DJO Oct- Dec 2022 Vol 32 issue (2)

DJO Vol. 32, No. 2, October-December 2021

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DJO Vol. 32, No. 2, October-December 2021

Delhi Journal of Ophthalmology DJO

Official Journal of Delhi Ophthalmological Society Editorial Board Volume 32   Number 2   October-December, 2021

Editor-in-Chief
Kirti Singh

Associate Editors

Annu Joon Devesh Kumawat Divya Jain Mainak Bhattacharya

Arshi Singh    Assistant Editors Priya Saraf

Akanksha Ankita Bhardwaj Khushboo Chawla Bhumika Sharma Charu Khurana
Deepanjali Arya Himshika Aggarwal Jatinder Bali Jatinder Bhalla
Jigyasa Sahu Manisha Agarwal Section Editors Neha Rathi Nisha Choudhary
Palak Gupta Pooja Bansal Priyadarshi Gupta Priyanka Golhait
Prachi Dave Rahul Mayor Anjali Mehta Ritu Aurora Shipra Sharda
Shweta Vishwanath Siddharth Baindur Gunjan Budhiraja Sumit Grover Siddharth Madan
Tanvi Gaonkar V.Krishna Neha Chawla Vaibhav Nagpal Vineet Sehgal
Prateek Kumar
Rajat Jain
Suma Ganesh
Vaibhav Khanna

International & Emeritus Editor

Arun Nrayanswami Bhavna Chawla George L. Spaeth Milind Pandeya Sonal Dangda
Satish Kotta Ruchi Goel
Vinod Kumar Samira Khan M. Vanathi Rajesh Sinha

A. K. Grover Atul Kumar Advisory Board Deepak Verma Jolly Rohtagi
J.S.Titiyal N.P. Singh M.D. Singh M. Vanathi
Namrata Sharma Pawan Goyal Bithi Chowdhary Praveen Vashisht Rakesh Bhardwaj
Ramanjeet Sihota Ritu Arora Mahipal S. Sachdev Sarita Beri Suneeta Dubey
S.C. Dadeya Pradeep Sharma
Rajender Khanna

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DJO Vol. 32, No. 2, October-December 2021

Contents

Editorial
Image Image who is fairer!............................................................................................................................ 6
Kirti Singh
Review Article
Primary Congenital Glaucoma: An Update......................................................................................................7
Arshi Singh, Kirti Singh

Uveitis in Children...........................................................................................................................................14
Rahul Bhatia, Devesh Kumawat, Bharti Arya, Sarita Beri

Optic Disc Pit Maculopathy: Review of Recent Advances in Its Diagnosis and Management......................21
Monika Dahiya, Manisha Nada, Jitender Phogat, Sakshi Lochab, Preeti Yadav, Surender Kumar

Original Article
Refractive Errors and Concomitant Strabismus in Children and Adolescents
: A Hospital Based Observational Study..........................................................................................................24
Anupam Singh, Omna Chawla, Rupal Verma, Vartika Saxena, Ranjeeta Kumari

A Critical Analysis of Publications in The IJO Over 2006-15...........................................................................30
Shalini Gupta, A K Amitava
Changes in Intraocular Pressure (IOP) after Phacoemulsification in Eyes with
Occludable Angle Of Anterior Chamber.........................................................................................................36
Pramod Kumar Sahu, Gopal Krushna Das, Divya Jain, Siddharth Madan

Case Report
Malarial Retinopathy: Falci Not So Fan-See!..................................................................................................42
Yogya Reddy, Reshma Ramakrishnan, Anamika Pandey, Ayushi ojha, Purva deore, Priyanka Gandhi
Choroidal Osteoma with Choroidal Neovascularization and Focal Choroidal Excavation...........................48
Rajwinder Kaur, Raja Narayanan, Balbir Khan, Ekta Gupta, Rishabh Narula

Brow Revision Following Animal Bite: A Case Report and Review of Literature..........................................49
Anju Kochar, Shaheen Farooq

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DJO Vol. 32, No. 2, October-December 2021

Neuroretinitis and Covid19: An Astounding Correlation................................................................................51
Dhaivat Shah, Rahul Singh, Garima Vaishnav, Ravin Punamia

Photo Essay
The Zonular Stripes ........................................................................................................................................55
Prateek Jain, Pooja Sah, Anshuman Pattnaik

Multimodal Imaging in Diagnosis of Choroidal Metastasis in Breast Carcinoma.........................................57
Sriram Simakurthy, Madhu Kumar, Om Shankar, Ashok Kanakamedla,
Jayamadhury Gudimetla, Anurag Shandil

Bilateral Long Standing Exudative Retinal Detachment in Coat’s Disease...................................................60
Punita Kumari Sodhi

PG Snippet
Hurricane Keratopathy – “A Curlicue on The Cornea"...................................................................................63
Vishnu Teja Gonugunta, Srinivasan Muthaiah

Omnibus Humanus (Masters, Change makers, Out of box thoughts)
A Legends Narrative......................................................................................................................................65
Ritu Aurora & Kirti Singh

Theme Article
OCT RNFL and MACULAR GCC........................................................................................................................67
Nisha Choudhary, Neha Sachdeva, Umesh Sharma

Anterior Segment Optical Coherence Tomography in Glaucoma..................................................................75
Arshi Singh
UBM Imaging..................................................................................................................................................82
Shweta Viswanath, Shikha Gupta

OCT Angiography: A promising tool in Glaucoma.........................................................................................86
Priya Saraf, Neha Sachdeva, Hage Amung

Interpretation of Retinal OCT........................................................................................................................90
Khushboo Chawla

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DJO Vol. 32, No. 2, October-December 2021

Fundus Fluorescein Angiography...................................................................................................................97
Priyadarshi Gupta, Ekta Shaw
Oct Angiography: The Basics.........................................................................................................................102
Khushboo Chawla

Cross-Sectional Imaging In Ocular And Orbital Lesions A Review................................................................107
Apoorva Sehgal, Alpana Manchanda, Jyoti Kumar, Ruchi Goel, R K Saran, Ayush Jain

Corneal Topography: Sirius...........................................................................................................................119
Jigyasa Sahu

Cover Image
Anterior Segment Optical Coherence Tomography in Glaucoma
Arshi Singh

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DJO Vol. 32, No. 2, October-December 2021

DOS Executive Members 2021-2022

DOS Office Bearers

Dr. Pawan Goyal Dr. Rajendra Prasad Dr. Jitender Singh Bhalla Dr. Sandhya Makhija
President Vice President Secretary Joint Secretary

Dr.Alkesh Chaudhary Dr. Kirti Singh Dr. Jitender Bali
Treasurer Editor Library Officer

Executive Members

Dr. O. P. Anand Dr. Gagan Bhatia Dr. Vivek Gupta Dr. Vivek Kumar Jain

Dr. Prafulla Maharanaa Dr. Amar Pujari Dr. Bhupesh Singh Dr. Pankaj Varshney

DOS Representative to AIOS Ex-Officio Members

Dr. J.S. Titiyal Dr. M. Vanathi Dr. Subhash ChaDnrd. SDuabdheaysah C. Dadeya Dr. Namrata Sharma

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DJO Vol. 32, No. 2, October-December 2021

Editorial

From the Editor’s Desk

Image Image who is fairer!

Imaging in ophthalmology has progressed by leaps and bounds to delineate tissue level morphology and pathology. At
the turn of the century ocular imaging was limited to film based fundus photography, fluorescein angiography and low
resolution ultrasound B scans. Then came the era of retinal and corneal imaging by Ocular coherence tomography and
corneal topography with their exponential growth.

Resolution of images has gone beyond the micron level and rapidity of acquisition to milliseconds. Comprehensive
algorithms, dedicated softwares permit canvas of insides of the eye to be painted on screen. The treasure trove has opened
and the red, green, yellow and blue jewelled colour coded pictures facilitate understanding of hitherto unknown aspects
of ophthalmology. Patient friendly non-invasive modalities permit universal application allowing easier incorporation in
blindness prevention programs. Innovative imaging modalities have also made metabolic assessment possible like retinal
flavoprotein fluorescence being suggested as a biomarker of mitochondrial activity in diabetes and glaucoma. Aqueous
outflow may be measured by anterior segment Optical coherence tomography angiography techniques, phase sensitive
optical coherence tomography and haemoglobin video imaging.

BUT, the constantly evolving upgrading wheel of technology burns a deep hole in pockets of those surgeons who join the
bandwagon of feeding this hydra headed monster of new and newest. The race to capture the micron level often deviates
physician’s perspective from holistic picture of “eye in a patient”. The patient, a whirlpool of hopes, fears and expectations
is forgotten, the jewelled images dictate decisions, with far reaching consequences. The clinician inherent inside the doctor
gets submerged, images reign instead.

Macro and micro -economics demand constant change. Justification for this gets packaged as, reducing error or enhancing
image quality of machines. This results in constant upgradation and purchase of new machines. The errors symbolized by
Red Green disease (OCT vocabulary) mandate critical thinking Artificial intelligence algorithms providing diagnostic and
prognostic alternatives, fuelled by need for virtual consultations have impacted future roles of clinicians.

Nature of training now needs to be modified to keep up to date with this technical prowess. Mistakes in our profession being
costly, doctors thus need to keep making new engrams, erasing old ones. While this does keep alive our neuronal circuits,
it does after some time, become strenuous, sometimes frustrating. So what is the solution? The answer is having a realistic
approach of understanding machine language sans its noise, without devaluing clinical judgement.

With this learning objective in mind this DJO issue brings forth its first theme issue of “Imaging modalities”. It details
Fluorescein angiography, Ocular coherence tomography in its various avatars- anterior, posterior (glaucoma and retinal
algorithms), Ultrasound bio-microscopy, Corneal topography to Radiological imaging.

Patient care being the mantra, human behind the machine, human in front of the machine and human interpreting the
painted canvas would always remain pivotal.

Happy learning to all

Team DJO

Dr. Kirti Singh DOI : http://dx.doi.org/10.7869/djo.707
MD, DNB, FRCS, FAIMER, DHA
Editor, Delhi Journal of Ophthalmology
Dir Prof & Director GNEC, MAMC Mamc and associated hospital’s’
New Delhi State Progr. Officer, NPCB

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DJO Vol. 32, No. 2, October-December 2021

Review Article

Primary Congenital Glaucoma: An Update

Arshi Singh, Kirti Singh

Department of Ophthalmology, Guru Nanak Eye Centre, New Delhi, India.

Abstract Primary congenital glaucoma is a rare maldevelopment of the eye that affects children in the first few years of their lives.
Blindness invariably occurs in untreated individuals. Early diagnosis by detailed examination under anaesthesia followed by
prompt treatment is necessary for favourable visual outcomes. Surgery is the primary treatment modality due to limited role
of medical management. Visual rehabilitation with management of refractive errors and amblyopia is key for long term visual
success. This article gives an overview of the epidemiology, genetics, diagnosis and treatment of this devastating disease.

Delhi J Ophthalmol 2021; 32; 7-13; Doi http://dx.doi.org/10.7869/djo.708

Keywords: Primary Congenital Glaucoma (PCG), Childhood Glaucoma, Trabeculodysgenesis

Introduction anomalies (holes, colobomata, aniridia)

Primary congenital glaucoma occurs due to abnormal • Corneotrabeculodysgenesis - peripheral (Axenfeld’s
development of trabecular meshwork and anterior chamber anomaly), mid-peripheral (Rieger’s anomaly), central
angle leading to impaired aqueous outflow. The terminology (Peter’s anomaly)
has replaced the older term Buphthalmos (Greek: bous =
• Corneal size (micro/ macrocornea)

ox; ophthalmos = eye) which is now restricted to primary 3. Congenital Glaucoma Research Network (CGRN) -
congenital glaucoma occurring before 3 years of age with current classification
resultant increased growth in eye ball.1 Combines disease severity with associated ocular anomalies6,7

The dismal disease consequences emphasised by Anderson’s a. Primary glaucoma:
comment “Future of hydrophthalmia patients is dark and • PCG: glaucoma at birth to 1‑year, neonatal (0–1 month)
one seeks in vain for a best operation in treatment2 have • Infantile glaucoma: 1–3 years
changed over the last few decades to more promising • Juvenile open angle glaucoma: 3 to 16 years
outcomes. This review traces the improved understanding • Spontaneously arrested cases: normal IOP & signs of
and treatment evolution of this condition.
PCG

Historical Trail b. Secondary glaucoma associated with:
• Non-acquired ocular anomalies (anterior segment
Although abnormal large eyeballs in children were
recognized by Hippocrates (4th century BC) and Celsus dysgenesis)
(1st century AD)3, it was only in late nineteenth century • Systemic disease or syndrome ( Sturge–Weber)
that von Hippel and Parsons linked disease pathology to • Post congenital cataract surgery (aphakia/ pseudophakia)
malformation of anterior chamber angle. • Acquired conditions ( Steroid‑induced, traumatic, uveitic)

The first successful surgery ‘goniotomy’ by Otto Barkan, in Epidemiology
19384 was modification of de Vincentis goniotomy, name
derived from Greek gonio (angle), tomein (cut) by designing Prevalence has racial connects varying from 1 in 10,000/
a glass contact lens to visualize and knife to cut trabecular 68,254 live births. (Caucasians) 8,9 to 1 in 2,500 (high rate of
tissue. Two decades later ‘ab externo trabeculectomy’ was consanguinity eg. Slovakian Gypsies and Middle East) 2,10 to
simultaneously and independently performed by Burian 1 in 3,300 (India). 11 Consanguinity promotes founder effect
with metal probe and Redmond Smith with nylon filament, or frequent mutations of a key mutation in an ancestor with
which ushered in the era of Schlemm’s canal surgery.3 reported 45- 80% consanguinity in PCG cohorts. 12,13

Classification Age of presentation is also linked to race, with early disease
at 3- 6 months in Asians /Arabians to 11 -12 months in
Primary congenital glaucoma was classified according to Caucasians.14,15 British Infantile glaucoma study (BIG)
age or pathology. reports earlier age of presentation in children with Asian
1. According to age of onset background (50% present before 3 months) compared to
Caucasian peers (52% by 6 months)16 Disease phenotype
• Congenital glaucoma - disease presence at / before birth is more severe in developing countries, with almost 2/3rd
• Infantile glaucoma- birth until 3 years PCG children in India presenting with blindness and most
• Juvenile glaucoma- 3 years to teenage reporting late, after 6 months.11,17

2. Anatomical classification (Hoskins–Shaffer– Slight male predominance (65%) has sometimes been noted
Hetherington) 5 for both Western and Asian cohorts.3,15 Bilateral disease is
• Isolated trabeculodysgenesis as flat iris insertion or the rule (70- 85 %).14 Since asymmetric presentation occurs
and fellow “apparently normal, currently disease free” eye
concave (wrap-around) insertion develops subclinical disease, it is imperative that children of
• Irido-trabeculodysgenesis with anterior stromal defects unilateral PCG have lifetime screening.15,18

(hypo/hyperplasia), anomalous iris vessels (persistence of
tunica vasculosa lentis /anomalous vessels) or structural

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DJO Vol. 32, No. 2, October-December 2021

Genetics Haab’s striae due to breaks in Descemet’s membrane are
hallmark of disease. Typically horizontal and linear in
Occurrence is sporadic with familial inheritance in 10–40% central cornea or parallel /curvilinear to limbus in peripheral
cases (autosomal recessive with incomplete or variable cornea, they need to be differentiated from post forceps
penetrance).3,19 assisted birth trauma Descemet tears, the latter being aligned
vertically.
Genetic loci identified are GLC3A, 3B, 3C and 3D.19 Corneal edema progress is from epithelial to stromal edema
• GLC3A loci (chromosome 2p21): Around 120 and scarring in case of persistently raised pressures.

mutations have been described with mutations in Intra-Ocular Pressure And Its Measurement
CYP1B1 gene, encoding cytochrome P450 enzyme
being commonest at 42%. 20-24 Gene effect has been Intraocular pressure (IOP) measurement depends on
traced to monooxygenase enzyme in endogenous anaesthesia stage/agent, corneal thickness, corneal edema
steroid metabolism25 with Tyrosinase deficiency and corneal biomechanics. Scleral / corneal stretch with
concurrence resulting in severe phenotype. reduced corneal hysteresis may mask the IOP rise.
• GLC3B loci (chromosome 1p36): Implicated gene Effect of corneal hysteresis (CH) and corneal resistance
is CDT6/ANGPTL coding for extracellular matrix factor (CRF) on IOP is significant, with both indices being
organization (ECM) and formation reduced in PCG.28-33 Measured by Reichert’s Ocular Response
• GLC3C loci (chromosome 14q24): gene LTBP2 Analyzer (ORA), corneal compensated IOP is independent
• GLC3D loci (chromosome 14q24) : Gene implicated is of corneal thickness.
latent transforming growth factor β binding protein Cornea is thicker in diseased eyes, with same being confirmed
responsible for ECM organization and formation by studies excluding corneal edema situations.34-36 Few
• TIGR/MYOC gene (chromosome 1q24.3--q25.2): linked authors however report thinner corneas .37, 38 Poor corneal
to juvenile onset glaucoma, autosomal dominant clarity makes optical tools like IOL Master under-estimate
inheritance. 26 CCT significantly (almost 40μm) compared to ultrasound
• Other associated genes: COL1A1 (17q21.33 ) , FOXC1 pachymetry.34
(6p25.3) ,ANGTP1 (8q23.1), TEK (9p21.2). 26
Optic Disc Dimensions
Genetic unravelling introduces molecular screening as the
preferred prevention tool in asymptomatic individuals with Disc excavation of > 0.1 C: D ratio in children younger than 1
a high risk of disease, and for prenatal diagnosis. It is already yr should be regarded with caution and > 0.3 most certainly
being done for Slovak gypsies. investigated for glaucoma.39,40 Elasticity of scleral canal
sometimes partly reverse this cupping.41
Clinical Manifestations

Classical triad remains blepharospasm, epiphora and Examination Under Anaesthesia (Eua)
photophobia with large eyeball with hazy corneas emerging
to be more common in Indian subcontinent. 3,17 Ophthalmic examination under sedation (intravenous or
Corneal: Corneal enlargement implies disease onset prior to mask anesthesia) suffices for diagnosis, followed by general
3 years. Corneal diameter of >11 mm (newborn), > 12 mm anaesthesia for definitive surgery.
(infant) and >13 mm (any age) is suspicious (Table 1).
Protocol for EUA42
Table 1 : Normal milestones with age • Intraocular pressure (IOP): All anaesthetic agents
except ketamine, succinylcholine, and cyclopropane
Birth - 6 Normal Suspicious cause rapid lowering of IOP (30 % reduction).
months Corneal diameter (mm) Excitement, intubation and induction artificially
1-2 yr >12 elevate IOP.43 Protocol mandates IOP measurement
9.5-11.5 >12.5 immediately post intubation. Perkins hand-held
>2 yr > 13 applanation tonometer or Tonopen for edematous
10-12 cornea, are the preferred measuring tools. 44
Birth - 1 year < 12 • Corneal diameter - white to white dimensions are
1- 2 years IOP (mmHg) measured in both vertical and horizontal diameters.
3-5 10- 12 + 3.7 Corneal thickness measurement.
7-9 12 • Gonioscopy is done with Koeppe lens (14–16 mm
13.6 diameter) combined with hand-held slit-lamp or
New-born 14.3 operating room microscope. Iris insertion is often
10-75 days Axial length (mm) high (wrap around), obscuring trabecular meshwork.
< 18 Iris contour is usually flat, rarely concave. Vascular
10- 36 17.22 -18.77 loops from major arterial circle, “Loch Ness Monster
months 20.14- 22.0 phenomenon” maybe seen. Rarely fine, fluffy tissue
4-10 years obscures peripheral iris called “Lister’s morning
22.78 - 23.12 mist”. Alternatively, a four mirror Zeiss indirect
goniolens can also be used during EUA or on slit lamp
examination for older children. In addition ,we can

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DJO Vol. 32, No. 2, October-December 2021

photograph the angle for documentation using the Goniotomy
RETCAM® wide-angle lens.
• Ophthalmoscopy: Optic disc cup is usually The procedure requires a clear cornea for proper visualization
central, round with steep-walls. Cupping enlarges of angle structures. Tissue obstructing trabecular meshwork
circumferentially due to stretching of scleral canal, in is incised by Barraquer knife/ Worst knife/ long 25 or 30
variance with polar notching of adult glaucoma.3 gauge needle, under direct visualization of Swan-Jacob
• Refraction: Axial myopia is a result of uncontrolled gonioscopic lens in a viscoelastic formed chamber. Entry
IOP. Progressive myopia or myopic shift presages is from temporal clear cornea and nasal 3-4 clock hours
PCG and glaucoma secondary to aphakia. are treated.53 Globe rotation manoeuvre permit excision of
5-6 clock-hours of meshwork54,55 Trabecular meshwork is
Treatment incised circumferentially laying bare a white line, followed
by falling back of iris. A single goniotomy is effective
Surgical treatment is the mainstay of therapy. Medical in controlling pressures in almost 75% cases56, multiple
therapy serves as a stop gap, an adjunct post-surgery or goniotomies increase the success rate to 93%. 57,58
in interval between repeat surgery to allow time for visual
maturation. Modifications of the procedure are: twin site approach for
wider coverage, Kahook dual blade use for more controlled
Beta-blockers are first line drugs, resulting in 30% reduction incision and endoscopic goniotomy.59-62 Goniotomy has
in IOP.45 Systemic side effects are minimized by using 0.25% the advantage of short operating time, conjunctiva-sparing
dose or punctal occlusion. nature, with low incidence of complications. 53, 57 Hyphema is
Both systemic and topical carbonic anhydrase inhibitors a common complication, but it resolves with time.
(CAIs) are effective, with topical Dorzolamide being the 2nd
option.46 Oral acetazolamide is used with caution due to its Sclemm’s Canal Surgery
side effects of growth suppression, renal impairment and
metabolic acidosis, the latter may precipitate an occlusive • Trabeculotomy ab- externo (Burian and Smith)63:
episode in sickle cell anaemia. The drug is given in 10 mg/ Similar to traditional trabeculectomy until raising of
kg/day in three divided doses. superficial scleral flap. Schlemm’s canal (SC) is subsequently
de-roofed by incising at blue-gray zone (anterior) junction
Alpha-2 agonists are not recommended children younger with white scleral zone (posterior), followed by threading a
than 6-9 years for risk of central nervous system mediated side defined trabeculotome (metal probe)into open ends of
respiratory depression and fatigue47,48 Prostaglandin SC. Rotation of probe towards anterior chamber collapses
analogues are the last resort, majority of children being trabeculum, ruptures inner wall of SC thereby re-establishing
non-responders.49 Irreversible iris pigmentation and eyelash communication between anterior chamber and Schlemm’s
elongation side effects require counselling, especially in canal. The procedure is repeated on other side with another
unilateral PCG. Sustained release implants have been trabeculotome designed for that side, covering 120–180° of
developed.50 trabeculum. Both the scleral and conjunctival flap are then
sutured.
Surgical Treatment Success rates are high (90%) 64-66 however the procedure is
hampered by difficulty in identifying Sclemm’s canal in
Surgery is the mainstay with options dictated by corneal presence of limbus distorted by stretching. Hyphema, false
clarity and disease severity. Surgical interventions are of two passage creation, Descemet’s detachment, cyclodialysis,
types: vitreous loss, vitreous haemorrhage, lens damage and
choroidal detachment are other complications.
• Ab-interno surgeries: Goniotomy, circumferential • Ab externo Circumferential 360° trabeculotomy
Schlemm’s canal surgery First performed by Beck and Lynch in 1995, using a 6/0
polypropylene suture passed in a purse string manner
• Ab-externo surgeries: Trabeculectomy with or to create a 360 degree incision in inner wall of Schlemm’s
without Mitomycin C, trabeculotomy, combined canal.67 Sustained IOP control with a single session, with
trabeculectomy with trabeculotomy (CTT) or use success rates of 87- 93% over long term follow-up are
of Glaucoma drainage devices. CTT is the surgery reported. 68- 70 Few unacceptable complications being creation
preferred for severe disease and in eyes presenting of false passage due to suture misdirection, subretinal and
with cloudy cornea.51 Due to this aspect it remains suprachoroidal damage iris tear and prolonged hypotony.
the most often performed surgery for PCG in Indian
subcontinent at 80%. 14 However the recent Cochrane 69, 71
meta-analysis suggests that there may be little to no
difference between CTT and routine conventional • Illuminated micro-catheter assisted trabeculotomy
trabeculotomy, or between viscotrabeculotomy (IMCT) replaced suture guided trabeculotomy in an
and routine conventional trabeculotomy.52 It stated attempt to reduce complications. Fibreoptic devices used
that 360-degree circumferential trabeculotomy may are: Glaucolight (DORC International, Zuidland, The
show greater surgical success than conventional Netherlands) or iTrack microcatheter (Ellex, Adelaide, SA)
trabeculotomy.52 72. The iTrack microcatheter (200 µm diameter shaft, 70µm
central lumen permitting viscoelastic injection) differs
from Glaucolight microcatheter (smaller diameter of 150

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DJO Vol. 32, No. 2, October-December 2021

µm sans lumen). Both devices incorporate an optical fibre over time, from 80-97% during first year to 58-66 % by 2-4
illuminating the tip, permitting continuous visualization. years and 20-40% by 5 -10 years. 87,89,93 Comparative studies
After 360 degree catheterization, catheter is retrieved from score GDD to perform better than trabeculectomy with
other cut end of Schlemm’s canal and two ends of catheter success of 87 to 53% (at 1 and 6 year) compared to 36 and
are pulled like a purse string, resulting in 3600 rupture of 19% with trabeculectomy.90 The same group reported more
inner wall of Schlemm’s canal. Success rates range from postoperative complications with GDD use in children,
80-87%. 72,73 Complications reported are hyphema in 100%, especially tube repositioning.
canal malformation restricting catheterization in 7-20% and
cataract.72,74, 75 Long term complications are otherwise high with reported
pupil abnormalities (16%), cataract (20% of phakic
Trabeculectomy With Mitomycin-C eyes), corneal touch, corneal decompensation (10%).89
Hypertensive phase post tube shunts is less frequently
A tested surgery with long term success rates ranging from seen in children 87,93 and is controlled by prophylactic use of
60 -90 %.76,77 adjunctive anti-metabolites improve success 78,79 aqueous suppressants. 88 Conditions peculiar to the child’s
eye like eye rubbing, growth and elasticity result in specific
Mitomycin-C is used in doses ranging from 0.2 - 0.4 mg/ml complications namely corneal decompensation, tube erosion
for durations of 2 -3 minutes.80 Postoperative complications /extrusion and tube retraction.82,88 Elastic recoil of sclera
like prolonged hypotony, scleral and conjunctival fragility after normalization of IOP can cause tube rotation leading
and endophthalmitis are known consequences of MMC to corneal contact. Growth of eyeball carries the potential
use.81 risk of tube retraction. Treatment of latter is done with tube
The most problematic aspect of creation of bleb for life with extenders or by tube-in-tube technique (threading new tube
subsequent tear film abnormalities, corneal astigmatism and within existing tube lumen) 94
bleb related infections. Propensity to eye rubbing and trauma
often results in high rates of bleb related endophthalmitis in Deep Sclerectomy
children (7-14%). 78-80,82
Utility of non-penetrating deep sclerectomy is doubtful
Combined trabeculotomy with trabeculectomy (CTT) given that obstruction to aqueous humour flow is internal,
with only few studies supporting its success in PCG eyes. 95,96
This surgery combines conventional trabeculectomy with
Schlemm’s canal exploration and rupture of its inner walls Cyclophotocoagulation
by Harm’s trabeculotome. This is followed by excision of
deeper trabecular block and suturing of superficial scleral Cyclophotocoagulation by transscleral Nd:YAG , transscleral
flap and conjunctival flap. diode, endoscopic diode, or micropulse trans-scleral diode
are reserved for eyes with guarded visual prognosis , has a
Combined trabeculotomy and trabeculectomy with or success rate ranging from 28 - 79% 97-99
without MMC is the most commonly procedure for PCG
eyes in Asia, with figures as high as 80% in Indian scenario. Transscleral cyclophotocoagulation diode (TSCPC) is safe
14,83 Success of the procedure ranges from 85 % at 1 year and effective, however efficacy is often transient and several
declining to 75 -77 % over a 2-3 year follow up.51,84 A large sessions are required. Lens opacification requiring cataract
series of 624 eyes with a 3 year follow up and 299 with surgery is common in phakic eyes (40%) 100
over 8 year follow up report success rates of 77 and 63%
respectively. 84,85 The longest reported follow up till date of 21 Prognosis And Complications
years from India has documented 6% compete success with
63% qualified success.86 Visual gain in children depends on the degree of corneal
scarring, astigmatism, anisometropic amblyopia, extent of
Glaucoma Drainage Devices (GDD) glaucomatous damage and sequel like cataract or strabismus.
Timely surgery with amblyopia therapy can result in good
Glaucoma drainage implants (GDD) come to rescue in vision and a large series of 624 eyes from India reports 42%
situations of multiple failed angle and or limbal surgeries.87 children gaining near normal vision.84 Functional gain of
Bleb of GDD being posterior to limbus makes it less vision occurs in 50-80% patients when IOP, refractive error
susceptible to endophthalmitis. Tube implants currently and amblyopia are managed adequately. 58, 69,101 A long follow
used in pediatric glaucoma are Baerveldt and Ahmed tube up of 28 years in 121 children 102, 103 documented minimal
shunts. For smaller sized eyes, smaller area models of FP 8 visual impairment in 42 % and low vision in 42 %. 86
Ahmed valve (96 mm2 ) compared to FP7 (184 mm2) and 250
mm2 size Baerveldt compared to 350 mm2 (adult) version Axial myopia is common (50 % and more), with younger age
are recommended. 88 Keeping in mind anticipated growth of surgery related to worse retinal parameters.100 Cataract
of child’s eye with high incidence of tube retraction, some and strabismus often occur after successful PCG surgery.
recommend adult size implants if it can fit the paediatric Cataract surgery requirement is reported for 22% within 8
eye.19 years and strabismus for 38% over 5 years increasing to 50%
over 10 years.102-104 Risk factor for occurrence of cataract are
Efficacy of tube shunts in PCG has been reported to be number of anti-glaucomatous surgeries and tube shunts.
high from 87-90% at 1year 87-92 however survival declines Performing strabismus or cataract surgery in filtered eyes can
have an adverse effect on bleb longevity, due to liberation

E-ISSN: 2454-2784  P-ISSN: 0972-0200 10 www.djo.org.in

DJO Vol. 32, No. 2, October-December 2021

of inflammatory cytokines. Performing cataract surgery can 11. Dandona L, Williams BC, Rao GN. Population-based assessment
also result in refractive surprise primarily due to greater
myopic shift, subsequent to increased responsiveness to IOP of childhood blindness in Southern India. Arch Ophthalmol.
fluctuation.105 Visual acuity improvement post-surgery is
gratifying, however these children need to be monitored for 1998;116:545–6
lens subluxation, capsular phimosis and endophthalmitis.
Visual sequel to PCG surgery have clinical implications 12. Helmy H Combined trabeculotomy-trabeculectomy versus
in visual prognosis and counselling of parents. Consent
for probable need of strabismus surgery, cataract surgery, Ahmed valve implantation for refractory primary congenital
amblyopia therapy and low visual aids should be taken from
the parents prior to performing surgery. Management of glaucoma in Egyptian patients: a long-term follow-up. Electronic
residual vision with and lifelong follow-up and low vision
aids is integral to enable these children to retain functionality. Physician 2016, 8 (2): 1884-1891

Conclusion 13. Genicek A, Genicekova A, Ferak V. Population genetical aspects

Early diagnosis, appropriate surgical management before of primary congenital glaucoma. Incidence, prevalence, gene
irreversible damage ensues can result in functional visual
gain to most of the children of primary congenital glaucoma. frequency, and age of onset. Hum Genet. 1982; 61:193–7
Choice of surgery depends on the severity of glaucoma-
goniotomy being preferred in milder disease and clearer 14. Senthil S, Badakere S, Ganesh J, Krishnamurthy R, Dikshit S,
corneas, CTT or assisted goniotomy in severe PCG. We
reserve use of drainage implants or cyclodestruction for Choudhari N, Garudadri C, Mandal AK. Profile of childhood
cases with surgical failure. Medication can help pre- and
post-operatively for IOP spikes. Genetic counselling, glaucoma at a tertiary center in South India. Indian J Ophthalmol
molecular diagnosis, possibly antenatal and reversing the
trend of consanguineous marriage in certain populations are 2019;67:358-65
the means to reduce this devastating childhood blindness.
15. Fung DS, Roensch MA, Kooner KS, Cavanagh HD, Whitson
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Ahmed valve implant following a failed initial trabeculotomy congenital glaucoma: An update Delhi J Ophthalmol 2021; 32
and trabeculectomy in refractory primary congenital glaucoma. (2): 7 - 13.
Middle East Afr J Ophthalmol. 2015, 22 (1):64–68.
89. O’Malley Schotthoefer E, Yanovitch TL, Freedman SF. Aqueous Acknowledgments: Nil
drainage device surgery in refractory pediatric glaucomas:
Long-term outcomes. J AAPOS. 2008; 12: 33-39 Conflict of interest: None declared
90. Beck AD, Freedman S, Kammer J, Jin J. Aqueous shunt devices
compared with trabeculectomy with Mitomycin-C for children Source of Funding: None
in the first two years of life. Am J Ophthalmol. 2003; 136:994-1000
91. Razeghinejad MR, Kaffashan S, Nowroozzadeh MH. Results Date of Submission: 21 Nov 2021
of Ahmed glaucoma valve implantation in primary congenital Date of Acceptance: 06 Dec 2021
glaucoma. J AAPOS. 2014; 18(6):590–595.
92. Werner M, Grajewski A. Further surgical options in children. Address for correspondence
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editors. Glaucoma Surgical management. Vol. 2. Elsevier; 2009. Arshi Singh Senior Resident
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93. Chen TC, Bhatia LS, Walton DS. Ahmed Valve Surgery for Department of Ophthalmology, Guru
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Ophthalmology and Strabismus. 2005; 42(5):274-283 E-mail: [email protected]
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E-ISSN: 2454-2784  P-ISSN: 0972-0200 13 Delhi Journal of Ophthalmology

DJO Vol. 32, No. 2, October-December 2021

Review Article

Uveitis in Children

Rahul Bhatia, Devesh Kumawat, Bharti Arya, Sarita Beri

Department of Ophthalmology, LHMC and SSK Hospital, Delhi, India.

Abstract Uveitis in children is an important cause of ocular morbidity and severe vision loss. The diagnosis and the management
are challenging in such population due to some unique presentations. Majority of the cases are idiopathic with infectious
causes contributing to about one fifth of the cases. Various causes of infectious uveitis include tuberculosis, toxoplasmosis,
toxocariasis. Anterior uveitis is the most common anatomical type of uveitis in children followed by almost equal incidence of
posterior, intermediate and panuveitis. The examination is often difficult in children and diagnostic evaluation is challenging.
The management requires multidisciplinary approach involving the rheumatologist and uveitis specialist. Exclusion of infectious
and masquerade causes is of utmost importance before starting any immunomodulatory treatment. The primary aim of the
treatment is to reduce the inflammation as early as possible to prevent complications. Corticosteroids are the main pillar stone
for the management. The dosage and the route of the steroids should be optimized according to the growth and development of
the child as it may have to be given for a longer duration. Steroid sparing agents such as methotrexate, cyclosporine, azathioprine
and biologic agents are reserved for children refractory to treatment with steroids. Children tend to develop complications of
uveitis more often than in adults, which include band keratopathy, cataract, glaucoma, cystoid macular edema, amblyopia and
strabismus.

Delhi J Ophthalmol 2021; 32; 14-19; Doi http://dx.doi.org/10.7869/djo.709

Keywords: : Uveitis, JRA, Behçet, Ocular Tuberculosis, Toxoplasmosis, Toxocariasis

Introduction a chronic inflammatory course, JIA has asymmetric joint

Uveitis in children constitutes of 4-10% of all uveitis involvement and the children outgrow the disease with

population.1,2 Uveitis in such population is often challenging time. It is one of the common causes of anterior uveitis in

in terms of diagnosis and management as well as the children with prevalence of uveitis in patients of JIA varying

spectrum of complications associated with it. between 14 to 24%.5–7

The diagnosis of uveitis in children may be delayed due The oligoarticular subtype accounts of 50% of the JIA cases
to the varied presentation of uveitis. The anterior uveitis and is diagnosed when fewer than 5 joints are involved
often is asymptomatic without the classical symptoms of during the first 6 months of the disease. It is more common
pain, redness or photophobia (white iritis). Strabismus and in females and the onset is usually before 6 years of age.
leukocoria may be the presenting symptoms which can cause Chronic anterior uveitis (CAU) is most commonly associated
misdiagnosis and delay in treatment. Also, the examination with oligoarticular JIA. Approximately 30% to 50% of
in children is often difficult. Examination under anaesthesia oligoarticular JIA patients develop CAU. The polyarticular
may be required for complete examination of the child. subtype is diagnosed when more than 4 joints are involved
Some of the masquerade syndromes such as retinoblastoma, in the first 6 months of disease. It is more common in
leukemia, juvenile xanthogranuloma may present as uveitis
as a primary presentation. Therefore, the approach to uveitis Table 1: Causes of pediatric uveitis
in children is very different as compared with that in adults.
Anterior uveitis Juvenile idiopathic arthritis
Epidemiology Juvenile seronegative
Intermediate uveitis
Based on the anatomical site of involvement, anterior uveitis Posterior uveitis spondyloarthropathies
is the most common type of uveitis in children.2–4 Posterior, Sarcoidosis
intermediate and panuveitis constitute almost equal Tubulointerstitial nephritis and
percentage of pediatric uveitis. Majority of the pediatric
uveitis cases are idiopathic. Juvenile idiopathic arthritis uveitis
is the most common known etiology overall.2–4 Infectious Kawasaki disease
uveitis constitute nearly one-fifth of the cases. Among the Herpetic uveitis
infectious cases, tuberculosis and toxoplasmosis are the Syphilis
most common etiologies. Intermediate uveitis is commonly Trauma
idiopathic in children. Bilateral involvement is the norm in Idiopathic
pediatric uveitis with males being affected slightly more
than the females.2–4 The etiologies of pediatric uveitis are Pars planitis
mentioned in (Table 1) and the important causes are being Sarcoidosis
considered in the following section. Tuberculosis
Lyme disease

Tuberculosis
Toxoplasmosis
Toxocariasis
Syphilis
Sarcoidosis

Juvenile Idiopathic Arthritis Panuveitis Tuberculosis
Acute retinal necrosis
It is a group of arthritides, which often presents below the Sarcoidosis
age of 16 years and persists for at least 6 weeks. Unlike Bechet’s disease
the adult onset rheumatoid arthritis which has symmetric Sympathetic Ophthalmia
joint involvement, characteristic rheumatoid nodules and Vogt-Koyanagi Harada disease

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DJO Vol. 32, No. 2, October-December 2021

females and can be further subdivided based on rheumatoid Herpetic Anterior Uveitis
factor (RF) results. RF positive disease mimic adult onset
rheumatoid arthritis and is not associated with uveitis. RF Herpes simplex virus (HSV) and varicella zoster virus
negative polyarticular JIA is associated with uveitis in 5% (VZV) can cause unilateral acute granulomatous or non-
to 10% of cases. Systemic disease or Still disease is seen in granulomatous anterior uveitis in children.12 Patchy or
children less than 5 years of age and affect both sexes equally. sectoral iris atrophy may occur. The intraocular pressure
Uveitis develops in less than 5% of the cases. may be high in herpetic infections contrary to low intraocular
pressure seen in non-infectious anterior uveitis.
Psoriatic arthritis and enthesitis-related arthritis are less
common subtypes of JIA which may also be associated with VZV anterior uveitis is more severe and results in segmental
CAU. iris atrophy (from occlusive vasculitis) and pupillary
distortion as compared to relatively mild uveitis with round
Major risk factors for developing uveitis in patients with JIA iris atrophy in HSV infection.
are female sex, antinuclear antibody (ANA) seropositivity,
oligoarticular arthritis, rheumatoid factor sero-negativity Definitive diagnosis requires polymerase chain reaction
and early (less than 6 years) age of onset of arthritis. (PCR) of the aqueous humor sample to detect the viral DNA.

Clinical features: The most common extra articular Idiopathic Intermediate Uveitis (Pars Planitis)
manifestation of JIA is uveitis. Uveitis in JIA is usually
bilateral, anterior, non-granulomatous uveitis with a According to the anatomic classification of uveitis by the
chronic relapsing course. Children with CAU associated Standardization of Uveitis Nomenclature (SUN) Working
with JIA are typically asymptomatic and therefore routine Group, the term “intermediate uveitis” defines a subset of
ophthalmologic screening is essential for early diagnosis and uveitis where the vitreous is the primary site of inflammation.
timely treatment. Otherwise, affected subjects may present Pars planitis is a diagnostic term that defines a subset of
after development of serious ocular complications and idiopathic intermediate uveitis where there is snowbank or
severe visual impairment. These may include complicated snowball formation.13
cataract, band shaped keratopathy, hypotony, and phthisis
bulbi. It usually affects children and adolescents. Various
associations with HLA-DR2 and HLA-DR15 have been
Screening guidelines have been developed; patients with reported.14
oligo- or polyarticular JIA with onset of arthritis at 6 years
of age or younger, duration of arthritis 4 years or less, and Clinical features: Children with pars planitis may be
ANA-seropositivity should be screened for uveitis within 6 asymptomatic and are usually diagnosed during a routine
weeks of diagnosis and then at 3-month intervals.8 examination. Some children are diagnosed only after
significant visual impairment or the development of
Seronegative spondyloarthropathies (SpA) complications that cause leukocoria or strabismus. Typical
clinical findings include mild to moderate anterior segment
These include ankylosing spondylitis, reactive arthritis, inflammation, diffuse vitreous cells and haze, snowballs and
psoriatic arthritis, and enteropathic arthritis. The common snowbanks located inferiorly. Peripheral retinal vasculitis
systemic findings are axial arthritis and enthesitis. may also be present. Band keratopathy, peripheral corneal
Adolescent males are more commonly involved. endotheliopathy, and posterior synechiae may be seen in
Clinical features: Ocular manifestation is with unilateral childhood pars planitis but are very rare in adults. Optic disc
acute anterior uveitis, occuring in one-fourth of the patients. edema and cystoid macular edema are the most frequent
The diagnosis is made based on presence of systemic findings complications.15
supportive of SpA and negative rheumatoid factor.9
Dense vitreous condensation may sometimes cause
Tubulointerstitial nephritis and uveitis leukocoria. Vitreous haze and cataract may cause amblyopia
in a young child with pars planitis. Cystoid macular edema
Tubulointerstitial nephritis and uveitis (TINU) is a rare is the leading cause of visual morbidity in intermediate
immune mediated entity occuring in children with female uveitis.15
preponderance.10 The median age of onset is 15 years.
Behçet Uveitis
Clinical features: The child may have fever, flank tenderness,
anorexia, and weight loss. Ocular presentation may be Although Behçet disease is more common in third and
bilateral non-granulomatous anterior uveitis or posterior fourth decade of life, it may occur in childhood as well.
uveitis in form of chorioretinitis and multifocal choroiditis.11 There are no internationally accepted diagnostic criteria for
Suspected cases may be advised urinalysis which shows childhood-onset Behçet disease. Recurrent oral ulcers are
presence of glycosuria, proteinuria, aminoaciduria, and the most common presentation of Behçet disease in children.
microscopic hematuria. Renal biopsy is confirmatory. Uveitis is less common (34%) in this group than in adults.16
Although the disease usually resolves spontaneously, The age of presentation in pediatric Behçet disease is in
systemic steroids are often used to prevent structural renal adolescence (10-15 years).17 There is a male predominance
damage. in the pediatric age group, similar to adult-onset Behçet

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DJO Vol. 32, No. 2, October-December 2021

uveitis.17 A positive family history has been reported in 20– Clinical features: Anterior uveitis is the most common
47% of pediatric cases from endemic areas, implying the role presentation in children with sarcoidosis. Granulomatous
of genetic factors in the early onset of the Behçet disease.18 KPs, iris nodules, and peripheral and broad-based posterior
HLA-B51 haplotype is seen in 60% of the cases. B*5101 is the synechiae are typical findings. Other than iris nodules at the
most common allele in this serotype. pupillary margin (Koeppe nodule) and in the iris stroma
Clinical features: The majority of patients have bilateral (Busacca nodule), nodules may also be found in the anterior
involvement and recurrent panuveitis with retinal vasculitis. chamber angle (Berlin nodule). Uveitis in sarcoidosis has to
Cataract, intraocular pressure elevation, macular edema, be differentiated from JIA associated uveitis. Inflammation
and optic atrophy are the most common complications.18 of the posterior segment in the form of retinal vasculitis or
multifocal choroiditis can be seen in sarcoidosis; however,
Sarcoidosis uveitis is typically non-granulomatous and confined to the
anterior segment in JIA.
Childhood sarcoidosis is a multisystemic granulomatous
inflammatory disorder. While older children may present Serum angiotensin-converting enzyme levels may be
with pulmonary involvement, young children with early-
onset non-hereditary sarcoidosis (onset before age 5, familial misleading because children tend to have higher levels
autosomal dominant Blau syndrome) typically present with
a triad of arthritis (involving knee and wrist), skin lesions than adults.19 The diagnosis of sarcoidosis is usually clinical
and anterior uveitis.19
based on typical signs of ocular disease and laboratory
Figure 1: Fundus photograph of left eye of an infant with congenital
toxoplasmosis. The patient had hydrocephalus with seizures and positive abnormalities, although definitive diagnosis requires
toxoplasma serology. An ill-defined atrophic macular scar is seen in left eye
suggestive of healed toxoplasma retinochoroiditis scar. The right eye had biopsy specimen showing non-caseating granulomatous

similar lesion at the macula. inflammation.l Toxoplasmosis

Congenital toxoplasmosis occurs via transplacental

transmission of Toxoplasma gondii especially when the

mother acquires the disease later during the pregnancy.21

Congenital toxoplasmosis scars are an incidental finding and

are usually bilateral (Figure 1). Acquired infection can occur

in childhood but is unilateral. Reactivation of the congenital

disease with active satellite lesions adjacent to the healed

lesion is the primary presentation in childhood.22

Clinical features: Ocular toxoplasmosis presents as focal
necrotizing retinochoroiditis with vitritis (“headlight in fog “
appearance) in children, similar to that seen in adults. It may
also present as panuveitis. Complication such as choroidal
neovascularization in the affected healed area is relatively
more common in pediatric cases than adults.23

The diagnosis is clinical with laboratory tests assisting in the
diagnosis. Polymerase chain reaction and determination of
Goldmann–Witmer coefficient using aqueous humour or
serum are the important laboratory tools.24

Toxocara

Ocular toxocariasis manifests in children and early

Figure 2: Clinical spectrum of ocular tuberculosis. a. Multiple active tubercles with ill-defined edges. b. Healed tubercular subretinal abscess along the supero-
temporal arcade. c. Active macular serpigenoid choroiditis.

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DJO Vol. 32, No. 2, October-December 2021

adolescents. Infection is by ingestion of toxocara eggs. The anti-inflammatory and immunomodulatory treatment.
egg matures into larvae and migrates in various tissue
including the eye. Infectious uveitis

Clinical features: Ocular presentation is unilateral in The management of infectious uveitis requires tailored
90% of the cases. Toxocara retinochoroiditis appears as treatment approach. Treatment of herpetic anterior uveitis
a well demarcated, elevated mass lesion in the posterior is with systemic antivirals (oral acyclovir 400 mg five times
pole with overlying vitritis. It may also present as daily for 2-4 weeks) along with topical-corticosteroid and
peripheral granuloma or chronic dense vitritis mimicking cycloplegic agents.28 In recurrent cases, oral prophylaxis
endophthalmitis. Immunologic studies are generally not (acyclovir 400mg twice a day) may be given to prevent relapse.
reliable for toxocariasis. Herpetic eye disease study- 1 (HEDS-1), a randomized trial
performed in 1990s, studied the efficacy of oral acyclovir
Tuberculosis (400 mg five times daily for 10 weeks) in addition to topical
corticosteroids for treating HSV iridocyclitis.29 Although the
Clinical features: Ocular tuberculosis can have a variety originally planned recruitment could not be completed and
of presentations in children including granulomatous the trial was stopped, the treatment failure was significantly
anterior uveitis, intermediate uveitis, posterior uveitis in less with additional oral acyclovir treatment. HEDS-2 study
the form of choroidal tubercle, tuberculoma, subretinal demonstrated the efficacy of low dose acyclovir prophylaxis
abscess, serpigenoid choroiditis (Figure 2) or panuveitis.25 (400mg twice a day for 1 year) in preventing recurrence of
Broad based posterior synechiae, retinal vasculitis with any form of HSV eye disease including iritis.30
perivascular choroiditis/scars and multifocal serpiginoid Toxoplasma retinochoroiditis is aimed at preventing
choroiditis are suggestive of tubercular infection.26 multiplication of the protozoa. Treatment regimen includes
Laboratory diagnosis of intraocular TB is challenging in pyrimethamine (2 mg/kg first day then 1 mg/kg each day)
the children. Based on the clinical findings, microbiological combined with sulfadiazine (50-100mg/day in two divided
results, immunologic tests and radiology features, the doses) for 1 month. Folic acid supplementation (7.5 mg
diagnosis of confirm TB, probable TB or possible TB is per day) is required along with anti-protozoal treatment.
made.26 Mantoux test has limited use since there may be Trimethoprim and sulfamethoxazole combination in double
cross reactivity with antigens used in the test with previously strength may also be used for treatment. Intermittent therapy
administered BCG vaccines. Interferon gamma release assay with trimethoprim-sulfamethoxazole combination is useful
(IGRA) is more specific. High resolution CT chest is more in preventing recurrences.31
useful than X-ray in cases with suspected systemic focus of
infection. Aqueous or vitreous PCR may help in confirming Steroids are the mainstay treatment for toxocara which
ocular infection in suspect cases but is less sensitive and not is aimed at reducing the inflammation and preventing
feasible in real world scenario. secondary complications such as development of tractional
membranes and retinal detachment. Oral steroids are used
Masquerade Syndromes In Children in the dose of 0.5-1mg/kg/day. Anti-helminthic therapy is
of unproven value in management; although few studies
Masquerade syndromes are one of the rare cause of uveitis support the use of albendazole (15mg/kg in two divided
in children. A high index of suspicion is required for the dose) or thiabendazole to eradicate the organism.32
early diagnosis. These should be suspected in cases with
incomplete or poor response with anti-inflammatory therapy. The management of ocular tuberculosis is with 4 drug
The causes include tumors such as retinoblastoma, leukemia regimen (Rifampicin 15 mg/kg, maximum dose 600mg/
and juvenile xanthogranuloma, congenital abnormalities day; Isoniazid 10 mg/kg, maximum dose 300 mg/day;
such as Coats disease, retained intraocular foreign bodies Pyrazinamide 35 mg/kg; and ethambutol 20 mg/kg) for 6-9
following trauma and endogenous endophthalmitis.12,27 months.33 Once diagnosis is made and treatment is started,
it should not be stopped before 6 months because of lack of
Diagnosis response, unless an alternative diagnosis is reached. This is
because poor or suboptimal response can be due to immune
Early and correct diagnosis is a challenge for uveitis in reaction from death of the microorganism, which in addition
children. The diagnosis may be difficult in preverbal and needs anti-inflammatory therapy. Steroid are to be used
asymptomatic children. The examination in children is judiciously under cover of anti-tubercular treatment, as it
often difficult. Also, uveitis in children may have unique might lead to flare up or reactivation of disease if used alone.
presentations which may cause misdiagnosis. Diagnostic Treatment response is assessed at 2 months and at the end of
approach vary for pediatric uveitis as compared to uveitis treatment (6-9months) based on resolution of TB lesions and
in adults. compatible uveitis.33

Management Non-Infectious Uveitis

The primary aim of management is to reduce the active Corticosteroids remain first-line treatment for non-infectious
inflammation and hence decrease the complications uveitis in children. Topical corticosteroids are initially used
associated with it. The management principles for uveitis are for treatment of anterior segment inflammation. Periocular
different in children as compared with adults. Exclusion of an or subtenon corticosteroid injections may be used for
infectious cause of uveitis and masquerade syndromes is of treatment of intermediate or posterior uveitis, especially
utmost importance before the administration of nonspecific in unilateral cases or for the treatment cystoid macular

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DJO Vol. 32, No. 2, October-December 2021

edema. Systemic corticosteroids are used only for short- dose of 20-40 mg every 7 to 14 days. Some data suggest that
term treatment in children because of significant systemic adalimumab may be slightly more effective than infliximab
side effects associated with their prolonged use, including in achieving remission in JIA associated uveitis.36 A close
Cushing syndrome, growth retardation, weight gain, co-ordination is required between ophthalmologist and
hypertension, osteoporosis, gastrointestinal disturbance, pediatric rheumatologist for starting the biologic therapy
psychosis and electrolyte imbalance.34 There is increased risk and to monitor the systemic side effects.
of corticosteroid induced cataract and glaucoma. Steroid-
induced glaucoma occur more rapidly and may be refractory Complications
to treatment in children. It is difficult to detect and monitor
intraocular pressure elevation in young children. Children tend to develop complications of uveitis more often
than in adults, which include band keratopathy, peripheral
Due to such complications, early use of corticosteroid anterior synechiae, posterior synechiae, cataract, glaucoma,
sparing agents or immunosuppressants is advisable. cystoid macular edema, epiretinal membrane, retinal
Immunosuppressive therapy is also advised if there is detachment and phthisis bulbi.37 Surgical intervention
active systemic disease. Non-biologic agents such as for the complication of uveitis in children also tend to be
antimetabolites (methotrexate) and signal transduction complicated, with higher failure rates.
inhibitors (cyclosporine) are commonly in management of
chronic non-infectious uveitis in children.34 Methotrexate is Conclusions
the most widely used first-line immunomodulatory agent in
children with uveitis because of its long-term safety profile To conclude, multiple challenges are faced related to
in this age group.35 However, it takes 1-2 months to achieve the diagnosis and treatment of pediatric uveitis. Clinical
the desired plasma levels. It is a folic acid analogue given manifestations of uveitis are different in children as
at 0.15mg/kg orally once a week for at least 3months and/ compared to the adults. Active inflammation need to be
or until stable quiescence, entirely without corticosteroids, aggressively controlled with immunosuppressants to
is observed with concurrent folic acid supplementation. prevent ocular complications. Drug-related side effects
Folic acid supplementation of 1mg per day for 5 days per should be monitored and therapy should be modified
week is required to decrease the bone-marrow toxicity. The accordingly. Immunomodulatory therapy requires a close
dosage of methotrexate should be reassessed as the child coordination between the ophthalmologist and pediatric
grows according to the weight. Methotrexate can be given rheumatologist to maximize the efficacy of treatment and
in subcutaneous form if the dose exceeds 17.5mg or there minimize the ocular and systemic side effects.
is no clinical remission even with increased oral dosage.
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DJO Vol. 32, No. 2, October-December 2021

Review Article

Optic Disc Pit Maculopathy: Review of Recent Advances in
Its Diagnosis and Management

Abstract Monika Dahiya, Manisha Nada, Jitender Phogat, Sakshi Lochab, Preeti Yadav, Surender Kumar

Regional Institute of Ophthalmology, PGIMS Rohtak, Haryana, India.

Optic disc pit (ODP) is a rare congenital anomaly of the optic disc which is usually unilateral and sporadic. It can lead to visual
impairment secondary to ODP maculopathy characterised by intraretinal and subretinal fluid collection at macula with no
known trigger factors. We came across a case of ODP maculopathy being misdiagnosed and mistreated as chronic central serous
retinopathy which led us to review the advances in its diagnosis and management as ODP maculopathy left untreated carries poor
prognosis. Its management remains challenging as there are no clear guidelines and multiple modalities are available. Although
few cases of spontaneous resolution of maculopathy are reported in literature but current management of ODP maculopathy
is surgical intervention. The most commonly performed surgery for ODP maculopathy is 23 G Pars Plana Vitrectomy, either
alone or in combination with ILM peeling, gas tamponade or laser photocoagulation. Recent treatment modalities available are
intravitreal gas tamponade, macular buckling, inner retinal fenestration, glial tissue removal and autologous fibrin. Prognosis
is poor if multilayer intraretinal and subretinal fluid collection is seen on optical coherence tomographywhile cases where fluid
don’t reach to macular arcade vessels have better anatomical and visual outcomes. Even though there are many recent advances
in the diagnosis and management of disease, management remains challenging as there are no comparative studies of different
therapeutic modalities and their success rate, related to the anatomical and functional outcomes. This review discusses the
literature on possible pathogenesis of ODP maculopathy as well as the recent advances in its diagnosis and management.

Delhi J Ophthalmol 2021; 32; 20-23; Doi http://dx.doi.org/10.7869/djo.710
Keywords: Optic disk pit, laser, gas tamponade, vitrectomy, buckling, autologous fibrin.

Introduction The first possible source of fluid is vitreous in ODP
maculopathy. The pre-existing vitreous traction on macula
Optic disc pits (ODPs) are rare congenital excavation of the and optic disc may lead to development of a negative
optic nerve head and are considered as a part of spectrum of pressure leading to entrance of fluid through the disc pit into
congenital cavitary anomalies of the optic disc, which also sub macular space resulting into macular detachment.9,10
includes morning glory syndrome, optic disc coloboma and Multiple studies have demonstrated passage of gas or
extrapapillary cavitation.1 It typically appears as a single silicone oil from the vitreous cavity to the subretinal space in
oval shaped hypopigmented grey–white excavation of the eyes with cavitary anomalies of optic disc, including ODP.11-
optic disc and most commonly located at inferotemporal 13 However, glycosaminoglycans and mucopolysaccharides
quadrant, but may also be found elsewhere.2 ODPs are which are components of the vitreous, were not found in
rare with an incidence of 1 in 10,000 people with no gender the subretinal fluid on histopathological examination of 2
predilection.3 These are typically unilateral while in 15% human eyes with ODP maculopathy.
cases, bilateral optic disc pits are found.4 ODPs occurrence is
usually sporadic, but possible autosomal inheritance is also The second possible source of fluid is the cerebrospinal fluid
suggested in literature with multiple affected members of (CSF). It is proposed that there is direct communication of
same pedigree.5,6 subretinal and subarachnoid space through optic disc pit
which allow the entrance of CSF to intraretinal and subretinal
Histologically, an ODP is a herniation of dysplastic retina space leading to ODP maculopathy.14 In several OCT based
through a defect in lamina cribrosa which extends into the studies, direct communication was documented between
subarachnoid space creating an anomalous communication the subarachnoid and subretinal space.15,16 Intracranial
between the intraocular and extraocular spaces, a feature migration of silicone oil was also reported in a patient with
shared by all congenital cavitary anomalies of the optic ODP who underwent retinal detachment surgery.17 Similar
disc.7,8 connection between subarachnoid and subretinal spaces was
also reported in morning glory anomaly.18
Pathogenesis
The third possible source of fluid is leakage from blood
Despite ODP maculopathy being a well-known entity vessels at optic disc pit. It is based on the finding of FFA in
for several decades, the nature and origin of the fluid still ODP-M cases where late hyperfluoroscence was seen at the
remains controversial. On literature search, available data disc pit as well as in the area of macular edema.19,20
includes case reports and small case series with no large or
comparative studies.

Four different possible sources have been proposed for the The fourth possible source of fluid is from the choroid,
fluid seen in intraretinal and subretinal spaces leading to through the Bruch’s membrane and peripapillary atrophy
macular detachment in ODP maculopathy cases. There are but it is unlikely because no subretinal and intraretinal fluid
no known trigger factors for ODP maculopathy and it can was documented in other diseases which cause significant
occur at any age. peripapillary and chorioretinal atrophy.21

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DJO Vol. 32, No. 2, October-December 2021

Clinical Presentation Differential Diagnosis

ODP is usually asymptomatic and can be an incidental • Optic nerve coloboma
finding. If this defect is large, it can cause visual field defects; • Choroidal & scleral crescent
paracentral arcuate scotomas and enlarged blind spot • Tilted disc
(Figure 1). Vision is typically unaffected but if associated • Circumpapillary staphyloma
with ODP maculopathy it can cause significant visual • Hypoplastic disc
deterioration. There are no known trigger factors for ODP • Glaucomatous optic neuropathy (Pseudo-pit)
maculopathy development and it usually occurs in third and
fourth decade of life.22 In long standing cases, when there Management
is coexisting macular detachment, it can be associated with
cystoid changes, lamellar or full-thickness macular holes and Spontaneous resolution of ODP-M had been reported in
retinal pigment epithelium atrophy, leading to irreversible literature, however treatment is warranted as most cases
visual impairment with poor prognosis.23 suffer gradual deterioration with significant visual loss
secondary to maculopathy.
Diagnosis
Outdated Treatment Modalities
• 78D/90D bio microscopy: appears as a unilateral, small,
oval, hypo-pigmented grey–white excavation of optic Conservative management with oral corticosteroids has
disc (Figure 1). been tried but however resorbed fluid recurrs following
discontinuation of the steroids.24
• OCT: If on OCT, nasal limit of serous macular
detachment is not seen, disc should be checked If ODP Laser photocoagulation has been done for ODP maculopathy
is associated with maculopathy, a schisis like separation management with laser spots were applied temporally to
between inner and outer retina and a larger serous optic disc. The resultant laser scars act as a barrier between
macular detachment would be present (Figure 2). disc pit and subretinal space, thus preventing entrance
of fluid into macula.25 Argon-blue green laser has most
• Visual field testing: On VFA, enlarged blind spot or commonly being used; followed by green and krypton
arcuate scotoma can be seen, even in asymptomatic lasers.26,27 This technique has very low success rate; due to
cases. absorption of laser energy by RPE and choroid with minimal
effect on macular schisis. It is associated with significant
Figure 1: L/E fundus photo showing grey-white excavation in temporal visual defects post laser and carries poor prognosis in cases
quadrant of ONH with macular deem and pigmentary changes at macula s/o where schisis is associated with macular detachment.

ODP-Maculopathy Current Treatment Modalities

Figure 2: SD-OCT MM6 scan showing sub retinal and intraretinal fluid at Pars plana vitrectomy (PPV): is the treatment of choice for
macula which is communicating on nasal side s/o ODP-Maculopathy ODP maculopathy. Induction of complete posterior vitreous
detachment (PVD) and release of vitreous traction at
macula and optic disc pit are key factors to achieve macular
reattachment. A 23 guage PPV is most commonly used either
alone or in combination with laser application, ILM peeling
and/or gas tamponade. After doing core vitrectomy, PVD is
surgically induced by aspiration with a backflush needle. It
can be combined with inverted ILM flap technique in which
ILM peeling is done by using an intraocular end- gripping
forceps over the superior macular region and peeling was
extended in a circumferential manner over macular area
at an area of about two disk diameters around the fovea,
without touching retinal surface. The peeled ILM flap is used
to cover the optic disc including ODP excluding fovea. It can
be combined with laser application at the temporal side of
ODP as an adjunctive treatment. At the end, gas tamponade
is done either with SF6 or C3F8.28 Few studies in literature
using PPV for treatment of ODP maculopathy show
promising long-term results for both retinal re-attachment
and visual and functional improvement. The majority of
studies reported high anatomical success rate of ~50%–95%
with visual acuity improvement in 50% of cases.29-32
A recent study, suggested that optical coherence tomography
features of ODP maculopathy may predict the surgical
outcomes after PPV. Specifically, multilayer intraretinal

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DJO Vol. 32, No. 2, October-December 2021

and subretinal fluids were less likely to have visual success, References
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the management of ODP maculopathy remains challenging 10. Jain N, Johnson MW. Pathogenesis and treatment of maculopathy
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maculopathy management is PPV, either alone or in associated with cavitary optic disc anomalies. Am J Ophthalmol.
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acts equally well independently of fluid origin but procedure
needs experience and has a long learning curve. Recent 11. Dithmar S, Schuett F, Voelcker HE, Holz FG. Delayed sequential
treatment alternatives, such as inner retinal fenestration,
glial tissue removal, and autologous fibrin, show promising occurrence of perfluorodecalin and silicone oil in the subretinal
results, to confirm these preliminary outcomes.
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optic disc pit. Arch Ophthalmol 2004;122:409-11.

12. Salam A, Khan-Lim D, Luff AJ. Superior retinal detachment in

an oil-filled eye with a colobomatous optic disc. Retin Cases

Brief Rep 2008;2:124-5.

13. Coll GE, Chang S, Flynn TE, Brown GC. Communication between

the subretinal space and the vitreous cavity in the morning glory

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14. Türkçüoğlu P, Taskapan C. The origin of subretinal fluid in optic

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2016;47(3): 294-8.

15. Krivoy D, Gentile R, Liebmann JM, Stegman Z, Rosen R, Walsh

JB et al. Imaging congenital optic disc pits and associated

maculopathy using optical coherence tomography. Arch

Ophthalmol 1996;114:165-70.

16. Rutledge BK, Puliafito CA, Duker JS, Hee MR, Cox MS. Optical

coher- ence tomography of macular lesions associated with optic

nerve head pits. Ophthalmology 1996;103:1047-53.

17. Kuhn F, Kover F, Szabo I, Mester V. Intracranial migration of

silicone oil from an eye with optic pit. Graefes Arch Clin Exp

Ophthalmol 2006;244:1360-2.

18. Chang S, Haik BG, Ellsworth RM, St Louis L, Berrocal JA.

Treatment of total retinal detachment in morning glory

syndrome. Am J Ophthalmol 2012;97:596-600.

19. Gordon R, Chatfield RK. Pits in the optic disc associated with

macular degeneration. Br J Ophthalmol 1969;53:481-9.

20. Theodossiadis GP, Ladas ID, Panagiotidis DN, Kollia AC,

Voudouri AN, Theodossiadis PG. Fluorescein and indocyanine

green angiographic findings in congenital optic disk pit

associated with macular detachment. Retina 1999;19:6–11.

21. Sadun AA, Khaderi KH (2013) Optic disc anomalies, pits, and

associated serous macular detachment. In: Ryan SJ, Schachat AP,

Wilkinson CP, Hinton DR, Sadda SR, Wiedemann P (eds) Retina,

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22. Brockhurst RJ. Optic pits and posterior retinal detachment.

Trans Am Ophthalmol Soc.1975;73:264-91.

23. Georgalas I, Ladas I, Georgopoulos G, Petrou P. Optic disc pit: a

review. Graefes Arch Clin Exp Ophthalmol 2011;249:1113-22.

24. Reed D. Congenital pits of the optic nerve. Clin Eye Vis Care

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1999;11:75–80. Cite This Article as: Monika Dahiya, Manisha Nada,
25. Brockhurst RJ. Optic pits and posterior retinal detachment. Jitender Phogat, Sakshi Lochab, Preeti Yadav, Surender Kumar.
Optic Disc Pit Maculopathy: Review of Recent Advances in Its
Trans Am Ophthalmol Soc. 1975;73:264-91. Diagnosis and Management. Delhi J Ophthalmol 2021; 32 (2):
26. Bonnet M. Serous macular detachment associated with optic 20 - 23.
Acknowledgments: Nil
nerve pits. Graefes Arch Clin Exp Ophthalmol. 1991;229(6):526- Conflict of interest: None declared
32. Source of Funding: None
27. Theodossiadis G. Treatment of retinal detachment with Date of Submission: 21 May 2021
congenital optic pit by krypton laser photocoagulation. Graefes Date of Acceptance: 06 Dec 2021
Arch Clin Exp Ophthalmol. 1988;226(3):299.
28. Bartz-Schmidt KU, Heimann K, Esser P. Vitrectomy for macular Address for correspondence
detachment associated with optic nerve pits. Int Ophthalmol Monika Dahiya Senior Resident
1995;19:323-9.
29. Teke MY, Citirik M. 23 Gauge vitrectomy, endolaser, and Regional Institute of Opthalmology
gas tamponade versus vitrectomy alone for serous macular PGIMS Rohtak, India.
detachment associated with optic disc pit. Am J Ophthalmol. E-mail: [email protected]
2015;160(4):779.
30. Rayat JS, Rudnisky CJ, Waite C, et al. Long-term outcomes Quick Response Code
for optic disk pit maculopathy after vitrectomy. Retina.
2015;35(10):2011-7.
31. Avci R, Yilmaz S, Inan UU, et al. Long-term outcomes of pars
plana vitrectomy without internal limiting membrane peeling
for optic disc pit maculopathy. Eye. 2013;27(12):1359-67.
32. Avci R, Kapran Z, Ozdek Ş, et al. Multicenter study of pars plana
vitrectomy for optic disc pit maculopathy: MACPIT study. Eye.
2017;31(9):1266-73.
33. Steel DH, Williamson TH, Laidlaw DA, et al. Extent and location
of intraretinal and subretinal fluid as prognostic factors for the
outcome of patients with optic disk pit maculopathy. Retina.
2016;36(1):110-8.
34. Ziahosseini K, Sanghvi C, Muzaffar W, Stanga PE. Successful
surgical treatment of optic disc pit maculopathy. Eye
2009;23:1477-9.
35. Ishikawa K, Terasaki H, Mori M, Sugita K, Miyake Y. Optical
coherence tomography before and after vitrectomy with
internal limiting membrane removal in a child with optic disc
pit maculopathy. Jpn J Ophthalmol 2005;49:411-3.
36. Ehlers JP, Kernstine K, Farsiu S, Sarin N, Maldonado R, Toth
CA. Analysis of pars plana vitrectomy for optic pit-related
maculopathy with intraoperative optical coherence tomography:
a possible connection with the vitreous cavity. Arch Ophthalmol
2018;129:1483-6.
37. Schaal KB, Wrede J, Dithmar S. Internal drainage in optic pit
maculopathy. Br J Ophthalmol 2007;91:1093.
38. Lei L, Li T, Ding X, et al. Gas tamponade combined with
laser photocoagulation therapy for congenital optic disc pit
maculopathy. Eye. 2015;29(1):106–114.
39. Theodossiadis GP. Treatment of maculopathy associated
with optic disk pit by sponge explant. Am J Ophthalmol.
1996;121(6):630-7.
40. Theodossiadis GP, Theodossiadis PG. The macular buckling
technique in the treatment of optic disk pit maculopathy. Semin
Ophthalmol. 2000;15(2):108-15.
41. Theodossiadis GP, Theodossiadis PG. Optical coherence
tomography in optic disk pit maculopathy treated by the
macular buckling procedure. Am J Ophthalmol. 2001;132(2):184-
90.
42. Inoue M, Shinoda K, Ishida S. Vitrectomy combined with glial
tissue removal at the optic pit in a patient with optic disc pit
maculopathy: a case report. J Med Case Rep. 2008;2:103.
43. Rosenthal G, Bartz-Schmidt KU, Walter P, Heimann K.
Autologous platelet treatment for optic disc pit associated
with persistent macular detachment. Graefes Arch Clin Exp
Ophthalmol. 1998;236(2):151-53.
44. Ozdek S, Ozdemir HB. A new technique with autologous fibrin
for the treatment of persistent optic pit maculopathy. Retin
Cases Brief Rep. 2017;11(1):75-8.
45. Todorich B, Sharma S, Vajzovic L. Successful repair of recurrent
optic disk pit maculopathy with autologous platelet rich
plasma: report of a surgical technique. Retin Cases Brief Rep.
2017;11(1):15–17.

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DJO Vol. 32, No. 2, October-December 2021

Original Article

Refractive Errors and Concomitant Strabismus in Children and
Adolescents: A Hospital Based Observational Study

Anupam Singh,1 Omna Chawla,2 Rupal Verma,1 Vartika Saxena,3 Ranjeeta Kumari,1
Nisheeta Patnaik,1 Barun Kumar,4 Devesh Kumawat,1

1Department Of Ophthalmology, All India Institute of Medical Sciences, Rishikesh, India.
2Department Of Physiology, Seema Dental College. Rishikesh, India.

3Department of Community and Family Medicine, All India Institute of Medical Sciences, Rishikesh, India.
4Department of Cardiology, All India Institute of Medical Sciences, Rishikesh, India.

Purpose: To study the different types of concomitant strabismus and associated refractive errors in children and adolescents.

Material & Methods: In this observational study, 178 patients aged between 4-19 yrs with concomitant strabismus of ≥ 15
Prism Dioptre (PD) on prism bar cover test were included and examined by a strabismus expert for type and amount of deviation
between July 2017 to June 2020 at a tertiary health care hospital in north India. Non-cycloplegic and cycloplegic refraction was
performed by a trained optometrist. Glasses were prescribed to achieve the best corrected visual acuity (BCVA). Data collection
was done using a predesigned and pretested questionnaire.

Results: The mean age of the study population was 11.20 ± 5.49 years (Median: 10, range = 4 -19 years) and 97 patients
Abstract (55.5 %) were male and 81 (45.5%) were female. Seventy-five patients (42.13%) were esotropes, 87 (48.87%) were exotropes

and 16 (8.99%) were in combined deviation group. Hypermetropia (83/140, 59.28%) was the most common type of refractive
error among ametropes (140/178). Among the hypermetropes, 52 (62.7%) had esotropia and 25 (64.1%) of the myopes had
exotropia. Further, 52 (69.3%) of the esotropes had hypermetropia but there was no such trend of error noticed among the
exotropes (p value < 0.001, Chi-square test). There were 70 (39.32%) amblyopes in the study population, out of which 35 (50%)
had esotropia (p value: 0.032, Chi-square test).

Conclusion: Hypermetropia was the most common refractive error among the study population and hypermetropes were prone
to have esotropia and vice-versa. Esotropia and hypermetropia were more common among amblyopes.

Delhi J Ophthalmol 2021; 32; 24-29; Doi http://dx.doi.org/10.7869/djo.711
Keywords: Refractive Error, Concomitant Strabismus, Esotropia, Exotropia, Children, Adolescents, Amblyopia.

Introduction refractive errors and strabismus are considered significant
contributors to visual impairment and treatable blindness in
Strabismus is a condition wherein there is a misalignment the general population.
of visual axes of the two eyes leading to trouble in the
binocular vision. It's prevalence ranges from 0.8% to Prevalence of visual impairment due to refractive errors
5.65%.1,2,3,4,5 Strabismus which means “to squint,” arises varies from 0.4% to 42% in the general population and there
from the Greek word “strabismós,” and is a common ocular is a lot of age-specific variability from one country to another
disorder where normal binocular vision is not possible with and even from rural to urban areas of the same country.10 At
misaligned eye leading to poor depth perception. Further, the same time, several studies have pointed out that there
good depth perception is essential for the normal growth exists an association between concomitant strabismus and
and development of children as it is directly associated with refractive errors. Some have pointed that the children who
better reading ability and achievement of developmental had hyperopia had developed accommodative esotropia.11,12
milestones on time. However, the degree of increased risk of accommodative
esotropia in relation to the severity of hyperopia is not well
The minute movements in eye-to-eye reciprocity, duration documented. On the other hand, some researchers observed
and quality of gaze are also considered significant for the concomitant exotropia was more prevalent in Asia
effective learning and emotional communication as well and was also related to the higher prevalence of myopia.13
as the effectiveness of emotional regulation.6,7,8 In fact, However, there are still some gaps in literature on how
for the development of a personality, the integrated and different types of strabismus is related to refractive errors,
elaborate neural networks are substantially dependent on especially in India. Thus, our study focuses on exploring
the reciprocal gaze. Thus, strabismus can lead to varying these relationships as it has potential implication for public
degree of psychosocial impact and social prejudice which health.
has a negative impact on socialization and employability.9
The risk of strabismus is believed to be higher for those with In this hospital-based cross-sectional observational study,
a family history of strabismus, premature birth, low birth we aimed to evaluate the different types of concomitant
weight, birth asphyxia, refractive error, amblyopia, etc. In strabismus in relation to the types of refractive errors among
the recent past, there has been an interest in the scientific the children presenting to ophthalmic outpatient department
community for the association of errors of refraction with (OPD) of a tertiary health care Institute in North India.
the strabismus as there are considerable number of changes
in the refraction during infancy and childhood. Both the

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DJO Vol. 32, No. 2, October-December 2021

Material and Methods 1% eye drops. Refractive errors were classified according to
the spherical equivalents (SE). Emmetropia was defined as
This cross sectional observational study was conducted in SE -0.5 to <+0.5 diopters (D), myopia as SE <-0.5 D, hyperopia
the department of ophthalmology from July 2017 to June as SE > +0.50 D, and astigmatism as cylindrical error >1D.
2020. The study followed tenets of Declaration of Helsinki In case of anisometropia, SE of the less hyperopic eye was
and has clearance from Institutional Ethics Committee. A considered to define refractive error as accommodative
total of 1441 patients who presented to ophthalmic OPD convergence is driven by the less hyperopic eye. In patients
during the above-mentioned period were screened by with bilateral astigmatism, the more astigmatic eye was used
trained ophthalmology residents. Patients with age beyond to define astigmatic refractive error. Best corrected visual
the range of 4-19 years and with history of previous squint acuity (BCVA) of worse eye was considered for statistical
surgery or incomitant strabismus were excluded. Those who analysis. Unilateral amblyopia was defined as the difference
declined to participate (n=182) or those who did not meet of at least of two lines between visual acuities of both eyes,
the inclusion criteria (n=1026) were excluded from the study. and bilateral amblyopia was defined as decreased best
Written informed consent from at least 1 parent or guardian corrected visual acuity ≤ 6/12 or 20/40 in both eyes.
was obtained. Assent was obtained whenever applicable
before examination. A total of 233 patients of concomitant Data was collected using a predesigned, pretested
deviation who met the inclusion criteria of ≥ 7 0 of deviation questionnaire. The variables which were recorded included
on Hirschberg corneal light reflex test and deviation of ≥15 birth history, family history, onset and duration of deviation,
Prism Dioptre (PD) on prism bar cover test were referred history of use of glasses, duration of use of glasses, impact of
to strabismus expert for further assessment. Patients were glasses use on the amount of deviation etc. The questionnaire
reassessed by Hirschberg corneal light reflex test, cover- was pre-validated before initiating the data collection.
uncover test, prism bar cover test and Krimsky test. Patients
with constant or intermittent deviation ≥15 PD on prism bar Statistical analysis
cover test (for near or distance, with or without glasses) were
included in the study. Fifty-five participants (n=55) were Data were entered in Microsoft Excel spreadsheet (Microsoft
excluded because of incomplete data and 178 participants Corporation, USA) and analysed using SPSS 23.0 (Statistical
were included for final analysis. (Figure 1) shows a flow Package for the Social Sciences, SPSS Inc., USA). Mean
chart of enrolment of the participants to the study. and standard deviation was calculated for all the data. For
Visual acuity (both aided and unaided) was assessed by comparisons of means, t test was used and for proportions,
Snellen’s chart or its equivalent. All the patients underwent dry Chi-square test was used. Pearson Chi-square test, likelihood
retinoscopy and cycloplegic refraction under cyclopentolate ratio, Fisher’s exact test and linear by linear association were
calculated for association between all the data. In all cases,
Figure 1: Flow chart of recruitment of patients to the study. P<0.05 was considered statistically significant. For statistical
analysis, when the number of patients in the same group was
smaller than 30 (n < 30), the non-parametric tests of Kruskal–
Wallis (χ2) for three or more groups and Mann–Whitney (U)
for two groups were performed. ANOVA for three or more
groups and independent T-test for two groups were used to
compute statistics when the number of patients was at least
30 or larger than 30 (n ≥ 30). The confidence level (CI) used in
this study was 95%, with alpha = 0.05 (α = 0.05).

Results

The mean age of study population was 11.20 ± 5.49 years
(Median: 10, range = 4-19 years). Out of these 178 patients,
97 (55.5 %) were male and 81 (45.5%) were female. Seventy-
five patients (42.13%) were esotropes, 87 (48.87%) were
exotropes and 16 (8.99%) were in combined group who had
both horizontal and vertical deviation. The mean deviation
of the study population was 39.22 ± 11.74 PD.

No clinically significant refractive error (emmetropia) was
noticed in 38 (21.34%) patients in the study population,
whereas 83 (46.63%) had hypermetropia, 39 (21.91%)
had myopia, 5 (2.8%) had astigmatism and 13 (7.3%) had
combined refractive error. Among the hypermetropes, 52
(62.7%) had esotropia and 29 (34.9%) had exotropia, whereas
25 (64.1%) and 8 (20.5%) of the myopes had exotropia and
esotropia respectively. Further, 52 (69.3%) of the esotropes
had hypermetropia but there was no such trend noticed
among the exotropes (p value < 0.001, Chi-square Test)
(Table 1, Figure 2).

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DJO Vol. 32, No. 2, October-December 2021

Table 1: Associations of various types of deviation

Type of Refractive Esotropia Exotropia Combined P value
Error (Row %)

Emmetropia 11 22 5 (13.2%)
(28.9%) (57.9%)

Myopia 8 25 6 (15.4%) <0.001
Hypermetropia (20.5%) (64.1%) 2 (2.4%) (Chi-Square

52 29 test)
(62.7%) (34.9%)

Astigmatism 0 (0.0%) 3 2 (40.0%)
(60.0%)

Combined 4 8 1 (7.7%)
(30.8%) (61.5%)

Best Corrected Figure 3: Bar diagram showing association between BCVA and type of
Visual Acuity deviation.
(BCVA) (Row %)
There were 70 (39.32%) amblyopes in the study population,
Group 1 6 9 2 (11.8%) 0.022 out of which 35 (50%) had esotropia. Further, 40 patients
(≤6/60) (35.3%) (52.9%) 7 (14.9%) (Fisher’s exact (53.3%) of the esotropia group had amblyopia whereas there
7 (6.1%) were only 26 (29.9%) amblyopes among exotropia group
Group 2 26 14 test) (p value: 0.032, Chi-square Test) (Table 1). There was no
(6/60-6/18) (55.3%) (29.8%) association between type of squint and age, gender, amount
of refractive error, anisometropia, astigmatism and duration
Group 3 43 64 of use of glasses.
(6/12-6/6) (37.7%) (56.1%)
According to the amount of deviation, the study population
Amblyopia (Row%) was divided into 3 groups, group A included patients with
15-35 PD of deviation; group B included patients with 36-
Amblyope 35 (50.0%) 26 (37.1%) 9 (12.9%) 0.032 55 PD of deviation and group C included patients with 56-
Non-amblyope 40 (37.0%) 61 (56.5%) 7 (6.5%) (Chi-Square test) 75 PD of deviation. There were 72 (40.44%), 98 (55%) and 8
(4.49%) patients in group A, B and C respectively (Table 2).
There was a statistically significant association between
onset of deviation within 6 months of age and amount
of deviation. 71 patients had onset of deviation within 6
months of age, 40 (56.3%) of them belonged to group B (36-
55 PD) (p value: 0.007, Fisher’s Exact Test). Also, there was
a statistically significant association between BCVA and
amount of deviation. 63 out of 72 (87.5%) of group A patients
had BCVA of 6/12-6/6 in the worse eye (p value:<0.001, Chi-
square test) (Table 2), (Figure 4).

Table 2: Associations of various amount of deviation

Figure 2: Bar diagram showing association between type of refractive error Best Group A Group B Group C p-value
and type of deviation. Corrected (15-35PD (36-55PD (56-75PD
Visual deviation) deviation) deviation)
A total of 112 patients had history of use of glasses, out of Acuity
which 19 (16.96%) noticed decrease in amount of deviation, (BCVA)
3 (2.67%) had increase in deviation and 90 (80.35%) did not (Row %)
notice any change in deviation after using glasses. Out of 19
patients who noticed decrease in deviation, 10 (52.6%) were Group 1 1 (5.9%) 16 (94.1%) 0 (0.0%) <0.001
hypermetropes (p value:<0.001, Chi- square test). (≤6/60) 8 (17.0%) 37 (78.7%) 2 (4.3%) (Chi-
63 (55.3%) 45 (39.5%) 6 (5.3%) Square
Based on BCVA of the worse eye, the study population was Group 2 Test)
divided into 3 groups. Group 1 included patients with BCVA (6/60-6/18)
of < 6/60, group 2 had BCVA between 6/60 to 6/18 and group
3 had BCVA of 6/12 to 6/6 in the worse eye. There were 17 Group 3
(9.5%), 47 (26.4%) and 114 (64%) patients in group 1, 2 and 3 (6/12-6/6)
respectively. Most of the patients i.e., 43 (57.3%) of esotropes,
64 (73.6%) of exotropes, and 7 (43.8%) of combined group Amblyopia
had visual acuity between 6/12 to 6/6 in the worse eye (p (Row %)
value: 0.022, Fisher’s Exact Test) (Table 1 Figure 3).
Amblyope 10 (14.3%) 57 (81.4%) 3 (4.3%) <0.001
62 (57.4%) 41 (38.0%) 5 (4.6%) (Chi
Non- Square
Amblyope Test)

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DJO Vol. 32, No. 2, October-December 2021

Figure 4: Bar diagram showing association between BCVA and Amount of 10 (52.6%) were hypermetrope which suggest that use of
deviation hypermetropic correction relaxes accommodation and thus
decreases amount of deviation in esotropes.
Sixty-two patients (86.1%) of group A had no amblyopia
A population-based study done to investigate risk factors for
whereas 57 (58.2%) of group B patients had amblyopia. strabismus in infant and young children (age group of 6-72
months)12 found that hypermetropia and anisometropia
Further, 57 out of 70 amblyopes (81.4%) were in group B (p are risk factors for esotropia and patients with astigmatism
are more prone to develop exotropia. Esotropia was
value: <0.001, Chi- square test). No significant association more common in hypermetropic children than in non-
hypermetropes. Esotropia was more common in infants with
was found between amount of deviation and age, gender, moderate hypermetropia than emmetropic controls.20,21,22
They concluded that it is important to correct childhood
type of squint, type of refractive error, amount of refractive hypermetropia of ≥2.00 D. Findings of our study are partly
consistent with those of the above-mentioned study.
error, anisometropia, astigmatism and duration of use of
The present study revealed that myopia was related to the
glasses. occurrence of exotropia which is consistent with the findings
of a population based study.2 However, data from another
Discussion population-based observational study indicated that there
was a significant trend to develop myopia overtime in
Uncorrected refractive errors are common cause for children with intermittent exotropia.23 This association can be
explained by the fact that increased accommodative demand
blindness14 and are the leading cause for moderate to severe in patients with intermittent exotropia2 may promote
development of myopia as reduction of accommodation
visual impairment globally.14,15 Consequently, the Global slows down progression of moderate myopia.24 Thus, we do
not conclude that myopia increases risk for exotropia which
initiative of World Health Organization for the prevention of was the finding of our study. Further population-based
study is required to clarify this association.
avoidable blindness (Vision 2020) has pointed this as focus
Most of the patients of our study population (64%) had
area which needs urgent intervention for public health.16 BCVA between 6/12 to 6/6 in worse eye. Also, there was
a statistically significant association between BCVA and
Children and adolescents with uncorrected refractive amount of deviation. 87.5% of patients having deviation
within range of 15-35 PD had BCVA of 6/12-6/6 in the worse
error may experience reduced visual acuity and abnormal eye. It means that ensuring good BCVA with glasses cannot
prevent development of strabismus and factors other than
binocular interaction which can lead to strabismus.A research good vision do play a role in development of strabismus.
However, good BCVA leads to less amount of deviation
in this field has suggested that strabismus has multifactorial which can be explained by better binocular interaction and
fusion.
aetiology. Various studies have suggested that infants with Among amblyopes (39.32% of the study population), 50%
had esotropia and 53.3% of esotropes had amblyopia. Thus,
hypermetropia are more likely to develop strabismus.17 there was a statistically significant association between
amblyopia and esotropia which can be explained by the fact
Furthermore, childhood hypermetropia if left uncorrected that in esotropia, the non-dominant temporal retina of the
deviating eye has to compete with more dominant nasal half
can lead to refractive accommodative esotropia.18,19 of the other eye. Also, 81.4% of amblyopes had deviation
within range of 36-55 PD. It means that amblyopia and poor
However, there are few studies showing the relationship of binocular interaction lead to greater amount of deviation.
This finding is consistent with that of vision in preschoolers
hypermetropia with other types of strabismus and also how study group (VIP), a population-based study which found
that the presence and magnitude of hypermetropia among
other types of refractive errors influence strabismus. preschoolers was associated with an increased proportion of
amblyopia and strabismus.25
In our study, hypermetropia was found to be the most
common type of refractive error followed by myopia, Further, 56.3% of patients having history of onset of
combined refractive error and astigmatism. Among the deviation within 6 months of age had deviation between 36-
hypermetropes, 62.7% patients had esotropia and 34.9% 55 PD. This implies that onset within 6 months of age leads
had exotropia, whereas 20.5% and 64.1% of the myopes had to greater amount of deviation as compared to onset at later
esotropia and exotropia respectively. Further, 69.3% of the age.
esotropes had hypermetropia but there was no such trend
noticed among the exotropes. So, there was a statistically
significant bidirectional association between hypermetropia
and esotropia but the association was unidirectional between
myopia and exotropia (p value < 0.001, Chi-square Test). The
association of hypermetropia and esotropia can be explained
by increased demand of accommodation in hypermetropes
leading to more accommodative convergence, thus more risk
of developing esotropia. Also, 19 patients noticed decrease
in amount of deviation after use of glasses, out of which

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DJO Vol. 32, No. 2, October-December 2021

Our study did not find any significant association between References
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prone to have esotropia and vice-versa. Good BCVA with https://doi.org/10.1167/iovs.03-1051
glasses does not prevent development of strabismus but it
can help to reduce the amount of deviation by improving 6. Cañigueral R, Hamilton AF de C.The role of eye gaze during
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providers including parents in making decision regarding
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in time. However, further population based study with large SH. The negative psychosocial impact of strabismus in adults.
sample size is required to confirm the results of the study Journal of AAPOS.1999;3:209–211. https://doi.org/10.1016/S1091-
and to find any possible association between exotropia and 8531(99)70004-2
myopia.
8. Satterfield D, Keltner JL, Morrison TL. Psychosocial aspects of
Compliance with Ethical Standards: This study was strabismus study. Arch Ophthalmol. 1993 Aug;111(8):1100-5. doi:
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9. Atowa UC, Hansraj R, Wajuihian SO. Vision problems: A review
Funding: This study was a non-funded intramural project of prevalence studies on refractive errors in school-age children.
conducted at All India Institute of Medical Sciences, African Vision and Eye Health.2019; 78:1–7. https://doi.org/10.4102/
Rishikesh. (Project number: 117/IM/2015) aveh.v78i1.461

Data Availability: The data will be made available if 10. Naidoo KS, Leasher J, Bourne RR, Flaxman SR, Jonas JB, Keeffe J
required. et al. Global Vision Impairment and Blindness Due to Uncorrected
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Ethical approval: All procedures performed in studies doi: 10.1097/OPX.0000000000000796. PMID: 26905537.
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with the 1964 Helsinki declaration and its later amendments diagnosis of strabismus in a population-based sample of 12-year-
or comparable ethical standards. old Australian children. Am J Ophthalmol. 2006 Dec;142(6):1085-8.
doi: 10.1016/j.ajo.2006.06.053.
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Clinical trial registration: This was an observational study Lin J, Wen G, Wei J, et al.Risk factors associated with childhood
approved by institutional ethics committee, so it did not fit strabismus: The multi-ethnic pediatric eye disease and
into criteria for trial. baltimorepediatric eye disease studies. Ophthalmology. 2011;
118:2251–2261. https://doi.org/10.1016/j.ophtha.2011.06.032

13 McKean-Cowdin R, Cotter SA, Tarczy-Hornoch K, Wen G, Kim J,
Borchert M et al. Prevalence of amblyopia or strabismus in Asian
and non-Hispanic white preschool children: Multi-ethnic pediatric
eye disease study. Ophthalmology. 2013; 120:2117–2124. https://
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14. Lou L, Yao C, Jin Y, Perez V, Ye J.Global patterns in health burden
of uncorrected refractive error. Investigative Ophthalmology and
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T, Cicinelli MV, et al.Global causes of blindness and distance
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16. Pizzarello L, Abiose A, Ffytche T, Duerksen R, Thulasiraj R, Taylor
H, et al.VISION 2020: The Right to Sight - A Global Initiative to
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17. Walsh LA, Laroche GR, Tremblay F .The use of binocular visual

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DJO Vol. 32, No. 2, October-December 2021

acuity in the assessment of intermittent exotropia. Journal of Cite This Article as: Anupam Singh, Omna Chawla, Rupal
AAPOS : the official publication of the American Association for Verma, Vartika Saxena, Ranjeeta Kumari, Nisheeta Patnaik,
Pediatric Ophthalmology and Strabismus / American Association Barun Kumar, Devesh Kumawat. “Refractive errors and
for Pediatric Ophthalmology and Strabismus.2000; 4:154–157. concomitant strabismus in children and adolescents: A hospital
https://doi.org/10.1016/S1091-8531(00)70005-X based observational study” Delhi J Ophthalmol 2021 32 (2) 24 -29.
18. Fu J, Li SM, Liu LR, Li JL, Li SY, Zhu BD, et al. Prevalence of Acknowledgments: Nil
amblyopia and strabismus in a population of 7th-grade junior high Conflict of interest: None declared
school students in central china: The anyang childhood eye study Source of Funding: None
(ACES). Ophthalmic Epidemiology.2014; 21:197–203. https://doi.or Date of Submission 29 Jun 2021
g/10.3109/09286586.2014.904371 Date of Acceptance: 04 Dec 2021
19. Chia A, Lin X, Dirani M, Gazzard G, Ramamurthy D, Quah BL, et
al. Risk factors for strabismus and amblyopia in young Singapore Address for correspondence
Chinese children. Ophthalmic Epidemiology.2013; 20:138–147. Anupam Singh, Additional
https://doi.org/10.3109/09286586.2013.767354
20. Miller JM .Clinical applications of power vectors. Optometry and Professor,
Vision Science.2009; 86:599–602. Department of Ophthalmology,
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P. Methods for a population-based study of myopia and India.
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24. O'Connor AR, Stephenson TJ, Johnson A, Tobin MJ, Ratib S,
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1701 g. Arch Ophthalmol. 2002 Jun;120(6):767-73. doi: 10.1001/
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25. Marjean T. Kulp, Gui-Shuang Ying, Jiayan Huang, Maureen G.
Maguire, Elise Ciner, Lynn A. Cyert, et al. Vision In Preschoolers
(VIP) Study Group; Relationship of Hyperopia with Amblyopia,
Strabismus, and Stereoacuity in Preschool Children. Invest.
Ophthalmol. Vis. Sci. 2012;53(14):153.

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DJO Vol. 32, No. 2, October-December 2021

Original Article

A Critical Analysis of Publications in The IJO Over 2006-15

Shalini Gupta,1 A K Amitava2

1Department Of Ophthalmology, Dr Shroff charity eye Hospital Delhi, India.
2Department Of Ophthalmology, JNMCH, AMU, Aligarh, Utter Pardesh, India.

Purpose: To qualitatively evaluate original articles in the IJO published over the decade from 2006 to 2015, using specific critical
appraisal checklists (CACL), to assess whether publications have improved over time.

Methods: All articles were classified either as systematic reviews/meta-analysis; randomised control trials; before and after

studies; cohort studies; observational or descriptive and diagnostic test studies: and Economic analysis scored using CACLs.

Scores were converted to percentages and compared between the earlier five years (E5Ys) and the later five (L5Ys). Annual

trend in article counts and CACL scores were assessed. T-test and ANOVA, with significance set at p ≤ 0.05 was used. 95% CIs are

Abstract quoted.
Results: Of a total of 529 original articles, there were 159 in the E5Ys, in 28 issues, while in the L5Ys; there were 370 articles in 48

issues. The mean score for E5Ys was 81.9±9.8% compared to 80.9±12.4% for L5Ys: p=0.26. Maximum articles (134) were in 2014.

No significant trends in number and scores were observed.

Conclusions: There were no significant qualitative differences in the quality of articles during the decade 2006 to 2015.
Vitreoretinal subspecialty and observational studies formed the main bulk of articles published in the IJO during this decade.
Systematic reviews and meta-analysis, considered the highest level of evidence, have scored the least on CACL.

Delhi J Ophthalmol 2021; 32; 30-35; Doi http://dx.doi.org/10.7869/djo.712

Keywords: Critical Appraisal, Checklists, Evidence Based Medicine, Indian Journal Of Ophthalmology, Quality Of Research.

Introduction following them through time to identify which participants
develop the outcome(s) of interest or look back at data that
The explosion of research has resulted in over 15 million were created prior to the development of the outcome.10 In
articles in 5000 journals monthly as of 2000.1 How good are CCS, researchers identify study participants as diseased or
these articles? How much importance should we assign not diseased and allow for statistical associations between
to the evidence? Despite editorial boards and referees, exposure and outcomes to be established. CXS consist of
poor quality articles slip through. Studies may vary from assessing a population at a single point in time. DTS compare
case reports to the meta-analysis.2,3 Critical appraisal, the a new diagnostic method with the current “gold standard”
cornerstone of evidence based medicine involves: Asking diagnostic procedure in a cross-section of both diseased
questions about research methodology; data analyses; how and healthy participants. EA determine the optimum use
a particular sample size was arrived at; providing a list of of scarce resources, involving comparison of two or more
the inclusion and exclusion criteria; analytical methods alternatives in achieving a specific objective under the given
employed for interpretation of results. The recently constraints.
accessible critical appraisal checklists (CACLs) allow us to
qualitatively comment on the worth of an article.4,5,6,7,8 CACLs have a common theme and are scored as yes (a
We designed this study to qualitatively evaluate original value of 2), can’t say (a value of 1) and no (a value of 0) in
articles in the IJO published over the decade from 2006 to decreasing order of importance. The aspects covered are the
2015 using specific CACLs. following: Does the paper ask a clear research question? Are
the aims of the study clearly stated? Is the methods section
Material and Methods sufficiently clear to allow the research to be repeated by
others? Are the results clearly presented with appropriate
We searched through each article under the headings of graphics and statistical tests? Is the sampling, recruitment
review and original articles in the IJO between Jan 2006 to method and inclusion/exclusion criteria clearly stated? Are
Dec 2015, and included those identified as systematic reviews the strengths and weaknesses of the study fully described? Is
and meta-analysis (SR/MA), randomized control trial (RCT), the referencing adequate, with inclusion of relevant previous
before and after study (BFR/AFR), cohort study (CS), case work? Is there a statement of ethics committee approval?
control study (CCS), descriptive and cross-sectional study
(DS/CXS), economic analysis (EA) and diagnostic test Of the eight CACLs, six (for SR/MA, RCT, CS, CCS, EA, DTS)
study (DTS), and scored them on study-specific CACLs. A were available from Critical appraisal skills programme
“meta-analysis” is “a statistical analysis of the results from (CASP),4 and used with minor modifications.5,11 For
independent studies" 9 RCT take a homogenous group of example, out of 10 questions in the CASP checklist for SR/
participants and randomly divide them into two separate MA, two questions asking for the results and their precision
groups. The intervention is then implemented in one were omitted as they could not be graded ordinally as we
group and comparisons of intervention efficacy between intended to. For RCT, eight out of 11 were retained from
the two groups are analyzed. A BFR/AFR measures the the original, two asking for results were omitted because
occurrence of an outcome before and after a particular of the similar reason given above, while one question
intervention is implemented. CS involve identifying study
participants based on their exposure status and either

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DJO Vol. 32, No. 2, October-December 2021

originally asking whether subjects were masked was altered Most of the articles dealt with vitreo-retina (119) followed
to: whether masking was double, single or not carried out, by glaucoma (78), cataract (60), cornea and conjunctiva (52),
deserving scores of 2, 1 and 0. Six questions, as such or after while those on ocular trauma (10) and ophthalmic education
some modification, were added from Scottish Intercollegiate were the least (7) (Table 1). Study design wise, the DS/
Guidelines Network (SIGN)5,12 checklist for RCT, covering CXS (307, 58%) were the commonest, while EA (3, 0.6%)
the following issues: Was sample size explained? Were comprised the least (Table 2).
treatments the only difference between groups? Drop-out Overall, no significant differences were seen in the mean-
rate in the treatment arm: ≤10% Yes, 10-20% Can’t Say, percentage scores of the E5Ys and L5Ys; subspecialty-wise,
>20% No. If concealment was adequate? How well was only community-ophthalmology and neuro-ophthalmology
biased minimized? With responses being: High quality scores showed a significant drop (Table 1); while study
(Yes), Acceptable (Can’t say), Low (No). Were ethical issues design-wise, only SR/MA scores declined significantly (Table
explained? 2). No trend was noticed over the decade overall (Figure 1,
Table 3), or subspecialty or study-wise (Figure 2,3,4).
For BFR/AFT, we used the CL available from the National
Institute of Health Quality Assessment Tool,13 for DS/CXS, Figure 1: Year-wise trend of mean percentage scores of the articles published
we adopted the one from the Journal of American Medical in the Indian Journal of Ophthalmology during the decade 2006 to 2015. (n =
Association (JAMA) series.14 Maximum scores in specific
checklists were: SR/MA (16); RCT (30); BFR/AFR (24); CS Year-wise number of articles appears on top of the figure).
(28); CCS (20); DS/CXS (22); EA (22); and DTS (18). Question PS: Number of journals in the E5Y = 28 and L5Y = 48
asking whether “all important and relevant studies were
included” proved challenging: Using the keywords we Figure 2: Subspecialty wise distribution of Systematic reviews and meta-
would conduct a detailed search in PubMed, Scopus and analysis published in the Indian Journal of Ophthalmology during the decade
Google scholar, and widen our search by looking up ‘similar
articles’ option, there could still be some uncertainty; 2006 to 2015.
consensus would aid us in coming to our best decision.

Each article was perused and classified by two researchers
working together, on the basis of study-design and the
subspecialty it covered. Some unclassifiable articles were
placed in a miscellaneous group. Each article was then read
by the two researchers and scored. All the questions had an
ordinal response of three grades (yes, cannot say, no; scored
as 2, 1 and 0). This permitted aggregating to a total score
and converting to percentages. Data was uploaded to SPSS.
Mean scores of earlier five years (E5Ys) (2006-2010) were
compared with the later five years (L5Ys) articles (2011-2015).
Moreover, they were sub-analyzed according to study-
design and subspecialty. Annual mean scores, over the 10
years, were assessed for trend. We used t-test and ANOVA,
with significance set at p ≤ 0.05. 95% CIs are quoted.

Results

During the decade 2006 to 2015, the IJO had 76 issues, 28 in
the E5Ys and 48 in the L5Ys, with 2012 articles in all.

Total number of articles in the decade 2006 to 2015
(n=2012)
Editorials (95)
Symposiums (115)
Photo-essays (18)
Brief communications & case reports (578)
Letters to the editors (561)
Journal abstracts (17)
Community ophthalmology articles not classified
under original articles (36)
Articles on Ophthalmic Practice (42)
Others (21)
Original and review articles (529)

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DJO Vol. 32, No. 2, October-December 2021

Figure 3: Subspecialty wise distribution of Randomized control trials published Figure 4: : Subspecialty wise distribution of Descriptive and cross-sectional
in the Indian Journal of Ophthalmology during the decade 2006 to 2015. studies published in the Indian Journal of Ophthalmology during the decade

2006 to 2015..

Table 1: Total and differences in mean percentage scores of articles, compared subspecialty wise, between the
Earlier and later five years in the Indian Journal of Ophthalmology during the decade 2006 to 2015.

Subspecialty n(%) Total Earlier five years Later five years P Value Difference in Mean
60 (11.3) Mean (SD) n Mean (SD) n Mean (SD) (95%CI)
Cataract 82.6 (10.5) 20 81.9 (9.7) 40 82.9 (11.0)
Glaucoma 0.70
78 (14.7) 84.0 (10.4) 18 82.9 (11.6) 60 84.4(10.0) -1.1(-6.7 to 4.5)
Uvea &
Endophthalmitis 35 (6.6 ) 76.0 (12.4) 14 78.0 (12.0) 21 74.7 (12.7) 0.63
-1.5 (-7.7 to 4.7

0.45
3.3 (-5.4 to 12.0)

Vitreo-retina 119 (22.5) 82.2 (8.9) 30 81.1 (8.5 89 82.5 (9.1 0.43
1.5 (-5.1 to 2.2)

Strabismus & 30 (5.7) 81.5 (10.0) 14 83.2 (11.3) 16 79.9 (8.8) 0.39
Amblyopia 3.3 (-4.4 to 10.9)

Cornea & Conjunctiva 52 (9.8) 82.7 (11.4) 20 81.9 (9.6) 32 83.3 (12.6) 0.66
1.4 (-7.6 to 4.8)

Optics & refraction 28 (5.3) 83.6 (10.3) 5 89.3 (5.9) 23 82.3 (10.7) 0.07
6.93 (-0.7 to 14.5)

Oculoplasty 45 (8.5) 78.5 (9.9) 11 76.2 (9.0) 34 79.2 (10.1) 0.37
-3.0 (-9.7 to 3.8)

Neuro-ophthalmology 24 (4.5)- 82.7 (11.5) 7 88.5 (6.3) 17 80.3 (12.5) 0.046
8.2 (0.2 to 16.2)

Community 24 (4.5) 67.7 (20.1) 6 85.6 (5.3) 18 61.8 (19.7) <0.001
Ophthalmology 23.8 (13.2 to 34.5)

Ocular trauma 10 (1.9) 87.8 (3.9) 1 86.4 (*CD) 9 88.1 (4.2) 0.71
-1.7 (-11.8 to 8.5)

Ophthalmic education 7 (1.3) 77.9 (20.1) 4 82.9 (11.9) 3 71.2 (29.6) 0.57
11.7 (-53.0 to76.5)

Miscellaneous 17 (3.2) 78.4 (14.7) 9 82.6 (9.2) 8 73.7 (18.6) 0.25
8.9 (-7.3 to 25.1

Total 529 (100) 81.2 (11.7) 159 81.9 (9.8) 370 80.9 (12.4) 0.26
1.13 (-0.9 to 3.1)

PS: Number of journals in the E5Ys = 28 and L5Ys= 48. *CD: cannot be determined as only one article is present.

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DJO Vol. 32, No. 2, October-December 2021

Most of the 50 BFR/AFR were on vitreo-retina (13); with nor the long follow-up of CS: also, CXS being like a snapshot
none on community-ophthalmology, ocular trauma and are quick to perform. Understandably, we found only nine
ophthalmic education. Only nine articles were CS, three CS (1.7%). Only three articles were on EA: although more
each on glaucoma and vitreo-retina, two on strabismus and such articles are warranted given the importance of financial
amblyopia and one on cataract. Of 13 CCS, seven were on prudence needed in a developing country like ours (Table 2).
vitreo-retina, two on glaucoma and one each on cataract, uvea
and endophthalmitis, cornea and conjunctiva and neuro- Overall, the EA have the highest scores (90.91%) but since
ophthalmology. DTS were maximally glaucoma related there were only three articles, this may be a biased conclusion
(5), while there was no such study on cataract, oculoplasty, (Table 2). DS/CXS scored significantly more than RCT (mean-
neuro-ophthalmology, ocular trauma, community- difference 4.7%, 95%CI for difference:0.7 to 8.6), BFR/AFR
ophthalmology and ophthalmic education. There were only (mean-difference 6.0%, 95%CI for difference:1.3 to 10.7), and
three EA studies, one each on cataract, vitreo-retina and SR/MA (mean-difference 19.1%, 95%CI for difference:14.6
community-ophthalmology (Table 2). to 23.6). SR/MA have scored the least (65.7%) (Table 2): and
significantly lesser than the RCT (mean-difference 14.4%,
Discussion 95%CI for difference:8.9 to 19.9), BFR/AFR (mean-difference
13.1%, 95%CI for difference:7.1 to 19.1), CS (mean-difference
To the best of our knowledge this is the first review of articles 17.4%, 95%CI for difference:7.9 to 26.9), DS/CXS (mean-
published in the IJO. In our analysis of 529 original articles, difference 19.1%, 95%CI for difference:14.6 to 23.6), DTS
covering 76 issues, 28 in the E5Ys and 48 in the L5Ys, we (mean-difference 14.6%, 95%CI for difference:6.0 to 23.1),
found that the IJO has maintained its quality over the last and EA (mean-difference 25.2%, 95%CI for difference:7.0 to
decade, scoring on checklists in a band around 80% (figure 43.5).
1, table 3) The L5Ys has a much greater number of articles Nearly 23% articles were on vitreo-retina; mostly descriptive
(E5Ys: L5Ys, 159:370) which likely reflects the increased (Table 1). This may be because of the increasing use of anti
journal frequency in subsequent years. VEGF and the advances in the imaging modalities like
enhanced depth and multimodal imaging and advances in
More than half (58%) the articles were DS/CXS, possibly vitrectomy.
because such study designs do not need the rigors of RCT,

Table 2: Total and differences in mean percentage scores of articles, compared study design-wise, between
the Earlier and the Later five years in the Indian Journal of Ophthalmology during the decade 2006 to 2015.

Type of study Total Mean (SD) Earlier Mean (SD) Later five Mean (SD) P value
n (%) 65.7 (12.9) five years 72.9 (7.7) years 66.9 (13.5) Difference in Mean
n
Systemic 55 (10.4) n 43 (95% CI )
reviews and 75 (14.2)
meta-analysis 12 52 <0.004
9.2 (3.1 to 15.4)
Randomized
control trials 80.1 (11.1) 23 79.4 (13.7) 80.4 (9.8) 0.77
-0.9 (-7.4 to 5.5)

Before and 50 (9.5) 78.8 (8.4) 17 77.5 (6.8) 33 79.4 (9.2) 0.40
-1.9(-6.6 to 2.7)
after study

Cohort study 9 (1.7) 76.6 (12.0) 2 78.6 (15.2) 7 76.0 (12.3) 0.86
2.6 (-74.7 to 79.8)

Case control 83.1 (10.1) 2 80.0 (7.1) 11 83.6 (10.7) 0.60

study 13 (2.5) -3.6 (-29.5 to 22.2)

Descriptive 307 (58.0) 84.7 (9.2) 94 85.3 (7.6) 213 84.5 (9.8)
and cross-
sectional study 0.49
0.72(-1.3 to 2.8)

Diagnostic test 17 (3.2) 80.3 (14.1) 9 76.3 (12.4) 8 84.7 (15.4) 0.24
-8.4 (-23.1 to 6.3)
study

Economic 3 (0.6) 90.9 (11.7) 0 - 3 90.9 (15.7) *CD
analysis

Total 529 (100) 81.2 (11.7) 159 81.9 (9.8) 370 80.9 (12.4) 0.26

1.13 (-0.9 to 3.1)

PS: Number of journals in the E5Ys = 28 and L5Ys = 48.*CD: Cannot be determined as no article in E5Ys in EA.

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DJO Vol. 32, No. 2, October-December 2021

Table 3: Year-wise mean percentage scores of the articles published in (23.8%) (Table 1). These lower scores in community-
the Indian Journal of Ophthalmology during the decade 2006 to 2015 ophthalmology in the L5Ys, are likely attributed to the special
issue of IJO having 12 articles on community-ophthalmology
Year of journal Number of articles Mean scores (SD) in this period (volume 60, issue 5, September-October 2012),
(N) and as mentioned earlier not scoring well on the CACLs.17
2006 27 83.2 (9.3)
2007 86.2 (8.5) Study-design wise SR/MA had significantly lower scores in
2008 35 79.5 (12.0) the L5Ys compared to the E5Ys, 9.3% (Table 2). Once again,
2009 28 81.1 (8.6) the possible reason is the special IJO edition, consisting of
2010 34 79.7 (9.5) poorly scoring SRs (on community-ophthalmology). Since
2011 35 80.1 (10.3) there were no studies on EA in the E5Ys, difference and its
2012 39 76.1 (16.6) significance were not computable (Table 2).
2013 51 82.4 (10.1) Interestingly, one fourth of the total articles published were
2014 68 81.9 (11.4) in 2014 (134) (Figure 1). This was also evident subspecialty
2015 134 81.6 (12.8) wise, except for those in uvea and endophthalmitis, neuro-
Total 78 81.2 (11.7) ophthalmology, ocular trauma, community-ophthalmology,
529 ophthalmic education and miscellaneous; and study design-
wise, barring those in SR/MA and DTS.
Ocular trauma is a major cause of preventable visual
impairment in the world,15 with a cumulative incidence of Manuscripts reviewing research publications in medical
4.3% and cumulative life time prevalence at >40 years of 21.1%. literature are few: particularly in ophthalmology. Kumar
Despite its public health importance, publications on eye et al analyzed seven of top 20 general ophthalmic journals
injuries are few, both internationally,16 and in India,15 which during 2005 to 2009.18 They evaluated 12426 abstracts for
is also evident in their being just 10 articles on ocular trauma the trends over the five years in terms of study-design
in the IJO during the period under review (Table I). Ocular and subspecialties: something akin to us, except that our
trauma cases constitute a sizeable number of emergencies, yet analysis was based on articles (529) of our national general
prospective studies are lacking, most publications consisting ophthalmology journal and covered a decade. Most of the
of case series and retrospective analysis. This point towards articles came from retina (34.6%: this figure is derived from
the complexity of subject and with many subspecialties combining medical retina, 29.1% and surgical retina 5.5%),
involved like retina, cornea, glaucoma and many others it and least often from strabismus (2.3%). In our study, we
becomes difficult to carry out a research project smoothly. too have observed that the maximum articles are from the
vitreoretinal subspecialty (22.5%) including both the medical
Likewise, only seven articles covered the field of ophthalmic and surgical retina, and strabismus comprised a meager
education (Table 1), all descriptive in nature. It is likely that 5.7%. Interestingly in their study, almost 40.1% of the studies
the possibilities for carrying out research in this field are comprised CS or CCS, while in ours they formed just 4.2%, a
limited and so is the number of articles. value 1/10th of theirs. In their study SR/MA comprised 2.9%,
while we had 4.2%. Kumar’s paper had 28.7% non-analytical
Overall, the ocular trauma subspecialty scored the highest studies (which comprised of case series and case reports): we
(87.9%) and community-ophthalmology the least (67.7%) have no comparable figures, since we had excluded the case
(Table 1): with the latter scoring significantly lesser than most reports. In our case, DS/CXS (including case series) were
of the subspecialties except for uvea and endophthalmitis highest in number (58%). While they report 3.3% as RCT,
(mean-difference 8.3%, 95%CI for difference: -1.5 to 18.1), we had almost four times as many at 14.2%. Between the
ophthalmic education (mean-difference 10.2, 95%CI for beginning and the end of the period under review, in their
difference: -5.7 to 26.1), and miscellaneous group (mean- study of five years, the proportion of retina articles increased
difference 10.7%, 95%CI for difference: -1.0 to 22.4). One from 32.9% in 2005 to 36.7% in 2009, while in ours over the
possible explanation for the community-ophthalmology decade they increased from 18.5% in 2006 to 24.4% in 2015:
scoring poorly may be on account of the special edition of both being similarly a modest increase.
the IJO (volume 60, issue 5, September-October 2012) which
commemorated the theme of the 9th General Assembly of Lai evaluated 1919 articles, from four leading general
the International Agency for the Prevention of Blindness ophthalmological journals for the methodological quality
(IAPB) by including 12 review articles largely covering using the Hedges Project criteria, and level of evidence
community-ophthalmology that could not score well on the classified according to the Oxford Centre for Evidence-based
CACLs, being of a general narrative nature.17 Medicine Levels of Evidences.19 Compared to us, there are
important differences here too: They reported that 54.4%
Overall the E5Ys scored statistically insignificantly 1.1% of articles were original, 21.9% were case reports, and 1.4%
higher than the L5Ys. Subspecialty-wise the L5Ys articles were review articles, while in our study we have 23.5%
have scored significantly lower than the E5Ys in two areas: original articles, 28.7% case reports, and 2.7% SR/MA. They
neuro-ophthalmology (8%) and community-ophthalmology reported only five (0.4%) articles on the economic aspects of
ophthalmology (all published in the BJO), we also have only
three EA (0.6%) articles. They had 12.3% articles on DTS,

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DJO Vol. 32, No. 2, October-December 2021

whereas we had only 3.2%. In their study 18.1% of articles 13. Quality Assessment Tool for Before-After (Pre-Post) Studies with
were of the highest quality, while in our study 24.6% fall No Control Group - NHLBI, NIH. https://www.nhlbi.nih.gov/
under this level. If we consider higher scoring studies as health-pro/guidelines/in-develop/cardiovascular-risk-reduction/
qualitatively better and assume that those with scores ≥ 95% tools/before-after. Accessed January, 2022.
are superior then we have 56 (10.6%) articles of a high quality,
more than three fourth (78.6%) of which were descriptive. 14. Guyatt G, Sackett D, Cook D. Users’ guides to the medical
It is relevant to note that the SR/MA despite being the highest literature. II. How to use an article about therapy or prevention. J
level of evidence have scored the least. This is an interesting Am Med Assoc 1994; 59-63.
paradox. Researchers and journals need to assess the quality
of even these highest-level evidence studies. 15. Vats S, Murthy GVS, Chandra M, Gupta SK, Vashist P, Gogoi M.
Community eye care: Epidemiological study of ocular trauma in
Our study has some limitations. For instance, although we an urban slum population in Delhi, India. Indian J Ophthalmol
have used readily available checklists, we have carried out a 2008; 56: 313-6.
few modifications. We have allocated scores to the responses
to be able to get a comparable number. Not all questions 16. Janet Alteveer J ,Brian Lahmann, M. An Evidence-Based
may carry the same weight and therefore this approach may Approach to Traumatic Ocular Emergencies. Emerg Med Pract
be considered simplistic. The fact that there is a plethora of 2010;12(5):1-21
tools and checklists, implies that medical fraternity will need
more time to narrow down to globally accepted checklists to 17 Honavar SG. Introduction to Special Issue. Indian Journal of
assess the quality of publications across nations, specialties Ophthalmology 2012; 60: 345-6.
and study designs.
18. Kumar A, Cheeseman R, Durnian JM. Sub specialization of
In future, journal editors, reviewers, authors and researchers the ophthalmic literature: A review of the publishing trends of
should endeavor to devise a universally acceptable metric the top general, clinical ophthalmic journals. Ophthalmology
which factors in the hierarchy of evidence and a critical 2011;118:1211-4.
appraisal score to calculate a single number to assess the
value of a publication. Moreover all specialties should be 19. Lai TYY, Leung GM, Wong VWY, Lam RF, Cheng ACO,
given equal importance and a strong reviewer network Lam DSC. How Evidence-Based Are Publications in Clinical
should be built so there is no delay in the decisions. Ophthalmic Journals ? How Evidence-Based Are Publications in
Clinical Ophthalmic Journals ? Invest Ophthalmol Vis Sci 2006;
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Delhi Journal of Ophthalmology.2021; Vol 32, No (2): 30 - 35
2. Howick J. Introduction to Study Design. Lancet 2002; 359: 57-61.
3. Kocher MS, Zurakowski D. Clinical epidemiology and Acknowledgments: Nil

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in evidence based guidelines. BMJ 2001; 323: 334-6.

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DJO Vol. 32, No. 2, October-December 2021

Original Article

Changes in Intraocular Pressure (IOP) after Phacoemulsification
in Eyes with Occludable Angle Of Anterior Chamber

Pramod Kumar Sahu, Gopal Krushna Das, Divya Jain, Siddharth Madan

Department of Ophthalmology, University College of Medical Sciences and Associated GTB Hospital, University of Delhi, India.

Purpose: The study was aimed to evaluate changes in intraocular pressure (IOP) after phacoemulsification in eyes with
occludable angle of anterior chamber and compare the results with eyes having open angle in patients without previously
diagnosed glaucoma.

Methods: Study subjects were cataract patients scheduled for phacoemulsification, having open or occludable angles. The
patients were divided into two groups of 30 eyes each, having open angle and occludable angles after evaluation by gonioscopy.
A total of 60 eyes that underwent phacoemulsification were analyzed on post-operative day 1, day 7, day 30 and day 90.
Outcome measures included estimation of visual acuity, IOP and central corneal thickness.

Abstract Results: The two groups showed significant reduction in IOP (p<0.05) after phacoemulsification. Increase in IOP was observed at
day 7 but thereafter there was a gradual reduction in IOP throughout 12 weeks of follow-up period in both the groups. The mean

reduction in IOP in Group I with occludable angles was 1.9 mm of Hg (8.3%) and in Group II was 1.4 mm of Hg (6.3%). Comparison

between two groups has not shown any significant difference in terms of IOP reduction.

Conclusions: Removal of the cataract using phacoemulsification serves as a modality to decrease IOP in eyes showing borderline
raised IOP. We observed that the eyes with occludable angle showed a higher reduction in IOP levels after cataract surgery as
compared to the open angle group. However, both the groups demonstrated a significant reduction in IOP in the follow-up
period. Phacoemulsification does have a role in reduction of IOP which may affect the long-term prognosis in the management
of glaucoma with cataract.

Delhi J Ophthalmol 2021; 32; 36-41; Doi http://dx.doi.org/10.7869/djo.713

Keywords: Phacoemulsification, Occludable Angle, Open Angle Glaucoma, Anterior Chamber Depth, Central Corneal Thickness, Intraocular Pressure

Introduction study was proposed to examine whether phacoemulsification

Cataract and glaucoma frequently coexist in the elderly as a primary intervention could result in significant changes

population. Cataract with glaucoma is one of the major in IOP in patients with occludable angles over a follow-up

causes of blindness.1,2 Although primary open-angle period of 12-weeks.

glaucoma is more common worldwide, primary angle- Material and methods
closure glaucoma (PACG) is more common in South East

Asian.2 The current standard care for PACG is a step-wise This study was conducted over 1.5 years in tertiary level

approach of a combination of laser iridotomy and medical hospital of North India. Considering a standard deviation

treatment. If these treatments fail, glaucoma surgery is of 2.2 and 1.65 in Open angle and Occludable angle group

indicated. It is proposed that crystalline lens increases in size respectively at 80% power and 5% Type I Error and taking

as age progresses at the rate of 0.029 mm per year pushing into consideration a 10% loss to follow up, final sample size

iris forwards leading to a closure of anterior chamber angle taken was 60.

and might possibly result in a pupillary block glaucoma.3

Early lens extraction could improve glaucoma control by This study included 60 consecutive patients who

causing the iris to fall back and preventing pupillary block.4 underwent phacoemulsification with intraocular lens (IOL)

The procedure might also reduce the requirement for drugs implantation. The patients admitted at a tertiary care hospital

and glaucoma surgery, maintain good visual acuity and were recruited over a period of 1.5 years. One eye per patient

may improve quality of life compared with standard care. was included in the study. The institution's ethics committee

An improvement in visual acuity would allow a more approved the study and written informed consent was taken

accurate visual field monitoring, and a clear optical pathway from all patients.

enhances the quality and reproducibility of optic nerve

imaging. Patients between 50 to 80 years who were scheduled for

elective phacoemulsification were included. Exclusion

Previous studies have evaluated effect of phacoemulsification criteria included underlying ocular comorbidity other than

on anterior chamber depth and intra ocular pressure (IOP) cataract, open-angle glaucoma, normotensive glaucoma,

in eyes with open-angle or occludable-angle glaucoma.5,6 It uveitis, history of trauma, previous intraocular surgery,

is documented that cataract extraction can cause changes established primary angle closure with evidence of previous

in anterior chamber depth (ACD), the iridocorneal angle acute episode or established peripheral anterior synechiae or

width and intraocular pressure (IOP) in eyes with co- subjects on any medications that could alter the IOP.

existing open angle glaucoma and angle-closure glaucoma.7

However, there is a paucity of literature on the effect of Patients who fulfilled the selection criteria underwent

phacoemulsification in eyes with occludable angles. This a complete ocular examination including visual acuity

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DJO Vol. 32, No. 2, October-December 2021

assessment, slit lamp examination, Goldmann applanation A foldable intraocular lens was placed inside the capsular
tonometry and indentation gonioscopy. The best corrected bag. Viscoelastic material was removed and finally the
visual acuity (BCVA) was estimated using a Snellen chart incision was hydrated using a 30-gauge cannula. No sutures
and was converted to logMAR values. A detailed slit lamp were applied. Patients were prescribed a standard topical
examination was performed to exclude signs of ocular treatment regimen comprising of prednisolone acetate 1%,
surface pathology and intraocular inflammation. Goldman moxifloxacin 0.5 %, tropicamide 0.8%, all of which were
applanation tonometry was done on day 1 preoperatively and tapered over four weeks subsequently. Postoperative follow-
1, 4, and 12 weeks postoperatively. Central corneal thickness up visits were scheduled at day 1, day 4 and at 12 weeks.
was measured using a pachymeter (in micrometers) by
using standard technique (Pachette 2 ultrasonic pachymeter, Results
model number DGH 550, DGH Technology Incorporation;
Exton, Philadelphia (19341), USA.) Using a single mirror Of 60 patients studied, no patient was lost to follow- up. A
gonioscope (Volk Optical Inc, Mentor, OH, USA), the angle of total of 60 eyes were recruited over 1.5 years and studied.
anterior chamber was evaluated by the principal investigator Both the groups were gender and age matched. Mean
in dim illumination using a shortened slit beam that did not LogMAR BCVA improved significantly from a preoperative
fall upon the pupil. The angle was graded according to the level of 1.00 ± 0.43 and 0.996 ±0.38 in group I and group II to a
Scheie classification based on observed angle structures. postoperative level of 0.19±0.13 and 0.177 ± 0.14 respectively
The drainage angle was then graded as occludable or at day 90 (p value<0.001). It was measured pre-operatively,
non occludable using a dichotomous modification of the day 7, day 30 and at day 90. The pre-op visual acuity between
Scheie scheme (based on angle structures seen).8,9 The the two groups was not significant (p-value 0.975), therefore
International Society of Geographical and Epidemiological the two groups were matched and compared. There was no
Ophthalmology (ISGEO) nomenclature defines occludable statistically significant difference between the two groups on
angles (anatomically narrow angle) using gonioscopy day 7, 30 and at days 90 (p value 0.889) (Table 1), (Figure 1).
where the posterior trabecular meshwork is not visible in
270° or more of the angular extent and absence of any other The mean pre-operative IOP was 22.80 ± 2.92 mm of Hg in
clinical features as primary angle closure suspect (PACS).10 occludable angle Group and 22.33± 3.30 mm of Hg in Open
In our study if the posterior (usually pigmented) trabecular angle group. An increase in IOP was noted at day 7 to 23.23±
meshwork could be visualized for less than 90° of the angle 2.38mm of Hg in occludable angle group and 23.17 ± 2.61
circumference, the angle was classified as occludable.
Table 1: Best corrected visual acuity
Patients were allocated into either occludable angle group or
open angle group (30 each). Group I: occludable angle and BCVA Group I Group II p-value
Group II: open angle.
Preoperative 1.00±0.43 0.996±0.38 0.975 (NS)
All the patients then underwent phacoemulsification with a
foldable IOL implantation. Day 7 0.443±0.17 0.417±0.20 0.889 (NS)
Day 30 0.253±0.13 0.247±0.16
The accumulated data was tabulated using Microsoft
Excel software (version 2010). Statistical analysis was done Day 90 0.190±0.13 0.177±0.14
using SPSS (Statistical Package for the Social Sciences, IBM
Corporation) software. Repeated measure ANOVA was used p-value <0.001
for statistical analysis. (overall)

Surgical Technique p<0.05 is significant
NS-Not significant, S-Significant
All the subjects were operated by a single surgeon using Interaction between group and time p = 0.889
a standard technique. On the day of surgery, all patients Mauchly’s test of sphericity p< 0.001 , Green house Geisser correction was
received short-acting mydriatic agent (tropicamide 0.8%, applied.
phenylephrine 5.0%) for dilatation of the pupil. After
administration of peri bulbar anesthesia, a supero-temporal Figure 1: A line diagram depicting Best corrected visual acuity (BCVA) at
self-sealing corneal incision was made just in front of different time intervals.
the vascular arcades of the corneoscleral limbus using a
calibrated 3.2 mm keratome. Viscoelastic substance (Hydroxy
Propyl Methyl Cellulose 2%) was injected into the anterior
chamber. A paracentesis incision of 1 mm was made 600
apart with a calibrated MVR blade. After the capsulorrhexis,
hydrodissection followed by nucleus rotation, nucleotomy
was performed by a single method using stop and chop
technique. After emulsification of nuclear fragments,
irrigation aspiration of residual cortical matter was done.

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DJO Vol. 32, No. 2, October-December 2021

in Open angle Group. Thereafter, there was a statistically of reversible blindness worldwide.1 The treatment of either
significant reduction throughout the follow-up period of condition can influence the course of the other.11-15 It is
12 weeks in both the groups. At day 90, the mean IOP in widely accepted that the use of glaucoma laser treatments
occludable angle group was 20.90 ±2.67 and in open angle and surgical glaucoma procedures can accelerate cataract
group to 20.93 ± 2.99 mm of Hg (p value < 0.001). (Table 2 and formation.11-14,16 Glaucoma medications could cause cataract
Figure 2) The mean reduction in IOP in Group I (occludable progression apart from the compliance, availability and
angle) was 1.9 mm of Hg (8.3%) and in Group II was by 1.4 financial issues related to these medications.10 Further,
mm of Hg (6.3%). Comparison between two groups has not phacoemulsification might influence the IOP in glaucoma
shown any significant difference in terms of IOP reduction. patients.
Central corneal thickness (CCT) was 0.550 µm in occludable
angle group and 0.558 µm in open angle group preoperatively. Numerous clinical studies have reported the effect of
After surgery it increased at day 7, after which a declining intraocular surgery (phacoemulsification, extracapsular
trend was observed at day 30 and 90 (Table 3), (Figure 3). At cataract extraction) on IOP, ACD, and the angle opening
the last follow up (day 90) CCT was higher in both groups width in normal eyes.5-8,12,17,18 Phacoemulsification aids
in comparison to their pre-operative values, difference was in reducing the intraocular pressure and has distinct
statistically significant. (p value < 0.001) advantages.19-21 Various studies have shown the role of
thickened cataractous lens as a cause for increased IOP.
Discussion Therefore, after phacoemulsification the human lens being a
risk factor contributing to glaucoma is removed.19,20 The need
With increasing life expectancy, visual co-morbidity due to for anti-glaucoma medications might be reduced or in certain
glaucoma and cataract are becoming increasingly frequent. cases eliminated.22 However, there is little information on the
Glaucoma is the most important cause of irreversible effect of phacoemulsification on eyes with occludable angle
blindness worldwide. At least 70 million people are suffering comparing with open angle. This study was a comparative
from glaucoma of which 10% are bilaterally blind.2 evaluation of 60 eyes with a high baseline IOP (> 21 mm of

Cataract is an age-related disease. According to the world Table 3: Central Corneal Thickness
health organization (WHO), cataract is the leading cause

Table 2: Intraocular pressure CCT Group I Group II p-value

IOP Group I Group II p-value Preoperative 0.550±0.03 0.558±0.02 0.234
Day 7 0.595±0.02 0.602±0.02 (NS)
Preoperative 22.80±2.92 22.33±3.30 0.565 Day 30 0.561±0.03 0.577±0.02
Day 7 23.23±2.38 23.17±2.61 (NS) 0.169
Day 30 21.20±2.55 21.10±2.96 (NS)
0.918
(NS) 0.013
(S)
0.889
(NS) Day 90 0.554±0.02 0.565±0.02 0.090
(NS)

Day 90 20.90±2.67 20.93±2.99 0.964 p-value <0.001
(NS) (overall)

p-value < 0.001 p<0.05 is significant
(overall) NS-Not significant, S-Significant
Interaction between group and time p = 0.083
p<0.05 is significant Mauchly’s test of sphericity p< 0.001, Green house Geisser correction was
NS-Not significant, S-Significant applied.
Interaction between group and time p = 0.661
Mauchly’s test of sphericity p< 0.001 , Green house Geisser correction was
applied.

Figure 2: A line diagram showing the changes in intraocular pressure (IOP). Figure 3: A line diagram showing the trend of central corneal thickness (CCT)
at different time intervals

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DJO Vol. 32, No. 2, October-December 2021

Hg), of which 30 eyes had occludable angle and 30 eyes had of primary angle-closure glaucoma (EAGLE) trial was a
open angle. The two groups showed significant reduction in landmark trial that concluded that initial treatment with
IOP (p<0.001) after uneventful phacoemulsification which clear-lens extraction was superior to laser peripheral
was performed for cataract removal. iridotomy plus topical medical therapy for individuals with
primary angle closure and PACG. Although the subjects in
Several studies quantified the long-term reduction in this study were operated for cataract, the IOP lowering effect
IOP following cataract extraction. Shin HC et al reported on removal of the human crystalline lens is known.32
significant IOP reduction after phacoemulsification in
occludable-angle group (2.31 mm Hg; 14.6 %) than open- The mechanisms by which lens extraction influences IOP have
angle group (0.77 mm Hg; 5 %) at 12 weeks when mean been postulated to be: Increased trabecular outflow (removal
preoperative IOP was significantly higher in occludable- of lens causes deepening of angle with backward rotation of
angle group than open-angle group.8 A reduction of 8.3% ciliary body, relieving compression on trabecular meshwork
and 6.3% respectively in the two groups was observed in and canal of Schlemm)33 Widening of anterior chamber angle
this study. Mean preoperative IOP in the study by Shin HC and elimination of pupillary block to increased uveoscleral
et al was 12.5-14.8 mm Hg, which was lower than mean outflow due postoperative release of prostaglandin F-2 are
preoperative IOP in this study (19.8-25.7 mm Hg- occludable other mechanisms.24,34 Traction on ciliary body due to fibrosis
angle group; 19 – 25.6 mm Hg- open angle group). and contraction of posterior capsule after cataract surgery
causing hyposecretion of aqueous, Removal of 5-mm thick
Shingleton BJ et al compared IOP of OAG patients with crystalline lens that is subsequently replaced by 1-mm thick
controlled patients, glaucoma suspects and normal controls IOL results in an increase in AC volume that is required to be
3 and 5 years after phacoemulsification.23 An IOP reduction filled by aqueous humor35,36
(~ 1.5 mm Hg) was observed in all groups, which was
significant at 3 years and sustained after 5 years. Authors Conclusions
suggested that cataract extraction could be appropriate
for certain patients based on medication requirements and Phacoemulsification lowered IOP with effect being more
extent of optic nerve damage. Mathalone N et al reported pronounced in eyes with occludable angles. The current
a 2 mmHg of IOP reduction in primary OAG two years study observed the same over 3 months, and the same
after phaco-surgery.24 The need for fewer medications with could be extrapolated over long term. Persistent long-term
reduced financial burden would ensure better compliance reduction of IOP following phacoemulsification may have
and improved quality of life. a beneficial effect in management of patients suffering from
both cataract and glaucoma.
Poley et al in their study on eyes with ocular hypertension
(OHT) and controls, reported that higher the IOP before References
surgery, more significant was the postoperative lowering
of IOP.5 The authors observed a pressure lowering effect of 1. Malhotra S, Prasad M, Vashist P, Kalaivani M, Gupta
phacoemulsification in glaucoma patients (not categorized SK. Prevalence of blindness in India: A systematic
into angle closure/open angle) and found a similar outcome, review and meta-analysis. Nat Med J India. 2019
in their follow-up study.25 ;32(6):325–33.

Issa SA et al calculated a prediction index/ PD ratio 2. Quigley HA, Broman AT. The number of people
{(preoperative IOP (mmHg) divided by ACD (mm)} for with glaucoma worldwide in 2010 and 2020. Br J
IOP reduction post cataract surgery.26 They concluded Ophthalmol.2006; 90(3):262-7.
that this PD ratio is strongly predictive for IOP reduction
following cataract extraction and eyes with higher initial 3. Augusteyn RC. On the growth and internal structure
IOPs and a shallower ACD resulted in greater IOP of the human lens. Exp Eye Res. 2010;90(6):643–54.
reductions after cataract surgery, thereby suggesting that
phacoemulsification effect to decrease IOP is more in shallow 4. Potop V, Corbu C. The role of clear lens extraction in
AC (occludable angles). Salvi SM et al evaluated changes angle closure glaucoma. Rom J Ophthalmol. 2017;61
occurring in central corneal thickness (CCT) immediately (4):244–8.
after uneventful phacoemulsification surgery.27 The CCT
increased by approximately 13.81% in the immediate 5. Poley BJ, Lindstrom RL, Samuelson TW. Long-term
postoperative period. It remained elevated by 6.44% on day effects of phacoemulsification with intraocular lens
1 compared to the preoperative values. These observations implantation in normotensive and ocular hypertensive
were similar to our observations in this study. eyes. J Cataract Refract Surg. 2008; 34(5):735–42.

Several studies on effect of age-related lens growth on IOP 6. Shingleton BJ, Wadhwani RA, O’Donoghue MW,
linked to anatomical changes have been done.28-31 It is known Baylus S, Hoey H. Evaluation of intraocular pressure
that eyes with angle closure glaucoma or occludable angles in the immediate period after phacoemulsification. J
have shorter axial lengths, shallower anterior chamber Cataract Refract Surg. 2001; 27(4):524–7.
depths, and thicker crystalline lenses. 28,29
The Effectiveness of early lens extraction for the treatment 7. Dooley I, Charalampidou S, Malik A, Loughman J,
Molloy L, Beatty S. Changes in intraocular pressure
and anterior segment morphometry after uneventful
phacoemulsification cataract surgery. Eye (Lond).
2010 ;24(4):519–26;

8. Shin HC, Subrayan V, Tajunisah I. Changes in
anterior chamber depth and intraocular pressure after
phacoemulsification in eyes with occludable angles. J

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Cataract Refract Surg. 2010 ; 36(8):1289–95. and normal patients. J Glaucoma. 2006; 15(6):494-8
9. Schie HG. Width and pigmentation of the angle of the 24. Mathalone N, Hyams M, Neiman S, Buckman G, Hod

anterior chamber; a system of grading by gonioscopy. Y, Geyer O. Long-term intraocular pressure control
AMA Arch Ophthalmol. 1957; 58(4):510-2. after clear corneal phacoemulsification in glaucoma
10. Foster PJ, Buhrmann R, Quigley HA, Johnson GJ. The patients. J Cataract Refract Surg. 2005;31(3):479–83.
definition and classification of glaucoma in prevalence 25. Poley BJ, Lindstrom RL, Samuelson TW,
surveys. Br J Ophthalmol. 2002 ;86(2):238–42. Schulz R Jr. Intraocular pressure reduction
11. Agrawal S, Fledderjohann J, Ghosh S. Risk factors after phacoemulsification with intraocular lens
for self-reported cataract symptoms, diagnosis, and implantation in glaucomatous and nonglaucomatous
surgery uptake among older adults in India: Findings eyes: evaluation of a causal relationship between the
from the WHO SAGE data. Glob Public Health. 2021 natural lens and open-angle glaucoma. J Cataract
;16(11):1771–85. Refract Surg. 2009; 35(11):1946-55.
12. Ling JD, Bell NP. Role of Cataract Surgery in the 26. Issa SA, Pacheco J, Mahmood U, Nolan J, Beatty S.
Management of Glaucoma. Int Ophthalmol Clin. A novel index for predicting intraocular pressure
2018;58(3):87–100. reduction following cataract surgery. Br J Ophthalmol.
13. Husain R, Aung T, Gazzard G, Foster PJ, Devereux 2005 ; 89(5):543-6.
JG, Chew PT, et al. Effect of trabeculectomy on 27. Salvi SM, Soong TK, Kumar B V., Hawksworth
lens opacities in an East Asian population. Arch NR. Central corneal thickness changes after
Ophthalmol. 2006; 124:787–92. phacoemulsification cataract surgery. J Cataract
14. LimLS,HusainR,GazzardG,SeahSK,AungT.Cataract Refract Surg. 2007; 33(8):1426–8.
Progression after Prophylactic Laser Peripheral 28. Saxena S, Agrawal PK, Pratap VB, Nath R. Anterior
Iridotomy Potential Implications for the Prevention of chamber depth and lens thickness in primary angle-
Glaucoma Blindness. Ophthalmology.2005; 112:1355– closure glaucoma: a case-control study. Indian J
9. Ophthalmol. 1993 ;41(2):71–3.
15. Bobrow JC. Factors Influencing Cataract Formation 29. Sihota R, Gupta V, Agarwal HC, Pandey RM, Deepak
After Nd: YAG Laser Peripheral Iridotomy. Trans Am KK. Comparison of symptomatic and asymptomatic,
Ophthalmol Soc.2008; 106:93–7. chronic, primary angle-closure glaucoma, open-angle
16. He M, Jiang Y, Huang S, Chang DS, Munoz B, Aung T, glaucoma, and controls. J Glaucoma. 2000 ;9(3):208–
et al. Laser peripheral iridotomy for the prevention of 13.
angle closure: a single-centre, randomised controlled 30. Cheng C, Parreno J, Nowak RB, Biswas SK, Wang
trial. The Lancet. 2019 Apr 20;393(10181):1609–18. K, Hoshino M, et al. Age-related changes in eye
17. Wang SY, Azad AD, Lin SC, Hernandez-Boussard T, lens biomechanics, morphology, refractive index
Pershing S. Intraocular Pressure Changes after Cataract and transparency. Aging (Albany NY). 2019
Surgery in Patients with and without Glaucoma: An ,16;11(24):12497–531.
Informatics-Based Approach. Ophthalmol Glaucoma. 31. Tripathi RC, Tripathi BJ. Functional anatomy of
2020 ;3(5):343–9. the anterior chamber angle. In: Jakobiec FA, ed,
18. Nganga Ngabou CGF, Makita C, Ndalla SS, Nkokolo Ocular Anatomy, Embryology, and Teratology.
F, Madzou M. [Intraocular pressure decrease Philadelphia,PA, Harper & Row, 1982; 197–284.
after manual small incision cataract surgery]. J Fr 32. Azuara-Blanco A, Burr J, Ramsay C, Cooper D, Foster
Ophtalmol. 2017 ;40(5):397–402. PJ, Friedman DS, et al. Effectiveness of early lens
19. Carolan JA, Liu L, Alexeeff SE, Amsden LB, Shorstein extraction for the treatment of primary angle-closure
NH, Herrinton LJ. Intraocular Pressure Reduction glaucoma (EAGLE): a randomised controlled trial.
after Phacoemulsification: A Matched Cohort Study. Lancet. 2016 Oct 1;388(10052):1389–97.
Ophthalmol Glaucoma. 2021;4(3):277–85. 33. Panek WC, Christensen RE, Lee DA, Fazio DT,
20. Todorović M, Šarenac Vulović T, Petrović N, Fox LE, Scott TV. Biometric variables in patients
Todorović D, Srećković S. Intraocular pressure with occludable anterior chamber angles. Am J
changes after uneventful phacoemulsification in early Ophthalmol. 1990, 15;110(2):185–8.
postoperative period in healthy eyes. Acta Clin Croat. 34. Tarongoy P, Ho CL, Walton DS. Angle-closure
2019 ;58(3):467–72. glaucoma: the role of the lens in the pathogenesis,
21. Vizzeri G, Weinreb RN. Cataract surgery and prevention, and treatment. Surv Ophthalmol. 2009;
glaucoma. Curr Opin Ophthalmol. 2010 ;21(1):20–4. 54(2):211-25.
22. Zhang ML, Hirunyachote P, Jampel H. Combined 35. Kooner KS, Dulaney DD, Zimmerman TJ. Intraocular
surgery versus cataract surgery alone for eyes with pressure following ECCE and IOL implantation
cataract and glaucoma. Cochrane Database Syst Rev. in patients with glaucoma. Ophthalmic Surg.
2015,14;(7):CD008671. 1988;19(8):570–5.
23. Shingleton BJ, Pasternack JJ, Hung JW, O’donoghue 36. Kim KS, Kim JM, Park KH, Choi CY, Chang HR.
MW − Three and five year changes in intraocular The effect of cataract surgery on diurnal intraocular
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open angle glaucoma patients, glaucoma suspects,

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DJO Vol. 32, No. 2, October-December 2021

Cite This Article as: Pramod Kumar Sahu, Gopal Krushna Das,
Divya Jain, Siddharth Madan. Changes in Intraocular Pressure
(IOP) after Phacoemulsification in Eyes with Occludable Angle Of
Anterior Chamber. Delhi Journal of Ophthalmology.2021; Vol 32,
No (2): 36 - 41.

Acknowledgments: Nil

Conflict of interest: None declared

Source of Funding: None

Date of Submission: 11 Feb 2021
Date of Acceptance: 16 Jun 2021

Address for correspondence
Siddharth Madan, M.S, D.N.B, F.I.C.O,

FAICO (Retina), MNAMS, Assistant Professor,

Department of Ophthalmology,
University College of Medical Sciences
and Associated GTB Hospital, India
Email : [email protected]

Quick Response Code

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DJO Vol. 32, No. 2, October-December 2021

Case Report

Malarial Retinopathy: Falci Not So Fan-See!

Yogya Reddy, Reshma Ramakrishnan, Anamika Pandey, Ayushi ojha, Purva deore, Priyanka Gandhi

Department of Ophthalmology, MGM Institute of Health Sciences, Navi Mumbai, India

Abstract Malarial retinopathy is an established entity in cases of complicated falciparum malaria and cerebral malaria in children
whereas, in adults it is rarely reported. Here we report a case of a 21-year-old male, with uncomplicated falciparum
malaria, referred to the ophthalmology outpatient department with a chief complaint of sudden blurring of vision in both
eyes. On examination, both eyes showed mild blurring of nasal optic disc margins with multiple intraretinal, preretinal
haemorrhages, subhyaloid haemorrhage involving the macula and Roth spots, suggestive of malarial retinopathy.The
patient was observed closely for spontaneous resolution and was treated systemically with anti-malarial drugs including
injection artesunate and tablet doxycycline. On subsequent follow-up after 2 weeks, improvement in visual acuity with
complete resolution of haemorrhages occurred. We aspire to bring to light a rare entity of malarial retinopathy and the
need to not over-treat the condition.

Delhi J Ophthalmol 2021; 32; 42-45; Doi http://dx.doi.org/10.7869/djo.714

Keywords: Malaria Retinopathy, Roth spots, Disc Edema, Sub-hyaloid Haemorhage

Introduction permanent damage to the retina. However, retinal nerve fibre

Malaria is an acute febrile illness caused by Plasmodium thinning is a proposed late complication in a few cases. Here,

species specially vivax and falciparum. It remains a large we report a rare case of malarial retinopathy in a 21-year-

burden on society, especially on tropical countries. Cerebral old male with uncomplicated falciparum malaria. This case

malaria is the most dreaded neurological complication assumes its importance in literature to increase awareness

caused by falciparum malaria. Various ocular findings on the condition and help in future research on the same.

have been described in patients with malaria, particularly Case report
falciparum type.1 Malarial retinopathy was first described by
Lemallen et al in 1993.2 Retinal haemorrhages, sometimes A 21-year-old male patient of falciparum malaria was
with subhyaloid extension, papilloedema, retinal edema, referred to ophthalmology outpatient department in view
and retinal pallor are common findings of malarial of red eyes and sudden onset blurring of vision in both eyes
retinopathy. An association between the severity of malaria for 2 days. It was progressive and painless and occured in
and retinopathy was noted. These fundus findings are an a febrile context. On examination, distant visual acuity was
indicator of poor prognosis in critically ill patients.3 Malarial 6/24 in right eye, 6/18p in left eye, and near visual acuity
retinopathy is more often seen in fatal, cerebral and severe was N12 in right eye and N10 in left eye. Colour vision
falciparum malaria and is rarely reported in uncomplicated was normal on Ishihara's color vision chart and Amsler's
cases. The most commonly proposed mechanism of grid test showed metamorphopsia. He had subconjunctival
pathogenesis is sequestration of erythrocytes causing retinal haemorrhages on temporal side of the palpebral conjunctiva
changes. The condition is known to be reversible with no in both eyes (Figure 1). Dilated fundus examination
revealed nasally blurred optic disc margins, multiple

Figure 1: Slit lamp image showingsubconjunctival haemorrhages on the temporal side of the palpebral conjunctiva in both eyes

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DJO Vol. 32, No. 2, October-December 2021

Figure 2 : Fundus photo showing nasally blurred optic disc margins, multiple retinal haemorrhages, subhyaloid haemorrhage at macula along with multiple Roth
spots in both eyes

retinal haemorrhages, subhyaloid haemorrhage at macula The patient was treated with systemic artesunate and
along with multiple roths spots in both eyes.(Figure2) doxycycline along with vitamin supplements and followed
Optical Coherence Tomography showed hyporeflectivity up regularly with nil ophthalmologic intervention.
at multiple layers, with fluid accumulation at macular area
suggestive of intraretinal and preretinal haemorrhages with On first follow up at 5 days, there was an improvement
subhyaloid extension at the macula.(Figure 3) A diagnosis of in the retinal picture with complete resolution of retinal
malarial retinopathy was made on the basis of clinical signs. haemorrhages and significant reduction of both Roth spots
His peripheral blood smear showed Plasmodium falciparum and disc edema.(Figure 4)
parasite and the Complete blood picture was within normal
limits. On follow-up after 2 weeks, the patient's visual acuity
improved to 6/6 in both eyes. Amsler's grid test was normal
with no metamorphopsia.

Discussion

Retina is considered as an extension of the central nervous
system because of common embrolyogical origin of
both structures. A histological correlation is also noted
between retina and brain. This gives a luxury to observe
the vasculature of central nervous system through retinal
examination which helps in understanding mechanism and
course of many diseases, including malaria.4 The proposed
pathogenesis for malarial retinopathy is impaired perfusion,
which is likely to be due to sequestration of infected
erythrocytes occurring in retinal microvasculature which in
turn causes hypoxia and ischemia of the retina.5 The most
popular alternate hypothesis for pathogenesis is release of
inflammatory markers from the retinal blood vessels.6 Most
common symptoms are retinal haemorrhages at multiple
layers, disc edema, vessel discoloration, retinal whitening,
etc.

Figure 3 : Optical Coherence Tomography showed hyporeflectivity at multiple Malarial retinopathy is well documented in children by
layers, with fluid accumulation at macular area suggestive of intraretinal and various groups primarily in Africa and other endemic
countries. However, the condition is rarely reported in
preretinal haemorrhages with subhyaloid extension at the macula adults. In a study conducted in India, D K Kochar et al. stated
that 34% of cerebral malaria patients were found to have
retinopathy changes like retinal haemorrhages, blurring of
disc margins, retinal edema, disc pallor, hard exudates .7
S. Looareesuwan et al noticed retinal haemorrhages in

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DJO Vol. 32, No. 2, October-December 2021

Figure 4 :Fundus photo at first follow up after 5 days there was a drastic improvement in the retinal pathology with complete resolution of retinal haemorrhages
and significant reduction of roth spots and disc edema

14% of patients with cerebral malaria in a study conducted Hyg. 1983 Sep;32(5):911-5.
in Thailand.8 Although the above studies show that 4. Richard J. Maude, Arjen M. Dondorp, Abdullah Abu Sayeed,
retinopathy is well associated with very severe, complicated,
or cerebral malaria, its association with uncomplicated cases Nicholas P.J. Day, Nicholas J. White, Nicholas A.V. Beare, The
is very rarely reported. In one such case, quinine therapy eye in cerebral malaria: what can it teach us?, Transactions of
was considered effective.9 The Royal Society of Tropical Medicine and Hygiene, July 2009,
Volume 103, Issue 7, Pages 661–664.
A few cases of malarial retinopathy in vivax malaria patients 5. Beare NA, Taylor TE, Harding SP, Lewallen S, Molyneux ME.
have also been reported in India.10 It has been noticed that Malarial retinopathy: a newly established diagnostic sign in
even though malarial retinopathy is directly related to the severe malaria. Am J Trop Med Hyg. 2006 Nov;75(5):790-7.
severity of systemic disease, the post-recovery outcome of 6. Villaverde C, Namazzi R, Shabani E, Park GS, Datta D, Hanisch
visual acuity is remarkably good. Spontaneous resolution B et al. Retinopathy-Positive Cerebral Malaria Is Associated With
is noted in a majority of cases including the current case. Greater Inflammation, Blood-Brain Barrier Breakdown, and
A.M. Dondorp et al. proposed that the use of quinine Neuronal Damage Than Retinopathy-Negative Cerebral Malaria.
and levamisole has a role in reducing the sequestration of J Pediatric Infect Dis Soc. 2020 Nov 10;9(5):580-586.
erythrocytes.11 Levamisole inhibits sequestration of infected 7. Kochar DK, Shubhakaran, Kumawat BL, Thanvi I, Joshi A, Vyas
red blood cells in patients with falciparum malaria. The role SP. Ophthalmoscopic abnormalities in adults with falciparum
of artesunate and other agents in the condition is still under malaria. QJM. 1998 Dec;91(12):845-52.
study. We treated our patient with systemic artesunate and 8. Looareesuwan S, Warrell DA, White NJ, Chanthavanich P, Warrell
observed satisfactory results. MJ, Chantaratherakitti S et al. Retinal hemorrhage, a common sign
of prognostic significance in cerebral malaria. Am J Trop Med
This case report brings to light the rare entity of malarial Hyg. 1983 Sep;32(5):911-5.
retinopathy in an adult case of uncomplicated malaria. All 9. Nanfack CN, Bilong Y, Kagmeni G, Nathan NN, Bella LA.
patients with fever presenting with a sudden blurring of Malarial retinopathy in adult: a case report. Pan Afr Med J. 2017
vision and retinal haemorrhages need to be examined for Jul 25;27:224.
malaria. We wish to stress the diagnostic importance of the 10. Sharma, Sangeeta & Maheshwari, Ujwala & Bansal, Nidhi.
condition in an ophthalmologic point of view. Regardless of RETINAL HAEMORRHAGE IN PLASMODIUM VIVAX
the frightening nature of the condition, it is important to not PATIENTS- 2 RARE CASE REPORTS. Journal of Evolution of
overtreat it. Medical and Dental Sciences. 2012.1. 929-931.
11. Dondorp AM, Silamut K, Charunwatthana P, Chuasuwanchai
References S, Ruangveerayut R, Krintratun S, White NJ, Ho M, Day NP.
Levamisole inhibits sequestration of infected red blood cells
in patients with falciparum malaria. J Infect Dis. 2007 Aug
1;196(3):460-6.

1. Paquet-Durand F, Beck SC, Das S, Huber G, Le Chang, Schubert
T et al. A retinal model of cerebral malaria. Sci Rep. 2019 Mar
5;9(1):3470.

2. Lewallen S, Taylor TE, Molyneux ME, Wills BA, Courtright P.
Ocular fundus findings in Malawian children with cerebral
malaria. Ophthalmology. 1993 Jun;100(6):857-61.

3. Looareesuwan S, Warrell DA, White NJ, Chanthavanich P, Warrell
MJ, Chantaratherakitti S et al. Retinal hemorrhage, a common sign
of prognostic significance in cerebral malaria. Am J Trop Med

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DJO Vol. 32, No. 2, October-December 2021

Cite This Article as: Yogya Reddy, Reshma Ramakrishnan,
Anamika Pandey, Ayushi ojha, Purva deore, Priyanka Gandhi.
Malarial retinopathy: Falci not so fan-see! Delhi J Ophthalmol
2021;32; (2) 42 - 45.

Acknowledgments: Department of General Medicine

Conflict of interest: None declared

Source of Funding: None

Date of Submission: 05 Dec 2021
Date of Acceptance: 10 Dec 2021

Address for correspondence
Yogya Reddy Junior resident

Department of Ophthalmology,
MGM Institute of Health Sciences,
Navi Mumbai, India.
Email: [email protected]

Quick Response Code

E-ISSN: 2454-2784  P-ISSN: 0972-0200 45 Delhi Journal of Ophthalmology

DJO Vol. 32, No. 2, October-December 2021

Case Report

Choroidal Osteoma with Choroidal Neovascularization and
Focal Choroidal Excavation

Rajwinder Kaur,1 Raja Narayanan,2 Balbir Khan,3 Ekta Gupta,1 Rishabh Narula,1

1Department of Ophthalmology, Adesh Institute of Medical Sciences and Research, Barnala Road, Bathinda, Punjab, India.
2Department of Ophthalmology LV Prasad Eye Institute, Hyderabad, India.

3Department of Ophthalmology, Gian Sagar Medical College and Hospital, Banur, India.

A 28-year-old female presented with recent onset of metamorphopsia in left eye. Best corrected visual acuity was 20/20 in
right eye and 20/600 in left eye. Colour fundus photograph revealed orange yellow peripapillary lesion crowning the optic
disc. Fundus Fluorescein Angiography revealed presence of choroidal neovascular membrane (CNV) type 2 in juxta-foveal
Abstract region. Enhanced Depth Imaging Optical Coherence Tomography confirmed presence of CNV and conforming type of focal
choroidal excavation (FCE). Diagnosis of choroidal osteoma with conforming FCE and CNV type 2 was made. FCE may be
seen in choroidal osteoma with CNV, primarily in areas adjacent to decalcification.

Delhi J Ophthalmol 2021;32; 46-48s; Doi http://dx.doi.org/10.7869/djo.715

Keywords: Focal Choroidal Excavation, Choroidal Osteoma, Choroidal Neovascularization.

Introduction increased in size in later phase suggestive of juxta-
foveal CNV Figure 1d. Non-contrast CT of orbit (Figure
Choroidal osteoma, described by Gass et al., is a benign 1e) and ultrasound B scan (Figure 1f) demonstrated the
ossifying tumor with mature bone replacing the choroid. It osteoma.Optical Coherence Tomography (OCT) macula
usually presents as a unilateral slightly irregular elevated (Spectralis; Heidelberg Engineering, Germany) showed
yellow–orange lesion in juxta-papillary or macular region that altered foveal contour and distortion of foveal architecture
typically occurs in healthy females, who present in their 2nd by hyperreflective complex (CNV) and FCE on its edge
to 3rd decade. Associated complications such as choroidal (Figure 1g). Enhanced Depth Imaging-OCT showed a well-
neovascular membrane (CNV) and/or subretinal fluid, defined subretinal hyperreflective material corresponding to
haemorrhage and photoreceptor loss overlying deossified CNV type 2 with a streak of fluid. There was a depression
osteoma are factors responsible for poor visual acuity.1-6 slope in the RPE, Bruch’s membrane complex and choroid
The tumour undergoes involution either spontaneously or suggestive of conforming FCE adjacent to the edge of
secondary to treatment and is attributed to the development CNV Figure 1h. 3D imaging also confirmed the depression
of focal choroidal excavation (FCE) and CNV. FCE has slope of FCE(Figure 1i). Diagnosis of choroidal osteoma
recently been described as one or more localized areas of complicated with associated choroidal neovascularization
choroidal excavation on spectral-domain optical coherence and conforming focal choroidal excavation was made.
tomography (SD-OCT), found in correspondence with The patient was treated with three doses of intravitreal
tumour decalcification in the absence of any scleral ectasia ranibizumab ([0.5 mg] Lucentis; Genentech, Inc., South San
and staphyloma.7,8 Francisco, CA). There was resolution of subretinal fluid and
foveal contour regained its normal architecture. (Figure 2)
Case Report Visual acuity improved to 20/20 at the final visit at 5 months.

A 28-year-old female presented with complaints of diminution Discussion
of vision with metamorphopsia since 2 months in the left
eye. The best corrected visual acuity was 20/20 in the right Although choroidal osteoma is a benign lesion, calcification
eye and 20/600 in the left eye. Fundus examination of right occurs mostly in subretinal space disrupting the retinal
eye was normal. Left eye fundus examination demonstrated pigment epithelium and leading to CNV formation
a flat, opaque, peripapillary orange-yellow choroidal lesion with permanent loss of vision. Currently, there is no
above the optic disc, 5DD*3DD in size with well-defined standard treatment for choroidal osteoma. Management
margins suggestive of choroidal osteoma and a choroidal of CNV has been well described in the literature. Hence,
neovascular membrane at the macula. Pigmentary changes treatment is targeted toward resolving subretinal fluid and
on the surface of the lesion, suggestive of early decalcification management of CNV. Various treatment modalities like
and RPE atrophy were noticed supero-temporal to the disc laser photocoagulation,9 Transpupillary Thermo-Therapy
(Figure 1a). Fundus autofluorescence (FAF) imaging showed (TTT) and surgical removal of CNVM have been tried but all
a diffuse peripapillary hypo FAF area corresponding to had poor visual outcome.10 Photodynamic therapy has been
decalcification and preserved fluorescence in the calcified shown to have additional benefits resulting in improvement
region.CNV is also seen with irregular autofluorescence in visual acuity and metamorphopsia as well as a reduction
(Figure 1b). Fundus fluorescein angiography (FFA) showed in the overall size of the CNVM.11 Studies have reported that
early patchy hyperfluorescent choroidal filling with a anti-VEGF injections are effective in resolution of choroidal
well demarcated hyperfluorescent lesion in subsequent vessels and regression of CNV with improvement of vision.
frames, and late diffuse hyperfluorescence over the region The characteristics of the lesion and patient response
of choroidal osteoma (Figure 1c). There was a relatively may determine the frequency of anti-VEGF injections.
well defined area of hyperflourescence at macula which

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DJO Vol. 32, No. 2, October-December 2021

Figure 1: (a) Color Fundus Photograph shows a large peripapillary orange yellow osteoma measuring approximately 5DD horizontally and 3DD vertically
with pale area within the osteoma superior to optic disc with visibility of underlying choroidal vessels suggestive of decalcification. (b) FAF demonstrate
a diffuse peripapillary hypo FAF area corresponding to decalcification and preserved fluorescence in calcified region. Irregular FAF is seen in area of
CNV. (c) Fundus fluorescein angiography (FFA) shows early patchy hyperfluorescent choroidal filling with a well demarcated hyperfluorescent lesion
in subsequent frames, and late diffuse hyper fluorescence. (d) There is a relatively well defined area of hyperfluorescence at the macula which increases
in size in the later phase suggestive of juxta-foveal CNV. (e) NCCT of orbit shows plaque like calcification measuring 9mm×1.6mm in the posterior pole of
globe in the juxta-papillary region. (f) B scan ultrasonography of the left eye shows shallow elevated choroidal mass of 1.3 mm × 11.2 mm × 6 mm, with
high reflectivity and acoustic shadowing giving a pseudo-optic disc appearance, which is suggestive of choroidal osteoma. (g) OCT confirms CNV with
altered foveal contour and distortion of foveal architecture by hyperreflective complex (CNV) and FCE on its nasal edge. (h) EDI-OCT shows a well-defined
subretinal hyperreflective material corresponding to CNV with a streak of fluid. There is a depression in the RPE, Bruch’s membrane complex and choroid
suggestive of conforming focal choroidal excavation (FCE) adjacent to the edge of CNV and over the area of the tumour decalcification. (i)3 D view of FCE.

Figure 2: At 6 month post treatment, fundus photography (a) showed area of enlargement of decalcification. (b)There was resolution of subretinal fluid and
foveal contour regained its normal architecture as seen on OCT.

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DJO Vol. 32, No. 2, October-December 2021

Improvement in vision depends on the location of the decalcification. International ophthalmology.2001; 24:41-4
lesion, decalcification, RPE involvement, presence of CNV, 10. Aylward GW, Chang TS, Pautler SE, Gass JD. A long-term follow-
and resolution of subretinal fluid and hemorrhages.12,13,14
Our case had unilateral osteoma complicated with CNV up of choroidal osteoma. Arch Ophthalmol. 1998;116:1337–41
which was treated with 3 doses of intravitreal ranibizumab 11. Shields CL, Materin MA, Mehta S, Foxman BT, Shields
at monthly intervals. She had significant improvement in
the visual acuity but long-term follow-up is necessary to see JA. Regression of extrafoveal choroidal osteoma following
reactivation of CNV and possibility of bilateral occurrence. photodynamic therapy. Archives of Ophthalmology.2008;
126:135-7
In our case, CNV is in proximity of FCE as it is known to 12. Song WK, Koh HJ, Kwon OW, Byeon SH, Lee SC. Intravitreal
occur on the edge or the slope of FCE. FCE has been classified bevacizumab for choroidal neovascularization secondary to
into conforming lesion, in which overlying retina is close to choroidal osteoma. Acta Ophthalmol. 2009;87:100–1
RPE; non-conforming lesions, in which hyporeflective space 13. Kubota-Taniai M, Oshitari T, Handa M, Baba T, Yotsukura
is visible between retina and RPE on OCT scans.5 Pierro et J, Yamamoto S, et al. Long-term success of intravitreal
al has also described decalcification of CO as a common bevacizumab for choroidal neovascularization associated with
pathogenic pathway for the development of FCE and CNV choroidal osteoma. Clin Ophthalmol. 2011;5:1051–5
in choroidal osteoma.4 Our case had a rare combination of 14. Narayanan R,ShahVA.Intravitreal bevacizumab in the
conforming type FCE with CNVM with choroidal osteoma. management of choroidal neovascular membrane secondary to
In conforming type of FCE, there is no distortion of overlying choroidal osteoma.Eur J Ophthalmol2008 May-Jun;18(3):466-8.
retina and continuity of photoreceptor layer is maintained,
hence vision is preserved. EDI-OCT also allows detailed Cite This Article as: Rajwinder Kaur, Raja Narayanan, Balbir
study of FCE; decalcified areas have absent outer retina and Khan, Ekta Gupta, Rishabh Narula. Choroidal Osteoma with
photoreceptor layers with schisis of corresponding retinal Choroidal Neovascularization and Focal Choroidal Excavation.
layer in areas of proximity to the tumour whereas calcified Delhi Journal of Ophthalmology.2021; Vol 32, No (2): 46 - 48.
areas have intact photoreceptor layer. Intact outer retina and Acknowledgments: Nil
photoreceptor layer at subfoveal level may be attributing Conflict of interest: None declared
factor for a favourable outcome in our case.Our case also Source of Funding: None
adds to the information that decrease in vision is attributed Date of Submission: 10 Feb 2021
to the CNV in choroidal osteoma.Anti-VEGF treatment plays Date of Acceptance: 29 Jun 2021
a great role in preserving the vision in choroidal osteoma.
Address for correspondence
Conclusion Rajwinder Kaur, Professor

Focal choroidal excavation and CNV may be related to areas Department of Ophthalmology
of decalcification in choroidal osteoma. Future investigations Adesh Institute of Medical
are warranted to ascertain the association of CNV and impact Sciences and Research,Barnala
of FCE on the clinical course of choroidal osteoma. Road,Bathinda,Punjab,India.
Email : [email protected]
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