DJO July- Sep Volume 32 issue (1)

DJO Vol. 32, No. 1, July-September 2021

Original Article

Change in Perimetric Global Indices Following
Cataract Surgery in Patients with Glaucoma

Sandeep Arora, Sumita Karandikar
Department of Ophthalmology, Dr. D.Y. Patil Medical College & Hospital, Nerul, Navi Mumbai, India.

Purpose: To study the change in perimetric global indices following cataract surgery in patients with glaucoma & Comparison
of preoperative & postoperative perimetric indices.

Methodology: This Prospective study was conducted in a major tertiary care hospital. Approval of Institutional Ethics
Committee was taken before start of the study. 31 Eyes of 18 Patients with co-existing cataract & Primary Open Angle
Glaucoma (POAG), who were well controlled & stable on medical therapy were screened for the study & recruited as per
inclusion & exclusion criteria. Follow up visits were at one & three months. Appropriate statistical analysis was done.

Result: Mean MD (Mean Deviation) improved significantly from -10.77 ± 9.39 dB pre-operatively to -6.52 ± 6.75 dB post-
Abstract operatively at 3 months (i.e. by 4 dB)(P value < 0.0001). 39% eyes showed improvement in MD < 2dB; 26% eyes showed

improvement 2dB to 4dB & 23% showed change more than 8dB at 3 months. Mean PSD (Pattern Standard Deviation)
worsened post-operatively by approximately 1dB from 4.88 ± 3.82 dB pre-operatively to 5.78 ± 4.30 dB post-operatively at 3
months. Majority of eyes showed worsening less than 2dB. (84% at 1month which increased to 94% at 3months). This was
statistically significant (P value <0.004 at 1month; <0.006 at 3 months)

Conclusion: In POAG patients, with presence of coexisting cataract, MD is more affected than PSD. Hence, PSD is a more useful
index to monitor glaucoma in these patients. Moreover, predominantly posterior subcapsular cataract doesn’t affect PSD as
much as it affects the MD.

Delhi J Ophthalmol 2021;32;49-54; Doi http://dx.doi.org/10.7869/djo 688
Keywords: POAG, IOP, Mean Deviation, Pattern Standard Deviation, Perimetry.

Introduction visual field global indices in glaucoma patients treated with
medical therapy only.
Glaucoma: the Silent Thief of Sight, is a disease of the eye
that slowly & painlessly causes permanent loss of vision. Methodology
Glaucoma was defined as “pressure within the eye higher
than the statistical normal of the population” from early 19th This prospective study was conducted in a major tertiary
century till last quarter of the 20th century. According to care hospital. Approval of Institutional Ethics Committee
American Academy Of Ophthalmology, glaucoma is defined was taken before start of the study. 31 Eyes of 18 Patients
as “multifactorial optic neuropathy with characteristic were selected for the study. Patients with co-existing cataract
visual field defects and characteristic changes in optic & POAG, who were well controlled & stable on medical
nerve head.1" After cataract, glaucoma is one of the leading therapy were screened for the study. Patients with visually
cause of blindness accounting for 15% of global blindness.2 significant cataract affecting their daily activities were taken
Prevalence of Glaucoma in India is between 2-13%. up for the study. Each participant underwent a complete
ophthalmological evaluation of Anterior & Posterior
Glaucoma is a progressive optic neuropathy characterized Segment.
by pathological loss of retinal ganglion cells and their axons,
which become clinically manifest as structural changes in Inclusion Criteria
the optic nerve head, retinal nerve fiber layer and functional
changes in the visual fields. Clinically, glaucoma is diagnosed 1. Patients with glaucoma & cataract.
on the basis of loss of neuroretinal rim and increased cupping 2. Patients on medical management only
of the optic nerve head. This along with wedge shaped nerve 3. Age more than 35 yrs.
fiber layer defects help clinch the diagnosis. In diagnosed 4. Visual acuity more than 6/60.
glaucoma patients when 25% of fibers are affected functional 5. Cataract with ≤ grade III nuclear sclerosis
changes start appearing in the visual fields of the patient, 6. Uneventful cataract surgery
which are measured by automated perimeter. This modality
helps in diagnosing and in knowing the progression of Exclusion Criteria
glaucoma.
1. Prior Intraocular Surgery.
Glaucoma patients tend to have concurrent cataracts. 2. Any Other Retinal Disease Affecting Vision
Cataract formation, can affect the analysis of visual field 3. Cystoid macular edema post cataract surgery
global indices, therefore, following cataract surgery, 4. Corneal Pathology
glaucoma progression could be masked by improvement 5. Optic Nerve Disease other than Glaucoma
of the global indices. Hence, this study is being undertaken 6. Disc Anomaly
to study these effects before & after cataract surgery on 7. Uveitis
8. Media Haze due to causes other than Cataract
9. Pregnancy

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DJO Vol. 32, No. 1, July-September 2021

POAG was defined in the presence of an untreated intraocular Figure 1: Sex Distribution
pressure (IOP) of >21mm Hg, open anterior chamber angle
on gonioscopy, glaucomatous optic disc damage on clinical belonged to 51 - 70 yrs age group. Mean age of our study
examination, and corresponding VF defects & patients were population was 59.89 ± 10.05 yrs. Best Corrected Visual
put on medical management with anti-glaucoma drops. Also Acuity(BCVA) improved in all Eyes.(68% of eyes improved
patients with Cup/Disc (C/D) ratio more than 0.7 & cataract to 0.2-0.0) & Mean BCVA improved from 0.57±0.25 log MAR
who were stable & with well controlled IOP on medical pre-operatively to 0.20 ± 0.22 log MAR post-operatively. In
management were included in the study. our study 65% of the eyes had nuclear sclerosis & 35% of
the eyes had predominantly posterior subcapsular cataract
Cataract was graded according to the criteria of the Lens (Figure 2).
Opacities Classification System III (LOCS III).3 Participants Also most of the eyes had NS2 grade of nuclear sclerosis
were categorized according to their preoperative cataract with or without PSC. 90% of the Eyes had well controlled
type, as NS1, NS2, NS2-3 both with & without posterior IOP with medical line of management. Mean IOP of the eyes
subcapsular cataract (PSC) . The overall cataract severity was in our study was 16.19±5.01 mm Hg pre operatively reducing
defined as nuclear cataract or predominantly PSC. Cataract to 13.42±3.65 mmHg post-operatively.(P value <0.0001). 39%
surgery with in-the-bag IOL insertion was performed under eyes showed 0.7 cupping & 35% eyes showed 0.8 cupping.
local anesthesia after obtaining preoperative fitness & Mean Cup/Disc (C/D) Ratio 0.6870968±0.12039 (Figure 3).
informed consent in all cases by SICS or Phacoemulsification 61% eyes had GHT of Outside Normal Limits Pre-Op, 55%
technique by an experienced Surgeon. All the surgeries were
uneventful. Figure 2 : Grades Of Cataract

Perimetry – Measurement

Visual Field testing was performed on Humphrey Field
Analyzer Perimeter (SITA- Standard 30-2, HFA II 740 -
software version 5.1.2, Carl Zeiss Meditec, Dublin, CA) once
before & at 1 & 3 months after cataract surgery. All eyes were
refracted immediately before each visual field performance,
and the appropriate age & distance-adjusted correction
was used for visual testing. The global indices such as the
Mean Deviation (MD) , Pattern Standard Deviation (PSD)
& Glaucoma Hemifield Test (GHT) were noted at each
visit. Severity of glaucoma related field loss was noted as
mild, moderate & severe. Also their relation to the type &
grade of cataract were noted . VF defects were considered
glaucomatous if at least 2 of the 3 Anderson criteria [3 or
more nonedged points in a cluster depressed to P< 5% and
one of which depressed to P<1%, Glaucoma Hemifield Test
outside normal limits and pattern standard deviation (PSD)
depressed to P< 5%] were present.4 The VFs were evaluated
for reliability.

IOP was measured by Goldmann Applanation Tonometer
and Gonioscopy with a Goldmann 2 mirror lens. For
calculating mean visual acuity(VA), Snellen’s VA was
converted to log MAR VA using Conversion Charts.

Statistical Analysis

Descriptive statistics included Mean, SD, SE, Kurtosis,
Skewness, Maximum, Minimum & Range for normally
distributed variables. A two-tailed paired t test was used to
evaluate changes in mean deviation MD, pattern standard
deviation PSD, before & after cataract extraction, to analyze
which indices changed significantly. All statistical analyses
were performed using SPSS for Windows (version 26.0.0,
SPSS, Inc, Chicago, IL). A P value <0.05 was considered to be
statistically significant.

Results

31 eyes of 18 patients were selected in our study (44% - Male
& 56%-Female )(Figure 1). As for laterality 16 Right eyes &
15 Left eyes were studied. Majority of the patients (77%)

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DJO Vol. 32, No. 1, July-September 2021

Figure 3: C/D Ratio Figure 5: Change in Pattern Standard Deviation

Table 1 : Correlation Of Change In Various Parameters
Post -Operatively With Cataract Grading Pre-Operatively

Cat Mean Mean Pattern Std Pattern Std
GRADING Deviation Deviation Deviation Deviation
CHANGE CHANGE CHANGE CHANGE
Preop- 1 Preop- 3
Preop- Preop-
1 month 3 month month month
Postop Postop Postop Postop

Figure 4: Change in Mean Deviation Ns1 2.6433333 2.7866667 0.3433333 0.3666667
1.455 1.445 -0.03 -0.085
eyes had GHT of Outside Normal Limits post-operatively. Ns1 with PSC 2.7325
Most eyes showed improvement in MD in post-operative 2.2316667 6.262 1.2191667 0.9916667
period. Mean MD improved from -10.77 ± 9.39 dB pre- Ns 2 6.228 1.864 2.036
operatively to -6.52 ± 6.75 dB post-operatively at 3 months 3.99
(approx 4dB), 39% eyes showed improvement in MD < 2dB; Ns 2 with 4.028 9.135 0.384 0.314
26% eyes showed improvement 2dB to 4dB & 23% eyes PSC 9.45 0.9125 0.78
showed change more than 8 dB at 3 months (Figure 4). 3.055
Ns 2-3 2.7425 0.879 0.7285
Mean PSD worsened post-operatively by approx 1dB 6.4309091
from 4.88 ± 3.82 dB pre-operatively to 5.78 ± 4.30 dB post- Ns 2-3 with 6.5318182 1.1736364 1.1936364
operatively at 3 months. Majority of eyes showed worsening PSC
in PSD less than 2dB. (84% at 1month which increased to
94% at 3months) (Figure 5). Nuclear
Cataract
As Per (Table 1)
MD: As the NS grade increases, improvement in MD Predominatly
increases, the change is more when associated with PSC. PSC
Maximum improvement is seen with NS2-3 with PSC at both
1 & 3 months post-op. However, with predominantly PSC, Table 2 : Correlation Of Change In Various Parameters
Post-Operatively With Severity Scale Of Galucoma
( As Per Mean Deviation)

Pre op Mean Mean Pattern Std Pattern Std
Glaucoma Deviation Deviation Deviation Deviation
Sevirity as per CHANGE CHANGE CHANGE CHANGE
Preop- 1 Preop- 3 Preop- 1 Preop- 3
MD
month month month month
Postop Postop Postop Postop

<- 6 dB (Mild) 1.5207143 1.4714286 0.2392857 0.2235714
2.8483333 3.5866667 1.755 1.3966667
-6 dB to -12
dB (Moderate) 8.0290909 8.1563636 1.51 1.4718182

>-12 dB
(Severe)

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DJO Vol. 32, No. 1, July-September 2021

Tables 3 : Correlation Of Change In Various Parameters reported after cataract extraction.10 In our study mean MD
Post-Operatively With The Improvement In BCVA ( LogMAR) improved significantly from -10.77±9.39 dB pre-operatively
to -6.52±6.75 dB post-operatively at 3 months (i.e. by 4dB)(P
Change in Mean Mean Pattern Std Pattern value<0.0001). 39% eyes showed improvement in MD<2dB;
Vision Deviation Deviation Deviation Std 26% eyes showed improvement 2dB to 4dB & 23% showed
CHANGE CHANGE CHANGE change more than 8dB at 3 months. Chen PP et al found
>0.0 to ≤ 0.3 Preop- 1 Preop- 3 Preop- 1 Deviation that mean deviation improved significantly (P < 0.0001).11
>0.3 to ≤ 0.6 CHANGE Rehman et al found that SITA-standard mean MD improved
month month month Preop- 3 from -12.3±5.8 to-11.1±6.3(P=0.023).12 Rao et al found Median
>0.6 Postop Postop Postop MD (25th and 75th percentiles) after cataract surgery
month [-10.52dB (range,-19.25 to- 4.86dB)] was significantly better
4.72125 4.836875 Postop (P=0.003) than that before surgery [-11.74dB(range,-20.61 to-
7.15dB)].13 Koucheki et al found in the entire study group,
3.6523077 3.9153846 0.788125 0.8275 MD improved significantly after surgery (P<0.001).14 Our
study showed similar results.
1.84 1.775 1.17 0.9169231
Change In Pattern Standard Deviation
1.335 1.27 In our study mean PSD worsened post-operatively by approx
1dB from 4.88±3.82dB pre-operatively to 5.78±4.30dB post-
the changes are more enhanced with all grades of cataract. operatively at 3 months. Majority of eyes showed worsening
less than 2dB. (84% at 1month which increased to 94% at
PSD: Maximum worsening is seen with NS2 grade both 3months). This was statistically significant(P value <0.004 at
with & without PSC. 1month; <0.006 at 3 months). Various studies on PSD have
shown varied results. Chen PP et al found the mean pattern
As Per (Table 2) standard deviation and corrected pattern standard deviation
MD: More the pre-op severity of glaucoma, more the increased (worsened) significantly after cataract extraction
improvement in MD post-operatively. (P= 0.03 and 0.01, respectively). Rao et al found median PSD
after surgery [4.76dB(range,2.48 to 9.83)] was worse (P=0.01)
PSD: As the severity of glaucoma increases, PSD worsens than that before surgery [3.50dB(range,1.93 to 8.20dB)].
post-operatively However, Rehman et al found the change in mean PSD was
statistically nonsignificant (from 7.2±3.0 to 7.3±3.6;P=0.84)
As Per (Table 3) & hence, concluded that the PSD values of SITA are not
MD: Improvement in MD is seen even with a small affected by the lens opacities in glaucoma patients. SITA-
improvement in BCVA. standard PSD was, therefore, suggested as the best metric
for monitoring glaucomatous change. Cataract can induce
PSD: Maximum worsening of PSD is noted with maximum various degrees of relative scotoma, an actual glaucomatous
improvement in BCVA. field defect can be hidden to some extent and can lead to
worsening of pattern standard deviation after surgery.
Discussion
As Per (Table 1)
Cataract is one of the most commonly encountered MD: As the NS grade increases, improvement in Mean
pathological condition in the ophthalmology clinic, Deviation increases, the change is more when associated
& increased prevalence of glaucoma is seen in elderly with PSC. Maximum improvement is seen with NS2-3 with
population, they often co-exist, hence, it is important to PSC at both 1 & 3 months post-operatively in our study. Rao
understand the effect of cataract on various perimetric global et al evaluated the effect of the type of cataract on the global
indices in glaucoma patients on medical therapy, before & indices and found that MD was significantly depressed in
after cataract surgery. eyes with both nuclear sclerotic and posterior subcapsular
cataracts. However, the improvement in MD in our study was
GHT: GHT showed no significant change after cataract significantly more in predominantly posterior subcapsular
extraction. 61% Eyes had GHT of Outside Normal Limits cataracts as compared with nuclear sclerotic cataracts.
Pre-Op, 55% Eyes had GHT of Outside Normal Limits Post- Therefore, MD is an unreliable index in glaucoma patients
Op at 3 months. 35% eyes remained within normal limits with cataract especially if associated with predominantly
both pre-operatively & post-operatively. Zalta et al found posterior subcapsular cataracts.
that in early glaucoma, the Glaucoma Hemifield Test (GHT)
interpreted 27% of 1676 fields as “within normal limits” PSD: Maximum worsening is seen with NS2 grade both
despite the presence of real scotomas on numeric pattern with & without PSC. Hayashi et al noted in the group with
deviation displays (P<.0001). In early glaucoma, visual field dense scotoma, the mean pattern standard deviation and
analysis based on the GHT alone is at significant risk for corrected pattern standard deviation worsened significantly
misinterpretation, underdiagnosis, and under-treatment.5 after surgery, However, in the group without dense scotoma,

Change In Mean Deviation
Cataracts can diffusely depress retinal light sensitivity,
thereby lowering the overall hill of vision of the visual field.
This is measured as a reduction in the mean deviation.6
Cataract surgery in eyes with only mild glaucoma-related
visual field defects has been shown to result in significant
improvement in the mean deviation.7,8,9 Improvement and
even disappearance of arcuate visual field defects have been

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DJO Vol. 32, No. 1, July-September 2021

the pattern standard deviation(PSD) and corrected pattern Cataract diffusely depresses retinal light sensitivity,
standard deviation(CPSD) showed a slight improvement.15 thereby lowering the overall hill of vision of the visual field.
This is measured as a reduction in the mean deviation.
As Per (Table 2) Cataract surgery has been shown to result in significant
MD: More the preoperative severity of glaucoma, more improvement in the mean deviation. Improvement and even
the improvement in Mean Deviation post-operatively in disappearance of arcuate visual field defects may occur after
our study. Therefore, in advanced glaucoma with cataract cataract extraction.
this index becomes much more unreliable. Koucheki et al
noted that the threshold sensitivity in the deepest areas of Therefore, we infer that in presence of coexisting cataract,
scotomas remained unchanged, & eyes with a better MD MD is more affected than PSD. Hence, PSD is a more useful
before surgery had a greater magnitude of improvement index to monitor glaucoma in these patients. Moreover,
of MD when correlation analysis was performed based on predominantly posterior subcapsular cataract doesn’t affect
glaucoma severity. Chen PP et al divided the eyes into three PSD as much as it affects the MD. Hence, in cases of cataract
groups by preoperative mean deviation and found that eyes coexisting with glaucoma PSD on perimetry is a relatively
with the worst preoperative visual field loss(mean deviation more reliable index.
-21.0dB or worse) did not have significant postoperative
improvement in mean deviation (P=0.60). However, eyes References
in the two groups with less severe visual field loss (mean
deviation 2-13.0dB or between -13.0dB and -21.0dB) improved 1. Primary Open-Angle Glaucoma PPP - 2015 [Internet]. American
significantly (P<0.0001) & eyes with more severe visual field Academy of Ophthalmology. 2015 [cited 2019 Sep 2]. Available
loss had less improvement in the mean deviation.16 from: https://www.aao.org/preferred-practice-pattern/primary-
open-angle-glaucoma-ppp-2015
PSD: As the severity of glaucoma increases, PSD worsens
post-operatively. Koucheki et al found that indices of 2. Thylefors B, Négrel AD. The global impact of glaucoma. Bull
localized field loss, PSD and CPSD, became mildly worse World Health Organ. 1994;72(3):323–6.
after cataract extraction in the study group as a whole &
therefore, in patients with advanced glaucoma with cataract, 3. The Lens Opacities Classification System III | JAMA
preoperative PSD is falsely low. Ophthalmology | JAMA Network [Internet]. [cited 2019
Oct 28]. Available from: https://jamanetwork.com/journals/
As Per (Table 3) jamaophthalmology/article-abstract/640261
MD: Improvement in MD is seen even with a small
improvement in BCVA in our study. On improvement in 4. Automated static perimetry [Internet]. [cited 2019 Oct 28].
BCVA≤0.3 logMAR improvement in mean MD was 4.72dB at Available from: https://www.aao.org/bcscsnippetdetail.
1 month, 4.83dB at 3 months. On improvement in BCVA>0.3 aspx?id=d4fbc026-468e-4a83-abe4-ae80edadcc85
to ≤0.6 logMAR improvement in mean MD was 3.65dB at
1 month, 3.91dB at 3months. However, on improvement in 5. Zalta AH. Limitations of the glaucoma hemifield test in
BCVA by >0.6 logMAR an improvement in mean MD noted identifying early glaucomatous field loss. Ann Ophthalmol. 2000
was only 1.84dB. This was because there were only 2 eyes in Mar 1;32(1):33–45.
the study population whose BCVA improved by more than
0.6 logMAR, & their pre-operative MD itself was relatively 6. Costagliola C, Simone CD, Giacoia A, Iuliano G, Landolfo
less at -3.74dB(which improved to -1.38dB at 3 months) & V. Influence of Lens Opacities on Visual Field Indices.
-3.89dB(which improved to -2.70dB at 3 months) Siddiqui Ophthalmologica. 1990;201(4):180–6.
et al found that, the MD value improved after surgery
(p>0.001), however, the changes of MD and VA were not 7. Yao K, Flammer J. Relationship cataract density and visual field
correlated (p=0.252,R2=0.252).17 damage. Eur J Ophthalmol. 1993 Mar;3(1):1–5.

PSD: Maximum worsening of PSD is noted with maximum 8. Guthauser U, Flammer J. Quantifying visual field damage
improvement in BCVA in our study. Siddiqui et al found caused by cataract. Am J Ophthalmol. 1988 Oct 15;106(4):480–4.
that, the extent of VA improvement correlated with the
deterioration of PSD score. The Pearson correlation test 9. Lam BL, Alward WLM, Kolder HE. Effect of Cataract on
showed a statistically significant correlation between Automated Perimetry. Ophthalmology. 1991 Jul 1;98(7):1066–70.
the postoperative VA improvement and the PSD change
(p=0.024,R2=0.478). 10. Bigger JF, Becker B. Cataracts and open-angle glaucoma. The
effect of cataract extraction on visual fields. Am J Ophthalmol.
Conclusion 1971 Jan;71(1 Pt 2):335–40.

In conclusion, Automated perimetry is Vital to monitor 11. Chen PP, Budenz DL. The effects of cataract extraction on the
the progress of glaucoma. However, cataract especially the visual field of eyes with chronic open-angle glaucoma. Am J
posterior subcapsular type can depress the retinal sensitivity Ophthalmol. 1998 Mar;125(3):325–33.
more than the other types, thereby affecting the various
perimetric global indices. 12. Rehman MS, Khairy HA, Azuara-Blanco A. Effect of cataract
extraction on SITA perimetry in patients with glaucoma. J
Glaucoma. 2007 Mar;16(2):205–8.

13. Rao HL, Jonnadula GB, Addepalli UK, Senthil S, Garudadri CS.
Effect of cataract extraction on Visual Field Index in glaucoma. J
Glaucoma. 2013 Feb;22(2):164–8.

14. Koucheki B, Nouri-Mahdavi K, Patel G, Gaasterland D, Caprioli
J. Visual field changes after cataract extraction: The AGIS
experience. Am J Ophthalmol. 2004 Dec 1;138(6):1022–8.

15. Hayashi K, Hayashi H, Nakao F, Hayashi F. Influence of cataract
surgery on automated perimetry in patients with glaucoma. Am
J Ophthalmol. 2001 Jul 1;132(1):41–6.

16. Chen PP, Budenz DL. The effects of cataract extraction on the
visual field of eyes with chronic open-angle glaucoma. Am J
Ophthalmol. 1998 Mar 1;125(3):325–33.

17. Siddiqui M a. R, Azuara-Blanco A, Neville S. Effect of cataract
extraction on frequency doubling technology perimetry
in patients with glaucoma. Br J Ophthalmol. 2005 Dec

1;89(12):156971.

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DJO Vol. 32, No. 1, July-September 2021

Cite This Article as: Change in Perimetric Global Indices

Following Cataract Surgery in Patients with Glaucoma Sandeep
Arora, Sumita Karandikar Delhi J Ophthalmology 2021, 32 (1): 49-54.
Acknowledgments: Nil
Conflict of interest: None declared
Source of Funding: None
Date of Submission: 17 Nov 2020
Date of Acceptance: 28 Jan 2021

Address for correspondence
Sandeep Arora Resident

Department Of Ophthalmology,
Dr. D.Y. Patil Medical
College & Hospital, Nerul,
Navi Mumbai. India
Email - [email protected]

Quick Response Code

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DJO Vol. 32, No. 1, July-September 2021

Case Report

A Case Report of Unilateral Peripheral Necrotizing Keratitis
Following Lasik

Sudhakar Potti, Prasad Patil

Department of Cornea, Sankara Eye Hospital, Guntur, Andhra Pradesh, India.

Abstract Corneal infiltrates are a relatively uncommon complication of refractive surgery and can cause serious consequences which
may result in vision loss. We report a case of unilateral peripheral necrotizing keratitis at the edge of the flap presenting 3 days
after femto-LASIK. A 23 year old male underwent femto-LASIK for myopia. On 3rd post-operative day, patient came to clinic
with complaints watering in left eye. On Slit lamp examination right eye was normal but in left eye thick opacification noted at
the edge of the corneal flap from 2 to 5 o’clock involving stroma, flap and cornea beyond flap edge and AS-OCT showed hyper-
reflectivity at the interface. An intensive treatment started with topical steroids, antibiotics and lubricating eye drops. On last
visit which was after 2 weeks of surgery, corneal infiltrates were resolved and transparency of cornea was achieved.

Delhi J Ophthalmol 2021;31; 55-57; Doi http://dx.doi.org/10.7869/djo.689

Keywords: Sterile Corneal Infiltrates, Diffuse Lamellar Keratitis, Topical Steroids

Introduction

Femtosecond laser assisted in situ keratomileusis (femto-
LASIK) is a safe procedure to correct errors of refraction like
myopia, hypermetropia and astigmatism. Corneal infiltrates
are a relatively uncommon complication of refractive surgery
and can cause serious consequences which may result in
vision loss. Sterile corneal infiltrates must be distinguished
from the other infectious etiologies to facilitate proper
management. Infectious etiologies like bacterial keratitis
and fungal keratitis should be identified and managed.
Diffuse lamellar keratitis (DLK) is the most common form
of the sterile inflammation after LASIK. We report a case of
unilateral peripheral necrotizing keratitis at the edge of the
flap presenting 3 days after femto-LASIK.

Case report Figure 1a: Slit lamp image showing thick opacificaton at the edge of corneal
flap from 2 to 5 o’clock hours
A 23 year old male underwent femto-LASIK for refractive
error of -4D sphere -0.5D cylinder at 50 in right eye and Figure 1b: Anterior segment optical coherence tomography (AS-OCT) showing
-3.5D sphere –0.5D cylinder at 130 in left eye at Sankara hyper reflection in stroma, flap and cornea beyond flap edge.
Eye Hospital, Guntur. WaveLight FS200 femtosecond laser
system (Alcon Laboratories Inc., Fort Worth, TX) was used moxifloxacin 5mg 8 times a day, artificial tears 4 times a
for creation of flap. Pulse energy was 0.8 μJ and the spot day in left eye. As the presentation was atypical, the lesions
and line separation for the bed cut was 8 μm. For a side appearing immunological at the same time infectious
cut, spot separation of 5 μm and line separation of 3 μm etiology could not be ruled out with 100 percent certainty,
were used. Flap thickness was 100µm with side cut angle we stepped up to a stronger steroid and added topical
of 70° and hinge angle of 50°. Wavelight EX500 excimer fortified vancomycin (50mg/mL). For uninvolved eye we
laser (Alcon Laboratories Inc., Fort Worth, TX) was used continued topical loteprednol and moxifloxacin eye drop.
for the ablation. The procedure was uneventful. Three days 5 days after initiation of treatment, patient came for the
after surgery patient came with the complaints of watering second post-operative follow up. Watering was reduced
in left eye since the day of the surgery. Patient denied any
history of trauma or foreign body exposure. His uncorrected
visual acuity (UCVA) was 6/6 in both eyes. On Slit lamp
examination right eye was normal but in left eye thick
opacification noted at the edge of the corneal flap from 2
to 5 o’clock involving stroma, flap and cornea beyond flap
edge (Figure 1a). There was no conjuctival congestion and
ciliary flush. Epithelium was intact and anterior chamber
reaction was absent. Anterior segment optical coherence
tomography (AS-OCT) showed hyper reflectivity and
thickening in left eye stroma, flap and cornea beyond the
flap (Figure 1b). We started topical dexamethasone 1mg+

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DJO Vol. 32, No. 1, July-September 2021

and patient was symptomatically better. On slit lamp Singhal et.al.3 reported a case of bilateral peripheral
examination we noted area of opacification was smaller as infiltrative keratitis in which a thick band of infiltrate
compared to the last visit and there were no inflammatory developed between flap margin and limbus which extends
cells. AS-OCT also showed reduced reflectivity and edema from 2 o’clock to 9 o’clock in both the eyes without involving
in left stromal bed. So we tapered topical steroids to 6 times flap edge. They successfully treated infiltrate with topical
a day and frequency of fortified vancomycin reduced to 6 prednisolone acetate 1% eyedrop.
times a day. On last postoperative visit, which was after 2
weeks of surgery, patient was asymptomatic and his UCVA Ambrosio et.al.4 reported 2 cases of bilateral, marginal
was maintained 6/6 in both eyes. Slit lamp examination of sterile corneal infiltrates in early postoperative period after
left eye showed complete resolution of opacification and LASIK. Flap lifting and irrigation was done and topical
corneal transparency was achieved. (Figure 2a) AS OCT corticosteroids and fortified antibiotics were started in the
showed minimal hyper reflectivity in stromal bed. (Figure both eyes of both the patients. Both patients responded well
2b) So fortified vancomycin was stopped and steroids eye to the treatment.
drops tapered gradually.
The exact mechanism of sterile corneal infiltrates is unknown.
Figure 2a: Slit lamp photo showing resolution of opacification Previous studies has suggested various risk factors like
use of non-steroidal anti-inflammatory drugs without
Figure 2b: AS OCT showing decreased hyper-reflectivity concomitant use of topical steroids,5 topical anesthetic
abuse6 and immunological reactions.7 In addition, some
Discussion studies reported association of peripheral sterile corneal
infiltrates with chronic meibomian gland dysfunction and
Corneal infiltrates are relatively uncommon complication chronic blepharitis, systemic inflammatory and autoimmune
diseases.
of LASIK and it represent nonspecific accumulation of
In our case we did not use topical non-steroidal anti-
inflammatory cells. Infectious keratitis is probably the most inflammatory drugs pre or post operatively. There was
no evidence of chronic meibomitis or chronic blepharitis.
important complication and it can be vision threatening. Our patient showed no serological or clinical signs of
autoimmune disease. Absence of conjuctival congestion and
In 2005 Lifshitz et.al. first reported a case of peripheral ciliary flush, absence of epithelial defect, absence of anterior
sterile corneal infiltrates after LASIK.1 In 2012 Bucci and chamber reaction, and response to topical steroids in our
McCormick reported a case of peripheral necrotizing case suggest sterile infiltrate. Our case differed from other
keratitis after LASIK.2 Here we report a case of unilateral case reports in being unilateral case.
peripheral necrotizing keratitis after LASIK.
In conclusion, we report a case unilateral peripheral
necrotizing keratitis after LASIK. Differentiation from
infectious keratitis is essential for the proper management.
Appropriate management results in faster resolution of
infiltrates without affecting final visual outcome.

References

1. Lifshitz T, Levy J, Mahler O, et al. Peripheral sterile corneal
infiltrates after refractive surgery. J Cataract Refract
Surg.2005;31:1392–1395

2. Bucci MG, McCormick GJ. Idiopathic peripheral necrotizing
keratitis after femtosecond laser in situ keratomileusis. J Cataract
Refract Surg. 2012;38:544–547.

3. Singhal S, Sridhar MS, Garg P. Bilateral peripheral infiltrative
keratitis after LASIK. J Refract Surg 2005;21:402-4.

4. Ambrosio R, Periman LM, Netto MV, Wilson SE. Bilateral
marginal sterile infiltrates and diffuse lamellar keratitis after
laser in situ keratomileusis. J Refract Surg 2003;19;154-8.

5. Teal P, Breslin C, Arshinoff S, et al. Corneal sub epithelial
infiltrates following Excimer Laser photorefractive keratectomy.
J Cataract Refract Surg. 1995;21:516–518.

6 . Kim JY, Choi YS, Lee JH. Keratitis from corneal anesthetic
abuse after photorefractive keratectomy. J Cataract Refract Surg.
1997;23:447–449.

7. Rao SK, Fogla R, Rajagopal R, et al. Bilateral corneal infiltrates
after Excimer Laser photorefractive keratectomy. J Cataract
Refract Surg. 2000;26:456–459.

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DJO Vol. 32, No. 1, July-September 2021

Cite This Article as Sudhakar Potti, Prasad Patil,A Case
Report of Unilateral Peripheral Necrotizing Keratitis Post Lasik
Delhi J Ophthalmology 2021; 31 (1), 55 - 57.
Acknowledgments: Nil
Conflict of interest: None declared
Source of Funding: None
Date of Submission: 04 Jan 2021
Date of Acceptance: 08 Feb 2021

Address for correspondence
Sudhakar Potti M.S

Department of Cornea
Sankara Eye Hospital, Guntur
-Vijayawada expressway, Pedakakani,
Guntur, Andhra Pradesh, India
E-mail: [email protected]

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DJO Vol. 32, No. 1, July-September 2021

Case Report

Case Report: Accidental Ocular Blast Injury To A Farmer’s
Eye by Gandhak Potash

Nimisha Nagpal, Pawan Prasher, Inderjit Kaur

Department of Ophthalmology, Sri Guru Ram Das Institute of Medical Sciences (SGRD) Amritsar, Punjab, India.

Abstract Ocular exposure to chemical explosions constitute true emergency that can be associated with injuries leading to
significant visual morbidities. Herein, we report a case of bilateral eye injury in a 54 year old male farmer due to a
chemical explosion while preparing a Gandhak Potash mixture. He presented with multiple charred wounds around his
face and embedded particulate matter over the eyelids, conjunctiva and cornea. He underwent superficial debridement
of the particulate matter along with conservative management using topical antibiotics, cycloplegics and steroids with
good visual outcome. Gandhak potash explosions can be potentially visually devastating. Ophthalmologists, especially
those working in agricultural surroundings, should be aware of this mode of injury. Good visual outcome is possible with
urgent, appropriate medical intervention.

Delhi J Ophthalmol 2021;32; 58-61; Doi http://dx.doi.org/10.7869/djo.690

Keywords : Trauma, Gandhak, Chemical, Ocular, Injury

Introduction of the cornea along with surrounding corneal edema.
In the left eye (LE), a clump of granular material had
Eye trauma constitutes 7% of all bodily injuries and 10%– perforated through the cornea paracentrally and deposited
15% of all eye diseases.1 It has been estimated that 90% of on the anterior surface of the iris (Figure 1A, 1B).
all ocular injuries are preventable.2 The reported incidence
of ocular chemical injuries in developing countries is There was a marked anterior chamber reaction in both
approximately 1.25% to 4.4%.3 eyes. Lenses were unaffected and visual acuity was hand
motions close to face with intraocular pressures of 11 and 12
Chemical exposure is a potentially blinding ocular mm of Hg bilaterally. Fluorescein staining revealed a large
injury and constitutes a true ocular emergency requiring epithelial defect in the right eye (RE) and multiple focal
immediate assessment and initiation of treatment. The epithelial erosions in the LE (Figure 1C, 1D).
majority of victims are young and exposure occurs at home
or workplace.4 Gandhak Potash mixture is usually used by
farmers in an improvised iron gun to produce a sound blast
that deters birds, pests, and wildlife animals from damaging
their harvest.5

This consists of a chemical reaction along with a thermal
and sound blast which if held close by can cause significant
primary and secondary injuries. Herein, we present an
unusual case of bilateral ocular injuries secondary to the
explosion of this mixture. To the best of our knowledge,
this mechanism of ocular injuries has not been previously
reported in ophthalmology literature.

Case Report Figure 1: Slit lamp picture at presentation (A) showing multiple granular
deposits within the corneal epithelium and stroma along with surrounding
A 54-year-old male reported to our outpatient department conjunctiva along with conjunctival congestion and matted eyelashes in
with a history of pain, photophobia, and sudden onset the right eye (B) left eye showing sub conjunctival haemorrhage (C) diffuse
of decreased vision after a blast injury with Gandhak and epithelial defect in cobalt blue filter after Fluorescein staining in the right eye
Potash to produce a minor sound blast. This was a routine
activity in their agricultural practice as it helped ward off and (D) multiple punctuate staining in the left eye.
wild animals and protect their harvest. This time, for some
reason that blast happened prematurely leading to burn
injuries involving his face and eyes. Examination revealed
multiple charred wounds on the face with embedded
particulate matter on the swollen eyelids.

Slit-lamp examination showed conjunctival hyperemia
and subconjunctival hemorrhage bilaterally with
diffuse multiple yellowish-white granular foreign
bodies embedded in the superficial and deep layers

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DJO Vol. 32, No. 1, July-September 2021

Figure 2: B-scan shows both eyes showed attached retinas with no evidence of any foreign body in the vitreous cavity.

Seidel test was negative. The red glow was visible but fundus care. Potassium chlorate reacts with sulfuric acid (2 KClO3
details could not be visualized due to reduced media clarity. + H2SO4 →2 HClO3 + K2SO4) to form a highly reactive
B scan ultrasonography of the posterior segment was normal solution of chloric acid and potassium sulfate which is a
(Figure 2). kind of low order explosive (Figure 4A, 4B).6

The patient was managed conservatively. After vigorous The injury caused by this chemical is a combination of
saline wash, superficial debridement was done using 26 chemical and thermal injury closely resembling blast injuries
G needle and particulate matter was removed from the with firecrackers7 causing damage to the ocular surface
corneal stroma taking care not to go too close to Descemet’s eventually resulting in poor visual outcomes.
membrane. The patient was started on topical moxifloxacin
0.5% four times a day, prednisolone 0.5% four times a day, To the best of our knowledge, only one case of Gandhak
homatropine 2% three times a day hydroxypropyl methyl Potash explosion has been previously reported in India
cellulose 0.3% four times a day, along with oral doxycycline which was eventually fatal for the patient probably due to the
100 mg twice daily and tablet vitamin C 500 mg three times higher quantity of mixture resulting in a bigger explosion.
a day. In our case, the patient was fortunate as the quantity was
limited leading to predominantly superficially injuries.
Patient was followed up on day 1, 3, 7, 14, 30, 2 months and
3 months. In the first few days, the LE showed worsening The mainstays of primary management are immediate
of inflammation with the occurrence of hypopyon and an decontamination and copious irrigation. Our management
increase in IOP, which was managed by adding topical was along the established guidelines8 which aimed at
brimonidine and timolol combination ED twice daily (Figure providing symptomatic relief, reducing the inflammation
3A, 3B). and prevent any visually devastating complications in this
patient.
The hypopyon appeared to be sterile in nature and gradually
resolved over a period of 2 weeks with no evidence of corneal Ocular chemical burns can have profound psychological,
infiltrate (Figure 3C, 3D). economic, and social consequences for the patient. For this
reason, a proactive approach to prevention becomes the
On follow up after 3 months, scattered superficial corneal most effective approach to prevent ocular chemical injuries
opacities were seen in both eyes with no epithelial defect or from occurring.
anterior chamber reaction. The best-corrected visual acuity
was 20/20 in RE and 20/40 in LE and IOP were 11 mm Hg in Purchase and use of such combustible materials over-
both eyes (Figure 3E, 3F). the-counter should be regulated, mass health education
and awareness should be provided to prevent delay in
Discussion management and adoption of safety equipment, like
protective eyeglasses, should be focused in the hope for the
We report a unique case of ocular injury with Gandhak preservation of vision.
Potash mixture which comprises of sulfur powder and
potassium chlorate powder respectively. Potassium chlorate Ophthalmologists, especially those working around such
(KClO3) reacts vigorously and spontaneously explodes agricultural communities, should be aware of this unusual
when mixed with combustible materials. The sulfur powder entity to best manage such cases of ocular injuries.
is available over-the-counter containing different quantities
of sulphuric acids which needs to be handled with utmost

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DJO Vol. 32, No. 1, July-September 2021

Figure 3: Slit lamp biomicroscopy pictures on consecutive visits showing (A) minimal size of epithelial defect left in RE post treatment and (B) Resolving anterior
chamber hypopyon with corneal opacities and conjunctival congestion on day 14 (C,D) Reducing corneal opacities in number and size with resolving anterior
chamber reaction on 1 month follow up of right and left eye respectively (E,F) Showing resolved corneal opacities and quiet anterior chamber on 3 month follow up

Figure 4 : (A) Demonstrating Potash powder (KClO3) (B) Sulphur powder which is readily available over the counter.

References Res 2018; 7: 9212-4.
6. Pradyot Patnaik. Handbook of Inorganic Chemicals. McGraw-
1. Acar U, Tok OY, Acar DE et al. A new ocular trauma score in
pediatric penetrating eye injuries. Eye (Lond) 2011; 25: 370-4. Hill, 2002.
7. Reddy KSN, Murty OP. The essentials of Forensic Medicine
2. Hutton WL, Fuller DG. Factors influencing final visual results in
severely injured eyes. Am J Ophthalmol 1984; 97: 715-22. and Toxicology. 33rded. New Delhi: Jaypee Brothers Medical
Publishers 2017: 214.
3. Xie Y, Tan Y, Tang S. Epidemiology of 377 patients with chemical 8. Spector J, Fernandez WG. Chemical, Thermal, and Biological
burns in Guangdong province. Burns 2004; 30: 569‐72. Ocular Exposures. Emergency Medicine Clinics of North
America 2008; 26: 125-36.
4. Saini JS, Sharma A. Ocular chemical burns-clinical and
demographic profile. Burns 1993; 19: 67-9.

5. Giri SK, Sharma L, Kumar V et al. Accidental Blast of an
Improvised Bird Scarer Gun: A Case Report. Int. J. Curr. Adv.

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DJO Vol. 32, No. 1, July-September 2021

Cite This Article as: Nimisha Nagpal, Pawan Prasher,
Inderjit Kaur. Case Report: Accidental Ocular Blast Injury To
A Farmer’s Eye by Gandhak Potash . Delhi J Ophthalmol
2021;32 (1), 58 - 61.
Acknowledgments: Nil
Conflict of interest: None declared
Source of Funding: None
Date of Submission: 06 Jul 2020
Date of Acceptance: 02 Apr 2021

Address for correspondence
Pawan Prasher

Professor, Department. of Ophthalmology

Sri Guru Ram Das Institute of Medical
Sciences and Research, Vallah,
Amritsar, Punjab, India,
E-mail: [email protected]

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DJO Vol. 32, No. 1, July-September 2021

Case Report

Adult Onset Orbital Lymphangioma Presenting with Globe
Luxation: A Rare Presentation of A Rare Disease

Deepsekhar Das, Swechya Neupane, Sahil Agrawal, Mousumi Banerjee
Mandeep Singh Bajaj, Seema Kashyap

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

Abstract Lymphangiomas or lymphatic vascular malformations typically present in adolescent age groups. Adult onset lymphangiomas
are extremely rare occurrences. The condition usually presents with proptosis or extraocular motility disorder. The authors
here report a 50-year-old female who presented with a painless progressive proptosis of the left eye for the last 10 years.
She gave history of episodes, where the globe spontaneously came out of the socket, which she used to then push back.
Radiological imaging of left orbit revealed a large retrobulbar lesion occupying the whole of the left orbit with luxation of
the left globe. There were no obvious bony erosions or intracranial extension. She underwent an excisional biopsy which
came out to be positive for adult onset lymphatic vascular malformation. Although proptosis is a usual presenting feature
of an orbital lymphatic malformation, globe luxation as a presenting complaint has never been documented.

Delhi J Ophthalmol 2021;32;62-64; Doi http://dx.doi.org/10.7869/djo.691

Keywords: Lymphangioma, Globe Luxation, Lymphatic Malformation, Adult-Onset

Introduction causing globe dystopia. The left upper eyelid was lifted
by an underlying mass. There was exposure keratopathy
Orbital lymphangioma is a rare, diffusely infiltrating of the cornea and marked extraocular muscle movements
benign non-encapsulated vascular malformation, usually limitation was noted.
diagnosed in the first or second decade of life.1 Adult onset
lymphangioma or late onset lymphangioma is the one which On palpation, the globe almost luxated (Figure 2), we could
presents after 30 years of age.2 Here we report a case of adult feel a 5.5 cm x 5.5 cm well defined solid, non-tender, non-
onset lymphangioma in a 50 year old female. pulsatile mass extending from the superior orbital rim to
the lateral canthus, free from the overlying eyelid with fixity
Case Report to the underlying structures with no increase in size on
Valsalva maneuver.
A 50-year-old female presented with painless progressive
proptosis of the left eye for the past 10 years. (Figure 1) On examination, BCVA in her right eye was 20/20. However,
There was no history of trauma, weight loss, anorexia, or any she denied any perception of light in her left eye. Her right
significant systemic illnesses. There was history of episodes eye had brisk direct pupillary response whereas there was
of the eyeball coming out of the orbit spontaneously causing fixed, 5 mm dilated pupil noted in the left eye with no
pain. The patient used to push the eyeball back inside the appreciable fundal glow.
socket.
A Contrast Enhanced Computed Tomography (CECT)
On general examination, she was alert, conscious and well- brain and orbit revealed a 6 cm x 5 cm well defined orbital
oriented to time and place and systemically stable. mass with inferomedial globe dystopia with no intracranial

On local examination, there was gross abaxial proptosis of the
left globe with a visible mass in the supero-temporal aspect

Figure 1 : Clinical picture of the patient on presentation showing severe Figure 2 : Clinical picture showing luxated left globe with a mass located
proptosis with globe dystopia superotemporal to the globe along with exposure keratopathy

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DJO Vol. 32, No. 1, July-September 2021

Figure 3 : CECT images showing a well-defined mass in the left orbit with globe dystopia.

extension or bony changes (Figure 3). Ultrasonography of malformations, or late onset lymphatic malformations
the left eye revealed an anechoic vitreous cavity without and which are characterised by presentation after 30 years of age
obvious retinal pathology or optic nerve head cupping. Left are extremely rare with very limited number of cases to be
orbital ultrasound showed a well-defined lesion with few reported in literature. Acquired lymphangiomas occur due
cystic spaces behind the globe. to impaired lymphatic drainage as a consequence of surgery,
trauma, malignancy, or radiation therapy.
Left anterior orbitotomy with complete removal of mass
was planned. Under general anaesthesia, using a modified
Stallard Wright incision, a plane was created, and the lesion
was identified as a well-defined, non-encapsulated, soft
mass. The mass was then removed and its base was cauterised
making sure not to damage the optic nerve. In post-operative
phase, the patient was treated with oral antibiotics and
steroids post-operatively. Histopathology of the specimen
revealed numerous thin walled vascular channels lined
by flattened endothelial cells filled with lymph along with
lymphoid aggregates in the stroma (Figure 4).

At 1 week follow up, the globe dystopia and proptosis had Figure 4 : Histopathology of excised lesion showing numerous thin walled
completely gone away (Figure 5). There was no recurrence vascular channels lined by flattened endothelial cells (arrow) filled with lymph
noted on 6 months follow up.
along with lymphoid aggregates in the stroma.
Discussion
Figure 5 : Clinical picture of the patient on follow up with no proptosis.
Orbital and adnexal lymphangiomas comprise 20 % of all
lymphangiomas and only 2% of them account for orbital
biopsies.1 One of the most important features of orbital
lymphangioma is swelling that increases gradually in size.
Sudden increase in size of the swelling can be attributed
to intraorbital haemorrhage or respiratory infections.
Various signs include ocular proptosis, blepharoptosis and
cellulitis. Acute proptosis, compressive optic neuropathy
and loss of vision are attributed to spontaneous intraorbital
haemorrhage.1 There is a lot of controversy regarding
pathogenesis of orbital lymphangiomas. According to
Harris et al., lymphangioma is considered isolated from
normal orbital vasculature whereas Wright et al. considered
them to be venous anomalies.3 The congenital form occurs
because of improper connection of lymphatic channel to
the main lymphatic draining duct. Adult onset lymphatic

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DJO Vol. 32, No. 1, July-September 2021

Lymphangiomas show benign features on histopathology in References
contrast to their locally aggressive nature with friable and
non-encapsulated lesions on gross appearance, thus making 1. Tunç M, Sadri E, Char DH. Orbital lymphangioma: an analysis
recurrences common even after resection of the tumor. On of 26 patients. British Journal of Ophthalmology. 1999 Jan
histology, lesions have thin walled stroma, a single layer 1;83(1):76-80.
of flattened epithelium along with occasional breaks in
continuity and lymphoid infiltration. There may be presence 2. Bailey ST, Wojno TH, Shields CL, Grossniklaus HE. Late-onset
of lymphoid aggregates in subendothelial spaces and presentation of orbital lymphangioma. Ophthalmic Plast Reconstr
haemorrhagic material in cystic spaces. Lesions often show Surg. 2007;23(2):100-103. doi:10.1097/IOP.0b013e3180331798
large, irregular vascular spaces that are lined by a single
layer of endothelial cells within fibroblastic stroma.4 3. Salihu N, Sylaj A. Orbital lymphangioma. International Journal
of Case Reports and Images (IJCRI). 2014 Jan 1;5(1):28-31.
Diagnostic imaging like Magnetic Resonance Imaging (MRI)
plays an important role in anatomical demonstration of 4. Miceli A, Stewart KM. Lymphangioma. InStatPearls [Internet]
cystic nature and haemorrhages present in lymphangiomas. 2018 Oct 27. StatPearls Publishing.
On T2 weighted images, venous component enhances
whereas lymphatic component shows fine enhancement 5. Khan SN, Sepahdari AR. Orbital masses: CT and MRI of
of septations.5 Pathognomonic finding includes cyst with common vascular lesions, benign tumors, and malignancies.
multiple fluid-filled levels. However, the appearance may Saudi Journal of Ophthalmology. 2012 Oct 1;26(4):373-83.
be absent in cases of adult onset lymphangiomas. Treatment
options for orbital lymphangiomas are conservative 6. Sen S, Singh P, Bajaj MS, et al. Management of a case of orbital
management, partial resection of cyst, needle aspiration, lymphangioma presenting in adulthood with negative-pressure
anterior orbitotomy with total removal and intralesional aspiration and bleomycin injection BMJ Case Reports CP
injection of sclerosing agents like alcohol, bleomycin and 2019;12:e227697.
tetracycline. In presence of orbital haemorrhage leading to
compressive optic neuropathy, orbital decompression is the 7. Das D, Kuberappa RG, Kumari Meena S, et al Globe luxation
only option to restore vision. Apart from compressive optic following cow horn injury BMJ Case Reports CP 2019;12:e229994
neuropathy, other complications of orbital lymphangiomas
include astigmatism, corneal exposure, hyperopia Cite This Article as: Deepsekhar Das, Swechya Neupane
secondary to pressure on posterior globe, strabismus and MBBS, Sahil Agrawal, Mousumi Banerjee, Mandeep
glaucoma.5 Based on clinical findings and histopathological Singh Bajaj, Seema Kashyap, Adult Onset Orbital
examination, diagnosis of adult onset orbital lymphangioma Lymphangioma Presenting with Globe Luxation: A Rare
was made in this case. Adult onset orbital lymphangioma is Presentation of A Rare Disease Delhi J Ophthalmol 2021;
an exceedingly rare entity that has very limited literature. 32 (1), 62-64.
The usual management varies from surgical excision,
debulking to intralesional bleomycin application.6 Acknowledgments: Nil

Globe luxation is a situation when the eyeball comes in Conflict of interest: None declared
front of the orbital rim and appears caught in between the
eyelid aperture. Usual causes of globe luxation are trauma, Source of Funding: None
strenuous conditions like chronic obstructive pulmonary
disease or spontaneous luxation in cases of shallow orbital Date of Submission: 03 Mar 2020
cavity.7 After performing a thorough literature search, Date of Acceptance: 13 Mar 2020
we believe that this is the first time a case of adult onset
lymphangioma has presented with globe luxation. Address for correspondence

Sahil Agrawal

Oculoplasty and Orbital Tumor Services

Dr Rajendra prasad centre
for ophthalmic sciences,
All India Institute of Medical Sciences
New Delhi India
[email protected]

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DJO Vol. 32, No. 1, July-September 2021

Case Report

Diplopia Following External Dacryocystorhinostomy with
Intracystic Pawar Implant Implantation: The Tale of an
Unheard Complication

Deepsekhar Das,1 Mandeep Singh Bajaj,1 Swechya Neupane,1 Sahil Agrawal,1 Saloni Gupta2

1Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India.
2Northern Railway Central Hospital, New Delhi, India.

Abstract Dacryocystorhinostomy (DCR) with Pawar intracystic implant placement is a simple and faster surgical option for treating
epiphora. Its advantages being small length of incision(5-6mm), minimal intraoperative and postoperative bleeding, and
sparing of the medial palpebral ligament with no need of creating sac and nasal mucosal flaps anastomosis. The presence of
holes at upper and lower end act as extra drainage channel. This makes it superior to conventional DCR with better success
rate. Herein, we report a rare complication of diplopia following implantation of Pawar’s implant. We believe in the following
chain of events with anterior displacement of implant, local inflammation was evoked, resulting in subsequent scarring and
medial rectus fibrosis.

Delhi J Ophthalmol 2021;32; 65-67; Doi http://dx.doi.org/10.7869/djo.692

Keywords : Pawar Implant, Diplopia, Dcr, Dcr-Complications

Introduction Case History

Dacryocystorhinostomy with Pawar intracystic implant A 38-year-old male presented with a history of diplopia and
placement is a simple, fast, and effective surgical option for inward deviation of his right eye for the past six months. He
the management of epiphora. Its results are comparable to gave a history of undergoing external dacryocystorhinostomy
conventional dacryocystorhinostomy (DCR). The advantages with Pawar implant placement 4years back for right eye
of Pawar implant DCR over conventional DCR are small chronic dacryocystitis. Immediately after the surgery, his
length of incision of around 5-6mm, minimal intraoperative had reduction in epiphora, however; in the subsequent
and post-operative bleeding complication and sparing of month, a swelling developed in the right medial canthal
the medial palpebral ligament. In this procedure, there is no region which had subsided on the administration of topical
need for creating a sac and nasal mucosal flaps anastomosis. and oral antibiotics.

The presence of additional four holes at the upper end near On examination, his visual acuity was 6/6 in both eyes with
the collar and six holes at the lower end of the Pawar implant normal intraocular pressures. On checking extraocular
act as an extra drainage channel having 1 mm diameter. This motility, there was marked restriction of ocular movements
makes it superior to conventional DCR with a better success of the right eye in the attempted right lateral and up gaze
rate.1 Herein, we report a case of patient developing diplopia (Figure 1).
following implantation of Pawar's implant.

Figures 1: Nine gaze image of the patient showing limitation abduction and elevation of right eye.

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DJO Vol. 32, No. 1, July-September 2021

Figures 2: a) Coronal cut of a non-contrast CT Scan of the orbit showing implant in situ (marked by a black arrow).
b) Image of the explanted Pawar implant

There was dystopia of the right medial canthus with medial This implant has many advantages over conventional
conjunctival congestion along with exposure of the tube. DCR with few complications. In a prospective study by
There was a granuloma adjacent to the exposed implant Chandravanshi et al. published in 2019 where 30 patients
with symblepharon bridging the medial bulbar conjunctiva underwent DCR with Pawar implant, post-operative
and the lower eye. The left eye was within normal limits. A complications like lid oedema, incisional oedema,
computed tomography (CT) scan of head and orbit was done haemorrhage from nasal mucosa, sac infection, wound gape
which revealed the presence of a heterogeneous opacity in and hypertrophic scar were more with conventional DCR.
the right medial canthal region suggestive of a tube, along This was attributed to the minimally invasive nature of
with a deficient bone in right medial canthal region which intracystic implant surgery.
hinted a prior surgery (Figure 2a).
However, complications like obstruction of passage and
The patient was planned for surgery. After creating a extrusion were more common with Pawar implant. In another
localized conjunctival peritomy, the granuloma was excised, study by Mishra et al., where he compared conventional
and the was symblepharon was released. The anteriorly external DCR with DCR with Pawar's implant placement,
displaced intracystic implant was removed (Figure 2b). 33 patients underwent external DCR with Pawar's implant
placement there was a success rate of 97% at 6months follow
A forced duction test was performed where the MR was up.4 The most common cause of failure of Pawar implant was
found to be tight. On further exploration, the MR and its mucus plug formation leading to obstruction of the tube,
overlying tenon were found to be fibrosed, and the tenon was followed by sac infection, crust formation and extrusion of
released. On repeating the force duction test medial rectus the implant through a skin wound.
was mildly tight. A conjunctival autograft was obtained from
the superior bulbar conjunctiva and placed over bare sclera Literature regarding the complications with the use of this
around the medial canthus and sutured with an 8-0 vicryl device is limited. Mishra et al., in his study, documented
suture. The patient was started on topical Moxifloxacin eye intraoperative complications as nasal mucosal bleed, which
drops and carboxymethylcellulose drops. occurred in 37%, angular vein bleed in 9% and nasal mucosal
disruption in 15%. Long term complications documented
Discussion in his study were granulation tissue formation in 3% and
mucous plug formation in 6%. In a study by Gupta et al.5
Pawar implant is a funnel-shaped device which has a wider where she performed external DCR with Pawar's Implant in
end designed to protrude into the nasal cavity. The first 40 patients, the complications she documented were bleeding
of its kind was made in 1985 from silicone pieces which per-operatively (5%), longer duration of surgery(7.5%),
were leftover from Denver's hydrocephalus shunts after a recurrence of symptoms(5%), pain at the incision site (2.5%)
hydrocephalus surgery.2 A collar was added to the implant and mild ecchymosis(100%).6 However, complications
in 1987. Furthermore, a haptic was added in 1995.3 like medial rectus fibrosis and diplopia following implant
placement have never been reported.
The idea behind this invention was to allow a smooth
unhindered passage of tears without being affected by
lacrimal sac fibrosis or the phimosis of the bony ostium in
the long run.

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DJO Vol. 32, No. 1, July-September 2021

Conclusion Cite This Article as: Deepsekhar Das, Mandeep Singh
Bajaj, Swechya Neupane, Sahil Agrawal, Saloni Gupta.
The most likely reason for the situation would be continuous Diplopia following external dacryocystorhinostomy with
rubbing of the anteriorly displaced implant against the intracystic Pawar implant . Delhi J Ophthalmol 2021; 32 (1),
bulbar conjunctiva and the underlying medial rectus muscle. 65-67.
That would have evoked an inflammatory response leading Acknowledgments: Nil
to granulation tissue formation around the region leading to Conflict of interest: None declared
symblepharon and fibrosis of medial rectus muscle. Though Source of Funding: None
the MR was tight, on follow up, diplopia was corrected in Date of Submission: 19 Mar 2021
primary gaze. Date of Acceptance: 16 Apr 2021

References Address for correspondence
Sahil Agrawal
1. Chandravanshi SL, Shrivastava SK, Shakya DK, Tiwari US.
Conventional Dacryocystorhinostomy Versus Pawar’s Implant Oculoplasty and Orbital Tumor Services
Dacryocystorhinostomy-A Prospective Study. International
Journal of Innovative Research in Medical Science. 2019 Dr Rajendra prasad centre for
Jan;4(01):5-10. https://doi.org/10.23958/ijirms/vol04-i01/533. ophthalmic sciences, All India Institute
of Medical Sciences New Delhi India
2. Pawar MD. Implant dacryocystorhinostomy using modified Email:[email protected]
Pawar implant. Journal of medical science and clinical
research. February 2015; 3(2): 4008-18. Quick Response Code

3. Pawar MD, Sutaria SN. Intra-cystic tube implantation in
dacryocystorhinostomy. In Proceed-ings of the Xth Congress
of the Asia Pacific Academy of Ophthalmology. 1985 Jan; pp.
19091.

4. Mishra, D., Bhushan, P., Sinha, B. P., Bhaskar, G., & Rao, R.
(2019). External dacryocystorhi-nostomy conventional surgery
versus Pawar implant: A comparative study. Indian journal of
ophthalmology; 67(7):1143–1147.https://doi.org/10.4103/ijo.
IJO_1889_18

5. Gupta S, Mengi RK. Outcome of Dacryocystorhinosotomy
With Silicone Intracystic Implant In Patients With Chronic
Dacryocystitis, http://www.jkscience.org; Oct-December 2013;

15(4): 181-4.

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DJO Vol. 32, No. 1, July-September 2021

Case Report

A Rare Case of Upper Eyelid Cutaneous Horn

Neeraj Sharma, Rupali Kashyap, Priyanka Kumar, Sonakshi Sehrawat, Soumya Sharma

Department of Ophthalmology, Department, Shree Guru Gobind Singh Tricentenary, (S.G.T) University, Buddhera, Gurugram, Haryana, India.

Abstract Cutaneous horn is a conical shaped overhanging growth from the skin composed of keratinized material. We are presenting
a case report on cutaneous horn of upper eyelid in a middle-age man, which developed within a year of onset from a focal
swelling and turned out to be benign lesion on biopsy after its excision. Clinical diagnosis of these types of tumours is easy
but their malignant potential can only be assessed by a histopathological examination, that determines the further course
of treatment.

Delhi J Ophthalmol 2021;32;68-70; Doi http://dx.doi.org/10.7869/djo.693

Keywords: Cutaneous Horn, Benign Tumour, Upper Eyelid

Introduction swelling was excised under local anaesthesia and defect

Cutaneous horn (cornu cutaneum) is keratinized epidermal was left to heal by secondary intention. (Figure 3). Excised

growth. Cutaneous horn is relatively rare tumour, the Specimen was sent for histopathological examination.

precursor lesions are multiple and variable.1 Usually sun

exposed area of skin is involved in this tumour.2 History

of these cutaneous horns reported in various articles reveal

that they look like animal horn so have always been point of

interest to the clinicians. Excision and biopsy play a key role

in management of such tumours. Base pathology of such

tumours is the hidden iceberg and all we see clinically is just

the tip of it.3 Case Report


A 45-year-old male patient, working as a clerk in a private

firm, presented in ophthalmology department with primary

complaint of a solitary firm conical shape protrusion from

the right upper eyelid. Patient noticed a focal swelling of the

right upper eyelid which gradually increased to its present

size in one year.

There was no history of redness, pain, discharge, excoriation
of skin and ulceration. Family and personal history for
ocular and systemic diseases was not significant.

A detailed clinical examination demonstrated a cone shaped Figure 2: pre operative slit lamp photograph of cutaneous horn of upper
growth measuring length of 1cm & base of 0.5cm over the eyelid
outer 1/3rd of the right upper eyelid (Figure 1 and 2). The
growth was firm in consistency, non-tender, without any
discharge or bleeding.

Regional lymph node examination was normal.
A clinical diagnosis of cutaneous horn was made. The

Figure 1: pre operative photograph of cutaneous horn of upper eyelid Figure 3: intraoperative photograph of excised cutaneous horn

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DJO Vol. 32, No. 1, July-September 2021

Figure 4: Histopathological photograph showing hyperkeratosis, hyperplastic horns by Yu et al reported that 39% of cutaneous horns
stratified squamous epithelium without invasion of underlying tissue were derived from malignant or premalignant epidermal
lesions and 61% from benign lesions. The relative risk of a
Gross examination of specimen revealed a grey brown malignant base pathology in a cutaneous horn on the eyelids
conical tissue measuring 1.0 cm in length and a base of 0.5 is 2:1, more than that on other part of the body.13
cm.
Other treatment options include electrocautery,
On microscopic examination hyperkeratotic, hyperplastic cryotherapy, carbon dioxide or Nd-YAG laser in patients not
stratified squamous epithelium seen. All these features were willing for excision. Drawback of these methods is that no
suggestive of cutaneous horn with no underlying tissue histopathologic evaluation could be done which is required
invasion (Figure 4). for assessing the malignant potential of the base lesion.14,15

On follow-up, the patient had an uneventful course and the Conclusion
site of wound was healthy and healing.
Cutaneous horns are a rare lesion, which can be diagnosed
clinically as a cone shaped overhanging growth above
the surface of the skin. Histopathological examination of
Discussion these lesions, especially the base of horn is mandatory to
rule out malignant changes. So, it is recommended that
The cutaneous horn alludes to a tumour located on the in every patient with cutaneous horn, the diagnosis of the
epidermis with a hyperkeratotic conical shape, white- cardinal lesion should be done by appropriate biopsy and
yellowish in colour ranging from few millimetres to a few histopathological examination.
centimetres in size.4 In the true cutaneous horn, the height is  
at least one-half the width of the base.5 cutaneous horn arises
due to abnormality in the spinous layer of the epidermis References
that leads to excessive accumulation of keratin and there
is an unusual cohesion of keratinized material which is 1. Oludiran OO, Ekanem VJ. Cutaneous horns in an African
accumulated either superficially or deep in cutis forming population. J Cutan Aesthet Surg. 2011;4:197-200.
a mass of keratin.6 UV ray exposure triggers the condition,
as evident in the higher number of cases reported on face, 2. Requena L, Requena C, Cockerell CJ. Benign epidermal tumors
pinna, nose, dorsal forearms, and scalp. It can involve chest and proliferations. In: Bolognia J, Jorizzo JL, Schaffer JV, eds.
and penis. cutaneous horn is usually seen in males above Dermatology. 3rd ed. Philadelphia, PA: Elsevier Saunders,
sixty years of age.7 2012;1805–06

It accounts for 4% of all eyelid tumours. The composition of 3. Mantese SAO, Diago PM, Rocha A, Berbert AL, Ferreira AK,
these lesions is the same as that of keratotic material which Ferreira TC. Cutaneous Horn: A retrospective histopathological
resembles an animal horn and there is no bony core. study of 222 cases. An Bras Dermatl. 2010;85:157-63.

The most common types of lesions that can be identified 4. Durkee S.Human Horn (CornuCutaneum of Rokitansky). Boston
histopathologically at the base of the cutaneous horn include: Med Surg J. 1866;74:9-11
actinic keratosis, keratoacanthoma, seborrheic keratosis,
pyogenic granuloma, discoid lupus erythematosus, verruca 5. Bart RS, Andrade R, Kopf AW. Cutaneous horn. Acta Derm
vulgaris, epidermal nevus, trichilemmal cyst, trichilemmoma, Venereol (Stockh).1968;48: 507
prurigo nodules, intradermal nevi, Bowen’s disease, basal
cell carcinoma and squamous cell carcinoma.8-12 6. Requena L, Requena C, Cockerell CJ. Benign epidermal tumors
and proliferations. In: Bolognia J, Jorizzo JL, Schaffer JV, eds.
Complete surgical excision of horn is the method of choice Dermatology. 3rd ed. Philadelphia, PA: Elsevier Saunders,
and histopathological examination plays a vital role in 2012:1805–06
further treatment and follow up. It identifies the base lesion
and its malignant potential. In 1991, A study of 643 cutaneous 7. Mencia-Gutierrez E, Gutierrez-Diaz E, Redondo-Marcus I,
Ricoy JR, Garcia-Torre JP. Cutaneous horn of the eyelid; a
clinicopathological study of 48 cases. J Cutan Pathol 2004;31:539-43.

8. RushPS,CockerellCJ,ButlerDF,ChanEF,JamesWD,etal.Cutaneous
Horn: Background, Pathophysiology, Epidemiology. [online]
Emedicine.medscape.com.2015 Link: https://goo.gl/aBtRxU

9. Vano-Galvan S, Sanchez-Olaso A. Images in clinical medicine.
Squamous-cell carcinoma manifesting as a cutaneous horn. N
Engl J Med.2008;359: e10.

10. Findlay RF, Lapins NA.Pyogenic granuloma simulating a
cutaneous horn. Cutis.1983;13:610–12.

11. Dabski K, Stoll HL. Cutaneous horn arising in chronic discoid
lupus erythematosus. Arch Dermatol.1985;12:837–38.

12. Gould JW, Brodell RT. Giant cutaneous horn associated with
verruca vulgaris. Cutis: 1999; 64:111–12.

13. Yu RC, Pryce DW, Macfarlane AW, Stewart TW. A
histopathological study of 643 cutaneous horns. Br J Dermatol
1991; 124: 449.

14. Cruz Guerra NA, Sáenz Medina J, Ursúa Sarmiento I, Zamora
Martínez T, Madrigal Montero R, Diego Pinto D, et al. Malignant
recurrence of a penile cutaneous horn. Arch Esp Urol. 2005;58:61–
63. [PubMed] [Google Scholar]

15. Padmini R, and Pandey N. Eyelid Cutaneous Sebaceous Horn:
A Rare and Interesting Case Report. International Journal of
Scientific Study June 2015; 3:3:135

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DJO Vol. 32, No. 1, July-September 2021

Cite This Article as: Neeraj Sharma, Rupali Kashyap,
Priyanka Kumar, Sonakshi Sehrawat, Soumya Sharma.
A Rare Case Of Upper Eyelid Cutaneous Horn. Delhi J
Ophthalmol 2021; 32(1), 68-70.
Acknowledgments: Nil
Conflict of interest: None declared
Source of Funding: None
Date of Submission: 21 Mar 2020
Date of Acceptance: 31 Mar 2020

Address for correspondence
Neeraj Sharma

Professor Ophthalmology

Department of Ophthamology,
SGT University Gurugram,
Haryana, India
E-mail: [email protected]

Quick Response Code

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DJO Vol. 32, No. 1, July-September 2021

Case Report

Bilateral Non Arteritic Anterior Ischemic Optic Neuropathy
Associated With Optic Nerve Head Drusen And Vascular
Risk Factors

Eathi Tuli, Aakanksha Sharma, Gaurav Sharma, Shalu Gupta

Dr. Rajendra Prasad Government Medical College Kangra at Tanda, Himachal Pradesh, India.

Abstract We report case history of 52 year old male who presented with sudden painless diminution of vision in right eye and with
similar complaint of diminution of vision in left eye six months back. He was diagnosed as Nonarteritic Anterior Ischemic
Optic Neuropathy (NAION) in left eye with Optic Nerve Head Drusen (ONHD) in both the eyes. He was otherwise well and
his medical history revealed systemic hypertension. Fundus examination revealed hyperaemic disc in the right eye and
temporal pallor of the disc in the left eye. On visual field examination, inferior altitudinal visual field defect in both the eyes.
ONHD can mimic as disc oedema and it can very rarely cause NAION. In addition, multiple vascular risk factors can also
cause NAION, hence detailed investigations and periodic follow up should be done to rule out ischemic disc complication.

Delhi J Ophthalmol 2021;32;71-73; Doi http://dx.doi.org/10.7869/djo.694
Keywords: Nonarteritic Anterior Ischemic Optic Neuropathy, Optic Nerve Head Drusen, Inferior Altitudinal Defect.

Introduction was unremarkable in both the eyes. Intraocular pressure
by applanation tonometer was 18 mmHg and16 mm Hg in
Nonarteritic Anterior Ischemic Optic Neuropathy (NAION) right and left eye. Dilated fundus examination showed disc
is the most common acquired primary optic neuropathy oedema with blurring of the disc margin and tortuous blood
caused by infarction of the short posterior ciliary arteries vessels in the right eye and temporal pallor of the optic disc
that supply the anterior portion of the optic nerve head. in the left eye (Figure 1).
NAION typically presents with acute unilateral painless loss
of vision. Primary risk factor for NAION is small crowded Visual field analysis by computerized automatic perimetry
optic disc. Additional risk factors associated with NAION showed an inferior altitudinal defect in both the eyes (Figure
are diabetes mellitus, hypertension, hyperlipidaemia, 2). Optical coherence tomography showed lumpy bumpy
atherosclerosis, obesity.1 ONHD can also cause NAION due internal contour appearance at the optic disc suggestive of
to crowing and compression of axons. Here in, we report a ONHD (Figure 3). B scan showed ovoid echogenic lesion
male patient who had ONHD and associated vascular risk at the junction of retina and optic nerve head suggestive of
factors who developed NAION. ONHD (Figure 4). MRI Brain was within normal limits.

Case Report Laboratory parameters including complete haemogram,
lipid profile, ESR, CRP, ACE levels, peripheral eosinophil
52 year old male presented to eye outpatient department count, coagulation parameters all within normal ranges.
with sudden diminution of vision (DOV) in the right eye
since 2 days, which was painless and progressive in nature, Patient was started on oral steroids, tablet prednisolone70
he noticed sudden DOV while waking up in the morning. mg on tapering dose and was advised to continue
There was no history of ocular pain, redness, photophobia, antihypertensive with regular monitoring of BP and blood
fever or trauma. sugar levels and was counselled to maintain healthy diet.
He had similar episode of sudden DOV in the left eye 6
months back and was diagnosed as NAION in the left eye Discussion
associated with ONHD in both the eyes at our institute.
He was started on oral corticosteroids after which patient NAION presents with acute unilateral painless loss of
noticed mild improvement in his vision. His family vision accompanied by sector or diffuse optic nerve head
history was unremarkable and medical history revealed oedema.2 Generally affects people over 50 years of age, exact
systemic hypertension since 6 months. Patient was on anti- pathogenesis of NAION is unproven, it is a multifactorial
hypertensive drugs, amlodipine, telmisartan and was non- disease and presumed due to transient disruption in the
compliant with the treatment. He did not have any habits circulation of the optic nerve head leading to hypo perfusion
such as smoking or drinking alcohol and had no history of and ischemia.3
recent infections or toxic exposure. On examination, he was
conscious and oriented. His blood pressure was 132/88 mm Optic disc drusen are calcified deposits located in the optic
Hg, with a regular pulse of 72 beats/min. nerve head anterior to the lamina cribrosa. Incidence in the
general population varies from 0.4–2.4%.4 Involvement is
On ophthalmological examination, ocular adnexa, gaze bilateral in 75–86% of cases and is asymmetric.5 Most cases
motility were normal. Pupils were sluggish, with left relative are asymptomatic and diagnosis is established during routine
afferent pupillary defect. Visual acuity was 6/60 and 6/36 eye examination as an incidental fundus finding. Visual
in right and left eye respectively. Slit lamp examination

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DJO Vol. 32, No. 1, July-September 2021

Figure 1 : Dilated fundus examination showed disc oedema with blurring of the disc margin and tortuous blood vessels in the right eye and temporal pallor of
the optic disc in the left eye

Figure 2 : Visual field analysis by computerized automatic perimetry showed an inferior altitudinal defect in both the eyes

Figure 3 : Optical coherence tomography showed lumpy bumpy internal contour appearance at the optic disc suggestive of ONHD

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DJO Vol. 32, No. 1, July-September 2021

obscuration in patients with ONHD is due to increased optic 7 Mullie MA and Sanders M.D. Scleral canal size and optic
disc tissue pressure causing impairment of disc circulation.6 Nerve head drusen. Am J Ophthalmol. 1985; 99(3):356–59

Mullie7 reported small scleral canal with vascular anomalies 8 Gittinger JW, Lessell S, Bondar RL. Ischemic optic neuropathy
in optic disc of patients with ONHD indicating a mesodermal associated with optic disc drusen. J Clin Neuroophthalmol,
dysgenesis of the optic nerve head. Gittinger et al8 reported 1984; 4(2):79-84.
mechanical distortion of blood vessels located in the laminar
and prelaminar region in cases of ONHD predispose them to 9 Hayreh SS, Podhajsky P, Zimmerman MB. Role of nocturnal
develop NAION. Tan et al reported presence of ONHD as an arterial hypotension in optic nerve head ischemic disorders.
independent risk factor for NAION. Ophthalmologica. 1999; 213:76–96.

NAION is associated with many conditions that predispose 10 Purvin V, King R, Kawasaki A, Yee R. Anterior ischemic optic
to decreased optic nerve head perfusion via micro vascular Neuropathy in eyes with optic disc drusen. Arch Ophthalmol.
occlusion such as systemic hypertension, diabetes mellitus 2004; 122:48–53
and hyperlipidaemia. Nocturnal systemic hypotension has
a significant role in the development of NAION, particularly 11 Newman NJ, Scherer R, Langenberg P. The fellow eye
in patients with an exaggerated nocturnal dip.9 in NAION: report from the ischemic optic neuropathy
decompression trial follow-up study. Am J Ophthalmol. 2002;
Hayreh reported nocturnal hypotension as one of the 134(3):317–328
aetiology for NAION. Purvin10 found 50% NAION patients
to have systemic vascular risk factors, but each patient Cite This Article as: Eathi Tuli, Aakanksha Sharma,
had only one at the time of presentation. Ischemic optic Gaurav Sharma, Shalu Gupta Bilateral Non Arteritic
neuropathy decompression trial (IONDT) found 60% to Anterior Ischemic Optic Neuropathy Associated With Optic
have vascular risk factors associated with NAION.11 Nerve Head Drusen And Vascular Risk Factors Delhi J
Ophthalmlogy 2021; 32 (1) 71-73.
In our case report ONHD was documented by using recent
techniques such as OCT and B scan. Our patient was known Acknowledgments: Nil
hypertensive and was non complaint to the treatment,
presented with sudden painless diminution of vision upon Conflict of interest: None declared
awakening suggesting nocturnal hypotension could have
caused NAION. Our medical treatment consisted of systemic Source of Funding: None
steroids. Firm evidence is lacking about the corticosteroid
efficacy on visual function. Mild improvement noted in our Date of Submission: 21 Dec 2018
patient could be due to the natural course of the disease Date of Acceptance: 5 April 2020
other than the treatment effect.
Address for correspondence
Conclusion Eathi Tuli DOMS,DNB,

ONHD rarely can cause NAION, clinicians should be aware Senior Resident Ophthalmology
of this association. Patients with NAION and concomitant
ONHD can have multiple systemic vascular risk factors Department of ophthalmology,
and hence detailed investigation should be performed. DR RPGMC TANDA, KANGRA
Patients with ONHD should be followed up periodically for HIMACHAL PRADESH, India
possibility of ischemic disc complications. Email id:[email protected]

References Quick Response Code

1 Hayreh SS, Zimmerman MB. Nonarteritic anterior ischemic optic
neuropathy: natural history of visual outcome. Ophthalmology.
2008; 115(2):298–305

2 Berry S, Lin WV, Sadaka A, Lee A. Nonarteritic anterior ischemic
optic neuropathy: cause, effect, and management. Eye Brain.
2017; 9: 23–28.

3 Miller NR, Arnold AC. Current concepts in the diagnosis,
pathogenesis and management of nonarteritic anterior ischaemic
optic neuropathy. Eye (Lond) 2015; 29(1):65–79.

4 Perez-Alvarez F, Luque A, Penas A. Bilateral disc drusen as an
important differential diagnosis of pseudotumor cerebri. Brain
Dev. 2007; 29(6):387–388.

5 Hu K, Davis A, O’Sullivan E. Distinguishing optic disc drusen
from papilloedema. Br. Med. J. 2008; 337:a2360.

6 Tan DK, Tow SL. Acute visual loss in a patient with optic disc
drusen. Clin Ophthalmol. 2013; 7:795-99.

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DJO Vol. 32, No. 1, July-September 2021

Case Report

Resolution of Combined Hamartoma of Retina And Retinal
Pigment Epithelium (CHRRPE) with Following Pars Plana
Vitrectomy

Monika Kapoor,1 Siddharth Madan,2 Priyanka Sankaran,1 Preeti Singh,1 Sarita Beri1

1Department of Ophthalmology, Lady Hardinge Medical college and Associated Hospitals, University of Delhi, New Delhi, India.
2Department of Ophthalmology, University College of Medical Sciences and Associated GTB Hospital, University of Delhi, New Delhi, India.

Abstract Combined hamartoma of retina and retinal pigment epithelium (CHRRPE) is a benign tumor located at the posterior
pole. The pathogenesis of the lesion is unknown however some authors propose that the tumor may arise from
undifferentiated ectopic progenitor cells which were otherwise destined for RPE and demonstrate failure to completely
differentiate. These cells then undergo hyperplasia and accumulate in the neurosensory retina. Optical coherence
tomography (OCT) assists in classification of the lesions based on location and differentiates from an epiretinal
membrane (ERM). Surgical intervention is warranted in cases that produce macular distortion with/without associated
ERM or retinal detachment and those producing visual complaints. Similar was the case with a 26-year-old male
who presented with unilateral metamorphopsia that was attributed to CHRRPE with a grade two ERM. Pars plana
vitrectomy with ERM peeling resulted in resolution of disabling symptoms and anatomical success as visible on OCT.

Delhi J Ophthalmol 2021;32;74-75; Doi http://dx.doi.org/10.7869/djo 695

Keywords: CHRRPE, Epiretinal Membrane, Combined Hamartoma Of Retina And Retinal Pigment Epithelium, Pars Plana Vitrectomy, Omega Sign

Introduction Discussion

Combined hamartoma of retina and retinal pigment Dragging of the fovea is seen in all CHRRPE lesions

epithelium (CHRRPE) are rare, solitary, nonhereditary involving the macula and in around 42 % of extra-

unilateral oval to bean shaped tumors that are benign with macular lesions. Fundus photography is very helpful

no malignant potential. These tumors are composed of glial, for documentation and follow-up. OCT findings include

vascular, and RPE components. The lesion is usually an omega-shaped in¬filtration of the inner retina that is lined

ill-defined gray retinal mass with the presence of tortuous posteriorly by the outer plexiform layer. OCT classifies the

or straightened retinal blood vessels. Retinal traction CHRRPE lesions into three zones based on their location i.e.

might also be observed. The lesions are usually stable. macular/peripapillary- Zone1; midperiphery-Zone 2 and

The CHRRPE lesions involve either the optic nerve or the located in far periphery as Zone 3. The lesions in Zone 1

macula. CHRRPE involving the macula show progressive need frequent follow-up however the peripheral lesions can

loss of vision as in the case of a 26-year-old male due to the be observed. The complications associated with CHRRPE

presence of epiretinal glial tissue and vitreous traction.1,2 include reduced VA due to ERM, amblyopia, retinoschisis,

Case report holes, choroidal neovascular membrane or an accompanying
retinal detachment.3
The patient presented with diminution and distortion of

vision in his right eye (OD) for last one year. Best corrected Epiretinal membranes that are associated with eye diseases
visual acuity (BCVA) was 6/12 partial OD with a refractive such as retinal vascular occlusion, diabetic retinopathy,
correction of -0.75 DS/-0.50 DC x 180 degrees and 6/6 (with trauma, retinal detachment, exudation, haemorrhage,
plano) in the left eye (OS). Anterior segment examination and choroidal involvement or any ocular surgical intervention
intraocular pressures were normal in both the eyes. Fundus are called secondary ERM’s. This patient had no signs of
examination OD showed grade two epiretinal membrane intraocular inflammation. Spontaneous separation of ERM’s
(ERM) based on the Gass classification, manifesting with is observed in cases of CHRRPE.4 Combined hamartoma of
macular distortion along-with a CHRRPE (Figure 1a). retina and retinal pig¬ment epithelium contains retinal blood
Fundus examination OS was normal. Fundus fluorescein vessels which appear abnormal and have a certain degree of
angiography showed distortion of vessels near the lesion traction at the vitreoretinal interface and are accompanied
and ERM (Figure 1b). All secondary causes for ERM were with prominent pre-retinal gliosis and tortuosity of the
ruled out. Optical coherence tomography (OCT) OD showed retinal blood vessels. These clinical features of CHRRPE can
the typical omega sign (Figure 1c) and incomplete posterior create diagnostic dilemma with the clinical appearance of
vitreous detachment. In view of deteriorating visual acuity, complex ERM.
vitreoretinal traction (Figure 1d) and surface irregularity,

pars plana vitrectomy (PPV) with ERM peeling without any A diagnosis of CHRRPE is more likely as it usually presents
internal limiting membrane peeling along-with intravitreal early in first or second decade of life and is not associated
20 % sulfur hexafluoride (SF6) injection in the vitreous cavity with underlying causes as mentioned. Moreover OCT helps
was performed. A VA of 6/9 was achieved on post-operative in distinguishing the two entities as CHHRPE lesions have a
day 40 with amelioration of metamorphopsia that was characteristic omega-shaped disorganization of inner retinal
documented on Amsler’s chart (Figure 1e-f).

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DJO Vol. 32, No. 1, July-September 2021

Figure 1: Fundus photograph OD at presentation showing grade two ERM Pigment Epithelium. Retina. 2018 Jan;38(1):12-19.
with an elevated lesion on the superotemporal aspect suggestive of CHRRPE 4. Kumar V. Spontaneous Separation of ERM in Combined
(1a). FFA showed distortion of vessels (1b). OCT OD showed the typical omega
sign characterized by localized thickening of the retina with epiretinal glial Hamartoma of Retina and Retinal Pigment Epithelium.
tissue accompanied-with vitreous traction and bounded posteriorly by the Ophthalmology. 2017 Sep 1;124:1402.
outer plexiform layer (1c-d). Incomplete PVD was also seen on OCT (Fig. 1c- 5. Bruè C, Saitta A, Nicolai M, Mariotti C, Giovannini A. Epiretinal
d). Post- operative fundus (1e) and OCT (1f) at day 40 shows resolution of membrane surgery for combined hamartoma of the retina and
retinal pigment epithelium: role of multimodal analysis. Clin
CHRRPE, vitreous traction and regularization of epiretinal surface. Ophthalmol. 2013;7:179-84.
6. Zhang X, Dong F, Dai R, Yu W. Surgical management of
layers, are bounded posteriorly by the outer plexiform epiretinal membrane in combined hamartomas of the retina and
layer. This was also the case in this patient who no other retinal pigment epithelium. Retina. 2010 Feb;30(2):305-9.
underlying ocular pathology and therefore CHRRPE was a
likely diagnosis. Cite This Article as: Monika Kapoor, Siddharth Madan,
Priyanka Sankaran, Preeti Singh, Sarita Beri. Resolution
Surgery aims to reduce the vitreous traction and establish of Combined hamartoma of retina and retinal pigment
surface regularity. Overlying epiretinal membrane is closely epithelium (CHRRPE) with epiretinal membrane following
linked to the underlying hamartoma therefore forceful pars plana vitrectomy. Delhi J Ophthalmol 2021; 32 (1) 74-75.
peeling of ERM is not advocated due to the anticipated Acknowledgments: Nil
complications.5,6 Reports on resolution of CHRRPE with Conflict of interest: None declared
epi-retinal membrane removal performed during PPV Source of Funding: None
accompanied-with disappearance of omega sign are few. Date of Submission: 06 Feb 2021
Epiretinal membranes may recur after surgery however Date of Acceptance: 02 Apr 2021
VA does improve after primary surgery even if the ERM
recurs. Timely surgical interventions may yield gratifying Address for correspondence
functional and anatomical success. Siddharth Madan

References M.S., D.N.B, FICO, FAICO ( Retina)

1. Kumar V, Chawla R, Tripathy K. Omega Sign: A Distinct Assistant Professor,
Optical Coherence Tomography Finding in Macular Combined Department of Ophthalmology,
Hamartoma of Retina and Retinal Pigment Epithelium. University College of Medical
Ophthalmic Surg Lasers Imaging Retina. 2017 Feb 1;48(2):122125. Sciences and Associated GTB Hospital,
University of Delhi, New Delhi
2. Shields CL, Thangappan A, Hartzell K, Valente P, Pirondini Email: [email protected]
C, Shields JA. Combined hamartoma of the retina and retinal
pigment epithelium in 77 consecutive patients visual outcome Quick Response Code
based on macular versus extramacular tumor location.
Ophthalmology. 2008 Dec;115(12):2246-2252.e3.

3. Dedania Vs, Ozgonul C, Zacks Dn, Besirli Cg. Novel Classification
System For Combined Hamartoma Of The Retina And Retinal

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Case Report

Penetrating Injury Into The Orbit By A Needle Fish:
An Unusual Case of Ocular Trauma

Rajender Singh Chauhan, Apoorva Goel, Chetan Chhikara, Ashok Rathi

Department of Ophthalmology, Pandit Bhagwat Dayal Sharma Post Graduate Institute of Medical Sciences, (PGIMS) Rohtak, Haryana, India.

Abstract Ocular trauma is the most common cause of monocular blindness worldwide. Some cases of ocular trauma can be
challenging to diagnose and manage due to their unique presentation as in this case where a 12 years old boy presented
to our hospital with symptoms mimicking orbital cellulitis. The patient had no obvious signs of injury at presentation
and was not improving on routine treatment. Later on, the patient was found to have an intra-orbital foreign body on
radiological investigations and was managed accordingly. The foreign body was correlated to be a broken needlefish beak
which injured him while swimming in a pond.

Delhi J Ophthalmol 2021;32;76-78; Doi http://dx.doi.org/10.7869/djo.696

Keywords: Ocular Trauma, Orbital Cellulitis, Needle Fish, Foreign Body

Introduction while swimming. However, injury with aquatic organism
like needlefish is rare and only few cases of Orbital injury
Many species of fish are known to cause injuries in human have been reported so far.3,4 Needlefish injuries reported,
beings, some of which may even be fatal. One such fish is consisted of puncture wounds to the orbit, chest, abdomen,
the marine needlefish (garfish), a member of the family spine, arms, legs and neck. Here, we report our experience
Belonidae (Figure 1 and 2). They can be found leapfrogging with penetrating injury into the orbit by a needlefish.
over objects on surface of water. During this leapfrogging
movement in water, they may traumatize humans or other Case Report
living beings by plunging their sharp beaks into anyone in
their flight path.1 Swimming and diving into the local water A 12 years old male presented to our emergency at Sohar
bodies like lakes and pond is a common practice in rural hospital, Sultanate of Oman following one week of treatment
areas. Hence, penetrating injury with organic foreign body in primary health center for orbital cellulitis with systemic
can occur in such people. When a patient presents with a antibiotics. Patient did not get any relief from the treatment
history of motility disturbance, a chronic congestion, irritated and was having persistent edema, redness and pain in left
eye unresponsive to treatment, decreased vision, a fistula in orbit. On careful history, patient revealed that he developed
the area of previous foreign body injury, localized pain or symptoms of lid edema, pain and drooping of eyelid two
pain on eye movement then penetrating orbital injury with days after swimming in sea of Saham area of North Batinah
organic foreign body must be suspected.2 Many case reports region of ‘Sultanate of Oman’. While swimming he felt
have been documented earlier describing penetrating that something had hit his left eye, which was probably a
injuries with wooden foreign bodies and metallic objects flying fish following which he had momentary pain. On
examination in emergency, the visual acuity was 6/12 in
the affected eye. There was lid edema, proptosis (4 mm),
mechanical ptosis, conjunctival congestion, chemosis and
restriction of ocular movements in adduction and abduction
were present and a provisional diagnosis of orbital cellulitis
was made (Figure 3). A trivial mark of injury was seen on
medial canthal region. Plain X-ray of head and orbit did not
reveal anything suspicious. On CT scan, the globe was intact

Figure 1: Upper and lower jaws of needlefish are elongated with long beak
that have small sharp teeth

Figure 2: The needle fish is elongate, silver in color, with a dark blue stripe. Figure 3: Patient’s left eye showing features suggestive of orbital cellulitis

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DJO Vol. 32, No. 1, July-September 2021

but a linear radio-opaque foreign body was seen in medial Discussion
compartment of orbit adjacent to medial rectus.(Figure 4,5,6).
Systemic investigations favored an infective etiology. A Diagnosis and management of penetrating orbital injuries
non- metallic organic foreign body was suspected and on with organic foreign body may present as a diagnostic
taking further history, patient’s parents brought a fish later dilemma and therapeutic challenge as it may follow a
on showing that probably this fish had hit the eye of their relatively minor trauma and may not be identified clinically
child as such injuries were common in that area. However, or on preliminary radiological investigations or might mimic
ocular injuries were uncommon. A trans-conjunctival some other readily diagnosed clinical disease. Radiological
medial orbitotomy was done and bone like structure of investigations help in exact localization of the foreign body,
approximately 3 mm length was removed. Patient was estimation of its consistency and size, confirming integrity
continued on intravenous antibiotics and topical broad- of the globe and evaluation of the response of surrounding
spectrum antibiotics. At postoperative 1 week follow up, the orbital tissue. NCCT scan has been recommended as the
lid edema and proptosis subsided. At 1-month follow-up, imaging modality of choice in all cases of head and ocular
the infection had completely resolved, and visual acuity in trauma. Magnetic resonance imaging (MRI) can be done
the left eye improved to 6/6. Hence, it was concluded that after ruling out ferromagnetic foreign body.5
this injury had occurred while swimming in the sea by the
flying fish, the beak of which was seen as a remnant in the Penetrating orbital injury due to needlefish is a rare
orbit. occurrence. The needlefish is elongate, silver in color, with

Figure 4: Coronal section of CT scan showing radiopaque hyperdense foreign Figure 5: Transverse section of CT scan showing radiopaque hyperdense
body lodged in medial compartment of left orbit. foreign body lodged in medial compartment of left orbit.

Figure 6: Plain X-ray skull PNS view not showing any radiopaque foreign body. a dark blue stripe. The upper and lower jaws are elongated
with long beaks that have small sharp teeth. Needlefish do
not produce venom. Therefore, treatment of the injury is
similar to penetrating injury, and it is determined primarily
by the site of damage.6

In our patient, the foreign body was trapped in the medial
compartment of the left orbit after traumatizing the medial
canthus and crossing the medial rectus muscle. The wound
was small and obscured but resulted in eye movement
disorder due to the retained organic foreign body in the
orbit. CT scan was beneficial for discovery of the orbital
foreign body whose Hounsfield (HF) unit value was more
than that of bone (700) but less than that of metal (> 3000)
which was consistent with the long elongated beak of the
Needlefish.

Conclusion

Our case report highlights the fact that a retained foreign
body should be kept in mind as a differential diagnosis
in cases of ocular trauma masquerading cellulitis after a

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DJO Vol. 32, No. 1, July-September 2021

high velocity mechanism of injury, such as swimming or Cite This Article as: Rajender Singh Chauhan, Apoorva
diving in waterbodies. These foreign bodies can be both Goel, Chetan Chhikara, Ashok Rathi. Penetrating Injury
organic like wood, plant or aquatic organism or inorganic into the Orbit by a Needlefish: An Unusual Case of Ocular
like metal or glass. These injuries represent a true threat to Trauma Delhi J Ophthalmol 2021; 32 (1), 76-78.
vision. Hence, emphasis should be laid on thorough history Acknowledgments: Nil
taking to explore the mechanism of injury and meticulous Conflict of interest: None declared
ophthalmological examination. Source of Funding: None
Date of Submission:17 Nov 2020
References Date of Acceptance: 19 Apr 2021

1. McCabe MJ, Hammon WM, Halstead BW, Newton TH. A fatal Address for correspondence
Rajender Singh Chauhan MS
brain injury caused by a needlefish. Neuroradiology 1978;
Department of Ophthalmology, Pandit
15:137–9. Bhagwat Dayal Sharma Post Graduate
Institute of Medical Sciences, (PGIMS)
2. Liu D. Common denominators in retained orbital wooden Rohtak, Haryana, India.
Email: [email protected]
foreign body. Ophthalmic Plast Reconstr Surg 2010; 26(6):454-8.

3. Ohtsubo M, Fujita K, Tsunekawa K, Yuzuriha S, Matsuo K.

CASE REPORT Penetrating Injury of the Orbit by a Needlefish.

Eplasty. 2013 Aug 6;13:e41.

4. Miller KE, Coan EB. Penetrating Orbital Injury From a

Needlefish. Mil Med. 2016 Aug;181(8):e962-4.5. Krimmel

M, Cornelius CP, Stojadinovic S, Hoffmann J, Reinert S. Wooden

foreign bodies in facial injury: a radiological pitfall. Int J Oral

Maxillofac Surg 2001; 30(5):445-7.

6. Al-Mujaini A, Al-Senawi R, Ganesh A, Al-Zuhaibi S, Al-Dhuhli

H. Intraorbital foreign body: clinical presentation, radiological

appearance and management. Sultan Qaboos Univ Med J 2008;

8(1):69-74.

Quick Response Code

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Case Report

An Unusual Case of Surma (Eye Cosmetic) Induced Conjunctival
and Scleral Ulceration

Chandana Chakraborti,1 Nabanita Barua,2 Ajoy Kr Saha,3 Rupanjili Lakra3

1Department of Ophthalmology, Regional Institute of Ophthalmology, Medical College and Hospital, West Bengal, India.
2Department of Ophthalmology, Deben Mahata Medical College and Hospital, Purulia, West Bengal, India.
3Department of Ophthalmology, North Bengal Medical College (NBMCH) Siliguri, West Bengal, India.

Abstract Surma is traditionally applied to conjunctiva and eyelids for cosmetic purpose in many centuries. It contains preservatives
and additives like lead, nickel, mercury that can lead to dry eye , blepharitis, allergic conjunctivitis, iritis and periocular
dermatitis. We report a case of “surma” induced conjunctival & partial thickness scleral ulceration in a 60 years old female.
She presented with sudden swelling and burning sensation in right eye after applying ‘surma’ in her right eye. Conjunctival
and scleral ulceration was noted subsequently. She was treated conservatively with topical and oral steroid. This unusual
complication of surma is not documented in literature.

Delhi J Ophthalmol 2021;32;79-81; Doi http://dx.doi.org/10.7869/djo.697

Keywords: Surma, Sterile Ulcer, Sclera, Conjunctiva

Introduction (Figure 1a,1b), with a normal pupillary reaction. The
pigmentation could not be removed with cotton pellet and
Worldwide, from prehistoric time female population has saline irrigation .
been using various eye cosmetics due to various reasons,
from improving appearance to increasing self-confidence, She was started with topical Moxifloxacin+ Dexamethasone
cultural practices.1-3 There are reports of two kinds of surface along with preservative free topical lubricant 6 times a day
application, first on periocular skin just external to lash line, in right eye.
second on muco-cutaneous junction along lid margin (called
lightlining).4 Various ocular side effects are lead toxicity, Inflammatory signs started reducing from 3rd day onward.
evaporative dry eye, reduced Ocular Surface Disease Index As the pigmentation gradually started to fade away,
(OSDI), ocular inflammatory changes are well established 5-7 conjunctival and scleral ulceration was revealed (Figure2).
in literature specially in contact lens users. We report a rare Swab from ulcer bed and lower fornix was sent for
case where conjunctival ulceration and scleral thining was microbiological investigation and it was negative.
noted after using surma. Patient was started on oral prednisolone 40mg (1mg/kg/
day). The patient improved clinically after fifth day. Steroid
Case Report: A 60- years -old female presented with sudden was tapered over a period of 2 weeks. Her vision and IOP
swelling and burning sensation in right eye after applying was stable. We thought of scleral patch graft, but the patient
‘surma’ in right eye. Patient had history of occasional use refused any surgical intervention. At 1 month follow up, a
of ‘surma’ in past without any reactions. She was suffering vascularized scar (Figure 3) was found covering the thinned
from mild itching in right eye for 2 days prior to this sclera. Subsequently, the patient was lost in follow up.
incidence. As a part of prevalent therapy in her locality she
applied ‘surma’ in her right eye to get relief from itching. The used surma bottle (Figure 1b) was sent to central forensic
This current episode occurred after applying a new bottle of lab to look for the nature of the material. Report shows a
‘surma’ from her locality. heterogeneous mixture of Manganese and Potassium
compounds, mainly in the form of oxides or carbonates.
Her visual acuity was 20/40 in right eye and 20/20 in left eye. Elemental carbon, Fluorine, Molybdenum compounds
Right eye had periorbital swelling, severe chemosis, blackish (preferably oxides) were also present in trace amount.
pigmentation of lower fornix and lower tarsal conjunctiva We counseled the patient to discontinue the eye cosmetic to

Figure 1a: Right eyelid swelling with conjunctival chemosis Figure 1b: : Black colored pigmentation of lower fornix and lower tarsal
conjunctiva of right eye with inset showing used bottle of surma
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79 Delhi Journal of Ophthalmology

DJO Vol. 32, No. 1, July-September 2021

Figure 2: Ulceration of sclero-conjunctiva but for till 1 year of follow up the thinning area was non
progressive and symptoms also improved. So we decided
prevent further exposure to offending substance. We also to wait and observe. Worldwide there is sufficient literature
advised her for regular follow up, seek proper advice from on over use of eye cosmetics but this large dose toxicity and
ophthalmologists in case of any ocular ailment (blepharitis grave consequence has never been reported. There is a need
in her case) and to avoid self medication. of creating awareness through education, via media, panel
discussion etc regarding the correct method, side effects of
Discussion continual and over use of ocular cosmetics. Many researchers
have called for stricter governmental regulations of kohl
The ocular surface spread of externally applied cosmetic trade and quality control and improved public education
products are well documented in literature.8 It may affect regarding its hazards and some governments have adopted
the lipid layer due to more debris or blockage in meibomian import controls and educational campaigns to alert users to
gland leading to dry eye.9 It is also shown that deeper the hazards of using Pb-containing kohl.
application (on muco-cutaneous junction ) incites poorer
OSDI than surface (periorbital skin). In our case the sample of used surma was found to contain
some unusual components that are not reported earlier.
Ocular dermatitis can occur in response to fragrances, These components could be the cause of such serious ocular
preservatives, antioxidants, nickel, lead, mercury containing complications. As the microbiological report was negative
pigments, and various additives. Inflammatory mediators so, we diagnosed our case as sterile ulceration. As the
released in abundance in tears during the process lead to episode started immediately after application of the surma
epithelial damage, goblet cell loss, and disturbed glycocalyx so, we can assume that the ulceration was surma induced.
mucin expression exacerbating dry eye condition.10
We planned autologous lamellar sclera graft in this patient Organic colourants fall into two classes: dyes and pigment.
The differentiation point is that dyes are soluble in water or in
organic solvent, while pigments are insoluble. Dyes can color
substances for which they have affinity whereas pigments
can be used to colour any substance that too surface-only
colouration. The bottle also showed black colored particles
which stained superficial layer of conjunctiva. The coloring
could be removed after few days repeated washing and
steroid combination. So surma can be considered as pigments
and not chemical dye.

Worldwide, there is sufficient literature on use of eye
cosmetics and its complications, but such grave consequence
has never been reported. There is a need of creating public
awareness through regarding the correct method and
possible side effects of continual and over use of ocular
cosmetics.

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80

DJO Vol. 32, No. 1, July-September 2021

References Cite This Article as: Chakraborti Chandana, Barua
Nabanita, Ajoy Kr Saha, Rupanjili Lakra. An unusual
1. Murube J. Ocular cosmetics in ancient times. Ocul Surf. case of Surma (eye cosmetic) induced conjunctival and
2013;11(1):2–7. scleral ulceration. Delhi J Ophthalmol 2021; 32; (1) 79-81.
Acknowledgments: Nil
2. Coroneo MT, Rosenberg ML, Cheung LM. Ocular effects of Conflict of interest: None declared
cosmetic products and procedures. Ocul Surf. 2006;4(2):94–102. Source of Funding: None
Date of Submission: 16 Sep 2020
3. Murube J. Ocular cosmetics in modern times. Ocul Surf. 2013; Date of Acceptance: 08 May 2021
11(2):60–4.
Address for correspondence
4. Ng A, Evans K, North RV, Jones L, Purslow C. Impact of eye Nabanita Barua Assistant Professor
cosmetics on the eye, adnexa, and ocular surface. Eye Contact
Lens. 2016;42(4): 211–20. Department of Ophthalmology,
Deben Mahata Medical College and
5. Wang MT, Craig JP. Investigating the effect of eye cosmetics on Hospital, Purulia, West Bengal, India.
the tear film: current insights. Clin Optom (Auckl). 2018;10:33-40 Email: [email protected]

6. El Safoury OS, El Fatah DS, Ibrahim M. Treatment of periocular
hyperpigmentation due to lead of kohl (surma) by penicillamine:
a single group non-randomized clinical trial. Indian J Dermatol. Quick Response Code
2009;54(4):361-3.

7. Dogra A, Dua A. Cosmetic dermatitis. Indian Journal of
Dermatology; 50(4):191-5.

10. V, Jain V, Gupta P. Structural and functional outcome of scleral
patch graft. Eye. 2007;21(7):930-5.

8. Goto T, Zheng X, Gibbon L, Ohashi Y. Cosmetic product
migration onto the ocular surface: exacerbation of migration
after eyedrop instillation. Cornea. 2010;29(4):400-3.

9. Ng A, Evans K, North RV, Purslow C. Migration of cosmetic
products into the tear film. Eye Contact Lens. 2015;41(5):304–9.

10. Bron AJ, de Paiva CS, Chauhan SK, et al. TFOS DEWS II
Pathophysiology Report. Ocul Surf. 2017;15(3):438–510.

11. Allen RLM (1971). Colour Chemistry. London: Thomas Nelson
and Sons Ltd. pp. 11–13.

12. IARC Working Group on the Evaluation of Carcinogenic
Risks to Humans. Some Aromatic Amines, Organic Dyes,
and Related Exposures. Lyon (FR): International Agency for
Research on Cancer; 2010. (IARC Monographs on the Evaluation
of Carcinogenic Risks to Humans, No. 99.) GENERAL
INTRODUCTION TO THE CHEMISTRY OF DYES.

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Case Report

Chronic Anterior Uveitis: Diagnosing Primary Sjogren’s

Srijita Mitra,1 Sahil Sarpal,1 Abhijit Chattopadhyay,2 Subhankarsri Paul,2 Jonaki Ghosh Roy2

1Department of Ophthalmology, Sarpal Eye Hospital, Amritsar, Punjab, India.
2Department of Retina And Uvea Services, Priyamvada Birla Aravind Eye Hospital, Kolkata, India.

Abstract Chronic anterior uveitis is a rare ocular manifestation in primary Sjogren’s syndrome and is not commonly reported. In our
case report, we discuss how ophthalmological findings in a patient supported her being diagnosed with primary Sjogren’s.
Meticulous history taking, Anterior segment photography, FFA, serological examination, aid of a rheumatologist was
taken. Following ophthalmic evaluation and extensive laboratory tests, patient was diagnosed as primary Sjogren’s. Close
monitoring and combination immunosuppressives were relevant in the treatment process.

Delhi J Ophthalmol 2021;32; 82-83; Doi http://dx.doi.org/10.7869/djo.698

Keywords: Autoimmune Complexes, Chronic Anterior Uveitis, , Immunosuppressives, Primary Sjogren’s

Introduction During her treatment, she had a recurrent attack after
a quiescent phase of 6 months in the same eye. In the
Sjogren’s syndrome is characterized by autoimmune following months, Methotrexate was dosed upto 7.5mg/
inflammation of exocrine glands. The condition is classified week. She had a relapse after 3 months with severe AC
as primary when it exists in isolation and secondary when reaction along with mild vitritis. Her fundus during this
associated with diseases such as rheumatoid arthritis, SLE, episode revealed a few hypopigmented spots which showed
systemic sclerosis, mixed connective tissue disease, primary no leakage on FFA. Her systemic medications at this point
biliary cirrhosis, chronic active hepatitis and myasthenia were, Hydroxychloroquine (300mg), Methotrexate (15mg),
gravis.1 Primary Sjogren’s syndrome affects females more Deflazacort (6mg) and Folic acid. During her treatment at
commonly than males (9:1) and begins in 4th- 5th decade our facility for a period of 18 months, she has had relapsing
running a course over 8-10 years.2 episodes atleast 4 times, each episode treated for atleast 3
months.
The cardinal ocular sign in Sjogren’s is keratoconjunctivitis
sicca (KCS). The ocular manifestations are posterior Discussion
blepharitis, meibomian gland dysfunction, punctate
epithelial erosions in cornea and anterior uveitis. Posterior Primary symptoms of Sjogren’s are a dry mouth and eyes.
segment involvement is very rare. Other symptoms can include dry skin, vaginal dryness,
chronic cough, numbness in the limbs, tiredness, muscle and
Case report joint pains and thyroid problems.

A 43 year old woman presented with complaints of occasional Our reported case was not a diagnosed patient of Sjogren’s
redness and floaters and history of use of topical steroids before being examined in our clinics for her ophthalmic
since 15 years. She had a systemic history of hypothyroidism condition. On probing into her personal history, she
(started on medication recently) Her BCVA at presentation had only to account for frequent gritty sensation in eyes,
was 6/9 (RE) and 6/12 (LE). Her right eye anterior segment hypothyroidism, peripheral tingling sensation and myalgia;
revealed granulomatous KP’s , heterochromia, AC cells 3+, symptoms which are vaguely suggestive of a possible
flare 2+, pseudophakia, no evidence of synechiae or iris connective tissue disorder. Our laboratory findings directed
nodules (Figure 1,2). She had cells in AVF and mild vitritis us to a provisional diagnosis and finally with the help of
and no abnormality in the fundus. She was started on the rheumatologist we could give specific treatment to our
topical steroids , cycloplegics and given a battery of blood patient.
investigations. She had raised ESR and a positive ANA (1:
1280 speckled pattern). Rosenbaum and Bennett described a series of eight patients
reporting that in all cases uveitis was bilateral and chronic;
She was referred to a Rheumatologist who diagnosed anterior and posterior disease (but no chorioretinitis) with
her as a possible case of primary Sjogren’s. Her SS-A, posterior synechiae, cataract and pars plana exudation being
RO-52 and SSB antibodies were strongly positive. She common .3 These signs are comparable in our patient except
was started on Deflazacort (6mg), Methotrexate (5mg), for unilaterality. Circulating immune complexes namely
Hydroxychloroquine (200mg) and Folic acid and also cryoglobulins and autoantibodies have been associated
advised lip biopsy. Her topicals were continued and tapered with both Sjogren and anterior uveitis. Other manifestations
during relapsing episodes. Her dry eye work up revealed: of immune complex-mediated disease include palpable
Schirmers (I &II) <5 mm, corneal/conjunctival staining score, purpura suggestive of leukoclastic vasculitis as described
TBUT> 10s, Tear film height < 0.5ml, aqueous deficiency in a case by Bridges and Burns.4 in their article they have
noted. She was started on cycloimmune topical and advised stressed the efficacy of combination of immunosuppressive
punctual plugging. therapy in treating acute conditions and close monitoring

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DJO Vol. 32, No. 1, July-September 2021

of such cases. Cytopenia, uveitis, pericarditis, pleuritis, Cite This Article as: Srijita Mitra, Sahil Sarpal,
interstitial lung disease, vasculitis, monoclonal gammapathy Abhijit Chattopadhyay, Subhankarsri Paul, Jonaki
of unknown significance and non-Hodgkin lymphoma only Ghosh Roy. Chronic Anterior Uveitis: Diagnosing
occurred in the patients without wide spread pain and high Primary Sjogren’s. Delhi J Ophthalmol 2021; 32; (1),
anti-SSB antibodies in primary Sjogren as noted by Borg and 82-83.
Kelder.5 Acknowledgments: Nil
Conflict of interest: None declared
Conclusion Source of Funding: None
Date of Submission: 06 Aug 2020
Thorough history taking and close monitoring of cases Date of Acceptance: 21 Dec 2020
of uveitis are very relevant in treating conditions with
autoimmune etiology. Combination of immunosuppressives Address for correspondence
under proper guidance of a rheumatologist is the key to Srijita Mitra MS
treating such refractory cases.
Department of Vitreo retina
References consultant, Sarpal Eye Hospital,
Amritsar, Punjab, India.
1. Kanski & Bowling’s Clinical Ophthalmology: a systematic Email: [email protected]
approach. Editors: Ken Nischal, Andrew Pearson.7th
edition;p.124 Quick Response Code

2. Stephen J. Ryan’s Retina. Editors : Andrew Schachat et al. 6th
edition ; p.1609

3. Rosenbaum JT1, Bennett RM. Chronic anterior and posterior
uveitis and primary Sjögren's syndrome. Am J Ophthalmol. 1987
Oct 15;104(4):346-52.

4. Bridges AJ1, Burns RP. Acute iritis associated with primary
Sjögren's syndrome and high-titer anti-SS-A/Ro and anti-SS-B/
La antibodies. Treatment with combination immunosuppressive
therapy. Arthritis Rheum. 1992 May; 35(5):560-3.

5. ter Borg EJ1, Kelder JC. Lower prevalence of extra-glandular
manifestations and anti-SSB antibodies in patients with primary
Sjögren's syndrome and widespread pain: evidence for a
relatively benign subset. Clin Exp Rheumatol. 2014 May-Jun;
32(3):349-53. Epub 2014 Feb 11.

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DJO Vol. 32, No. 1, July-September 2021

Case Report

Choroidal Detachment in Idiopathic CD4 Lymphocytopenia

Avadhesh Oli,1 Raja Narayanan,2 INHS Asvini,1 Navy Nagar, Colaba,2 Smt Kanuri Santhamma

1Department of Center for Vitreo Retina Services L. V. Prasad Eye Institute, Hyderabad, India.
2Department of Center for Vitreo Retina Services L. V. Prasad Eye Institute, Mumbai , India.

Usually reduction in CD4 cell count is seen with HIV infection, however CD4 lymphocytopenia in absence of HIV infection

is called as Idiopathic CD4 Lymphocytopenia (ICL). The patients usually present with features of immunosuppression and

opportunistic infections. Ophthalmic manifestations of this rare entity are not well known and very few cases are reported

till date. We present a case of a 69 year old male patient who reported with profound reduction in his visual acuity in

Abstract his left eye for 15 days. On examination of his left eye the visual acuity was hand motion close to face and he had 360
degree choroidal detachment which was confirmed on a B scan. He had evidence of subretinal fluid on optical coherence

tomography (OCT). His detailed uveitis workup was unremarkable and HIV test was negative twice repeated at two

occasions six weeks apart. His CD4 count was 57 cells/ mm3 with all other investigations normal. This report highlights

that iIdiopathic CD4 lymphocytopenia is a rare entity which usually presents as opportunistic infections as reported in the

literature. This case of ICL had choroidal detachment which is unusual presentation of this rare entity.

Delhi J Ophthalmol 2021;32; 84-87; Doi http://dx.doi.org/10.7869/djo.699

Keywords: Choroidal Detachment, Idiopathic CD4 Lymphocytopenia, Immunosuppression

Introduction A) Extra ocular movements were full and free. Intraocular
pressure was 14 mm Hg by non-contact tonometry in both
CD4 cells have important role in the immune mechanism the eyes. He was subjected to multimodal imaging.
of the body and reduction in CD4 cell count may lead
to opportunistic infections and reduced immunity. Low On fundus fluorescein angiography (FFA) multiple pin point
CD4 counts are commonly reported in the setting of HIV areas of hyper fluorescence were noted with late staining
infection, however few patients without HIV who present of the disc. (Figure 1 B,C) Optical coherence tomography
with opportunistic infections may have low CD4 cell counts (OCT) showed a neuro-sensory detachment with sub retinal
on extensive investigations. This entity was later reported precipitates without any evidence of intraretinal fluid.
as idiopathic CD4 lymphocytopenia (ICL), which is a rare (Figure 1 D) Ultrasound B scan in the left eye showed 360
condition with low CD4 counts in absence of HIV infection. degrees choroidal detachment. There was no intra-ocular
The CD4 cell counts are usually less than 300 cell/mm3 or mass noted on B-scan. No subtenon fluid was evident.
20% of total T cells on two separate occasions1. Most of (Figure 2 A-C) UBM confirmed a ciliochoroidal detachment.
the cases of ICL have been described with systemic and No gross pathological choroidal or orbital mass lesion was
ophthalmic opportunistic infections like viral retinitis. We noted in the orbit on MRI however orbital inflammation was
present a rather rare case of elderly male reporting with suspected.
reduced vision and choroidal detachment (CD). He was
investigated thoroughly and was found to have low CD4 Baseline blood investigations including a complete
counts with a HIV negative status. hemogram, peripheral blood smear, lipid profile, renal and
liver function test and thyroid profile were normal. Detailed
Case report uveitis workup including ESR, CRP, Mantoux test, serum
angiotensin converting enzyme levels, chest x ray, syphilis
A 69 year old patient presented with profound reduction of serology, TORCH titers and HIV serology were normal.
vision in his left eye of 15 days duration. He gave a history
of intermittent fever preceding the episode. He denied any LE Orbital inflammation was suspected on MRI, and a trial
history of pain, redness, flashes floaters, trauma or surgery of oral steroids along with antibiotic cover was given. Over
to the fellow eye and unexplained weight loss . He was a a period of next 2 weeks, he showed slight improvement in
known case of diabetes mellitus and hypertension for the vision in left eye to counting fingers close to face (CFCF).
past 10 years, optimized on medication. (Figure 3) Over a period of 1 month his CD were persistent
but reduced in height, however his poor vision was
At presentation, the visual acuity was 20/20 in right eye (RE) unexplained. He was further investigated for CD4 counts,
and hand motions in left eye (LE). RE anterior segment and and a repeat HIV test. The HIV was repeated with Tridot
fundus was normal. He had relative afferent pupillary defect and ELISA which was negative. His CD4 count was 57 cells/
in the LE. Rest of the anterior segment was normal except mm3 and was reconfirmed twice. During his last follow-
posterior chamber intraocular lens in the capsular bag. up after 4 months his CDs had resolved with reduction in
Anterior chamber was quiet with no evidence of cells or flare. subretinal fluid on OCT but the vision remained stable.
Fundus evaluation revealed elevated brownish mounds 360 (Figure 3 A-D)
degree in LE suggestive of peripheral choroidal detachment
(CD). The retina was attached without any evidence of
vasculitis, retinal hemorrhages or disc edema. (Figure 1

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DJO Vol. 32, No. 1, July-September 2021

Figure 1: (A) LE Fundus photograph at presentation (B,C) LE FFA showing peripheral CD and multiple hyper florescent dots (D) LE OCT showing neurosensory
detachment and choroidal granularity

Figure 2: (A) LE Ultrasound biomicroscopy (UBM) showing Cilio-choroidal effusion,
(B, C) Ultra sound B scan LE showing thickened choroid and peripheral choroidal detachment

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DJO Vol. 32, No. 1, July-September 2021

Figure 3: (A) LE fundus picture at 4 months follow up
(B) LE OCT showing reduced neurosensory detachment and choroidal granularity at 4 months
(C, D) Ultra sound B scan LE resolved peripheral CDs seen with reduction of choroidal thickening.

Discussion This case of choroidal detachment in ICL is unique because
of its rarity. CD in this case occurred most probably due
Idiopathic CD4 lymphopenia (ICL) is a rare disorder to unexplained inflammation in the choroid. The exact
characterized by low CD4 counts in the absence of HIV cause of CD in ICL is not known but the disc leak on FFA
infection. A cut off value of 300 cells/ mm3 or count less and resolution of effusion on oral steroids point towards
than 20% of total WBC on two separate occasions has inflammatory pathology. This case highlights an unreported
been used in various studies.2 The common manifestations infrequent presentation of a rather rare disease. The
of ICL are opportunistic infections like cryptococcal ophthalmologist should have a high index of suspicion to
meningitis, tuberculosis, human papilloma virus infection. identify other life-threatening diseases and opportunistic
The ophthalmic manifestations are very rare. On peer infections associated with ICL. The challenge in our case lies
reviewed literature search, very limited data is available in avoiding the similar complications in the fellow eye, in
on patients of ICL presenting with only ophthalmic contrast to HIV where in drugs can help to improve the
manifestations. Progressive outer retinal necrosis (PORN) CD4 cell count.
has been reported by Gupta et al in 2 cases of ICL.3 They
concluded that Varicella zoster necrotizing retinitis, which References
is classic for PORN can occur in HIV negative patients.
Idiopathic CD4 lymphocytopenia should be considered in 1. Smith DK, Neal JJ, Holmberg SD. Unexplained opportunistic
healthy patients who develop ocular infections as seen in infections and CD4+ T-lymphocytopenia without HIV infection.
the immunocompromised patients. Hoang et al reported a An investigation of cases in the United States. The Centers for
case of recurrent cytomegalovirus (CMV) retinitis in an HIV- Disease Control Idiopathic CD4+ T-lymphocytopenia Task Force.
negative patient with CD4+ T lymphocytopenia.4 A similar The New England journal of medicine. 1993;328(6):373379.
case of CMV retinitis was presented by Sloan et al in HIV
negative patient.5 2. Zonios D, Sheikh V, Sereti I. Idiopathic CD4 lymphocytopenia:
a case of missing, wandering or ineffective T cells. Arthritis
Research & Therapy. 2012;14(4):222.

3. Gupta M, Jardeleza MS, Kim I, Durand ML, Kim L, Lobo AM.
Varicella Zoster Virus Necrotizing Retinitis in Two Patients with

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DJO Vol. 32, No. 1, July-September 2021

Idiopathic CD4 Lymphocytopenia. Ocular immunology and Cite This Article as: Avadhesh Oli, Raja Narayanan,
inflammation. 2016;24(5):544-548. INHS Asvini, Navy Nagar, Colaba, Smt Kanuri
4. Hoang QV, Simon DM, Kumar GN, Oh F, Goldstein DA. Santhamma. Choroidal Detachment in Idiopathic CD4
Recurrent CMV retinitis in a non-HIV patient with drug- Lymphocytopenia. Delhi J Ophthalmol 2021; 32; 1, 84-87.
resistant CMV. Graefe's archive for clinical and experimental
ophthalmology = Albrecht von Graefes Archiv fur klinische und Acknowledgments: Nil
experimentelle Ophthalmologie. 2010;248(5):737-740.
5. Sloan DJ, Taegtmeyer M, Pearce IA, Hart IJ, Miller AR, Conflict of interest: None declared
Beeching NJ. Cytomegalovirus retinitis in the absence of HIV
or immunosuppression. European journal of ophthalmology. Source of Funding: None
2008;18(5):813-815.
Date of Submission: 22 May 2020
Date of Acceptance: 26 July 2020

Address for correspondence
Raja Narayanan MS

Director and Network Head
Department of Center for Vitreo
Retina Services L. V. Prasad Eye
Institute, Mumbai, India.
Email: [email protected]

Quick Response Code

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DJO Vol. 32, No. 1, July-September 2021

Case Report

Bilateral Buried Optic Nerve Head Drusen Mimicking Idiopathic
Intracranial Hypertension

Juhi Sahu, Muthukrishnan Vallinayagam, C Alekhya

Department of Ophthalmology, Mahatma Gandhi Medical College & Research Institute, Pondicherry, Sri Balaji Vidyapeeth, India.

Abstract Optic nerve head (ONH) drusen are buried deposits in the optic nerve head simulating papilloedema. Idiopathic
Intracranial Hypertension (IIH) signifies raised intracranial pressure in the absence of intracranial space occupying lesion.
A 35-year old woman diagnosed elsewhere with IIH and treated with oral acetazolamide for six months, presented with
non-specific headache. Fundus showed bilateral blurring of disc margins, elevated disc and obscuration of cup. MRI of
brain was normal. The non- resolution of disc edema despite six months of acetazolamide therapy raised suspicion of an
alternate diagnosis. Ultrasound B-scan confirmed the diagnosis of ONH drusen. ONH drusen should be excluded before
entertaining the diagnosis of IIH.

Delhi J Ophthalmol 2021;32; 88-90; Doi http://dx.doi.org/10.7869/djo.700

Keywords:Buried Optic Nerve Head Drusen, Idiopathic Intracranial Hypertension, Pseudopapilloedema

Introduction acetazolamide therapy for six months, presented with
intermittent episodes of “vague” headache. She denied
Optic nerve head (ONH) drusen are abnormal calcified ocular complaints. Barring the headache, there was no
mitochondrial deposits resulting from impaired axonal history suggestive of raised intracranial pressure. The visual
metabolism.1 Idiopathic Intracranial Hypertension (IIH) is acuity and pupillary examination were normal bilaterally.
a headache syndrome characterized by papilloedema with The fundus examination revealed bilateral blurring of disc
normal cerebrospinal fluid (CSF) composition and normal margins, elevated disc and obscuration of physiological
neuroimaging.2,3 Pseudopapilledema is an apparent optic cup (Figure 1). MRI of brain was normal (Figure 2). Visual
disc swelling secondary to a benign process such as buried field charting (HFA 24-2) was unremarkable (Figure 3).
Optic Nerve Head (ONH) drusen, hypoplastic, tilted or The persistence of disc edema despite strict compliance
hyperopic disc. ONH drusen accounts for 75% of clinically with medical therapy for six months aroused a clinical
diagnosed cases of pseudopapilledema.4 suspicion with respect to an alternate diagnosis. Ultrasound
B-scan showed highly reflective deposits which persisted
Case Report on reducing gain, suggesting ONH drusen (Figure 4). The

A 35-year old woman diagnosed with Idiopathic Intracranial
Hypertension (IIH) elsewhere and commenced on oral

Figure 1: Fundus photograph showing bilateral blurring of disc margins, elevated disc and obscuration of physiological cup

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DJO Vol. 32, No. 1, July-September 2021

Figure 2: MRI of brain showing normal picture optic nerve sheath diameter was within normal limits.

Acetazolamide therapy was discontinued and patient was

reassured. Discussion

Idiopathic Intracranial Hypertension (IIH) or pseudotumor
cerebri is characterized by papilledema in the presence
of normal cerebrospinal fluid composition and normal
neuroimaging. Modified Dandy’s criteria for the diagnosis
of IIH includes signs and symptoms of raised intracranial
pressure (ICP), absence of localising findings on neurological
examination, normal neuroimaging, elevated CSF opening
pressure >200 mm of water (>250 in obese) in an awake and
alert patient, with no other cause for raised ICP.
ONH drusen is a rare congenital optic disc anomaly seen in
2% population. It is hypothesized to occur due to abnormal
optic disc vasculature causing axonal ischemia and impaired
axonal metabolism. The optic disc appears elevated with
indistinct margins and a localized lumpy appearance. ONH
drusen tends to evolve over the years, from being buried
to becoming exposed in the setting of optic atrophy and
reduction of retinal nerve fiber layer (RNFL) thickness.
Although often asymptomatic, ONH drusen is associated
with visual field defects in 87% of cases which may be
arcuate defects, enlargement of blind spot or peripheral
constriction of visual field. Vision threatening complications
like Anterior ischemic optic neuropathy (AION), retinal
vascular occlusions, choroidal neovascular membrane and
retinal haemorrhages have been reported.5 Though the
classical fundus appearance confirms an exposed drusen, a
definitive diagnosis of buried drusen remains a challenge.4

Figure 3: Visual field charting (HFA 24-2) was unremarkable

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DJO Vol. 32, No. 1, July-September 2021

Figure 4: Ultrasound B-scan showed highly reflective deposits which persisted on reducing gain, suggesting ONH drusen

A buried ONH drusen can cause an elevated disc with Raised Intracranial Pressure: A Cross-Sectional Study.
blurred margins and thereby mimic papilloedema.5 Orbital de Carvalho M, editor. Neurol Res Int. 2015 Mar
ultrasound offers a non-invasive modality in establishing 22;2015:146059.
the diagnosis.
5. Palmer E, Gale J, Crowston JG, Wells AP. Optic Nerve Head
The diagnosis of IIH is to be reconsidered in a patient with an Drusen: An Update. Neuro-Ophthalmol. 2018 Nov 2;42(6):367–84.
atypical clinical picture. The non-resolution of disc elevation
following compliant medical therapy for IIH is unusual Cite This Article as: Juhi Sahu, Muthukrishnan
and hints towards an alternative diagnosis. ONH drusen as Vallinayagam, C Alekhya. Bilateral Buried Optic
a differential for pseudopapilloedema should be excluded Nerve Head Drusen Mimicking Idiopathic Intracranial
before initiating therapy for IIH. Hypertension . Delhi J Ophthalmol 2021; 32; (1) 88 - 90.

Declaration Of Patient Consent Acknowledgments: Nil
Conflict of interest: None declared
The authors certify that they have obtained all appropriate Source of Funding: None
patient consent forms. In the form the patient(s) has/have Date of Submission: 01 Sep 2020
given his/her/their consent for his/her/their images and Date of Acceptance: 03 Apr 2021
other clinical information to be reported in the journal. The
patients understand that their names and initials will not Address for correspondence
be published and due efforts will be made to conceal their Muthukrishnan Vallinayagam
identity, but anonymity cannot be guaranteed.
MS, DNB, FRCS, FICO, Associate Professor
References
Department of Ophthalmology,
1. Birnbaum FA, Johnson GM, Johnson LN, Jun B, Machan Mahatma Gandhi Medical College
JT. Increased Prevalence of Optic Disc Drusen after & Research Institute, Sri Balaji
Papilloedema from Idiopathic Intracranial Hypertension: Vidyapeeth, Pondicherry India.
On the Possible Formation of Optic Disc Drusen. Neuro- Email: [email protected]
Ophthalmol. 2016 Jul 3;40(4):171–80.
Quick Response Code
2. Venkataramana P, Patil P, Koti S, Shetty V. Coexistence
of optic disc drusen and idiopathic intracranial
hypertension in a non-obese female. J Clin Ophthalmol
Res. 2015;3(3):150.

3. Ambika S, Arjundas D, Noronha V, Anshuman. Clinical
profile, evaluation, management and visual outcome
of idiopathic intracranial hypertension in a neuro-
ophthalmology clinic of a tertiary referral ophthalmic
center in India. Ann Indian Acad Neurol. 2010;13(1):37.

4. Mehrpour M, Oliaee Torshizi F, Esmaeeli S, Taghipour
S, Abdollahi S. Optic Nerve Sonography in the
Diagnostic Evaluation of Pseudopapilledema and

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DJO Vol. 32, No. 1, July-September 2021

PG Snippet

Ocular Manifestations Of Goldenhar Syndrome; At A Glance

Isha Acharya, Jolly Rohatgi, Ashit Handa, Anchal Arora, Saini Aarushi, Tadu Nampi

Department of Ophthalmology, University College of Medical Sciences (UCMS),Guru Teg Bahadur (GTB) Hospital, Delhi, India.

Goldenhar syndrome (oculo-auriculo-vertebral dysplasia, OAVS) is a rare, congenital disease with an incidence of 1:3500
and 1:5600. It occurs due to abnormal development of the first and second branchial arches. It has a multifactorial
Abstract etiopathogenesis. The typical features of Goldenhar syndrome include ocular anomalies - epibulbar dermoids,
microphthalmia and coloboma, auricular features such as preauricular tags, hearing loss, micrognathia, and vertebral
anomalies such as scoliosis or hemivertebrae.

Delhi J Ophthalmol 2021;32; 91-92; Doi http://dx.doi.org/10.7869/djo.701

Keywords: Congenital, Auricular, Dermoid, Coloboma

Introduction Table 1: Common ocular manifestations of Goldenhar syndrome

Goldenhar syndrome (oculo-auriculo-vertebral dysplasia, Eyeball, Eyelid Ocular manifestations
OAVS) is a rare, congenital Lacrima system
disease occurring due to abnormal development of the first Eyelid coloboma,
and second branchial arches.1 blepharoptosis,
It has a multifactorial etiopathogenesis and dependent on microphthalmos,
genetic and environmental factors.2 It is associated with anophthalmos,
constellation of anomalies including ocular, craniofacial cryptophthalmos.7-9
(cleft lip / palate, mandibular hypoplasia, etc) (Figure 1),
auricular (preauricular appendages / fistulas, ear asymmetry, Associated ocular complications
microtia,etc), (Figure 2) and skeletal (vertebral defects, etc) with eyelid coloboma:
deformities.3-6 Exposure keratopathy, corneal
ulceration, corneal perforation,
Ocular Manifestations corneal leukoma, amblyopia

The classic ocular features of this syndrome include Congenital dacryostenosis,
microphthalmia, epibulbar dermoids, lipodermoids(Figure3) lacrimal drainage obstruction,
and coloboma. However, there are many additional ocular lacrimal fistula, lacrimal sinus
dimple.10

signs that can be found in a patient of Goldenhar syndrome Strabismus Esotropia followed by
associated with systemic features. (Table 1) exotropia, congenital
ophthalmoplegia, Duane’s
retraction syndrome.11

Conjunctiva Dermolipomas, caruncular
abnormalities(dysplatic and/
or bilobed caruncles, ectopic
caruncles)12

Cornea, limbus Limbal dermoids, microcornea,
corneal anesthesia,
Figure 1: 12 year old child with Figure 2: Auricular features showing Iris /choroid neuroparalytic keratitis.13
Goldenhar syndrome; showing microtia with external auditory canal Lens Associated ocular
atresia and a pre-auricular skin tag Optic nerve/disc complications with limbal
mandibular hypoplasia dermoids: corneal astigmatism,
(blue arrow) of right ear anisometropic amblyopia,
visual axis obscuration, ocular
surface abnormalities, dellen,
inadequate lid closure.14

Coloboma15

Cataract(polar cataract)

tilted optic disc, optic nerve
hypoplasia, disc coloboma

Retina Vessel tortuosity, retinal
hypopigmentation, hypoplastic
macula

Figure 3: Ocular findings showing dermolipoma in right eye(red arrow) & Exudative Vitelliform
Limbal dermoid in left eye encroaching visual axis (yellow arrow). Maculopathy has also been
found associated16

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DJO Vol. 32, No. 1, July-September 2021

References Cite This Article as: Isha Acharya, Jolly Rohatgi, Ashit
Handa, Anchal Arora, Saini Aarushi, Tadu Nampi.
1. Goswami M, Bhushan U, Jangra B. Goldenhar Syndrome: A Ocular Manifestations Of Goldenhar Syndrome; At A
Glance Delhi J Ophthalmol 2021;32; (1) 91-92.
Case Report with Review. Vol. 9, International journal of clinical Acknowledgments: Nil
Conflict of interest: None declared
pediatric dentistry. 2016. p. 278–80. Source of Funding: None
Date of Submission: 18 Dec 2020
2. Strömland K, Miller M, Sjögreen L, Johansson M, Joelsson B-ME, Date of Acceptance: 08 Feb 2021

Billstedt E, et al. Oculo-auriculo-vertebral spectrum: associated Address for correspondence
Isha Acharya MBBS
anomalies, functional deficits and possible developmental risk
Department of Ophthalmology,
factors. Am J Med Genet A. 2007 Jun;143A(12):1317–25. University College of Medical Sciences
(UCMS),Guru Teg Bahadur (GTB)
3. Heike CL, Luquetti D V, Hing A V. Craniofacial Microsomia Hospital, Delhi, India.
Email – [email protected]
Overview. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE,
Quick Response Code
Bean LJH, Stephens K, et al. Seattle (WA); 1993.

4. Mehta B, Nayak C, Savant S, Amladi S. Goldenhar syndrome

with unusual features. Indian J Dermatol Venereol Leprol. 2008

May 1 [cited 2020 Dec 16];74(3):254.

5. Wilson GN. Cranial defects in the Goldenhar syndrome. Am J

Med Genet. 1983 Mar;14(3):435–43.

6. Lima M de DM de, Marques YMFS, Alves S de MJ, Ortega KL,

Soares MM, Magalhães MHCG de. Distraction osteogenesis in

Goldenhar Syndrome: case report and 8-year follow-up. Med

Oral Patol Oral Cir Bucal. 2007 Nov;12(7):E528-31.

7. Baum JL, Feingold M. Ocular aspects of Goldenhar’s syndrome.

Am J Ophthalmol. 1973 Feb;75(2):250–7.

8. Tawfik HA, Abdulhafez MH, Fouad YA. Congenital upper eyelid

coloboma: embryologic, nomenclatorial, nosologic, etiologic,

pathogenetic, epidemiologic, clinical, and management

perspectives. Ophthal Plast Reconstr Surg. 2015;31(1):1–12.

9. Mansour AM, Wang F, Henkind P, Goldberg R, Shprintzen

R. Ocular findings in the facioauriculovertebral sequence

(Goldenhar-Gorlin syndrome). Am J Ophthalmol. 1985 Oct

1;100(4):555–9.

10. Lüchtenberg M, Blotiu A, Lindemann G, Emmerich KH.

[Anomaliesof the efferent lacrimal ducts in Goldenhar syndrome].

Vol. 213, Klinische Monatsblatter fur Augenheilkunde. Germany;

1998. p. aA8-9.

11. Aleksic S, Budzilovich G, Choy A, Reuben R, Randt C, Finegold

M, et al. Congenital ophthalmoplegia in oculoauriculovertebral

dysplasia-hemifacial microsomia (Goldenhar-Gorlin

syndrome). A clinicopathologic study and review of the

literature. Neurology. 1976 Jul;26(7):638–44.

12. Nijhawan N, Morad Y, Seigel-Bartelt J, Levin A V. Caruncle

abnormalities in the oculo-auriculo-vertebral spectrum. Am J

Med Genet. 2002 Dec;113(4):320–5.

13. Mohandessan MM, Romano PE. Neuroparalytic keratitis

in Goldenhar-Gorlin syndrome. Am J Ophthalmol. 1978

Jan;85(1):111–3.

14. Spierer O, Gologorsky D, Adler E, Forster RK. Lamellar

keratoplasty with corneoscleral graft for limbal dermoids. Int J

Ophthalmol. 2018;11(3):512–5.

15. Margolis S, Aleksic S, Charles N, McCarthy J, Greco A,

Budzilovich G. Retinal and optic nerve findings in Goldenhar-

Gorlin syndrome. Ophthalmology. 1984 Nov;91(11):1327–33.

16. Bruè C, Mariotti C, Celani S, Rossiello I, Giovannini A. A

Case of Goldenhar Syndrome Associated with a New Retinal

Presentation: Exudative Vitelliform Maculopathy. Case Rep

Ophthalmol Med. 2015;2015:626027.

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PG Snippet

Ophthalmic Manifestations of Immunoglobulin
G4-Related Disease (IgG4-RD)

Ayushi Agarwal, Samreen Khanam, Akash Raut, Ruchi Goel

Department of Ophthalmology, Guru Nanak Eye Centre, Maulana Azad Medical College, New Delhi, India.

Abstract Immunoglobulin-G4 related disease (IgG4-RD) is an inflammatory disorder of unknown etiology that comprises a wide
spectrum of presentation, involving nearly all the bodily systems. A number of clinical conditions which were previously
identified as distinct entities, such as autoimmune pancreatitis, Reidel thyroiditis, and Mikulicz syndrome, have now come
within the purview of this disease. The clinical expression of IgG4-RD varies depending upon the organ involved. The
authors in this article aim to highlight the various ophthalmic manifestations of IgG4-RD.

Delhi J Ophthalmol 2021;32; 93-94 Doi http://dx.doi.org/10.7869/djo.702

Keywords: Immunoglobulin G4-Related Disease, Immunoglobulin G4-Related Ophthalmic Disease, Non-Specific Orbital Inflammation

Introduction

First identified in 2001 as an extra-pancreatic manifestation Figure 1: IgG4-ROD presenting as bilateral painless dacryoadenitis. Note the
of type 1 auto-immune pancreatitis (AIP), IgG4-related bilateral S-shaped lids.
disease (IgG4-RD) is a novel fibroinflammatory disorder
of unknown etiology.1 Presence of one or more ophthalmic
features along with the distinct histopathological appearance
is termed as IgG4-related ophthalmic disease (IgG4-ROD)
(Table 1). Approximately 5% - 35% cases of IgG4-RD possess
one or more ophthalmic manifestations, which can be
broadly classified as:

Table 1. Ophthalmic manifestations of IgG4-RD

Orbit and Adnexa Uvea

Lacrimal gland lesions, including Anterior uveitis frontal nerve, is highly suggestive of IgG4-ROD, especially
dacryoadenitis Pan-uveitis when bilateral. This may or may not be associated with
adjacent bony remodeling. Histologically, the perineurium
Enlarged orbital nerves is the primary site of involvement accompanied by the
absence of fibrosis.
Extraocular muscle enlargement/
Myositis

Lacrimal sac lesions like diverticulitis Intermediate uveitis Enlargement of Extra-Ocular Muscles (EOMs), most
Lacrimal drainage apparatus lesions commonly inferior rectus, followed by superior rectus,
Intraocular mass (simulating uveal lateral rectus, medial rectus, inferior oblique, and superior
Orbital mass melanomas) oblique, presents as myositis and may be confused with
Medial canthal swelling myopathy associated with Thyroid Eye Disease (TED).
Eyelid involvement, ptosis Cornea, Sclera and Conjunctiva Unlike IgG4-ROD, thyroid-associated myopathy is
Orbital fat and bone involvement Diffuse scleritis, Nodular scleritis, characterized by involvement of muscle belly with relative
sparing of tendinous insertion.
Vitreo-Retina posterior scleritis, necrotizing
Exudative Retinal Detachment scleritis IgG4-ROD may manifest as painless orbital mass, clinically
Dry eye presenting as bilateral or unilateral proptosis, medial canthal
Retinal vasculitis swelling, or even ptosis. Not only does IgG4-ROD simulates
Neural Corneal ulcer orbital lymphoma, it is also associated with an increased risk
Peripheral Ulcerative Keratitis of developing lymphoma.2 On Magnetic Resonance Imaging
Enlarged orbital nerves (MRI), adnexal IgG4-ROD typically appears isointense on
Optic nerve mass lesion T1-weighted Imaging (T1WI) and hyperintense on T2-WI
with homogenous contrast enhancement.3

Optic neuropathy Conjunctivitis Involvement of orbital fat as well as bone has also been
reported. Fibrosclerosis of the surrounding soft tissue as well
Orbital And Adnexal IgG4-ROD as the lacrimal drainage apparatus fall within the spectrum
of IgG4-ROD.
Lacrimal gland involvement, the commonest ophthalmic
feature of IgG4-RD, manifests as painless, bilateral (more Corneoscleral IgG4-ROD (Outer Coat)4
commonly) or unilateral dacryoadenitis. Mikulicz syndrome
refers to bilateral salivary gland enlargement in presence of Scleral involvement in the form of anterior diffuse scleritis,
bilateral dacryoadenitis ( Figure 1). Enlargement of orbital nodular scleritis, necrotizing scleritis, and posterior scleritis
nerves, most commonly infra-orbital nerve followed by has been documented in literature (Figure 2). Corneal

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DJO Vol. 32, No. 1, July-September 2021

and ocular surface manifestations include corneal ulcer, with or without systemic disease. The diagnosis is extremely
peripheral ulcerative keratitis, and dry eye which is partly challenging and must be confirmed by integrating
attributed to involvement of lacrimal gland and decrease clinico-radiological features with laboratory findings and
in the sub basal nerve plexus number as well as density, histopathological examination. Recurrences and relapses are
leading to tear film alterations. Conjunctivitis may be an not uncommon and thus, close monitoring and long-term
associated feature. follow-up is recommended in all cases.

Uveal IgG4-ROD (Middle Coat) References

Occasionally, IgG4-ROD may present as an elevated 1. McNab AA and McKelvie P. IgG4-Related Ophthalmic
intraocular mass lesion and simulate uveal neoplasms such Disease. Part II: Clinical Aspects. Ophthalmic Plast Reconstr
as choroidal melanoma, and ciliary body tumor.5-7 Anterior Surg 2015; 31: 167-178.
uveitis, vitritis, or pan-uveitis are the other associated
manifestations of IgG4-ROD ( Figure 3). 2. Yu W-K, Tsai C-C, Kao S-C, et al. Immunoglobulin G4-related
ophthalmic disease. Taiwan J Ophthalmol 2018; 8: 9-14.
Figure 2: IgG4-ROD presenting as left-sided sclero-uveitis.
3. Song YS, Choung HK, Park SW, Kim JH, Khwarg SI, Jeon YK.
Retinal IgG4-ROD (Inner Coat) Ocular adnexal IgG4-related disease: CT and MRI findings. Br
J Ophthalmol. 2013 Apr;97(4):412-8.
Retinal involvement in IgG4-RD is usually secondary.
Exudative retinal detachment, associated with an intraocular 4. Kocabeyoglu S, Karadag O, Mocan MC, Erden A, Irkec M.
mass and ocular inflammation, is not uncommon. IgG4-ROD Orbital Involvement and Ocular Surface Changes in IgG4-
may clinically present as retinal vasculitis, masquerading Related Systemic Disease. Cornea 2016 Nov;35(11):1449-1453.
autoimmune conditions such as granulomatosis with
polyangiitis.8 5. Ohno K, Sato Y, Ohshima K, et al. IgG4-related disease
involving the sclera. Mod Rheumatol 2014; 24: 195-198.
Neural IgG4-ROD
6. Das D, Deka P, Verma G, et al. IgG4-related intraocular
Besides the involvement of the orbital nerves, IgG4-ROD inflammation masquerading as ciliary body melanoma in a
may also present as an optic nerve mass lesion, mimicking young girl. Indian J Ophthalmol 2016; 64: 601-603.
a meningioma. Compressive effects due to expansion of an
orbital IgG4-related mass may lead to optic neuropathy.9 7. Khanam S, Agarwal A, Goel R, Saran RK. Immunoglobulin
G4related disease masquerading as metastatic malignant
A high index of suspicion for IgG4-ROD must be maintained melanoma of the choroid. BMJ Case Rep. 2021 Apr
in any case with the above enumerated clinical features, 1;14(4):e241605.

8. Lee CS, Harocopos GJ, Kraus CL, Lee AY, Van Stavern GP,
Couch SM, Rao PK. IgG4-associated orbital and ocular
inflammation. J Ophthalmic Inflamm Infect. 2015 May 29;5:15

9. Noshiro S, Wanibuchi M, Akiyama Y, Okawa S, Ohtaki S,
Sugino T, Iihoshi S, Mikami T, Sugita S, Hasegawa T, Mikuni
N. IgG4-related disease initially presented as an orbital mass
lesion mimicking optic nerve sheath meningioma. Brain
Tumor Pathol. 2015 Oct;32(4):286-90.

Cite This Article as: Ayushi Agarwal, Samreen
Khanam, Akash Raut, Ruchi Goel. Ophthalmic
Manifestations of Immunoglobulin G4-Related Disease
(IgG4-RD). Delhi J Ophthalmol 2021; 32; (1) 93- 94.

Acknowledgments: Nil

Conflict of interest: None declared

Source of Funding: None

Date of Submission: 15 Apr 2021
Date of Acceptance: 04 May 2021

Address for correspondence
Ayushi Agarwal Resident

Department of Ophthalmology,
Guru Nanak Eye Centre, Maulana Azad
Medical College, New Delhi, India.
Email: [email protected]

Figure 3: Non-Contrast Computed Tomography (NCCT) scan, axial view, Quick Response Code
showing a right-sided heterogeneously enhancing hyperdense intra-ocular www.djo.org.in

lesion. The patient was diagnosed as IgG4-ROD on tissue analysis.

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DJO Vol. 32, No. 1, July-September 2021

PG Snippet

Coats’ White Ring: The Embellished and Spruced up Iron
Remnant in the Cornea

Vishnu Teja Gonugunta

Department of of Cornea & Refractive Surgery Services, Aravind Eye Hospital, Madurai, Tamil Nadu, India.

Abstract Coats’ white ring can be a common finding in the cornea following incomplete removal of the iron rust ring after ocular
trauma with iron particles. Though the finding is common and innocuous, it’s etiology is rarely understood and commonly
considered as a corneal opacity following an ocular injury and less recognised as a distinct entity. It can be diagnosed from
the history and the typical appearance as a ring of discrete white dots in the subepithelial layers with intact epithelium.
Patients are asymptomatic and treatment is not required.

Delhi J Ophthalmol 2021;32; 95-96; Doi http://dx.doi.org/10.7869/djo 703

Keywords: Coats’ White Ring, Rust Ring, Iron Deposition, Cornea, Ocular Trauma.

Introduction Figure 1a: 10X magnification shows the location of the lesion in the cornea
near the limbus.
A 34-year-old male patient, welder by occupation came for
his routine eye check-up. He had past history of episodes of
iron metal chips flown into both of his eyes while working
without protective eye wear few years back. Examination of
his right eye revealed macular corneal opacity, whereas his
left eye revealed Coats’ white ring of 1.5 mm diameter near
the temporal limbus (Figure 1A and 1B). Coats’ white ring is
a form of iron deposition in the cornea which is often circular
or oval in shape and was first described by George Coats. It
is located within the Bowman’s membrane or the anterior
stroma, and is composed of a complete or incomplete ring of
discrete white dots. The overlying epithelium is smooth and
intact. The lesion(s) are associated with previous metallic
corneal foreign bodies.1,2 It is usually seen in the area of
incompletely removed rust ring in the cornea. Calcium was
thought to be the cause of the lesion as an occupational
injury in limestone workers.3 But the lesion doesn’t contain
calcium or lipid as it was thought before. Histochemical
analysis confirmed the presence of iron within the lesion.4
Patients with Coats ring are asymptomatic. These incidental
findings don’t require any treatment.

References

1. Reidy, JJ . Corneal and Conjunctival Degenerations. In:
Mannis MJ, Holland EJ. Cornea: Fundamentals, Diagnosis and
Management Vol:1 4th ed. New Delhi: Elsevier; 2016. pp. 858-
859

2. Stallard HB. White Rings In The Cornea. Br J Ophthalmol.
1934;18:452-453

3. Miller EM. Genesis of white rings of the cornea. Am J
Ophthalmol.1966 ;61:904-7.

4. Nevins RC, Davis WH, Elliott JH. Coats' white ring of the
cornea—unsettled metal fettle. Arch ophthalmol. 1968;80:145–
146.

E-ISSN: 2454-2784  P-ISSN: 0972-0200 Figure 1b: 16X magnification shows the typical ring of discrete white dots in
the cornea with few white dots in the centre of the ring.

95 Delhi Journal of Ophthalmology

DJO Vol. 32, No. 1, July-September 2021

Cite This Article as: Vishnu Teja Gonugunta. Coats’
White Ring: The Embellished and Spruced up Iron
Remnant in the Cornea. Delhi J Ophthalmol 2021; 32;
(1) 95 - 96.

Acknowledgments: Nil
Conflict of interest: None declared
Source of Funding: None
Date of Submission: 25 Apr 2021
Date of Acceptance: 08 May 2021

Address for correspondence
Vishnu Teja Gonugunta MS

Department of Cornea & Refractive
Surgery Services, Aravind Eye
Hospital, Madurai, Tamil Nadu, India.
Email: [email protected]

Quick Response Code

E-ISSN: 2454-2784  P-ISSN: 0972-0200 96 www.djo.org.in

DJO Vol. 32, No. 1, July-September 2021

Pictorial CME

Anomaly In Development and Regression of Hyaloid Artery -
The Bergmeister's Papillae

Prajnya Ray,1 Shruthy Vaishali Ramesh,2 Prasanna Venkatesh Ramesh,3

1Department of Optometry, Mahathma Eye Hospital Private Limited, Tiruchirappalli, Tamil Nadu, India.
2Department of Cataract and Refractive Surgery, Mahathma Eye Hospital Private limited, Tiruchirappalli, Tamil Nadu, India.

3Department of Glaucoma and Research, Mahathma Eye Hospital Private limited, Tiruchirappalli, Tamil Nadu, India.

Abstract In this article we discuss about one of the commonest developmental anomaly of the hyaloid system known as the
Bergmeister's papillae. It was first described by an Austrian ophthalmologist O. Bergmeister. It is a sign of persistent fetal
vasculature which is seen projecting from the disc. It is frequently observed as an incidental finding. Literature suggests
certain rare scenarios where it leads to strong vitreoretinal adhesions leading to complications like retinal detachment and
vitreous hemorrhage. Considering this presentation we have stressed the need for optical coherence tomography evaluation
for careful monitoring of the patient.

Delhi J Ophthalmol 2021;32; 97-98; Doi http://dx.doi.org/10.7869/djo 704

Keywords: Bergmeister's Papillae, Persistent Hyaloid Artery, Optical Coherence Tomography, Vitreoretinal Adhesion

Full Text

Bergmeister's papillae are the commonest congenital
anomaly of the hyaloid system. As a part of the normal
development, the embryogenic hyaloid artery appears at the
third week of gestation and reaches the lens by the fourth
or the fifth week of gestation, to form tunica vasculosa
lentis, which undergoes complete atrophy and disappears at
birth.1,2

Due to some reasons unknown, there may occur a temporary
arrest of this process, resulting in the persistence of the
vascular system in parts and in different forms depending
upon the time of action.3 The Anterior anatomical remnant
of the avascular hyaloid system may occur and manifest
clinically as Mittendorf's dot (remnants that remain at the
back of the lens) or the posterior anatomical remnant of the
avascular hyaloid system may occur and manifest clinically
as Bergmeister's papillae (remnants that persist at the optic
disc).4 It is characterized by raised glial tissue on the optic
disc surface (Figure 1).

Normal visual acuity, absence of inflammatory signs, the
stationary character of the glial tissue, and the presence
of persistent pupillary membrane will help to clinch
the diagnosis. In most severe cases it can be associated
with pigmentary changes, amblyopia, posterior polar
cataract, microphthalmia, the persistence of the primitive
vitreous and vitreomacular traction. Careful monitoring
is needed pertaining to eventual vitreous thickening on
the peripapillary area which might lead to vitreo-retinal
adhesion leading on to secondary complications like
vitreomacular traction, tractional retinal detachment and
vitreous hemorrhages.5,6 Optical coherence tomography
(OCT) evaluation of Bergmeister’s papillae will throw
light on the need for careful monitoring and intervention
if required. In conditions where there is no traction further
monitoring won't be necessary (Figure 2a), whereas cases
with vitreoretinal adhesion and traction will need careful
monitoring (Figure 2b).

Figure 1: (a-h) Clinical presentations of Bergmeister's papillae.

E-ISSN: 2454-2784  P-ISSN: 0972-0200 97 Delhi Journal of Ophthalmology

DJO Vol. 32, No. 1, July-September 2021

Figure 2: (a) Optical coherence tomography (OCT) of Bergmeister’s papillae showing hyper-reflective tissue overlying the optic nerve head with no evidence of
traction (red arrow).

(b) OCT of Bergmeister’s papillae showing hyper-reflective elevated tissue attached to the optic nerve head causing traction (red arrow) due to vitreoretinal
adhesion at the peripapillary region.

Reference Cite This Article as: Prajnya Ray, Shruthy Vaishali Ramesh,
Prasanna Venkatesh Ramesh. Anomaly in Development
1. Amador-Patarroyo MJ, Pérez-Rueda MA, Tellez CH. Congenital and Regression of Hyaloid Artery - The Bergmeister’s
anomalies of the optic nerve. Saudi J Ophthalmol 2015;29(1):328. Papillae. Delhi J Ophthalmol 2021; 32; (1) 97 - 98.

2. Mann I. The development of the human eye. New York: Grune & Acknowledgments: Nil
Stratton; 1964.
Conflict of interest: None declared
3. Bhattacharjee H. A family with rare multiple congenital ocular
abnormalities- Indian J Ophthalmol 1985:33(2):117-120 Source of Funding: None

4. Zimmerman LE, Font RL. Congenital Malformations of the Eye: Date of Submission: 10 Feb 2021
Some Recent Advances in Knowledge of the Pathogenesis and Date of Acceptance: 02 Apr 2021
Histopathological Characteristics. JAMA 1966;196(8):684–92.
Address for correspondence
5. Kaur R, Khan B, Syal E, Sidhu H, Kaur M. Persistent hyaloid Shruthy Vaishali Ramesh MS
artery with posterior polar cataract in a young male: A rare
presentation2018;30(3):203-205. Department of Cataract and
Refractive Surgery, Mahathma
6. Rossi S, Gesualdo C, Tartaglione A, Bilo L, Coppola A, Simonelli Eye Hospital Private limited,
F. Bergmeister's papilla in a young patient with type 1 sialidosis: Tiruchirappalli, Tamil Nadu, India.
case report. BMC Ophthalmol 2020;20(1):356. Email: vaishusmail@gmail.
com

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