years and has a male preponderance. Presentation is with posteriorly located tumours present with visual loss prior to
rapidly progressing proptosis, usually involving the superior orbit occurrence of proptosis. Evaluation includes screening for
(Figure 5a). This may originate in the ethmoid sinus or nasal cavity Neurofibromatosis. CT scan reveals a fusiform tumour with the
with orbital extension.17 Occasionally the tumour may present presence of kinks in the tumour. Enlargement of the optic foramen
with a subconjunctival mass. The alveolar variant is more aggressive may be present. MRI is useful when there is intracranial extension
with regional lymph node metastasis. Distant metastasis occurs to of the tumour. Histopathology may reveal eosinophilic Rosenthal
the lungs and cervical lymph nodes by hematogenous fibres, which represent areas of degeneration. Surgical intervention
dissemination. CT scan demonstrates an irregular orbital mass is recommended for unsightly proptosis or obstructive

54 DOS Times - Vol. 14, No.3, September 2008

Figure 6a: Optic nerve glioma in a 5 year old boy Peripheral nerve tumours
in the left eye with downward and outward
displacement of the globe Neurofibroma

This benign tumor is a proliferation of Schwann cells, fibroblasts
and axons. The localized variety is common in adults, (Figure
7a,7b&7c) while the plexiform type can present in the first decade.
S shaped ptosis is present due to neurofibromatous thickening of
the underlying tissues. Pulsating proptosis may occur due to
transmission of cerebral pulsations when there is a defect of the
orbital roof. Presence of signs of neurofibromatosis is almost
universal in patients with plexiform neurofibromatosis. Plexiform
neurofibromatosis is seen as an irregular, diffuse soft tissue mass.20
A localized lesion can be removed by surgical excision. The plexiform
variety can be debulked. Though benign, the plexiform type can be
highly invasive.

Figure 6b: CT scan axial view Figure 7a: Neurofibroma of the right orbit;
showing the presence of a fusiform mass a diffuse mass is palpable in the right lower lid

posterior to the globe Figure 7b: CT scan, axial view revealed the
presence of an ill-defined, soft tissue mass in the
hydrocephalus. If visual acuity is good, observation with imaging right lower lid involving the orbital structures.
every 6 months is sufficient. Neurosurgical intervention may be
necessary if the tumour extends intracranially. Chemotherapy may Figure 7c: CT scan, coronal view showed
be given in children less than 4 years, radiotherapy is recommended the diffuse soft tissue mass involving the right
for older children or in cases with opticohypothalamic tumours.
Usually benign, these tumours usually lead to irreversible unilateral orbit, especially the superior, lateral and
visual loss.18 inferior sectors

Meningioma 55

This tumour originates from the meningothelial cells of the
arachnoid of the optic nerve sheath (primary) or adjacent to the
orbit (secondary).19 Primary tumours can occur in children. Bilateral
lesions may also be seen in children. These are more common with
neurofibromatosis and a female preponderance is present.
Presentation is with axial proptosis and slowly progressive unilateral
visual loss. The visual loss is generally severe and color vision is
abnormal. Fundus examination reveals optic disc edema and venous
congestion. Retinochoroidal collateral vessels called optociliary shunt
vessels are sometimes present. CT scan shows a thickened optic
nerve shadow with a linear negative shadow, ‘railroad sign’. Foci of
calcification are present. If the visual acuity is good with the tumour
confined to the optic nerve, no intervention is required. If there is
progression, debulking and/or radiotherapy is employed.
Eventhough this is a benign tumour, invasion of adjacent structures
leads to higher mortality. The prognosis for vision is poor.

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(8a) (8b) (8c)

Figure 8a: Burkitt’s lymphoma in a 12 year old boy who presented with rapidly progressive swelling of the right side of the face,
proptosis of the right eye and upward displacement of the globe. Figure 8b: CT scan coronal view, showed the presence of a large soft
tissue mass in the right orbit with erosion of the inferior orbital wall. Figure 8c: CT scan axial view showed a soft tissue swelling in the

temporal fossa region with extension into the right orbit.

Neurilemmoma Secondary orbital tumours

This is predominantly a tumour of adults. Usually unilateral and Amongst these possibly the most important include orbital spread
solitary, presentation is with slowly progressive proptosis with of retinoblastoma and squamous cell carcinoma of the conjunctiva.
downward displacement of the globe as these tumours arise from Orbital retinoblastoma occurs following extrascleral extension or
branches of the supraorbital or supratrochlear nerves. CT scan by spread along the optic nerve. These cases have a high risk of
reveals a well circumscribed, fusiform mass. Histopathology shows systemic metastasis and require bone marrow biopsy and CSF
the presence of Antoni A (palisading configuration of spindle cells) examination. Management is by high dose chemotherapy followed
or Antoni B pattern (less orderly arrangement of cells in a mucoid by irradiation.24 Squamous cell carcinoma of the conjunctiva can
matrix).21 Surgical excision is the treatment of choice for these spread to the orbit especially in children with Xeroderma
radioresistant tumours. pigmentosa who have a defect in the DNA repair mechanism.
Management is usually radical with exenteration and the prognosis
Lymphoid and leukemic tumours is poor.

Burkitt’s lymphoma Lacrimal gland tumours

This comprises about 50% of tumours in children in East Africa. Adenoid cystic carcinoma of the lacrimal gland
Three distinct forms are seen - African, Non-African and
associated with AIDS. The African form affects the jaw bones, This is the most common primary malignancy of the lacrimal gland.
orbit and abdominal viscera, while the American form involves Though it usually affects young adults, it can occur in children.
the lymph nodes, bone marrow and abdomen and the AIDS- Presentation is with unilateral progressive proptosis and downward
related form mainly involves the CNS. 22 Presentation is with rapid and medial displacement of the eyeball. CT scan reveals an ovoid
proptosis and upward displacement of the globe (Figure 8a). CT soft tissue mass with irregular outlines and erosion of bone.
demonstrates a large mass in the maxilla with secondary Management is by biopsy to establish the diagnosis or complete
involvement of the orbital soft tissues (Figure 8b,8c). The Epstein- excision with removal of orbital bone if involved, followed by
Barr virus has been implicated in the pathogenesis of this disorder. chemotherapy and irradiation. 25 Prognosis is poor especially with
Management is by biopsy to establish the diagnosis with debulking the presence of the basaloid pattern on histopathology with high
followed by chemotherapy and radiotherapy. recurrences.

Granulocytic sarcoma Pleomorphic adenocarcinoma of the lacrimal gland

This is the orbital form of acute myelogenous leukemia. This tumour This arises by malignant transformation of a pleomorphic adenoma
has been called a choloroma because the myeloperoxidase within of the lacrimal gland especially following incomplete excision.
the tumor imparts a green hue to it. Presentation is with rapid Management is by complete excision followed by chemotherapy
onset of unilateral proptosis occasionally with a mass involving the and radiotherapy. These tumours have a better prognosis than
lateral wall of the orbit. Orbital manifestation may precede the adenoid cystic carcinoma of the lacrimal gland.
systemic involvement. CT reveals a large, irregular mass which
may erode bone. A peripheral smear and bone marrow biopsy is Histiocytic and fibrocytic tumours
usually advocated in suspicious presentations to confirm the
diagnosis. Management is by aggressive chemotherapy. Bone Benign fibrous histiocytoma
marrow transplantation may be needed in systemic involvement.
While the prognosis for vision is good, the five year survival is poor This is the most common mesenchymal tumour of the orbit in
inspite of treatment.23 adults. Preschool age children are also known to get affected. This
is usually unilateral involving the superior or nasal orbit with
corresponding proptosis. CT scan reveals a well circumscribed

56 DOS Times - Vol. 14, No.3, September 2008

Figure 9a: Ossifying fibroma in a 13 year Figure 9b: CT scan coronal view Figure 9c: CT scan axial
old girl in the left eye who presented with demonstrating the presence of an view showing the large
expansile bone lesion of the medial wall bony lesion arising from
left eye proptosis and lateral globe of the orbit, with areas of lucency the medial orbital wall
displacement

mass. Management is by complete surgical excision. It can be or multiple bones (polyostotic) usually as part of Albright’s
aggressive in younger patients. Recurrence is known to occur, but syndrome. Presentation is in the first or second decade with
survival rate is excellent. proptosis and globe displacement. Optic nerve compression can
occur if the sphenoid bone is involved. CT scan shows translucent
Histiocytosis X or Langerhans cell histiocytosis zones and areas of sclerosis. The lesion is best observed if there are
no signs of optic nerve compression.27 Cosmetically problematic
This group of tumors is characterized by the proliferation of lesions are surgically excised. Visual prognosis is poor if the sphenoid
Langerhans cells which have characteristic tennis racket shaped bone is involved.
granules known as Birbeck granules. Of the three subtypes,
Eosinophilic granuloma, Hand-Schuller-Christian disease and Metastatic tumours
Letterer-Siwe disease, orbital involvement is more common with
eosinophilic granuloma. Most cases are seen below 5 years of age. Wilms’ tumour presents in early childhood with rapidly progressive
Eosinophilic granuloma presents with a painful tender swelling with unilateral proptosis. Ecchymosis of the lids and conjunctiva may be
overlying erythema in the superolateral aspect of the orbit present. CT scan demonstrates a large irregular tumour with
anteriorly. The frontal and zygomatic bones are most commonly extensive invasion of the paranasal sinuses and cranial cavity.
affected. CT scan shows an osteolytic lesion in the superotemporal Management is by incisional biopsy followed by radiotherapy and
orbit with an irregular contour and marginal hyperostosis. chemotherapy. Prognosis for life is poor. Neuroblastoma arises in
Spontaneous healing may occur. Management is controversial. the first 2 years of life with the primary arising from the adrenal
Intralesional injection of steroids is known to hasten resolution. 26 medulla. Ecchymosis of the lids is present and the tumour is usually
Surgical curettage, chemotherapy and radiotherapy has been bilateral. Increased urinary secretion of vanillyl mandelic acid is
employed for these lesions. present. Management includes a combination of chemotherapy
and radiation. Prognosis for life is generally poor.
Osseous tumours
Miscellaneous tumours
Aneurysmal bone cyst
Alveolar soft part sarcoma
This benign tumour usually affects children in the second decade.
Presentation is with proptosis, globe displacement, pain and a This tumour presents with rapidly progressive proptosis or
palpable mass. Usually arising from the frontal bone, this tumour displacement of the globe. It is more common in females.
causes inferior globe displacement. CT scan reveals an expansile, Histopathology reveals the presence of PAS positive crystalline
mildly enhancing loculated cystic mass. Surgical curettage is the cytoplasmic structures. CT scan demonstrates a well defined mass
treatment of choice; supplemental low dose radiotherapy has also in the superior or temporal quadrants of the orbit. Complete surgical
been given. excision followed by chemotherapy and radiotherapy has been
used in the management of these cases. Orbital tumours have a
Ossifying fibroma better prognosis than systemic lesions. 28 Metastasis is known to
occur to the lungs.
This tumour usually appears in the second decade of life with slowly
progressive proptosis and globe displacement (Figure 9a). CT shows Primitive neuroectodermal tumours (PNETs)
a well defined mass with localized bone expansion (Figure 9b&9c).
This locally aggressive benign lesion is best managed by early surgical The PNETs and Ewing’s sarcoma are supposed to be the same
removal. spectrum of lesions, with the PNETs having better neural
differentiation. Ewing’s sarcoma is poorly differentiated and can
Fibrous dysplasia occur as a primary or metastatic tumour in the orbit.29 It can
metastasise to the bones and lungs. Usually unilateral, management
This hamartomatous malformation results from idiopathic arrest is by incisional biopsy to establish the diagnosis followed by
of maturation of woven bone. It can involve one bone (monostotic)

www.dosonline.org 57

chemotherapy and radiotherapy. Prognosis is poor due to the of the orbit. A review of 35 cases. Ophthalmology 1988;95:1027-
widespread metastasis. 1032.

Dermolipoma 13. Haik BG, Karcioglu ZA, Gordon RA, Pechous B. Capillary
hemangioma. Surv Ophthalmol 1994;38:399-426.
This is a yellowish white tumour usually present in the
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skin and adnexal elements with excess fat and involves the cases. Br J Ophthalmol 1999;83:76-80.
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Collins syndrome, hemifacial microsomia and linear nevus J Ophthalmol 1984;197:108-110.
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First Author
Ramesh Murthy MD, FRCS

58 DOS Times - Vol. 14, No.3, September 2008