Editor-in-chief Subhash C. Dadeya, Editor-in-chief Savleen Kaur, Editor Volume 24 No. 1, July-August, 2018
Subhash C. Dadeya
Shibal Bhartiya
Patrons Executive Editor
A.K. Gupta G. Mukherjee
Gurbax Singh L.D. Sota APasslliasvtai nDtoEkdaintoiar MAsosnisictaanLtoEhdcihtoabr Abhilasha SEadnitoorria
Madan Mohan M.S. Boparai, P.K. Khosla Assistant
Chief Advisory Board Editorial 49 Pharmacotherapy in Amblyopia
Atul Kumar, Cyrus Shroff Harbansh Lal, Lalit Verma 5 Universal Health for all - New Drugs
Mahipal Sachdev Radhika Tandon
Ritu Arora, Rajendra Khanna, Rishi Mohan, R.V. Azad Featuring Sections Case Reports
S. Bharti, Sudarshan Khokhar, Sushil Kumar, Y.R. Sharma 53 Phacomatosis
Expert Corner Pigmentovascularis with Klippel
Editors 9 Accommodative Esotropia Trenaunay Weber Syndrome –
Savleen Kaur Shibal Bhartiya Case Report with Review of
Review Article Literature
Assistant Editors 17 Surgical Timing in Infantile 56 Inferior Rectus Loss and its
Abhilasha Sanoria, Monica Lohchab Esotropia Management Following Trauma
Pallavi Dokania 22 Idiopathic Infantile Nystagmus 59 Bilateral Acquired Superior
Syndrome Oblique Palsy with Left Tonic
International Advisory Board 25 Overview of Inferior Oblique Ocular Tilt Reaction Following
Arvind Chandna Derek Sprunger, Frank Martin Surgery Resection of a Left Posterior
J. Panarelli, Larson Scott Saurabh Jain, Seyhan Obzkon 29 Stereovision in Strabismus fossa mass; An Esoteric
Sonal Ferzavandi Surabhi Shalini Presentation
Perspective
National Advisory Board 33 Failed Conventional Probing or News Watch
A.K. Khurana, B.N. Gupta B.S. Goel, Barun Nayak Persistent CNLDO- what next? 61 DOS Times Quiz
C haitra Jaidev, Chand Singh Dhull Deepak Mishra, D. Ramamurthy 37 Pediatric Cataract Surgery: What 63 DOS Crossword
Dharmender Nath Gursatinder Singh Roles do Study Methodology and 65 Author Guidelines
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R.K. Bansal, Ragini Parekh Rakesh Porwal Recent Trends and Advance
S.P. Singh, Sandeep Saxena Sanjeev Nainiwal 42 Childhood Glaucoma- The CGRN
Sudesh Arya, Santhan Gopal Santosh Honavar Classification Tear Sheet
Shreya Shah, Sudhir Kumar Swapan Samantha, T.S. Surendran 45 Intra-vitreal Anti-vascular 71 Leucocoria: White Pupillary
V. Saharanamam Vandana Jain, Vinita Singh Endothelial Growth Factor Drugs Reflex
Virendra Agarwal Yogesh Shukla for Treatment of Retinopathy of
Prematurity
Delhi Advisory Board
A.K. Grover, Abhinandan Jain Ajay Aurora, Ajay Sharma
Alkesh Chaudhary Amit Chopra, Amit Khosla
Anju Rastogi Anuj Mehta, Anup Goswami
Arun Baweja, Arun Sangal Ashwini Ghai, Ashu Agarwal,
B.P. Guliani, Bhavna Chawla Dinesh Talwar
D.K. Mehta, G.K. Das H. Gandhi, H.S. Sethi, H.S. Trehan
J.K.S. Parihar, J.S. Titiyal Jatinder Bali, J.L. Goyal
J olly Rohatgi, Kamlesh, K.P.S. Malik K.R. Kuldeep, Kamal Kapoor
Kirti Singh, Lopa Sarkar, M. Vanathi M.C. Agarwal, M.L. Bharti
Mahesh Chandra Manisha Agarwal
Meenakshi Thakkar Mohita Sharma
Mukesh Sharma N.Z. Farooqui, Nabin Pattnaik
Namrata Sharma, Neeraj Sanduja, Noshir Shroff
Om Prakash O.P. Anand
P.K. Pandey, P.K. Sahu, P.N. Seth Pawan Goyal, Piyush Kapur
Poonam Jain, Pradeep Sharma Praveen Malik, Punita K. Sodhi
Rajendra Prasad, Rajesh Sinha Rajiv Bajaj, Rajiv Garg,
Rajiv Mohan, Rajiv Sudan Rajpal, Rakesh Bhardwaj
R.B. Jain, Ramanjit Sihota Rakesh Mahajan, Rohit Saxena
Sagarika Patyal Sandhya Makhija
Sanjay Chaudhary Sangeeta Abrol, Sarita Beri
Sarika Jindal, Satish Mehta Shashi Vashisht
Sunil Chakravarty, S.N. Jha Suma Ganesh, S.M. Betharia
Sushil Kumar, Tanuj Dada Taru Dewan, Tushar Agarwal
Tinku Bali, Umang Mathur Usha K. Raina, V.K. Dada
V.P. Gupta, V. Rajsekhar V.S. Gupta, Vinay Garodia
Viney Gupta, Vipul Nayar Yogesh Gupta
Section Editors
Strabismus & Oculoplasty
Paediatric Hardeep Singh
Ophthalmology Mridula Mehta
Abhishek Sharma Rachna Meel
Annu Joon, Anirudh Singh Seema Das
Deepali Mathur Sumita Sethi
Geetha Srinivasan Neuro-ophthalmology
Rasheena Bansal Promita Dutta, Satya Karna
Renu Grover, Sumit Monga Swati Phuljhele, V. Krishna
Glaucoma Retina
Deven Tuli, J.S. Bhalla Bhumika Sharma
Mainak Bhattacharya Bhuvan Chanana
Manavdeep Singh Darius Shroff
Reena Chaudhary Deependra Vikram Singh
Cataract & Refractive Devesh Kumawat
Abhishek Dagar Koushik Tripathy
Charu Khurana Naginder Vashisht
Reena Sethi Raghav Ravani
Ritika Sachdev R.P. Singh, Ritesh Narula
Sanjiv Mohan Vinod Kumar Agarwal
DOS Correspondents Cornea & Oular Surface
Divya Kishore Manisha Acharya
Manisha Mishra Noopur Gupta
Mohit Chhabara Parul Jain, Pranita Sahay
Prateeksha Sharma Rajat Jain
Richa Agarwal, Shweta Dhiman Uma Sridhar
Sumit Grover, Yashpal Goel Vikas Veerwal
DOS TIMES
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DOS EXECUTIVE MEMBERS
Executive Committee: DOS 2017-2019
DOS Office Bearers
Dr. Sudarshan Kumar Khokhar Dr. Subhash C. Dadeya
President Secretary
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Vice President Treasurer Joint Secretary Editor Library Officer
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Ex-Officio Members
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Ex-President Ex-Secretary Ex-Treasurer
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5 DOS Times - July-August 2018
Editorial
Universal Health for all!!
Dear colleagues and friends!! Dr. (Prof.) Subhash C. Dadeya
We as ophthalmologists have witnessed a rapid and striking technological
evolution with aims that are beyond just 6/6 vision. During the last few years,
there has been upheaval in surgical instrumentation, imaging and intraocular
lenses. But there is a single sub-speciality which is minimally or least affected
by our recent armamentarium i.e. pediatric ophthalmology and strabismus.
The basic principles to examine the pediatric eye have remained grossly the
same. But the residents and most academicians need access to a recapitulation
of these basic examination techniques. The present issue highlights these
techniques along with a pinch of newer diagnostic tools. The anti VEGFs have
carved their way into pediatrics through preterm babies and stereovision
is the new goal of a strabismologist. We have experts teaching us the basic
surgical steps as well small cases highlighting largely accepted norms.
In summary, this issue transforms a whole chunk of pediatrics in ophthalmology
to a booklet in your hand.
Happy reading!!
Thanks
Dr. (Prof.) Subhash C. Dadeya
Secretary - Delhi Ophthalmological Society
Room No 114, 1st Floor, OPD Block,
Guru Nanak Eye Centre, Maharaja Ranjit Singh Marg,
New Delhi - 110002
Email: [email protected], [email protected]
Mobile: 9968604336, 9810575899
WhatsApp: 8448871622
www. dos-times.org 7
Expert Corner
Accommodative Esotropia
Dr. Frank Martin, Australia Mr. John Sloper, UK Dr. Derek T Sprunger, USA Dr. Scott A. Larson, USA
Dr. Stacy L. Pineles, USA Dr. Maria Rosario Gomez De Dr. Subhash Dadeya, India Dr. Savleen Kaur, India
Liaño Sanchez, Spain
Esotropias are one of the most discussed and researched entities of Strabismus. With this article we aim to discuss the
most common of the esotropias that is the accommodative esotropias. Experts around the world were questioned about
their practice patterns in treating patients with accommodative esotropia.
(FM): Frank J. Martin, Clinical Professor University of Sydney VMO Sydney Children’s Hospitals Network and Sydney Eye Hospital
(JS): John Sloper, FRCS, FRCOphth, Honorary Consultant, Strabismus and Paediatric Service, Moorfields Eye Hospital, City Road,
LONDON.
(DS): Derek T Sprunger, MD, FAAO, MAAPOS. Associate Clinical Professor, IUSOM. Indianapolis, in USA
(SL): Scott Larson, Scott A. Larson, MD. William E. Scott Professor of Pediatric Ophthalmology Department of Ophthalmology
and Visual Sciences, University of Iowa. USA
(SP): Stacy L. Pineles, MD, MS, Jerome and Joan Snyder Chair in Ophthalmology, Director, Ophthalmology Residency Program,
Pediatric Ophthalmology, Adult Strabismus, Neuro-Ophthalmology, Stein Eye Institute, UCLA Department of Ophthalmology, Los
Angeles, California, USA.
(RG): Maria Rosario Gomez De Liaño Sanchez. Professor of Ophthalmology at Universidad Complutense de Madrid (Spain), Head
of the Department of Motility Unit at Hospital Clinico San Carlos (Madrid - Spain). ISA President (International Strabismological
Association). IPOSC Secretary - Treasurer (International Pediatric Ophthalmology and Strabismus Council).
(SD); Dr. Subhash Dadeya, Director Professor of Ophthalmology, Guru Nanak Eye Center, Maulana Azad Medical College, New
Delhi, India. President Strabismus and Pediatric Oph Society of India 2017-18 and Secretary, Delhi Ophthalmological Society).
The questions have been prepared by (SK): Dr. Savleen Kaur, Senior Research Associate, Advanced Eye Center, Post Graduate
Institute of Medical Education and Research, Chandigarh, India. Executive Editor DOS Times.
www. dos-times.org 11
Expert Corner (SD): Most cases of Accommodative esotropia usually
present between age of 3-5 years however in our
(SK): Do you see esotropia more commonly than setting some patients report at later age. Strabismus
exotropia in your clinical practice? is the most common symptom for which these
patients are brought to the hospital by parents.
(FM): In our clinical practice we see an equal number of
esotropes and exotropes. (SK): How do you like to classify accommodative
esotropia?
(JS): Yes, considerable more frequently.
(DS): Intermittent is most common patient in my clinical (FM): We like to classify accommodative esotropia as fully
accommodative with or without a high AC/A ratio
practice. or partially accommodative with or without a high
(SL): Yes. The majority of children I see with strabismus AC/A ratio.
have accommodative esotropia. I see many who have (JS): Based on age of onset, the presence of refractive
Northern European heritage who are more likely to error and its effect on the angle of squint, the
be hyperopic. presence of any normal or anomalous binocular
(SP): No, I see more exotropia. function and any difference between near and
(RG): Yes, I do. distance angle and binocularity. But there are many
(SD): No, in our setting esotropia is more commonly seen children who don’t fit into a neat classification and
than esotropia. I try to understand the pathophysiology of each
individual child using the above features.
(SK): At what age and with what symptoms do these
patients present to you? (DS): I classify them as A) Fully accommodative (eyes
aligned with spectacle) vs partially accommodative
(FM): Congenital esotropes present between the age of 3-6 (eyes not aligned with spectacles). B) High AC/A)
months. Their parents or a health professional has Eyes aligned at distance with spectacles and
observed their strabismus. Very occasionally they esotropia at near) vs Normal AC/A.
present later as the child’s family had been given
incorrect information that strabismus in the first year (SL): Fully accommodative and partially accommodative.
of life is not unusual. Distance/Near Disparity with or without high AC/A.
Accommodative esotropes usually present between (SP): I tend to classify them as fully accommodative or
the age of 2½-4 years. Their parents or carers partially accommodative and then group by normal
observe that the child has intermittent esotropia or high AC/A ratio. Of course, there are the rarer
that is noticeable when the child is fixating on a near cases that are non-refractive and only present at
object. The strabismus tends to be more obvious near but those are not typically seen.
when the child is tired. Accommodative esotropia
may at times present in infancy as an esotropia with (SD): I classify them as fully accommodative and partially
an accommodative element. accommodative with high AC/A and Normal AC/A
ratio.
Exotropia may present as intermittent exotropia at
any time between the age of 6 months to 4-5 years. (SK): Which cycloplegic do you prefer for children
The child is observed to have intermittent exotropia with esotropia?
that manifests at distance fixation. The child very
often has a tendency to close one eye in sunlight. (FM): We prefer to use Cyclopentolate 1%. In most
children one drop is sufficient to achieve cycloplegia,
Constant exotropia may present at any age. There is however, in some children with dark brown eyes
often a history of premature birth, developmental two drops are needed. We like to wait at least 30
delay, or poor health. Parents may also observe that minutes before refracting the child.
these children have poor vision in one eye.
(JS): I usually use cyclopentolate 1% unless the child’s
(JS): A few children present in the first year of life, but irides are very dark or cyclopentolate doesn’t fully
more at age 2 to 4 years. They can present at any age. relax their accommodation. For these children I
Parents usually bring them because they have noticed use atropine 1% twice a day for 3 days before the
the appearance of a squint refraction.
(DS): All ages for esotropia and exotropia (DS): Cyclopentolate 1% for > 1 year of age; cyclopentolate
(SL): The peak age is between 2 and 3 years old. Most of 0.5% for < 1 year.
the time the family has noticed intermittent crossing (SL): Cyclopentolate 1%.
of the eyes. (SP): I prefer Cyclogyl 1%.
(SP): Patients either present as infants less than 6 months (RG): Cyclopentolate 1%, below 9 months cyclopentolate
with infantile onset esotropia, at a later stage such as
2-4 years of age with accommodative esotropia, or 0.5%. Atropine 1% if refractive ET/ high AC/A ratio.
later with diplopia. (SD): I prefer 1% atropine till 7 year of age and 1%
(RG): Being a referential centre Infantile ET comes too
late-usually between 8 -12 months. Acquired ET Cyclopentolate after 7 year of age.
presents at all ages and often after receiving different
treatments.
12 DOS Times - July-August 2018
(SK): What are the most important clinical Expert Corner
considerations while examining patients with
esotropia? usually has significant hyperopia. The child with
accommodative esotropia almost invariably has a
(FM): The most important consideration is the visual period of their life before the strabismus develops
acuity in each eye. We measure the esotropia both where the child’s eyes are straight and the child is
at near and distance fixation. Ocular motility is binocular.
checked looking for any restriction to exclude a (JS): I try to determine the effect of full refractive
restrictive problem such as Duane syndrome or a correction on esotropia at whatever age it presents.
cranial nerve palsy. Careful optic nerve and retinal (DS): If eyes are fully aligned with hyperopia, this rules
examination is critical to exclude underlying out infantile esotropia.
pathology. It is important to ensure the child is fully (SL): Age of onset. The children with onset of esotropia
cyclopleged for an accurate refraction. In children before age 6 months do behave differently than
who appear to have a high AC/A ratio we use +3.00D those with and onset at age 2-3. These younger
lenses to ascertain the response at near fixation. children are much less likely to respond to spectacle
Visual acuity is assessed using an age appropriate treatment. Magnitude of Esotropia and degree
test. Accurate visual acuity is very important for of hyperopia are also considerations. Experience
amblyopia management. suggests that children with low hyperopia and large
angle esotropia respond differently (and less well)
(JS): Excluding lateral rectus weakness and assessing than those with larger degrees of hyperopia.
acuity and any potential for binocular function. Also (SP): Typically based on refractive error and response to
determining the full cycloplegic refractive error and hyperopic correction. However, age of onset is also
assessing its effect on the esotropia. important. If the age of onset is less than one year
of age, the patient typically has infantile esotropia
(DS): Whether the eyes are aligned with spectacle but if there is significant hyperopia, I will still try to
correction (from full cycloplegic refraction) and treat them with spectacles. If there are other signs
presence or absence of amblyopia. of infantile esotropia such as DVD, latent nystagmus,
or over-elevation in adduction, these signs will
(SL): Amblyopia is the major impediment to developing sway me more towards infantile esotropia.
binocular vision. A careful visual acuity assessment (RG): Higher Refraction, trial of plus lenses, usually they
is critical. have a variable angle of esotropia.
(SD): Based on age of on set, refractive error and response
(SP): For me, the most important initial consideration is to hyperopic corrections and other associations of
refractive error. Once we correct refractive error, infantile esotropia.
then it is important to determine whether there is
any amblyopia. Once refractive error and amblyopia (SK): Do you prefer a partial or full correction with
are treated, if there is still esotropia manifest with glasses? Do you prefer a step wise increase in
correction, then we start to consider near: distance power of the glasses?
disparity. If the patient is fusing at distance but not
at near, then bifocal spectacles can be added. (FM): In children with accommodative esotropia we
generally prescribe the full correction. If the child
(RG): 1. To out rule any systemic and ocular aetiology. is unable to tolerate their glasses, we use either a
2 Refractive value. 3. Amblyopia 4. It is really short term or long term cycloplegic agent to allow
a concomitant ET, or it has limitations or if is the child to relax into their hyperopic correction.
mainly Nystagmus with ET 5. Vertical associated
strabismus. 6. Head turn. 7. Evolution over time. (JS): I give full cycloplegic correction from the start.
The number of squint operations performed for
(SD): Visual acuity, refractive error, amblyopia, AC/A esotropia in the UK is now half of what it was
ratio, ocular motility to rule out VIth nerve palsy 25 years ago because we are much better at
and fundus examination are important clinical fully correction hypermetropia in children with
considerations while examining patients with esotropia.
esotropia.
(DS): Full correction with no stepwise increase.
(SK): How do you differentiate these patients from (SL): The major obstacle to binocular vision after
infantile esotropia with an accommodative
component? amblyopia is persistent strabismus. I would like
the child to get as straight as possible as soon
(FM): Patients with infantile esotropia with an as possible. I prefer prescribing full cycloplegic
accommodative componentarelikelytohaveanearly refraction and have not found benefit in prescribing
onset of their strabismus. They will have the typical less than the full plus at the outset of treatment.
features of infantile esotropia, including a tendency (SP): I typically prescribe the full correction. I only treat
to cross-fixate, pseudo abduction weakness and with partial plus if the patient does not tolerate the
possibly nystagmus. In due course, the child may full hyperopic correction.
develop either dissociated vertical deviation (DVD) (RG): Full correction if it is accommodative and no
or inferior oblique muscle overaction or both. They increase if possible.
generally have a low hyperopic error as opposed (SD): I prefer full correction.
to the child with accommodative esotropia who
www. dos-times.org 13
Expert Corner are not significant changes to the glasses, I usually
see the children yearly.
(SK): In which type of patients with esotropia do you (SP): I usually like to follow up after two to three months.
prefer bifocals or progressive addition lenses? (RG): 4-6 weeks but not only to see improvement of
the deviation but amblyopia and fusion may also
(FM): We prescribe bifocals or progressive additional improve over time. Some patients have initial
lenses only if the child is straight at distance and has improvement with the glasses and deviation recurs
residual esotropia at near fixation. In the partially after months.
accommodative esotrope we usually repeat (SD): After 4-6 weeks.
refraction 2-3 months after the initial pair of glasses
are prescribed as repeat refraction may uncover (SK): How do you treat Amblyopia in these patients of
additional hyperopia. We prefer to use bifocals and accommodative esotropia?
specify to the dispensing optician that the bifocals
should be a large segment with the upper part of (FM): Our management of amblyopia is no different to that
the segment bisecting the pupil. in other patients. We follow the PEDIG guidelines
and prefer to use face occlusion to pharmacological
(JS): I use bifocals in children with convergence excess penalisation. Occasionally we will use a filter
esotropia (binocular for distance and esotropic occlusion Bangerter filter).
for near) when parents prefer bifocals to surgery. I
don’t use progressive lenses in children. (JS): By patching as a first choice, usually initially for two
hours a day increasing if needed. I generally reserve
(DS): If there is esotropia at near fixation while eyes are atropine penalisation for a child who won’t wear a
aligned at distance fixation. patch.
(SL): I would not typically start children in bifocals even if (DS): I prefer part time occlusion and atropine 1%
there is an apparent distance / near disparity from penalization if non-compliant with patch.
a high AC/A ratio. Most children in this situation
will get better alignment at near with single vision (SL): My goal with amblyopia treatment is equal vision
glasses. For those children in their full hyperopic at the youngest age possible. For this reason, I
correction who still have crossing at near > 8 prism recommend full time patching in children if their
diopters and their alignment improves with +3.00 vision has not improved with the glasses. I prefer
lenses at near viewing over their glasses, I would full time patching to gain vision and part time
consider bifocals then. I warn the parents at the patching to maintain vision. With that said, I try to
outset that we may have to change the lenses more make the parents a partner in the decision-making
than once in the beginning, so this is not a surprise for their child so if they make an informed decision
for them. Luckily most opticians in our area are to do part time patching or atropine at the outset, I
willing to change the lenses at no additional cost support them.
if we do the change in the first few months of
purchase. (SP): I start with refractive correction and if the
amblyopia does not improve, I start 2 hours per day
I prefer flat top bifocals, so the power of the bifocal of patching.
is high. I find progressive-add bifocals are more
likely to fail given the add power is at the bottom (RG): Mild to moderate amblyopia is treated by partial
of the lens. Children are much more likely to ignore occlusion; Severe cases by total occlusion, then
the progressive bifocals. depends on the rhythm of recovery, deviation and
refraction I combine it with penalisation, atropine,
(SP): I only treat with bifocals if the patient is fusing at filters etc.
distance but not at near.
(SD): Refractive correction followed by patching and if
(RG): AC/A ratio with no or very small strabismus at required television and mobile games.
distance, the first glasses are Bifocals then I go for
progressive lenses. (SK): Which patients with accommodative esotropia
are the best candidates for surgery?
(SD): I prefer bifocals if there is residual esotropia at near
while eyes are ortho at distance. (FM): The best candidates for surgery are children with
partial accommodative esotropia. The surgical
(SK): When do you like to follow up a child with outcome in these children is usually extremely good
accommodative esotropia after prescribing as they have been binocular during the early part
him/her glasses? of their life before the accommodative esotropia
manifested.
(FM): We generally follow-up within 2-3 months after
issuing the prescription. If the child’s strabismus is (JS): Those with a recent onset of esotropia who do not
not fully corrected with the hyperopic correction, fully correct when they have fully adapted to their
we will frequently repeat the refraction. full hypermetropic correction because they have
the potential to recover binocular function. I only
(JS): Usually six weeks after first glasses prescription to operate on children with no binocular potential if
check that they are wearing the glasses properly they still have a cosmetically poor squint when they
and to assess their effect are wearing their full hypermetropic correction.
(DS): I like to follow up after 2 months.
(SL): For the initial glasses, I check within 2 months.
Once the child has been stable for a year and there
14 DOS Times - July-August 2018
(DS): Those with residual deviation while using Expert Corner
spectacles and no amblyopia.
(SK): Do you prefer a bilateral symmetrical surgery in
(SL): Those who partially respond to the glasses could be these patients?
considered. Ideally, they have equal vision before
surgery. This includes those who may respond with (FM): In children with accommodative esotropia we
good distance alignment but still have crossing at prefer symmetrical surgery – bimedial rectus
near. muscle recession.
(SP): I treat any patient with more than 10-12 PD of (JS): No, I generally do a recess/resect procedure on one
esotropia with their full hyperopic correction with eye unless there is a significantly greater angle for
surgery. near, when I do bimedial recessions.
(RG): Early onset cases, normal retinal correspondence, (DS): Yes, if no amblyopia. No if amblyopia present.
Decompensated microtropia or any other case with (SL): Not necessarily. I often do bilateral symmetrical
good potential of binocularity.
surgery but often also use recess/resect. I honestly
(SD): Cases of partially accommodative esotropia. don’t know that one is superior than the other.
However, I offer pre-operative prism adaptation for
(SK): According to you what is the best time to operate those who partially respond to glasses, in that case, I
these patients? use bilateral symmetrical surgery. I also use bilateral
surgery for Esotropia associated with a pattern
(FM): If the child’s accommodative esotropia is fully or for distance near disparity. If the family doesn’t
controlled with glasses, there is absolutely no want to do pre-operative prism adaptation and the
indication for surgery. We will operate on children angle of esotropia is less than 35 prism diopters, I
with partial accommodative esotropia at any age if would consider a recess/resect procedure. Also, if
the esotropia is uncosmetic. there is significant residual amblyopia, I prefer to
do a recess/resect procedure in the weaker eye.
Early surgery also improves the opportunity for (SP): Yes, I prefer a Bilateral symmetric surgery
regaining binocular visual function. In children (RG): In Acquired Accommodative ET it will depend
with accommodative esotropia with high AC/A on the amount of deviation (for large deviations
ratio where the eyes are straight at distance and I prefer R+Rs); If the deviation is greater at near
esotropia continues to manifest at near, we will distance- Bimedial recessions. If the AC/A is very
operate on these children after the age of 13-14 large and deviation at distance is very small I prefer
years to help take them out of bifocals or multifocals. faden surgery alone or combined with unilateral
MR recession. Small deviations up to 25 PD, no
We have found that by waiting until the child’s vertical surgery is needed and children below age
accommodative component has significantly of 3-4; I usually treat them with Botox. In cases of
decreased in the teenage years, we rarely have to Infantile ET in children my preferred technique is
consider surgery in this group. Bimedial recessions or Botox if deviation is up to 30
PD.
(JS): If I think that there is a chance of recovering (SD): In most of cases I prefer bilateral symmetrical
binocular function when the squint is of recent medial rectus recession.
onset I operate as soon as practicable once they are
fully adapted to their glasses. Otherwise it is when (SK): What is your procedure of choice-recession
the child and parent wish for surgery. with or without augmentation, faden/posterior
fixation suture?
(DS): As soon as possible after determining that
spectacles alone do not align eyes. (FM): We prefer bimedial rectus muscle recession. We
have found that there is no need for augmentation.
(SL): When amblyopia treatment has reached an end
point (equal vision or as good as can be obtained (JS): Straightforward medial rectus recession and lateral
with treatment) and there are two strabismus rectus resection or bimedial rectus recessions.
measurements that correspond within about 5
prism diopters, surgery can be considered. (DS): Bilateral medial rectus recession alone.
(SL): For distance/near disparity I would operate for
(SP): As soon as I am sure that they are still esotropic
with their full cycloplegic refraction and amblyopia the near angle or the prism adapted angle for
is treated. those with a High AC/A. If they have a distance
near disparity without a high AC/A, I would use a
(RG): When the deviation is almost stable and there are posterior fixation suture.
no signs of spontaneous recovery. (SP): I typically do a bilateral medial rectus recession for
the average between the distance and near angles
(SD): In fully accommodative esotropia surgery is not and will add posterior fixation sutures if there is a
recommended, however in cases of partially high AC: A ratio.
accommodative esotropia and non-accommodative (RG): I prefer faden for very cases with very High AC/A
esotropia surgery should be performed for ratio, usually bridge technique.
cosmetically unacceptable deviation after treating (SD): Bilateral medial rectus recession.
the amblyopia when child is cooperative for
examination. www. dos-times.org 15
Expert Corner change of glasses. I will do if I think the refraction
can be weaned completely off (small hyperopia,
(SK): What is your early postoperative goal in such small astigmatism, and small anisometropia).
patients? (SD): I don’t recommend weaning.
(FM): Our goal at surgery is to regain binocular visual (SK): What are the risk factors for consecutive
function. We aim for orthotropia or a very small exotropia in these patients?
residual esotropia (within 10 prism dioptres)
which would still allow the child to regain binocular (FM): The risk factors are uncorrected amblyopia, not
visual function. decreasing the hyperopic correction at appropriate
interval and most importantly operating on children
(JS): If I think they have the potential to regain binocular with fully accommodative esotropia. The child with
function I aim for orthotropia. If not, I aim to leave partial accommodative esotropia we tend not to
them slightly esotropic in their glasses. over-weaken the medial rectus muscles.
(DS): Alignment (no E or X). (JS): Doing surgery in a child whose hypermetropia is
(SL): For all post-op esotropia patients, I hope for a small not fully corrected.
exotropia (<8) in the first 1-2 weeks. I am hoping by (DS): Prime risk factor is operation on patient not
this to break up suppression if it is present. wearing spectacles for his or her accommodative
(SP): My postoperative goal is orthotropia at distance component.
and near with their full cycloplegic refraction.
(RG): Ortho or small monofixation syndrome. (SL): Consecutive exotropia to me means a previously
(SD): Goal of surgery in such patients is orthophoria or fully corrected accommodative esotrope who now
residual esotropia within 8 prism dioptres. is exotropic in the full cycloplegic refraction. I know
others use this term for post-operative exotropia.
(SK): How do you wean glasses in patients with If we are discussing the first scenario, I don’t know
accommodative esotropia? that it can be predicted, there are a certain number
of children that develop exotropia in their glasses as
(FM): As the child grows older there is a tendency their accommodative convergence decreases.
to become less hyperopic and the hyperopic
correction can gradually be decreased. As control (SP): Over-correction after surgery or needing to
of their accommodative esotropia increases we decrease the plus immediately post-operatively.
slowly decrease the hyperopic correction giving
the minimum amount required to maintain (RG): Initial overcorrection after surgery, not compliant
binocularity both at distance and at near. Older with glasses specially in cases of anisometropia,
children will often present for review complaining large MR Recessions, high hyperopia, amblyopia,
of some blur of their distance vision. This is usually residual large alphabetic patterns or vertical
a good indication that we can safely decrease the deviations, psychomotor delayed children,
hyperopic correction. In children with good control, hyperactive children.
we encourage them to wear their glasses less for
outdoors and recreational activities. We insist that (SD): Uncorrected refractive error, amblyopia, surgery on
they continue to use their glasses for schoolwork. fully accommodative esotropia.
Orthoptic exercises (clear and misty) can be (SK): What is your take on hyperopic refractive
helpful in giving the child an understanding how surgery in these patients?
they can “flex” their accommodation/convergence
relationship and learn to hold their eyes straight on (FM): We do not recommend refractive surgery before the
occasions, such as for school photographs without age of 21 years. At that age we ensure that before the
the need to wear their glasses. child undergoes refractive surgery, their refraction
has been stable for at least 2-3 years. Surgery can
(JS): I don’t. be successful if hypermetropia falls in correctable
(DS): I do not wean unless cycloplegic refraction range.
decreases. I prescribe enough hyperopia (up to full We have found that contact lenses are an excellent
refraction) to keep eyes aligned. alternative to glasses and can be worn very
(SL): For fully controlled accommodative esotropia, successfully in children from a young age (5-6
meaning orthotropia with glasses on at distance years).
and near, I have my orthoptists test the child with
loose minus lenses held over their glasses to find at (JS): I don’t recommend it. The refractive correction
what point they become esotropic. I typically don’t required often changes with time and it is important
reduce the glasses power by more than one diopter to keep then full corrected.
in this case. I’d shoot to keep them under-plussed
to the point that they can have straight eyes but still (DS): I do not recommend refractive surgery before
have normal vision. 21 years old. Response to surgery after 21 is
(SP): I typically measure them with -0.50 over their satisfactory.
glasses 2-3x/year and if they are still orthotropic, I
will decrease their prescription. (SL): I know some young adults may want to get out
(RG): Generally, after age 9 -10 weaken them by 0.75 D of their glasses who still need them to maintain
straight eyes and they don’t want to do contact
16 DOS Times - July-August 2018 lenses. I think it is not a bad option for those >20
years. I have seen it done successfully. Expert Corner
(SP): I have not used it so do not have any experience
(SL): A majority will have less hyperopia over time. A
with it. substantial minority will be straight without glasses
(RG): I usually do not suggest refractive surgery in at some point before age 12 years but many will
need glasses into young adulthood or adulthood to
children. Over life I speak with them on the keep straight eyes.
possibility of a future refractive surgery. Small
moderate hyperopes may decrease and may need (SP): I think that the component that is corrected with
a refractive surgery if Accommodative ET persists. spectacles slowly improves as the child grows and
High hyperopia usually does not decrease over typically resolves by the mid-teens.
years and some have too shallow anterior chambers
and cannot have an ICL so in those patients I am (RG): Some improve spontaneously over years - partially
cautious with my conversations. accommodative ET of smaller amount and greater
(SD): I don’t recommend refractive surgery in these at near.
patients.
(SD): Majority of patients become less hyperopic during
(SK): What do you think is the natural course of course of time.
patients with accommodative esotropia?
(SK): Do you have any experience in botulinum
(FM): This is one group of patients who do extremely toxin in esotropia with an accommodative
well with conservative management (correction component?
of refractive error, bifocals where indicated and
management of any amblyopia). Maintaining (FM): I have not managed accommodative esotropia with
excellent communication with the family as to what Botulinum Toxin. I have seen a few patients for a
to expect from the start is extremely important. second opinion where the Botulinum Toxin has
Many of the children with accommodative esotropia proved to be unsuccessful. I would not recommend
are very intelligent and learn to understand why the use of Botulinum Toxin which is an intervention
they have strabismus and what techniques they can with risk attached to manage a problem that can
use to help control the accommodative esotropia. be safely and successfully managed with optical
Fully accommodative esotropes usually depend correction.
on optical correction for their control throughout
their life. The hyperopic correction over time can (JS): No, I don’t have any experience.
generally be decreased. Many become successful (DS): I have no experience with injectable toxins for this
contact lens wearers and look forward to refractive
surgery when they are older. clinical entity.
(SL): No.
Those with a high AC/A ratio can usually be weaned (SP): No, I have not used it for this situation.
out of their bifocal or multifocal by the age of 13- (RG): It is a very good option for ET until 25/30 PD and
14 years. If they continue to rely on their near
correction, then surgery to weaken the medial for residual deviations.
rectus muscle can be considered after the age of 13- (SD): No, I don’t used botulinum toxin in esotropia with
14 years.
an accommodative component.
Partial accommodative esotropes, especially those
with a low hyperopic correction generally improve Compiled by:
over time and many can manage their activities Dr Savleen Kaur
apart from close work without the need for glasses. Senior Research Associate, PGIMER, Chandigarh
I have found that children with accommodative Email: [email protected]
esotropia of any form with a high hyperopic
correction usually prefer to continue to wear their
glasses throughout life.
(JS): Most will stay stable provided they continue to
wear full correction for their hypermetropia.
(DS): The usual course is to becomes less hyperopic over
time and need less correction for good alignment.
www. dos-times.org 17
Review Article
Surgical Timing in Infantile Esotropia
Dr. Suma Ganesh MS, DNB, Dr. Soveeta Rath DNB, FICO
Department of Pediatric Ophthalmology, Strabismus and Neuro Ophthalmology,
Dr Shroff Charity Eye Hospital, Daryaganj, New Delhi, India
Summary: Infantile esotropia is classically defined as an esotropia with an onset before the age of 6 months. It affects 1 in every 100
to 500 persons. The most beneficial timing of surgery of infantile esotropia still remains controversial. Helveston describes intervention
between 4-6 months of age as “Ultra-early”, surgery before the age of 2 years as “Early” and any intervention after 2 years as “Late”
surgery. Basic research proves that alignment of eyes during the critical period of development of sensory system ensures development of
binocular vision. The benefit of prompt surgery must be balanced with the rate of reoperations and spontaneous resolution while deciding
on the surgical management of Infantile esotropia and the same must be meticulously counselled to parents.
Infantile esotropia (IE) is classically defined as an 3. Bilateral MR recession with unilateral LR resection.
esotropia with an onset before the age of 6 months. It 4. Bilateral MR recession with bilateral LR resection.
affects 1 in every 100 to 500 persons1,2,3. It presents
with a large angle of strabismus (> 30 prism diopters However, in many cases single surgery does not result in
(PD), no or mild amblyopia, hypermetropia < 2 dioptres, complete motor outcome and a second surgery for correction of
cross fixation and pseudo limitation of abduction, latent residual esotropia is warranted. In some children development
nystagmus, dissociated vertical deviation (DVD) and absent or of dissociated vertical deviation and inferior oblique overaction
reduced binocular vision, in the absence of any neurological usually develops later (after 2 years of age) desiring second
disorder. Affected children exhibit abnormal stereopsis4,5 and surgery for the treatment of resultant vertical deviation then.
naso-temporal asymmetries of smooth pursuit, optokinetic
responses6,7 and abnormal visual motion processing8,9,10. Surgical dosage
Multiple factors like sufficient patient cooperation for
evaluation and planning, horizontal deviation stability, In children with IE, bilateral medial rectus recession is
estimation of vertical deviation (if any), any oblique muscle the most commonly preferred surgical approach although size
overaction, absence of accommodative factors, presence of of the growing eyeball and unstable angle of deviation have
alternating fixation determine the appropriate surgical option always been a deciding factor. The dosage which is usually
and timing. Although surgical correction remains the gold followed varies with the angle of deviation. In these children
standard management option for infantile esotropia, the most amount of recession to be performed is usually measured from
beneficial timing of surgery still remains controversial. This limbus rather than insertion.
review focusses on appropriate timing of surgical repair in
infantile esotropia to attain the best motor as well as sensory WHY IS TIMING IMPORTANT FOR INFANTILE
outcomes. ESOTROPIA
Management of Infantile Esotropia The question of timing of surgery has always been an issue,
because basic research proves that alignment of eyes during
Management of IE begins with assessment of visual acuity, the critical period of development of sensory system ensures
refractive error, amblyopia. Though not very common, yet development of binocular vision. This critical period occurs
amblyopia and refractive error has to be treated first before between first 4-6 months of age. Stereopsis usually develops
planning any surgical intervention. during this period and attains maturity by 6-12 months of age15.
Studies on animal models showed that prism induced esotropia
The Congenital Esotropia Observation Study (CEOS) found caused irreversible loss of cortical cells and stereopsis as
that infants presenting with esotropia before 20 weeks of age early as within 3 weeks16. This equally corelates in humans
have a greater variation of size and character of the deviation. that increased tendency for abnormal binocular experience
Yet in 98% of these infants esotropia persists, especially those usually occurs during the development of stereopsis17. In a
who have large-magnitude (≥20° or 40 PD) esotropia with study by Birch and Stager, prism-corrected esotropic patients
onset after 10 weeks of age and refractive error ≤3.00 diopters, by 5 months of age developed stereopsis similar to infants
warranting early surgery11,12. of the same age with a normal visual development in 40% of
them whereas only 20% developed stereopsis if corrected after
Surgical options 5 months of age18. Thus this forms the basis of concept early
surgical correction of infantile esotropia.
The available surgical approaches for correction of
infantile esotropia includes13,14. OPTIMUM TIMING OF SURGERY: HOW EARLY?
1. Bilateral medial rectus (MR) recession
2. Unilateral MR recession combined with lateral rectus (LR) The unresolved debate related to optimal timing of surgery
dates back to 1939 when Chavasse proposed that the ability of
resection. developing fusion in infants with congenital squint was possible
Ganesh S. et al. Surgical Timing in Infantile Esotropia www. dosonline.org 19
th onset after 10 weeks of age and refractive error ≤3.00 diopters,
rranting early surgery. 11,12
Review Article
e ultimate goal of treatment of IE is
The ultimate goal of treatment of IE is Absence of nystagmus or head
bobbing
Absence of any previous
extraocular muscle surgery
Review article • To align the • To promote the Absence of any structural ocular.
development of Absence of following birth
visual axes
history:
binocular vision
Gestational age < 34 weeks
which is usually followed varies with the angle of deviation. In these Birth weight <1500 grams
children amount of recession to be performed is usually measured from Ventilator treatment in the
limbus rather than insertion. newborn period
rgical optioTnhes:figure below shows the amount of recession for different angle 14 History of meningitis or
any major medical event.
e available surgical approaches for correction of infantile esSoetnrsoorpyiaand Motor Outcomes :
ludes13,14 Early Vs Late Surgery:
Small Medium Large Larger SENSORY: Stereopsis is considered
1. Bilateral me82.05d--39i0amPmlD rectus (MR)9.5 -10 mrPDemcession10.54-51P1Dmm 12-12.5 mm the gold standard in evaluation of
2. 30- 45
50- 60 PD and binocular status in patients with
UnilateRreavlieMw aRrtricelecession combined with lateral rectus g(reLaRter) resectiosatsnrsaebsissemdubsy. Tmhiusltpiaprleamsteutderiehsaisnththues been
light
3. Bilateral MR recession with unilateral LR resection
of surgical timing of IE. Surgery around
4. BilaterctaholenMgaegneRitoarfletswcqoueiysnetsaiwrosa.n1s9pwosistihblebiiflathteedreavliaLtRionrecosueldctbieofnully corrected before 1 year of age is associated with better
stereopsis has been reported in multiple
studies. Other group of authors found that
However, Ainuthmorsahnayvecraepsoerstedsivnargioleus stiumregferarmye dapoperosprniaotetforressuurgletryinto cacohmievpeletea hmighoetroperrcentage of children operated
outcomeWHgdaYoifnofIeSddrTemInMaotttIoNersrGmeanIcsMdoresPnegOnadRrsdoTirAsnyguNosrTuugtFrcegOoircRmyaeIlNsti.fFmBoAiaNrnsgTedIucLsoEoenrEdrSabeOgyecTHtRoeifOolcvPhneIisAltd:ooantfainrerte2e0r:sviedntuioanl, theesotrsboteefproerioea-a2cyuiesitayrsthoafnatgheosaelsoopaecrhatieevdebdeygoonodd
warrantTehde. qIunesstioomn oef octihmtvhieialndrtgareaoclftnigisonudmnregeeuvnrtesyulhooaafplslmyeaylewdesnaeytvsdeoublrfeoinepdgnsisatslnhaoeticsesicurareitt(,eiacbadfeltcevapreuesrr2eitoiydbceaasaloicfrdseao2olfvsysofeitaaebarrteseiegooonepfnqsa)iugseefs3o3tl.iloHonwoewidnegbvyeeraorttlhhyeesrasut3r4tg.aeCinreymntheranastl
and inferreisoearrcohblpiqrouves
desiringdesveecloopnmdenst uofrgseenrsyoryfosyrstetmheentsruereastdmeveenlotpmoefntroefsbuinltoacnultar vveisirotnic. Tahlisdeanvdiapteiroipnheral fusion has also been
tSIpnhureercgnfhe.iicrldraerliSbcdeedredrtdqxyeeriuneptvuvodiveeeacBsiesrllawleiooluirralyessppcrinighpsetcbgocohedeleaniderrc:nieusuoIlatdoalrEsadeilnsunt,rSsieeagmtoboasaolclaftgyppicihllnecusaopiorisrmsc,htsrrcteuppsooiuibcmrerdmraiaerassealictimolllnwcdsahdes-umreclsrleottashihmoanenrnboaarugoleddewintltntfnstctwdiihurtefriahaathrneasdeotgsseleeltcantetdaontaruertdrertfe4ysil4reh-ngeeyaa-ea-s-gao6vs6nshcooetefeypttmlmdirtuosnphosiatporssteepitiemnrszubaivencmrsteesreeahadeofinrpontesmfeltycryataii2enon-oeetrosoyncbtidlufeehfysyfyaruaengsgascraeftasi6tteseogsrogehy-dreew1rwne.eb2aiiteoybtShishpmntsio5gensooatirrtsmrr3enomo.hn1otowp7woheapnierslastmIieimnhbnsokasifgsnalcuo.oo1aasvs6sfcuugtieTstuuaseauhlyedgl.acal1ideyyesr5lobmemdsrffaooeuveuleplamrslalongolcusewrdrITuoatirthabityrnheneeeotngadsdomnlseudyefaarastoniirsholmnltsllmeyogooptswefhrbbtuobehiaernviesmegnegioneocfatirchjrryotusseethretlafpraeonboyrrslsiepreotIttnaayEurerar3y.dd5as(.oiomTetofthasueelabitmtftelcweeorpehmowaifrrco1aealhyssr)l
unstabledeavnelgoplemeonft deinvi4a0t%ioonf hthaevme wahlweraeaayftsesro2bnyeleyaersn20a% ddeevceildoipnegd fsatecrteooprs.isThifeadsyomsmaegtreies of both motion detection
corrected after 5 months of age.18 Thus this forms the basis of concept early and mVEPs37,38.
surgical correction of infantile esotropia. Though all above studies focus on
potential benefit of improved sensory
nesh et al. InifHfatehnlevteidlseetvoienas’tsioopntrrcoooppuoildasabl eoffuulllytrcao-reraercltyedsurgciocuanlttriimesinsugrgwearys issuppeprfoorrtmeeddbayt tWheraigghet, outcomes following early surgery,
OPbITenIfgMoraeUntMdheBTaiIgrMcehoINfetGtwaoOl.yF2e1aS,2rU2s,21R39.GSuErRgYic:aHl OinWterEvAeonRf t2LiYoo?nr 3byeefaorrse28t. he second birthday has stereopsis beyond intermediate grade
GwThahnebSicebenisbrcIeotauyeinnetortenunrarsaettglnmghelsehnyrnW.iCiisHnvmdrIceddaenevhreaaetenasentnieylclasrgaeuvryntsoneovhlrdieri.hund.otvnEaglts,2Iavist,nentiu4sso‘eascoeLn,ifs2rbornadgIanadt5oenvbylnien,e’t2boespgeetSd6rpnneeoi’eetehleritneoyfaxabmidiobrroopanneefHacpainirycdn‘snaorbScootootsdgnseteobptnwtsat,BsrSeou2edni‘iosreimfidsEwnddreumop2yaveactarlariielehraayornrergseaitlrendtgeeyhdeartz’e’srhsosratadtytaasuhfresitosnsenpuotfeafdatosontpfr‘lheteaEgr.uaMoom2ccoergage1lrtIootv,ae2Erepeterrn2egeanlnya,dtyi2iddlinbstit-3ssi’imrtsiboclaiioypaovptnyleenpaIocSNtrra srrhroifsbeinomDoradyfyexfrbteIwEBidfifhmCnemrrliSsaeeiqaeeAgnoheascevadTetigdidatkoetnrrdiIeve‘nsovadOlfLao-roelpdobiaytssNcc6pinoetuaaatES1eium,thtarmnSle2i’tglenoFogdicipuomoenotdnOealrwotfmsttrogR.uyih‘s2pit1eSnewtsis7hldEshst8izehAootoae2oaAe9fesfmnsrnu-taRn3rtaee2dt2nuetpLgo.isaaddnYeeonhedribbrretadSvaiMehasnr3e’sUt,clcsrfwytafuy,kaRsrobgeeerewnsGltlereluaaitmooeEhnerrstwneegRsmno1w2ettritY19n8oereUin0yg3arantolhS9st. has been achieved only by few of these .
regimen24,25,26. However the controversy Constant esotropia >40PD at near
lingers and there has been no consensus MOTOR: The literature is replete with
on exact timing as noted by Shaffiq and (1/3 metres) on 2 examination, reports suggesting enhanced binocular
separated by 2-4 weeks vision following prompt surgery for
Elliot27. As per current US standard, age Refractive error < 3.00 dioptres. IE, however concerns affecting motor
of first surgery for IE is approximately 12 Absence of Incomitant or outcomes include:
to 18 months, whereas in many European 1. Instability of angle of deviation and
paralytic strabismus
accuracy of measurement
2. Chances of reoperation
3. Tendency of spontaneous resolution
4. Onset of DVD
5. Need of hyperopic glasses
Instability of angle of deviation and
accuracy of measurement: It is believed
that surgical outcomes usually get
20 DOS Times - July-August 2018 Ganesh S. et al. Surgical Timing in Infantile Esotropia
Review Article
Author Year No of Children Timing of Surgery Steropsis Achieved/Not Tests Used Fusion
Birch 1990 81 Before 1 year 35%
Charles 1992 24 9-23 months 19.04% Randot Not available (NA)
Wright 1994 7 13-19 weeks 71.4%
Ing 1995 16 4 months 68.7% Frisby/Titmus NA
Birch 1995 73 5-16 months 41.1%
Birch 1998 80 Before15 months 30% Randot NA
Shirabe 2000 9 Before 8 months 55.5%
Titmus/Randot 68.7%
Simonz 2005
Random dot NA
Birch 2006
Random dot NA
Cerman 2014
Magli 2016 231 6-24 months 13.5% vs 3.9% Titmus 44.4%
Randot stereo test NA
(RDS) Dynamic RDS
Housefly
301 32-60 months
50 0-6 months 38 % vs 16% Randot 78% vs 61%
128 7-12 months
38 13- 39 months 50% Randot/Titmus NA
Lang and Titmus
188 Before and after 2 50.7% 78.3%
63.8%
years in < 2 years
36.1%
in > 2years
Early Late smaller angle of deviation, intermittent
Advantages: and variable angles and hyperopia
1.Increased potential for binocularity Advantages: >3D requiring spectacle correction39. A
2.Reduced muscle contracture 1. Stable and reliable measurement constant esotropia > 40 PD presenting
2. Better amblyopia management after 10 weeks of age has a low likelihood
Disadvantages: 3. Lesser chances of reoperations of spontaneous resolution. Surgery on
1. Unstable measurement 4. Decreased risks of general anaesthesia patients in whom the esotropia may
2. Chances of recurrent/residual spontaneously resolve may lead to
esotropia and spontaneous resolution Disadvantages: consecutive exotropia. The tendency of
3. Cannot rule out accommodative 1.Reduced chances of binocularity spontaneous resolution depends mainly
component 2.Increased muscle contracture can lead on constancy of deviation rather than the
4. Technical difficulties due to exact to mechanical component of squint size of deviation has been described by Fu
measurements in growing eyeball et al.
affected while operating on unstable and group and 0.99 in the late group. In a Thus the benefit of prompt
imprecise angle of deviation in children meta-regression analysis of the ELISSS surgery has to be balanced with the
with IE. However Birch et al reports and 12 other studies it was found that rate of reoperations and spontaneous
comparable motor as well as sensory reoperation rates were 60-80% for resolution while deciding on the surgical
outcomes at 1 year post- operative follow children first operated around age 1 and management of IE and the same has to be
up both for stable and unstable angle 25% for children operated around age meticulously counselled to parents.
of deviation. Unstable deviation was 4.28 In another recent study by Magli et
considered if there was >10 PD variation al, patients who underwent surgery ≤2 Dissociated vertical deviation: Shin
at 2 preoperative visits at an interval of 6 years of age had a reoperation rate of and Paik identified association of DVD
weeks. 12.1% vs a reoperation rate of 8.6% in with later surgery and large preoperative
children operated on at >2 years of age. angle by multivariate analysis in a
Studies have reported good retrospective review of 90 patients
post operative alignment (<10PD of The cause for second surgery was of IE who had undergone surgery40.
orthophoria) following early intervention either residual or recurrent esotropia Surgery before 24 months of age for IE
for IE only with single surgery. These or consecutive exotropia. Onset of new reduces the incidence and the severity of
provide an inference that neither the vertical deviation or DVD and inferior spontaneous DVD, especially in cases with
angle instability nor does the accuracy oblique overaction were the other a large amount of esodeviation, lowering
of measurement affects motor outcomes indications for another surgery. the need for a second operation to correct
adversely. DVD was also reported by Yagashaki et
Spontaneous resolution: The other al41.
Reoperation rates: Several studies issue in a small subset of children with
has shown that surgery at a very early age IE is tendency of spontaneous resolution. Hyperopic glasses: Pre operative
increases the number of reoperations. In children with early onset esotropia hyperopic glass prescription is an
In the ELISS study ,the mean number factors favouring spontaneous resolution important determinant of surgical
of operations was 1.18 in the early include younger age at presentation, planning and outcomes. ELISS study
found that spherical equivalent of the
glasses was 3.0 D in the early vs. 2.9
Ganesh S. et al. Surgical Timing in Infantile Esotropia www. dosonline.org 21
Review Article
D in the late group. Glasses had been underwent surgery for IE before 2 years childhood esotropia: a population-
prescribed previously in 19.1% children of age (“early group”) whereas 126 were based study. Ophthalmology
in the early group vs. 24.3% in the late operated after their second birthday 2007;114:170–4.
group 25 Birch et al reports postoperative (“late group”). The mean preoperative 2. Mohney BG, Erie JC, Hodge DO, Jacobsen
hyperopia of +4.00 D to +5.50 D was found deviation was comparable in both the SJ. Congenital esotropia in Olmsted
in 9% of those in the early surgery group groups (49.07PD vs 46.41PD). In the County, Minnesota. Ophthalmology
and 6 % of those in the standard surgery. early group, mean age at surgery was 1998;105:846–50.
Hyperopia as a baseline parameter did 16 months and after a mean follow up 3. Friedman Z, Neumann E, Hyams SW,
not vary between groups with regard to of 36 months, 41 (75.92%) achieved an Peleg B. Ophthalmic screening of 38000
surgical timing . alignment within 10PD of orthophoria children, age 1 to 2.5 years, in child
and 13/54 (24.07%) needed reoperation welfare clinics. J Pediatr Ophthalmol
Other factors for residual esotropia and consecutive Strabismus 1980;17:261–267.
exotropia. The mean age at surgery for 4. von Noorden, GK. Binocular Vision
Social concern: Besides above those operated >2 years of age was 60 and Ocular Motility: Theory and
parameters, social stigma has always months. Ninety eight (76.5%) children Management of Strabismus. 5. St. Louis,
been a prominent determinant while achieved good alignment within 10PD Mo: CV Mosby; 1996.
discussing about strabismus. There are orthophoria whereas 28 (22.2%) needed 5. Birch EE, Gwiazda J, Held R.
chances of poor parent- child relation second surgery. Stereo-acuity between Stereoacuity development for crossed
due to the cosmetic appearance of the 3000-200 seconds of arc was attained in and uncrossed disparities in human
child. Moreover, straight eyes following 55% children in the early group versus infants. Vision Res 1982;22:507–13.
early surgery alleviates psychomotor only 27% in the late group in those who 6. Westall CA, Eizenman M, Kraft SP et al.
development of the growing child. were cooperative for evaluation at final Cortical binocularity and monocular
follow up. optokinetic asymmetry in early-onset
Type of surgery: There has been esotropia. Invest Ophthalmol Vis Sci
no consensus so far regarding the best To conclude this data from the north 1998;39:1352–1360.
surgical approach for IE. Although Indian cohort also supports the current 7. Westall CA, Shute RH. OKN asymmetries
various techniques have been proposed literature that early surgery results in in orthoptic patients: contributing
by different studies, none have a clear comparable motor but better sensory factors and effect of treatment. Behav
advantage over other. Magli et al analysed outcomes irrespective of stability of Brain Res 1992;49:77–84.
9 different surgical approaches and found deviation. 8. Birch EE, Fawcett S, Stager D. Co-
that a six muscle approach (Bilateral MR development of VEP motion response
recession+ LR resection+ Inferior oblique CONCLUSION and binocular vision in normal
weakening) had 11 times higher chance infants and infantile esotropes. Invest
of improving binocular vision compared Infantile esotropia and its Ophthalmol Vis Sci 2000;41:1719–
to 3 muscle approach (Bilateral MR management still remains a controversial 1723.
recession+ Unilateral LR resection). issue though there is no dearth of literature 9. Norcia AM, Hamer RD, Jampolsky A,
related to surgical outcomes. Majority of Orel-Bixler D. Plasticity of human
Duration of misalignment: It has also studies favour surgery before 2 years of motion processing mechanisms
been found that duration of misalignment age or earlier to enhance the potential of following surgery for infantile
determines binocular vision development. binocularity, in the absence of any adverse esotropia. Vision Res 1995;35:3279–
Better stereoacuity is seen with stable motor or refractive outcomes. However, it 3296.
long-term eye alignment42. is noted that only coarse stereopsis and 10. Tychsen L, Burkhalter A, Boothe
peripheral fusion are achieved in most RG. Neural mechanisms in infantile
Early Vs Late: Risks And Benefits: of them. Another concern that has to esotropia—what goes wrong? Am
be borne by surgeons is post-operative Orthoptic J 1996;46:18–28.
An extensive literature review now stability of deviation on a long term basis 11. Pediatric Eye Disease Investigator
provides enough evidence favouring is essential to maintain binocularity. Group. The Clinical Spectrum of Early-
prompt and early surgical management Preoperative instability of deviation does onset Esotropia: Experience of the
for infantile esotropia. Yet, late surgery not influence the surgical outcomes. Thus, Congenital Esotropia Observational
is still being practised by several prompt surgical intervention during the Study. Am J Ophthalmol 2002;133:102–
proponents. In the following table we critical period based on the rationale of 08.
summarize the advantages and dis- restoration of cortical connections has to 12. Pediatric Eye Disease Investigator
advantages of early vs late surgery. be followed for management of IE. Yet, the Group. Spontaneous resolution of
surgical decision is based on the clinical early-onset esotropia: experience of
Although above issues may pose examination and a meticulous orthoptic the Congenital Esotropia Observational
some difficulties in planning early evaluation by the surgeon. Optimal Study. Am J Ophthalmol 2002;133:109–
surgery the major factor that outweighs surgical timing definitely varies as per 18.
these problems are preservation and clinical scenario. 13. Rosenbaum AL, Santiago AP. Clinical
restoration of binocular vision by early strabismus management. Philadelphia:
surgery. REFERENCES WB Saunders;1999;pp 117-23.
14. Helveston EM. Atlas of strabismus
OUR DATA: (unpublished) 1. Greenberg AE, Mohney BG, Diehl surgery. Mosby;1985;pp 108-110.
NN, Burke JP. Incidence and types of 15. Birch EE, Gwiazda J, Held R.
A retrospective chart review of Stereoacuity development for crossed
children who presented with infantile and uncrossed disparities in human
esotropia at out tertiary eye care center infants. Vision Res. 1982;22:507–13.
between 2012-2017 showed 54 children 16. Crawford ML, Von Noorden GK.
Optically induced concomitant
strabismus in monkeys. Investig
Ophthalmol Vis Sci. 1980;19:1105–9.
17. Kumagami T, Zhang B, Smith E, Chino
Y. Effect of onset age of strabismus on
the binocular responses of neurons
in the monkey visual cortex. Invest
Ophthalmol Vis Sci 2000;41:948-54.
22 DOS Times - July-August 2018 Ganesh S. et al. Surgical Timing in Infantile Esotropia
Review Article
18. Birch EE, Stager DR. Monocular Paediatr Neurol. 2011;15:205-8. children with infantile esotropia. Invest
29. Birch E, Stager D, Wright K, Beck R. The Ophthalmol Vis Sci. 2007;48:5523–31.
acuity and stereopsis in infantile 38. Peckford L ,Wright T Gerth, C.; Wong,
natural history of infantile esotropia AM. Annual Meeting Abstract and
esotropia. Invest Ophthalmol Vis Sci during the first six months of life. Program Planner. Association for
Journal of the American Association Research in Vision and Ophthalmology;
1985;26:1624–30. for Pediatric Ophthalmology and 2007. Early vs. delayed surgery for
Strabismus 1998;2:325–8. infantile esotropia in human infants:
19. Chavasse FB. Worth’s Squint—or the 30. Birch EE, Felius J, Stager DR Sr, Weakley effects on cortical visual motion
binocular reflexes and the treatment of DR Jr, Bosworth RG. Pre-operative processing. Abstract 4868.
stability of infantile esotropia and post- 39. Friedrich D, de Decker W. Prospective
strabismus. 7th ed. London: Bailliere, operative outcome. Am J Ophthalmol. study of the development of strabismus
2004;138:1003–9. during the first 6 months of life.
Tyndal & Cox, 1939. 31. Wong AM. Timing of surgery for Orthoptic Horizons 1987:21–28.
infantile esotropia: sensory and 40. Shin KH, Paik HJ. Factors influencing
20. Helveston EM, Ellis FD, Plager DW, motor outcomes. Can J Ophthalmol. the development and severity of
2008;43:643-51. & dissociated vertical deviation in
Miller KK. Early surgery for essential 32. Fu VL, Stager DR, Birch EE. Progression patients with infantile esotropia. J
of intermittent, small-angle, and AAPOS 2014;18:389–391.
infantile esotropia. Journal of Pediatric variable esotropia in infancy. Invest 41. Yagasaki T, Yokoyama YO, Maeda M.
Ophthalmol Vis Sci. 2007;48:661–664. Influence of timing of initial surgery
Ophthalmology and Strabismus 33. Çerman E, Eraslan M, Öğüt MS. The for infantile esotropia on the severity
relationship of age when motor of dissociated vertical deviation. Jpn J
1990;27:115–9. alignment is achieved and the Ophthalmol 2011;55:383–8.
subsequent development of stereopsis 42. Birch EE, Fawcett S, Stager DR. Why
21. Wright KW, Edelman PM, McVey JH, et in infantile esotropia. JAAPOS. does early surgical alignment improve
2014;18:222-5. stereopsis outcomes in infantile
al. High-grade stereo acuity after early 34. Charles SJ, Moore AT. Results of early esotropia? J AAPOS. 2000;4:10–4.
surgery for infantile esotropia in
surgery for congenital esotropia. Arch normal and neurologically impaired Correspondence to:
infants. Eye (Lond). 1992;6:603-6. Dr. Suma Ganesh
Ophthalmol 1994;112:913-19. 35. Birch EE, Stager DR, Berry P, Everett Department of Pediatric ophthalmology,
ME. Prospective assessment of acuity Strabismus and Neuro ophthalmology,
22. Ing MR. Surgical alignment prior and stereopsis in amblyopic infantile Dr Shroff Charity Eye Hospital,
esotropes following early surgery. Daryaganj, New Delhi, India
to six months of age for congenital Invest Ophthalmol Vis Sci 1990;31:758-
65.
esotropia. Trans Am Ophthalmol Soc. 36. Birch E, Stager D, Everett M. Random
dot stereoacuity following surgical
1995;93:135–46. correction of infantile esotropia.
Journal of Pediatric Ophthalmology
23. Birch EE, Stager DR Sr. Long-term and Strabismus 1995;32:231–5.
37. Bosworth RG, Birch EE. Direction-
motor and sensory outcomes after of-motion detection and motion VEP
asymmetries in normal children and
early surgery for infantile esotropia. J
AAPOS. 2006;10:409–13.
24. Shirabe H, Mori Y, Dogru M, Yamamoto
M. Early surgery for infantile esotropia.
Br J Ophthalmol 2000;84:536-8.
25. Simonsz HJ, Kolling GH, Unnebrink K.
Final report of the early vs. late infantile
strabismus surgery study (ELISSS), a
controlled, prospective, multicenter
study. Strabismus 2005;13:169–199.
26. Magli A, Carelli R, Esposito F,
Bruzzese D. Essential Infantile
Esotropia:Postoperative Sensory
Outcomes of Strabismus Surgery.
Semin Ophthalmol 2017;32:663-67.
27. Elliott S, Shafiq A. Interventions for
infantile esotropia. The Cochrane
Database of Systematic Reviews. 2005,
Issue 1. Art. No. CD004917.
28. Simonsz HJ, Kolling GH. Best age for
surgery for infantile esotropia. Eur
Ganesh S. et al. Surgical Timing in Infantile Esotropia www. dosonline.org 23
Review Article
Idiopathic Infantile Nystagmus Syndrome
Dr. Nripen Gaur MD, Dr. Pradeep Sharma MD
Department of Pediatric Ophthalmology, Strabismus Services,
Dr. R.P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
Ocular nystagmus is a regular, rhythmic and Table 1 : Clinical characteristics of infantile nystagmus
involuntary to and fro movement of the eye syndrome
which leads to the disruption of the steady
fixation. Nystagmus cycle consists of a slow 1. Almost always bilateral and conjugate in direction and
ocular movement away from fixation which is frequency
followed by a corrective movement towards
fixation in the opposite direction. These oscillations may either 2. Uniplanar: usually horizontal. Rarely can be vertical or
consist of slow eye movements (pendular nystagmus) or as a torsional.
combination of slow and fast movements (jerk nystagmus).
Pendular nystagmus is a smooth sinusoidal oscillation which is 3. Similar amplitude on both sides. An accelerating slow
equal on both sides whereas in jerk nystagmus the eyes appear phase is present.
to beat in one direction.
Infantile nystagmus syndrome (INS) is a common type of 4. Dampened by convergence, so near vision is better.
early-onset nystagmus. The incidence of this disease is about 5. No oscillopsia.
one in thousand1 and there is a male preponderance. The 6. A null zone is usually present and the head posture is
pathophysiology of this syndrome is still unclear. It is also
called congenital nystagmus, however, it is rarely seen at birth assumed to bring the eyes in null zone.
and presents in the first few months of life. The Classification 7. Disappears during sleep or general anaesthesia.
of Eye Movements Abnormalities and Strabismus (CEMAS) 8. Distinctive waveforms are present: jerk /pendular/
Working Group have proposed a classification to bring about
clarity to the eye movement and strabismus literature2. They mixed.
have two criteria for infantile nystagmus syndrome: infantile-
onset and accelerating slow phases on ocular motor recordings. The prevalence of anterior visual pathway abnormalities
According to CEMAS, INS is a different clinical entity from other has been noted in patients having INS. It ranges from 38 per
forms of early-onset nystagmus like fusion maldevelopment cent to 91 percent4. These include congenital cataracts, albinism
syndromes (manifest latent nystagmus), spasmus nutans and and various optic nerve disorders5.
the ones localizing to brainstem or cerebellum.
Pathogenesis
Clinical features
The pathophysiology of INS remains unclear, however
This condition usually begins at 2 to 3 months of age. many theories have been proposed to support the pathogenesis
Clinically the nystagmus is binocular and conjugate. It is of this condition. These theories can be grouped into two
uniplanar and usually horizontal, rarely it can be vertical or categories: those which consider INS resulting from a primary
torsional. It can have distinctive waveforms: pendular, jerk or defect in the ocular motor control system6 and those which
mixed. These waveforms may vary in different gaze positions consider it to result from a defect in the visual sensory system7.
and can vary at different times. The nystagmus is accentuated
by fixation effort and improves on eye closure. It is dampened by Diagnosis and clinical examination
convergence effort, hence the near vision is usually better. A null As per CEMAS classification, the waveform analysis of
zone is often present where the amplitude or frequency or both
is minimum. Hence, the patient usually assumes a head posture eye movements is required for the diagnosis. The nystagmus
to bring the eyes to the null zone. Table 1 enlists the clinical movements must be distinguished from nystagmoid
features of infantile nystagmus syndrome. Congenital periodic movements which include oculopalatal myoclonus, opsoclonus,
alternating nystagmus (PAN) is a primary-position nystagmus ocular bobbing and Heimann-Bielchowsky’s unilateral vertical
that periodically switches direction after approximately 120 bobbing. A detailed history regarding the onset of nystagmus,
seconds. Between each cycle there is a quiet phase of a few abnormal head posture, developmental delay, family history of
seconds before the nystagmus begins to beat in the opposite nystagmus must be taken. A detailed ocular examination must
direction3. Acquired forms of PAN often occur with pathology be done to rule out the presence of any anterior visual pathway
involving the cerebellum, such as cerebellar degenerations, defect. The nystagmus waveform, frequency, amplitude,
tumours and multiple sclerosis. Arnold chiary malformation direction and plane of oscillation must be recorded. Presence
can also present with PAN. Hence these conditions should be or absence of null zone must be looked for. A dilated fundus
always ruled out in a patient presenting with PAN. examination is essential to rule our retinal pathologies. INS
must be distinguished from FMS with which it can be confused.
24 DOS Times - July-August 2018 Table 2 lists salient differences between INS and FMS. An ERG
may be done to rule out retinal degeneration8. A magnetic
resonance imaging may be ordered if any neurologic pathology
is suspected.
Sharma P. et al.Idiopathic Infantile Nystagmus Syndrome
Review Article
Table 2 : Differences between infantile nystagmus syndrome(manifest ophthalmic division of oculomotor nerve.
nystagmus) and Fusional maldevelopment syndrome (manifest latent Additionally, acupuncture applied to the
sternocleidomastoid muscle may also
nystagmus) work to reduce the nystagmus. However,
in a study conducted at our centre, the
Manifest nystagmus Manifest latent nystagmus effect was found to be shortlived.
1. Biphasic, mostly planar Mostly jerk or mixed Pharmacologic therapy for
nystagmus has also been tried. Both
2. No change on abduction Increased on abduction memantine and gabapentin have been
tried for nystagmus, both of them
3. Accelerating slow phase Decelerating slow phase reduced the intensity but had no effect
on visual acuity. Oral and topical carbonic
4. No change in covering one eye Increase on covering one eye anhydrase inhibitors have also been
tried11, In a study conducted by Hertle et al
5. Direction independent of fixing eye but Fast phase always towards fixing eye to compare Topical brinzolamide (Azopt)
versus placebo in the treatment of INS,
may change on the either side of null zone topical Azopt significantly improved; INS
waveform characteristics in the primary
6. Less commonly associated with Nearly always associated with infantile position null zone, group mean values of
the nystagmus acuity function across gaze
infantile esotropia(10-50%) esotropia(95%) and group mean ETDRS binocular letter
visual acuity. However further studies are
7. Binocular visual acuity same as Binocular visual acuity better than needed in this area to establish anything
concrete. Baclofen prescribed at 30 mg
uniocular monocular vision. per day in three divided doses is useful
and commonly decreases acquired PAN.
Table 3 : Treatment options for nystagmus However, congenital PAN appears to
respond less so to baclofen12. Similarly,
Non-surgical treatment baclofen has no effect on INS.
1. Optical devices: glasses, contact lenses , prisms
2. Occlusion and pleoptics Surgical management
3. Medical therapy
4. Acupuncture The principle for nystagmus surgery
5. Auditory biofeedback in INS is to correct the abnormal head
6. Botulinum toxin posture by shifting the null zone to
the primary position. This can be done
Surgical treatment with the help of surgery on horizontal
1. To shift the null position to primary: Modified Kestenbaum surgery recti muscles. Kestenbaum devised the
2. To induce adduction / convergence : Bimedial rectus recession /faden first surgical approach using recession
3. To reduce the amplitude of nystagmus: supramaximal recession or faden on all – resection of all four horizontal recti.
horizontal recti. Anderson advocated only recessions of
yoke muscles to correct face turn. We
Figure 1: Showing 7-year child having INS presenting with 30 degree faceturn (1A) corrected by follow Augmented Anderson approach
Augmented Anderson procedure (1B). to correct up to 30 degrees of face turn
(Figure 1)13. This involves 9 mm of
Management Non-surgical therapy medial rectus recession and 12 mm of
lateral rectus recession on yoke muscles
Management of INS involves The use of optical devices has been depending on the direction of face
symptomatic therapy for nystagmus. tried in the treatment of nystagmus. turn. Hertle Dell’Osso procedure which
Any underlying systemic and ocular The use of over minus lenses stimulates involves disinsertion of all recti and
disorders must be looked for and treated accommodative convergence and thus can their reinsertion has been found to be
accordingly. Asymptomatic forms with dampen nystagmus. Base –out prisms can effective for short durations in decreasing
good vision do not require specific be additionally used to induce fusional amplitude of nystagmus in patients
therapy, however, these patients must be convergence by using 7-prism dioptre having no eccentric null14.
screened for refractive errors and should base out prism in front of each eye9.
be screened accordingly. The patients Contact lenses have been used to achieve In cases of vertical nystagmus having
having a significant visual impairment fusional control in these patients10. a chin elevation of depression of 25
and head postures must be treated Soft contact lenses may additionally degrees or more, a vertical Kestenbaum
must for the same. Following are the dampen nystagmus through a sensory surgery can be done which involves the
management options of INS. feedback mechanism by stimulating the same principle as that of Kestenbaum
procedure. For torsional nystagmus,
three modalities have been described:
the vertical shifting of horizontal recti,
horizontal shifting of vertical recti
Sharma P. et al.Idiopathic Infantile Nystagmus Syndrome www. dosonline.org 25
Review Article
and lastly selective advancement and of infantile nystagmus: What to do Ophthalmol. 2015;99:471–6.
recession of the anterior fibres of the four first and why. Ophthalmic Genet. 12. McLean RJ, Gottlob I. The
obliques. Table 3 enlists the management 2017;38:22–33.
options for nystagmus. 3. Kim SH, Chung W-K, Kim BG, Hwang CS, pharmacological treatment of
Kim MJ, Lee W-S. Periodic alternating nystagmus: a review. Expert Opin
Conclusion nystagmus of peripheral vestibular Pharmacother. 2009;10:1805–16.
origin. Laryngoscope. 2014;124:980–3. 13. Gupta R, Sharma P, Menon V. A
INS remains and challenging disorder 4. Weiss AH, Biersdorf WR. Visual sensory prospective clinical evaluation of
for the paediatric ophthalmologists. The disorders in congenital nystagmus. augmented Anderson procedure
aetiology still remains obscure. Care Ophthalmology. 1989;96:517–23. for idiopathic infantile nystagmus. J
must be taken to uncover any associated 5. Abadi RV, Bjerre A. Motor and sensory AAPOS. 2006;10:312–7.
neurological and visual pathways characteristics of infantile nystagmus. 14. Singh A, Ashar J, Sharma P, Saxena R,
disorder. Proper refractive correction Br J Ophthalmol. 2002;86:1152–60. Menon V. A prospective evaluation of
and amblyopia management must be 6. Jacobs JB, Dell’Osso LF. Congenital retroequatorial recession of horizontal
assured. Timely management in cases nystagmus: hypotheses for its genesis rectus muscles and Hertle-Dell’Osso
of patients having face turn can assure and complex waveforms within a tenotomy procedure in patients with
prevention of torticollis. The surgical behavioral ocular motor system model. infantile nystagmus with no definite
procedures to dampen the nystagmus J Vis. 2004;4:604–25. null position. J AAPOS. 2016;20:96–9.
have not been able to provide satisfactory 7. Kelly DH. Visual processing of moving
results. With newer studies in the area of stimuli. J Opt Soc Am A. 1985;2:216–25. Correspondence to:
pharmacological therapy for nystagmus 8. Cibis GW, Fitzgerald KM. Dr. Pradeep Sharma
being undertaken, the future holds Electroretinography in congenital Pediatric Ophthalmology & Strabismus Services,
promise for the medical treatment of this idiopathic nystagmus. Pediatr Neurol. Dr. R.P. Centre for Ophthalmic Sciences,
disease. 1993;9:369–71. All India Institute of Medical Sciences,
9. Thurtell MJ, Leigh RJ. Treatment of Ansari Nagar, New Delhi, India.
References nystagmus. Curr Treat Options Neurol.
2012;14:60–72.
1. Forssman B, Ringnér B. Prevalence and 10. Biousse V, Tusa RJ, Russell B, Azran
inheritance of congenital nystagmus in MS, Das V, Schubert MS, et al. The
a Swedish population. Ann Hum Genet. use of contact lenses to treat visually
1971;35:139–47. symptomatic congenital nystagmus.
J Neurol Neurosurg Psychiatry.
2. Bertsch M, Floyd M, Kehoe T, Pfeifer 2004;75:314–6.
W, Drack AV. The clinical evaluation 11. Hertle RW, Yang D, Adkinson T, Reed M.
Topical brinzolamide (Azopt) versus
placebo in the treatment of infantile
nystagmus syndrome (INS). Br J
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26 DOS Times - July-August 2018 Sharma P. et al.Idiopathic Infantile Nystagmus Syndrome
Review Article
Overview of Inferior Oblique Surgery
Dr. Subhash Dadeya MD, MNAMS, FIMSA
Director-Professor Ophthalmology, Guru Nanak Eye Centre, Maulana Azad Medical College, Delhi, India
Inferior oblique surgery remains a challenge for 5. chin-down head position in patients with nystagmus.
strabismologist. Various types of Inferior oblique 6. Inferior rectus aplasia.
weakening procedures in the past decade have been
useful addition to our armamentarium of surgical PRIMARY INFERIOR OBLIQUE SURGICAL PROCEDURES
procedures. Technical advances have translated a better Recession
outcome, however the art has to be mastered & skill
need to be acquired. This article will provide an insight into the The inferior oblique muscle can be recessed along its
surgical skill required for various types of commonly performed anatomical path by as much as 14 mm. The inferior oblique
Inferior oblique surgeries recession is commonly used as a primary weakening procedure
Anatomy: The inferior oblique originates at the periosteum in patients with over elevation in adduction. 10 mm Inferior
of the maxillary bone near the lacrimal fossa and inserts into oblique recession is a simple, safe and effective method for the
the posterior globe near the inferior border of the lateral rectus, cosmetic and functional treatment of horizontal deviation and
passing under the inferior rectus. With the eye in the primary V pattern with primary inferior oblique overaction.
position, the inferior oblique makes a 51° angle with the visual
axis and acts an excycloductor. The secondary and tertiary Surgical technique
actions are elevation and abduction, respectively. The inferior After inserting the speculum and applying the fixation
oblique is innervated by the lower portion of the coulometer
nerve. suture, Retract globe in extreme elevation and adduction.
Surgery is performed more commonly on the inferior Make incision in infero temporal bulbar conjunctiva 7 to
oblique muscle than on any other cyclovertical muscle. Surgical
access to the inferior oblique muscle and procedures designed 9mm from the limbus a point between the lateral rectus
to alter the effect of contraction of the inferior oblique muscle and inferior rectus.
on the globe are relatively straightforward and complications The incision extended downwards and backwards.
are infrequent in experienced hands. Blunt dissection of facial attachments is done.
The anatomy of the inferior oblique muscle is somewhat Muscle is isolated over by muscle hook facilitating direct
atypical compared to the other extraocular muscles, which is visualization of the inferior vortex vein underneath.
important for understanding the effects of the various surgical Do not hook LR/IR fibres.
procedures on eye position and ocular motility. In particular, Care should also be taken to hook the entire muscle and be
innervation of the muscle occurs through the neurofibrovascular aware of the possibility of multiple congenital insertions.
bundle (NFVB), which attaches midway between the origin and 60 vicryl is inserted into the anterior aspect of the
the insertion 2 mm temporal to the inferior rectus muscle. After inferioroblique muscle near its insertion and the suture is
anterior transposition of the muscle, the NFVB ligament will looked.
mechanically act as the muscle’s new origin. The inferior oblique is severed from its insertion 3 mm
from the globe.
BRIEF HISTORY The interior oblique is sutured 7 mm lateral and 7 mm
The first studies of the effects of inferior oblique weakening inferior end of the lateral rectus insertion (Figure 1).
This method ensured that there was no bunching of the
that include anterior transposition were published in the 1940s. muscle and the muscle remained in its plane of action.
Accordingly, most operations on the inferior oblique muscle are When needed, this surgery was combined with the
designed to diminish its function. Weakening procedures on the appropriate horizontal muscle surgery either in the same
inferior oblique muscle are numerous and include myotomy, sitting or at a second sitting.
myectomy, recession, marginal myotomy, disinsertion, anterior Most commonly performed Inferior Oblique Surgery is by:
transposition, Hang Back recession and denervation and Parks (IR) or Fink’s (LR) technique).
extirpation.
Anterior transposition
INDICATIONS
Many current inferior oblique recession techniques
1. Primary inferior oblique overaction. include various ranges of transposition of the insertion and
2. Superior oblique palsy. are categorized under “anterior transpositions.” The main
3. V, Y, or X pattern strabismus with or without difference between these procedures and true recessions is
that anterior transposition alters the path of the muscle, which
craniosynostosis. is generally more effective at weakening the inferior oblique.
4. Duane’s syndrome patients with upshoot. Anterior transposition of the inferior oblique muscle has been
Dadeya S. Overview of Inferior Oblique Surgery www. dosonline.org 27
Review Article
Figure 1
widely used for the treatment of increased 2. Over elevation combined with severe change or adjustment to the inferior
adduction associated with DVD (Table 1). excyclotorsion. oblique muscle.
It is achieved by placing the IO Myectomy Nasal myectomy. In this procedure,
insertion at any point anterior to its a 5-mm section of the muscle is excised
normal course can be placed just below Inferior oblique myectomy typically along the muscle belly between the
the lower border of LR muscle or involves the surgical removal of a NFVB and the origin. The most recently
temporal to the IR insertion. segment of muscle between the NFVB and described weakening operations on
the insertion of the muscle. The muscle the inferior oblique muscle are nasal
It is stronger weakening procedure segment is cauterized prior to removal. myectomy and anterior and nasal
as compared to recession. The primary advantage is convenience as transposition.
this procedure is faster than a recession.
It causes antielevation syndrome In addition, there is less risk of surgical Both anterior transposition and
(AES). Hence this procedure when intraoperative complication because myectomy reduced the function of the
performed unilaterally is reserved for there is no scleral pass required to overactive inferior oblique muscle, but
patients with significant hypertropia in reattach the muscle. The disadvantage eyes with anterior transposition tended
upgaze and patients of SO paresis with is the possibility that the muscle may to be more effective.
atleast 25PD hypertropia in primary reattach and lead to recurrent overaction.
position. Hang Back Recessions
SECONDARY INFERIOR OBLIQUE
Anterior nasal transposition SURGICAL PROCEDURES Although hang-back recession
has widely been used as a weakening
Anterior nasal transposition (ANT) Residual or recurrent symptoms of procedure on extraocular muscles, its
is a relatively new procedure, in which the inferior oblique overaction are common. effectiveness has mostly been studied for
insertion is transposed to a location over Further weakening of the inferior oblique rectus muscles. A surgical technique for
the nasal half of the inferior rectus muscle muscle can be accomplished by a further recessing the inferior oblique muscle by
typically 2 mm nasal to the nasal border recession (re-recession). However, if the Hang Back technique its effectiveness in
of the inferior rectus muscle and 2 mm muscle was already maximally recessed V-pattern strabismus with inferior oblique
posterior to the inferior rectus insertion. or previously transposed, or if severe overaction (IOOA) has been described by
The inferior oblique is thus transformed weakening is the objective, re-recession Kamlesh et al. and they concluded 10 mm
from an extorter into an intorter and may not be an option. Inferior oblique recession is a simple, safe
from an elevator to a depressor. The and effective method for the cosmetic
ANT can be used to eliminate or reduce Traditional drastic inferior and functional treatment of horizontal
severe excyclotorsion. An advantage of oblique weakening procedures, such deviation and V pattern with primary
this procedure over temporal anterior as denervation with extirpation or inferior oblique overaction. The surgical
transposition is that it avoids AES. myectomy, are disadvantageous; because technique consisted of free suspension of
they render the muscle definitively one or both inferior oblique muscles 10
Indications for ANT are nonfunctional, prohibiting any further mm along their physiological path using
1. Bilateral 4th nerve palsy
28 DOS Times - July-August 2018 Dadeya S. Overview of Inferior Oblique Surgery
Hang Back Recessions
Although hang-back recession has widely been used as a weakening procedure on extraocular Review Article
muscles, its effectiveness has mostly been studied for rectus muscles. A surgical technique for
recessing the inferior oblique muscle by HaTnabgleB1ack technique its effectiveness in V-opfarettcesrsnion done.
strabismus wOivtherainctfieornior oblique overaTcrtainosnpo(IsOitOioAn)Suhragserbyeen described by Kamlesh etAadlv.aanntdages
they conclud+e4d 10 mm Inferior obliqueAtriencfeersiosriornecitsusa simple, safe and effective method1f. o rItthiselandmark free surgery
cosmetic and+3functional treatment of 1hmormiztoon2tamlmdepovsiatetrioionr taonIRd iVnsepratitotnern with primary2i.n feEraiosirly gradable from 6-12 mm
oblique over+a2ction There are studies o3nmvmartioou4smimnfpeorsioterrioobr ltioqIuReinmseurtsicolne weakening proc3e. d uMreinsi.mal manipulation
However, no+n1e of them are full proo4f manmdpoesatecrhiocraasned 2hmasmtloatebreal toreIRatIendserdtieopnending up4.o n Mthusecle is always available for
situation. DVD and IOOA Full anteriorization reoperation and adjustments
CONTRAINDICATION
INFERIOR PERFORM 1. V pattern strabismus without IOOA
OBLIQUE SURGERY IF >+2 2. X pattern in patients with long
OVERACTION
GRADED +1 TO OVERACTION standing exotropia causing
+4 (V PATTERN overshoot in upgaze simulating
IOOA.
AND UPSHOOT) 3. Pseudo IOOA in craniofacial
disorders.
PRIMARY MORE 4. Restrictive pathologies like after
OVERACTION- EFFECTIVE retinal detachment surgery,
ANTERIORIZATI where IOOA may have associated
USUALLY incyclotorsion.
BILATERAL ON
REQUIRING COMPLICATIONS
BILATERAL There are several possible
SURGERY complications of inferior oblique surgery
that can occur.
EVEN IF IN AMBLYOPIA NO VERTICAL ASYMMETRIC IF 1. The most common may be recurrent
ASSYMETRIC DO MAY RESTRICT DEVIATION IN HYPERTROPIA
SURGERY TO IN PRIMARY or persistent inferior oblique
BILATERAL AFFECTED EYE- PRIMARY overaction, for which a secondary,
SURGERY EVEN POSITION- more profound weakening
IF MINIMAL TO MAY DO SYMMETRICAL
AVOID MONOCULAR SURGERY
UNMASKING SURGERY
Figure 2
6-0 polyglactin 910 sutures bridging the them are full proof and each case has to procedure is often performed.
cut ends of muscle (Figure 2). There are be treated depending upon the situation. Overcorrection, less common than
studies on various inferior oblique muscle undercorrection, may respond to
weakening procedures. However, none of IO will retract into its sheath and conversion of an inferior oblique
shall stay recessed according to amount
Dadeya S. Overview of Inferior Oblique Surgery www. dosonline.org 29
Review Article
transposition into a less profound, inferior oblique muscle but also modify, REFERENCES
traditional recession or alternatively quantitatively and qualitatively, the field
one of several superior oblique of action. A well-known example is the 1. Kamlesh, Dadeya S, Kohli V, Fatima S.
weakening procedures. limitation of elevation in abduction after Primary inferior oblique overaction-
2. Damage to vortex vein: the vortex anterior transposition and the concurring management by inferior oblique
vein is vulnerable in most inferior apparent inferior oblique overaction in recession. Indian J Ophthalmol
oblique recession and transposition the contralateral eye, or AES. Placing the 2002;50:97-101
procedures. Damaging the vortex new insertion that is less temporal, or
vein may result in excessive even nasal (as in the ANT procedure), Apt L, Call MB. Inferior oblique
hemorrhaging (bleeding). to the inferior rectus border avoids the recession. Am J Ophthalmol 1978;
3. Retrobulbar hemorrhages have been limitations of elevation in abduction. 85:95-100.
reported
4. Transient pupillary dilation As excyclorotation is the primary 2. Cooper EL, Sondall GS. Recession
action of the inferior oblique in its natural versus free myotomy at the insertion
DISCUSSION position, some surgical procedures of the inferior oblique muscle. J Pediatr
of the inferior oblique may alleviate Ophthalmol 1969; 6:6-10.
A wide variety of surgical procedures torsional deviations. Regardless whether
can weaken the action of the inferior the preoperative torsional deviation is 3. Ziffer AJ, Isenberg SJ, Elliott RL, Leonard
oblique muscle. However recession, the reason for surgery, the potential for APT. The effect of anterior transposition
anterior transposition and Hang Back torsional effects of any inferior oblique of the inferior oblique muscle. Am J
recession are commonly used. procedure warrants attention. Preferably, Ophthalmol 1993; 116:224-27.
torsional changes should also be
10 mm Inferior oblique recession is measured with the double-Maddox rod 4. Pradeep Sharma, MD, S Thanikachalam,
a simple, safe and effective method for test or fundus imaging, MD, Sachin Kedar, MD, and Rahul
the cosmetic and functional treatment of Bhola, MS. Evaluation of subjective
V pattern with primary inferior oblique Currently, there is good and objective cyclodeviation following
overaction. understanding of the mechanical oblique muscle weakening procedures.
functionality of the NFVB after inferior Indian J Ophthalmol. 2008; 56: 39–43.
Kamlesh et al concluded 10 mm oblique anterior transposition. However,
Inferior oblique recession by hang back not all aspects of the effects of inferior 5. Kumar K, MS, Hari Narayan Prasad, MS,
technique is a simple, safe and effective oblique muscle surgery can be explained Sumit Monga, MS, FRCS, Rahul Bhola,
method for the cosmetic and functional in terms of the geometry of the new MD’; Jaapos; 2008;12,4: 401–404
treatment of horizontal deviation and insertion. This, in turn, indicates that
V pattern with primary inferior oblique greater study is needed regarding the 6. Potter WS, Nelson LB. Hangback and
overaction response of connective tissue pulleys and Hemi-hangback recession techniques
pulley bands to inferior oblique surgery. in strabismus surgery. Ophthalmology
One challenge in understanding Clinics of North America 1922; 5:47-
(and thus predicting) the effects of 56.
these techniques is that most inferior
oblique procedures not only weaken the Correspondence to:
Dr. Subhash Dadeya
Director-Professor Ophthalmology, Guru Nanak
Eye Centre, Maulana Azad Medical College,
Delhi, India.
30 DOS Times - July-August 2018 Dadeya S. Overview of Inferior Oblique Surgery
Review Article
Stereovision in Strabismus
Dr. Jyoti Shakrawal MD, Dr. Rebika Dhiman MD, Dr. Rohit Saxena MD, PhD
Strabismus and Neuro-ophthalmology Services , Dr R P Centre for Ophthalmic Sciences,
All India Institute of Medical Sciences, New Delhi, India
Stereopsis is derived from the words ‘stereo’ meaning Figure 1: Panum’s Fusional Area along with an imaginary line
solid and ‘opsis’ meaning sight. Stereovision or connecting points corresponding to zero retinal image disparity,
binocular stereopsis is the perception of depth known as horopter.
after the transmission of visual information from
both the eyes. The range over which fusion has stereoacuity are listed in (Table 2) (Figure 2-8). The dissociation
occurred is known as the Panum’s Fusional Area. An of the two eyes is artificially induced, which forces the two eyes
imaginary line connecting points corresponding to zero retinal to fuse to form a single image of the object of interest. The
image disparity is known as a horopter. The left and right stereoacuity assessment has been made possible in infants and
retinal images are fused for objects located along the horopter children with the availability of special stereoacuity test cards,
and within Panum’s area. Beyond Panum’s area, either in front namely, Infant Randot stereoacuity cards, Randot preschool
or behind, diplopia occurs (Figure 1). Stereoacuity, which stereoacuity test, and the Distance Randot stereoacuity test.
is reciprocal of stereo threshold is the stereopsis that can be
resolved by minimal horizontal separation. Mean stereoacuity Esotropia (ET)
in a person with normal binocularity is around 20±10 arc sec.
Any impairment in stereopsis leads to poor quality of vision, Infantile and accommodative esotropias are the most
which can influence daily activities like difficulty in playing common forms of esodeviation seen in practice. Both are
sports or performing visuo-motor tasks, which in turns leads to associated with abnormal binocular functions and stereopsis.
limited career options in adults. Infantile ET has an onset at around 6 months of age, which is the
Stereoacuity develops during the first year of the life, critical period for the development of stereopsis. Studies have
followed by further improvement up to 18 months. It starts shown that the abnormal stereoacuity in infantile ET is due
from 3-4 months of age, and rapidly develops during next 4-12
months. The right and left ocular dominance columns develop
in harmony if normal visual input reaches from both eyes after
birth. Fine stereopsis is possible after development of both
ocular dominance columns. Lack of visual input by any eye
results in suppression of ocular dominance column of that side.
Normal alignment of two eyes with good vision, along with a
good cortical and ocular motor function is necessary for normal
stereovision. In strabismus, misalignment of the two eyes result
in formation of retinal images at non-corresponding locations,
which finally leads to abnormal stereovision.To restore normal
stereovision after strabismus surgery is always a challenge for
the strabismologists, especially in infantile and accommodative
esotropia. Achievement of stereoacuity after strabismus
surgery is usually associated with everlasting alignment of the
two eyes together and less risk of amblyopia.
Principle of stereotest Table 1: Various principles of dissociation used in stereoacuity testing
Stereoacuity for distance and near can Principle Details Examples
be tested in different ways. The dissociation
of the two eyes is artificially induced, Haploscopic Dissociation by using angled mirror Synaptophore
which forces the two eyes to fuse to form
a single image of the object of interest. The in front of each eye. Stereoscope
assessment of stereoacuity is based on the
following four principles of dissociation Anaglyph Dissociation by using coloured TNO test
(Table 1). Vectographic glasses
Titmus fly test
Abnormal stereopsis in Dissociation by using glasses with Randot stereopsis test
Strabismus polarized filters. It prevents from the
exasperation of seeing red-blue tints.
The commercially available tests for
Panographic Real depth stereogram which uses Lang’s test
cylinder or prisms. Frisby devis distance
stereotest (FD2 test)
Saxena R. et al. Stereovision in Strabismus www. dosonline.org 31
Review Article
Table 2: Near and distance streoacuity tests available in clinical settings. children with onset ≥24 months than in
those with early onset at 7-17 months.
Stereopsis test for near Principle Available disparities The strongest predictor of stereoacuity
(s of arc) outcome was the duration of constant
misalignment (>4 months). Intermittent
1. TNO (The Netherlands Organization) Anaglyph 480- 15 misalignment of <4 months duration had
test (Figure 2) Vectographic 3500- 40 a better outcomes5. Other studies have
found high AC/A ratio to be associated
2. Titmus fly test (Figure 3) with poor stereopsis6. This is because
cases with high AC/A ratios are difficult
3. Randot stereopsis test (Figure 4) Vectographic 660- 20 to treat, treatment is often delayed and
this leads to prolonged misalignment.
4. Lang’s test (Figure 5) Panographic 1200- 200
These observations go in favour of
5. Frisby devis near stereotest (Figure 6) Panographic 600- 5 prompt and aggressive management of
both infantile and accommodative ET to
6. Synaptophore Haploscopic 720- 90 restore binocular functions.
7. Lang 2 pencil test 5000- 3000 Exotropia (XT)
Stereopsis test for distance Intermittent exotropia is a common
exodeviation affecting nearly 1% of the
1. Frisby devis distance stereotest (FD2 Panographic 5- 50 population7. As the name suggests, it
test) (Figure 7) is intermittent to begin with that later
deteriorates into a constant XT. Distance
2. Distance randot test (Figure 8) Vectographic 400- 60 stereoacuity is the first to be affected8.
As the disease progresses, the deviation
3. AO-Vectographic Project-O-Chart Slide Vectographic 480- 30 starts to manifest for both distance as well
test near and this leads to disruption of near
stereoacuity as well. Sharma P et al, have
to prolonged misalignment rather than disruption by 3-4 months of age, it suggested a cut-off- 40 arc sec for near
being congenital in nature. In support of continues to be affected by abnormal and 400 arc sec for distance stereoacuity-
this hypothesis, normal stereoacuity has binocular experience even beyond 4 as an indication for prompting surgical
been documented in same proportion of years of age2. Abnormal stereopsis has intervention in intermittent XT9.
healthy infants and infantile esotropes at been noted in both intermittent as well
3-5 months of age. Decline in stereoacuity as constant accommodative ET. Despite Stereoacuity following
is noted later in life by prolonged the intermittency in deviation, 40% strabismus surgery
abnormal binocular feedback1. cases exhibit abnormal stereopsis in Esotropia (ET)
accommodative ET3. The stereodefects
Accomodative ET has a late in accommodative esotropia may be It is important to keep in mind
onset at around 18-48 months of age primary in nature; either genetic or pre- that strabismus surgery is not just a
and is commonly associated with existing before the onset of ET. Infact, cosmetic surgery. The ultimate target
hyperopic refractive error and/or hyperopia along with pre-existing after all strabismus surgery is to restore
abnormal accommodative convergence/ abnormal stereoacuity precipitate normal stereovision in addition to the
accommodation (AC/A) ratio. The accommodative ET4. binocular vision10. The presence or loss
deviation is intermittent in initial stage of stereovision is an important deciding
that gradually progresses to become A study by Fawcett S et al,5 has
constant. Despite late onset, abnormalities evaluated the effect of several factors
of binocular sensory functions are on stereoacuity in accommodative ET.
associated with accommodative ET. According to them, age of onset has
According to literature, although minimal effect on stereoacuity outcomes
stereoacuity is most susceptible to with better stereoacuity documented in
Figure 2: TNO test. Figure 3: Titmus Fly Stereotest.
32 DOS Times - July-August 2018 Saxena R. et al. Stereovision in Strabismus
Review Article
Figure 4: Near Randot Stereotest. Figure 5: Langs Stereotest.
Figure 6: Plexiglass plate for frisby David near Stereotest. Figure 7: Frisby David Distance Stereotest.
factor for the timing and outcomes of concluded that in infantile esotropia accommodative esotropia than infantile
the strabismus surgery. The ideal time to despite an early intervention and good esotropia due to following reasons: late
intervene for infantile ETis 4-6 months refractive correction only < 0.5% patients age of onset of accommodative esotropia
and for intermittent divergent strabismus gained normal stereoacuity by 5 years of (mostly after 18 months of age) and
is 4-6 years. Proper assessment of visual age. No stereoacuity was achieved in 60% presence of intermittency of deviation
acuity and stereoacuity should be done cases. This suggests that there are other early in the course of the disease. But
prior to any strabismus surgery by using factors that interplay and contribute to despite surgical alignment of strabismus,
various methods described earlier. poor stereoacuity outcomes in infantile stereo deficits persist in many cases. In
ET. These factors are inability of the a prospective study by Birch EE et al,12
In infantile and accommodative current surgical techniques to predict only 18% cases with accommodative ET
ET, several studies have been done to post-operative alignment and inability had normal Randot stereoacuity during
look for the outcomes of stereoacuity to achieve a post-operative residual a follow-up of 4-11 years. Rest had either
after strabismus surgery. An early deviation within 4-6pd in every case, subnormal or nil stereoacuity.
intervention and alignment within 8 which in turn affects the development
prism dioptre (pd) is considered essential of ocular dominance columns. Also, an Poor stereoacuity at immediate post-
for good stereovision. As mentioned intervention much earlier (2-3 months) operative period in both infantile and
earlier, duration of misalignment than the current recommendations may accommodative ET is a poor predictor. Nil
plays an important role in disruption be needed. stereoacuity in immediate post-operative
of stereoacuity in ET. Thus, an early period is associated with greater risk
intervention is expected to improve the Accommodative ET is mostly of permanent loss of stereoacuity
stereoacuity outcomes in such cases by managed with optical correction and and recurrence of deviation requiring
decreasing the duration of misalignment. surgical intervention may be needed resurgeries. Also, poor stereoacuity
However, the studies prove otherwise. in around 30% cases5. Stereoacuity is associated with greater risk of
A study published by Birch EE et al,11 outcomes are anticipated to be better in amblyopia13 and poor quality of life14.
Saxena R. et al. Stereovision in Strabismus www. dosonline.org 33
Review Article
2003;7:369–73.
4. Birch. Stereoacuity outcomes
following treatment of infantile and
accommodative esotropia. Optom Vis
Sci. 2009;86(6):647–52.
5. Fawcett S, Leffler J, Birch EE.
Factors influencing stereoacuity in
accommodative esotropia. JAAPOS
2004; 4:15-20.
6. Wilson ME, Bluestein EC,
Parks MM. Binocularity in
accommodativeesouopia. J
PediaaOphthalmolSuabismns 1993;
30:233-6.
7. Hutchinson AK. Intermittent
exotropia. OphthalmolClin North Am.
2001;14:399–406.
8. Stathacopoulos RA, Rosenbaum AL,
Zanoni D, Stager DR, McCall LC, Ziffer AJ,
et al. Distance stereoacuity: Assessing
control in intermittent exotropia.
Ophthalmology. 1993;100:495–500.
9. Sharma P, Singh R, Narvekar M, Gadia
R, Menon V. Evaluation of distance and
near stereoacuity and fusionalvergence
in intermittent exotropia. Indian J
Ophthalmol. 2008;56:121–5.
Figure 8: Distance Randot Stereotest. 10. Sharma P. Gaur N, Phuljhele S, Singh
R. What’s new for us in strabismus?
Indian J Ophthalmol 2017;65:184-90.
Exotropia (XT) operative stereoacuity improvement. 11. Birch EE, Fawcett S, Stager DR. Why
They also gave a cut-off of worsening of
Intermittent XT affects distance distance stereoacuity> 20 arc (FD2) as an does early surgical alignment improve
stereoacuity more than the near indication for surgery.
stereoacuity15. Stereoacuity assessment stereoacuity outcomesin infantile
is now used as an objective clinical Conclusion
method to look for the progression in esotropia? J AAPOS 2000;4:10–4.
intermittent XT. There are no specific Rapid stereoacuity development 12. Birch E, Stager Sr. D, Berry P, Leffler
guidelines concerning the timing for is seen in first two years of life. Any
surgical intervention. Various studies interruption of binocular input during J. Stereopsis and long-termstability
have been done to look for the change in this period leads to subnormal stereopsis.
stereoacuity after strabismus surgery in Both early-onset esotropia as well as of alignment in esotropia. J AAPOS;
intermittent exotropia. In a prospective exotropia are associated with stereo
study of 31 intermittent XT followed up deficits. A major contributing factor is 2004;8:146-50.
for 6 months by Sharma et al9 distance the greater duration of misalignment.
as well as near stereoacuity showed Therefore, early management of 13. Bosworth R, Birch E. Binocular
significant improvement after surgery. strabismus (optical or surgical) is
Distance steroacuity improved in considered indigenous to successful function and optotype-grating acuity
around 87% patients, while no change restoration of binocular sensory status.
was noted in 6.5% cases. Worsening of However, studies reveal that despite early discrepancies in amblyopicchildren.
stereoacuity was seen in 2 cases who intervention recovery of stereoacuity
developed consecutive esotropia. The remains a challenge, especially due to Invest Ophthalmol Vis Sci
percentage of patients with poor near the role several other factors influencing
stereoacuity (<40 arc sec) improved from its outcome. Better pre-operative 2003;43E-Abstract 3183
81% to 42% post-operatively. In another stereoacuity is predictor of good post-
study of 30 cases of intermittent XT by operative stereoacuity outcomes with 14. Menon V, Saha J, Tandon R, Mehta M,
Singh et al,16 distance as well as near lower rate of recurrent deviations.
stereoacuity improved significantly from Khokhar S. Study of the psychosocial
50 s of arc to 17.5 s of arc, and 240 s of References
arc to 90 s of arc respectively 6 months aspects of strabismus.J Pediatr
following surgery. They concluded that 1. Birch EE, Stager DR. Monocular acuity
pre-operative stereoacuity of the patient and stereopsis in infantileesotropia. Ophthalmol Strabismus 2002;39:203–
is an important predictor of stereoacuity Invest Ophthalmol Vis Sci
outcomes. In a prospective case control 1985;26:1624-30. 8.
study, Singh et al15. reported that a
distance stereoacuity worse than 70 s of 2. Birch EE. Binocular sensory outcomes 15. Singh A, Sharma P, Singh D, Singh R,
arc (FD2) is poor prognostic sign for post- in accommodative ET (Marshall Parks
Lecture). JAAPOS 2003; 7: 369-73. Sharma A, Menon V. Evaluation of FD2
3. Birch EE. Binocular sensory function (Frisby Davis distance) stereotest in
in accommodative esotropia. J AAPOS
surgical management of intermittent
exotropia. Br J Ophthalmol.
2013;97:1318–21.
16. Singh R, Kakkar A, Menon V, Sharma
P, Phuljhele S. Evaluation of factors
influencing distance stereoacuity on
Frisby-Davis distance test (FD2) in
intermittent exotropia. Br J Ophthalmol.
2011;95:1098–101.
Correspondence to:
Dr. Rohit Saxena
Professor of Ophthalmology
Dr. R.P. Centre for Ophthalmic Sciences
All India Institute of Medical Sciences
AnsariNagar, New Delhi, India.
34 DOS Times - July-August 2018 Saxena R. et al. Stereovision in Strabismus
Perspective
Failed Conventional Probing or Persistent
CNLDO- what next?
Dr. Manpreet Singh MS, DNB, FAICO, Dr. Manpreet Kaur MS, Dr. Manjula Sharma DNB,
Dr. Pankaj Gupta MS
Department of Ophthalmology, Advanced Eye Centre, Post Graduate Institute of Medical Education and
Research, Chandigarh, India
The incidence of congenital nasolacrimal duct Figure 1: (Left) Nasal endoscopy guided lacrimal probing after the
obstruction (CNLDO) ranges from 5%-20%, and medialization of inferior turbinate (IT). The lateral nasal wall, floor and
this condition usually resolves by 3-4 weeks IT is labelled.
after birth due to the negative pressure created
by crying, suckling and nasal breathing by the dilatation of the NLD.
neonate. Before one year of age, the CNLDO Advantages: The nasal endoscopy provides visualization
can be managed with conservative treatment like lacrimal
sac compressions with or without antibiotic eyedrops. After of the tip of the lacrimal probe, hence prevents the formation of
one year of age, these children are commonly subjected to a a false passage and even it is formed, it helps in the redirection
conventional probing at most of the places, but in few centers, of the probe to its desired location. The nasal endoscopy also
the primary/ first probing is performed under the nasal provides the information about the nature of IT and width of
endoscopic guidance. The primary probing provides >90% the inferior meatus.
success rate making it a reasonably satisfying procedure to
perform but in few, the conventional probing fails, or the Disadvantages: Require specialized skills, learning curve,
CNLDO persists. and expensive equipment.
Definition of persistent CNLDO or failed probing1: If the Success rate- 80-95%.
symptoms of CNLDO recur within 6 weeks of primary probing,
the condition is called as persistent CNLDO or failed probing. Balloon catheter dacryoplasty (BCD)3
The treatment options for a persistent CNLDO are: This is an effective treatment option for persistent CNLDO
1. Nasal endoscopy guided lacrimal probing ± inferior or as a primary procedure in children older than 48 months.
Lacrimal probing alone is much less effective in children with
turbinate medialisation stenosis, obstructions, or diffuse narrowing of the nasolacrimal
2. Balloon catheter dacryoplasty/ dilatation (BCD) of duct proximal to the level of the valve of Hasner. The BCD of
the NLD achieves true dilatation, is technically much easier
nasolacrimal duct (NLD)
3. Lacrimal intubation with silicone stents (mono or
bicanalicular)
4. Dacryocystorhinostomy (DCR)- external or endonasal
Nasal endoscopy guided lacrimal probing ±
inferior turbinate medialisation2
Procedure: A 2.7 mm pediatric Hopkins II telescope of 0°
or 30° is used for the visualization of the inferior turbinate (IT),
meatus and the floor of the nose. The Bowman’s lacrimal probe
is passed similarly as in the conventional probing, and the tip of
the probe is observed to appear from the inferior meatus region
(Figure 1).
In case of an impacted turbinate, where there is no space
between the lateral nasal wall and IT or the inferior meatus
is very narrow, the IT is medialized by passing a periosteum
elevator between the two structures and pulling it into the
nasal cavity, thereby enlarging the inferior meatus. This allows
the 2.7 mm telescope to go into the inferior meatus and observe
for the opening of NLD. The mucosa surrounding the inferior
meatus is preferably decongested for better visualization and
minimizes the bleeding. A few surgeons also prefer a serial
Singh M et al. Management of Failed Probing... www. dos-times.org 35
Perspective
Figure 2: (Left) The knot of bicanalicular silicone lacrimal stent in Figure 3: The loop of bicanalicular silicone lacrimal stent (black arrows
inferior meatus (IM). highlighting puncta). Note adequate length of loop to prevent punctum
cheese-wiring.
to perform than silicone intubation and (>30 months of age). The deflated intubation (with Masterka, Monoka-
balloon catheter (coated externally with
obviates the need to remove the tube or a viscoelastic) is pushed through the Crawford) and bicanalicular intubation
upper punctum, canaliculus, lacrimal sac,
concerns about the child pulling out the and down the nasolacrimal duct into the are comparable. The advantage of the
nasal cavity. Once in position, the end is
tube. A high success rate can be achieved connected to the inflation device. The monocanalicular stent is the easy removal
inflation is performed twice for 90 and
with BCD in cases of failed probings and 60 seconds sequentially, at a pressure under topical anesthesia in the clinic
of 8 atmospheres. At first, the distal
failed silicone intubation, as well. Hence nasolacrimal duct is dilated followed by without the need of sedation or general
the deflation of the balloon catheter and
the indications for BCD would be: pulling it proximally towards the lacrimal anesthesia.
sac-proximal NLD junction. The success
1. Failed probing at any age. rate of BCD can be upto 75% (age 24-48 Procedure: After surgical
months) and 82% (12–24 months).
2. Failed silicone intubation at any age. preparation, both upper and lower
Advantages: relatively non-invasive,
3. As a primary procedure in children single-step procedure, no need for the lacrimal puncta are gently dilated. After
additional procedure (stent removal etc.)
with trisomy 21 at any age. ensuring an adequate nasal decongestion,
Disadvantages: require specialized
Children with trisomy 21 have skills, availability, learning curve, the desired size of Bowman’s lacrimal
expensive equipment.
an inadequate response to probing or probe is passed, and its tip is visualized
Success rate- 75-100%.
silicone intubation. Silicone intubation with nasal endoscopy in the inferior
Lacrimal intubation with
should be reserved for patients silicone stents (mono or meatus region. The Bowman’s lacrimal
bicanalicular)4
with canalicular stenosis or used in probe is then removed, and the metal
Silicone intubation of the lacrimal
combination with BCD in patients who drainage system has been used for bodkin of Crawford’s lacrimal probe
the children having persistent CNLDO
have failed a primary BCD. or are older (>48 months of age) at is passed from either of punctum. The
presentation. The intubation system
Procedure: After surgical can be bicanalicular (common) or passage of the olive tip of the metal
monocanalicular. In both, the end of the bodkin requires a sufficiently dilated
preparation, the superior punctum silicone stent is retrieved from the nose
(inferior meatus), and one needs to keep
is gently dilated. After ensuring an the stent in-situ for 3 months. lacrimal punctum. The further passage
adequate nasal decongestion, the desired The outcomes of monocanalicular of bodkin is similarly as of a lacrimal
size Bowman probe is used to perform probe. Once the olive tip is visualized in
conventional probing. The tip of the the inferior meatus, Crawford’s hook is
probe can be directly visualized with an passed, and the olive tip is hooked safely.
endoscope or is indirectly confirmed with
Now the metal bodkin is pulled out of the
a metal-to-metal touch. This is to ensure nasal cavity. Now, the other metal bodkin
that the probe has perforated through is passed similarly and retrieved from the
all the obstructions and has entered the nasal cavity. The whole lacrimal tract is
nose. The probe is then removed and the now intubated with the silicone stents.
probe of the balloon catheter is placed in The metal bodkins are detached from
precisely similar fashion. the silicone stents, and the stents are tied
The lacrimal balloon catheter is a with each other, and the knot is placed in
cylindrical balloon along the length of the the inferior meatus (Figure 2).
probe making its handling and feels like a Care is taken to keep the stent loop
Bowman probe. It is available in 2 sizes- loose enough to prevent the cheese-
a 2-mm balloon catheter (≤ 30 months
wiring of the puncta. This is done by
of age) and a 3-mm balloon catheter opening the eyelids apart at the end of the
36 DOS Times - July-August 2018 Singh M et al. Management of Failed Probing...
Perspective
procedure (Figure 3). 2. The bones are softer than adults, Nasolacrimal Duct Obstruction: A
Advantages: failed BCD, canalicular ethmoid sinuses are not fully Report by the American Academy of
developed, and nasal cavity is of Ophthalmology. Ophthalmology. 2018
stenosis, cheaper than BCD, provide smaller size. Jun 7. pii: S0161-6420(18)31192-8.
constant dilatation of NLD. doi: 10.1016/j.ophtha.2018.05.005.
3. The skull base is lower, hence the 4. Eshraghi B, Jamshidian-Tehrani
Disadvantages: premature stent superior edge of bony osteotomy M, Mirmohammadsadeghi A.
prolapse, extrusion or loss, punctum should be strictly by the opening Comparison of the success rate
cheese wiring, nasal or conjunctival of the common internal opening of between monocanalicular and
mucosal granuloma, need for removal common canaliculus in the sac. bicanalicular intubations in incomplete
(under GA- bicanalicular) complex congenital nasolacrimal
4. The overall size of the sac is small duct obstruction. Orbit. 2017
Success rate- 80-98%. and intraoperative bleeding is crucial Aug;36(4):215-217
The intraoperative use of 5. Quaranta-Leoni FM, Verrilli S, Leonardi
Dacryocystorhinostomy A. Surgical outcome and unusual
(DCR)5,6 mitomycin-C and lacrimal intubation is complications of paediatric external
helpful in improving the overall success dacryocystorhinostomy. Orbit. 2018 Jul
This procedure is considered as the rate of the procedure. 30:1-5.
final surgical procedure for the persistent 6. Bothra N, Naik MN, Ali MJ. Outcomes
CNLDO patients in whom the lacrimal Bibliography in pediatric powered endoscopic
sac is opened up into the nasal cavity, in dacryocystorhinostomy: a single-
the middle turbinate region via a bony 1. Kamal S, Ali MJ, Gauba V, Qasem center experience. Orbit. 2018 May
osteotomy. This procedure has a success Q. Congenital nasolacrimal duct 22:1-5.
rate in pediatric age group- external obstruction. M. Javed Ali (ed.),
(93.5%) and endoscopic endonasal Principles and Practice of Lacrimal Correspondence to:
(95.6%). This is advisably performed in Surgery, pp117-131. Dr. Manpreet Singh
patients after the BCD and nasolacrimal Department of Ophthalmology,
duct intubation. 2. Galindo-Ferreiro A, Akaishi P, Cruz A, Advanced Eye Centre, Post Graduate Institute of
Khandekar R, Dossari S, Dufaileej M, Medical Education and Research, Chandigarh,
Critical points in pediatric DCR Galvez-Ruiz A, Schellini S. Success Rates India
1. The postoperative fibrosis is a of Conventional Versus Endoscope-
Assisted Probing for Congenital
crucial feature in the healing of Nasolacrimal Duct Obstruction in
the nasal mucosa and lacrimal sac Children 12 Years and Younger. J
mucosa leading to the formation of a Pediatr Ophthalmol Strabismus. 2016
functional ostium Sep 1;53:292-9.
3. Wladis EJ, Aakalu VK, Yen MT, Bilyk
JR, Sobel RK, Mawn LA. Balloon
Dacryoplasty for Congenital
Singh M et al. Management of Failed Probing... www. dos-times.org 37
Perspective
Pediatric Cataract Surgery:What Roles do Study
Methodology and Surgical Techniques Play?
Dr. Sumit Monga
Consultant, Pediatric Ophthalmology, Strabismus and Neuro-Ophthalmology Service,
Centre for Sight group of Eye Hospitals, Delhi NCR, India
The advances in technology and refinements in Opacification could be a primary response of the anterior
surgical techniques, over past two decades, have vitreous face when it occurs with the IOL optic rather than a
catapulted pediatric cataract surgery into a new secondary scaffold response caused by proliferating LECs,
era. This has led to vastly improved surgical and inflammatory cells, and exudate deposits.
functional outcomes. Various innovative studies
have contributed to the current understanding As per the current understanding, one can make the
and evolution of surgical techniques that have helped to partly following recommendations. In children younger than 5 years,
overcome the challenges of pediatric cataract management1. PCCC with anterior vitrectomy is advisable. Posterior CCC
However, pediatric cataract surgery has been associated with without anterior vitrectomy may be considered in children
many complex issues that have been debated in literature. between 5 years and 7 years. In older children, maintaining
We believe that possibly the information on key aspects of intact posterior capsules is advised.
pediatric cataract surgery may have been influenced by study
methodologies and surgical techniques that have been reported Primary IOL Implantation in Children
in the literature.
IOLs are being used increasingly for the optical correction
Surgical Technique of aphakia in infants following cataract surgery. In children
older than 2 years of age, primary IOL implantation of foldable
Pediatric cataract surgery has evolved from the procedure Acrylic IOL is the current standard of care. There is growing
of discussion and aspiration in 1930’s, lensectomy in 1970’s, evidence in literature to support the use of primary intraocular
extra-capsular cataract extraction in 1980s to the present lens implantation in children less than 2 years of age4-12.
surgical technique of anterior continuous capsulorrhexis While the surgical technique has mostly remained similar for
with phaco-aspiration of lens matter with primary posterior all studies, the study methodology was variable. Most of the
capsulorrhexis with limited anterior vitrectomy2. reports are retrospective, non-comparative series while others
have compared the outcomes of primary IOL implantation
The use of mechanized vitrectomy instrumentation to with the group receiving contact lens for visual rehabilitation.
selectively perform a primary posterior capsulectomy and The number of patients in these series failed to provide the
vitrectomy combined with IOL implantation resulted in statistical power necessary to adequately assess the outcomes
decreasing the scourge of visual axis opacification and has led of IOL implantation. Few studies depicted in Table 1 are
to fewer reoperations in younger children. In the literature, the prospective, out of only one is a randomized trial comparing
rate of PCO is up to 100% when the posterior capsule remains outcomes in the IOL versus contact lens group16. The reporting
intact. There is a strong relationship between age and incidence of results in the retrospective and non-randomized studies
of PCO. The rate of PCO membranes is high in young children, could have been marred by fallacies of inadequate sample size
reflecting greater tissue reactivity of lens epithelial cells (LECs). and lack of standardized protocols.
When primary posterior capsulectomy is not combined with
anterior vitrectomy, the incidence of posterior capsule closure The primary outcome measure in majority of these studies
is up to 60%. The main reason for the occurrence of VAO after has been the complication rate and overall safety profile of
performing a PCCC could be the increased LEC activity and IOLs in young children. Visual axis opacification has been the
also the presence of an intact anterior vitreous face, which most frequent complication in all studies, but its incidence
acts as a scaffold for LEC migration. Reported rates of VAO rate has differed between them. One glaring explanation could
after vitrectomy are less than 6%2. The importance of anterior be the variable follow up, as posterior capsular opacification
vitrectomy in pediatric surgery is emphasized in in cataract (PCO) can have a delayed onset. Particularly, the use of Acrylic
surgery in patients younger than 7 years by several authors.In foldable IOLs has been implicated with delayed and milder
a study, the technique of optic capture through the posterior variety of PCO3.
capsulorhexis has been shown to prevent PCO. However,
Vasavada et al found that optic capture without anterior Many studies, at the start of the decade, had some cases in
vitrectomy did not always ensure a clear visual axis. Eyes with which Polymethyl- methacrylate (PMMA) IOLs were implanted.
an obscured visual axis had reticular fibrosis of the anterior Research has conclusively established that IOL material and
vitreous face in the first 2 months after surgery. Hence, vitreous design has a direct influence on PCO rates. Acrylic foldable
IOLs have been documented lesser PCO rates and severity of
PCO compared to PMMA and silicone foldable IOLs. This trend
Monga S. Pediatric Cataract Surgery: What Roles do Study Methodology... www. dos-times.org 39
Perspective
Table 1: Studies on Primary IOL implantation in Children less than 2 years of age: Methodology
Authors, Type Laterality Coexisting N (No. of Age Mean FU Type of IOL Type
of Ocular / eyes) Group power of IOL
Year of of Study Cataract Systemic (Wks) (mo) IOL Calc Fixation
Pathology formula
Publication Bag
Lambert SR Prospective, UL Excluded 11 2-22 13±6 PMMA Holladay Bag
etal, 1999 Non- (5) SRK II
randomized AcrySof or I Bag
(6)
Bag
Lambert SR, Prospective, UL Excluded 12 3-22 - PMMA Holladay (86.2%)
2001 non- (6) I or SRK Sulcus
randomized AcrySof II (13.6%)
(6) Bag
Plager D Retrospective, UL,BL Included 15 3-20 NA AcrySof 3 SRK II or NA
etal, 2002 Comparative
piece SRK-T Bag,
Sulcus
Trivedi RH, Retrospective UL,BL Included 29 0.8-43.2 33.4 ± AcrySof Holladay
2004 16.1 single Bag
months piece (93%),
Sulcus
Lundvall A, Retrospective UL Included 28 1-40 36 HSM- NA (7%)
2006 Included 25 PMMA, Bag,
Excluded 45 AcrySof SRK-T Sulcus
Ashworth JL, Retrospective UL,BL Excluded 57 1-51 44.36 NA NA
2007 UL,BL 3 - 104 months
HSM-
Ram J etal, Prospective, UL 18± 9.3 PMMA
2007 non- mo
randomized
IATS, 2010 Prospective, 4-24 12 AcrySof Holladay
randomized, I
Comparative
Gupta A etal, Retrospective, UL,BL Excluded 120 1-23 mo 8.856 PMMA SRK II
± 7.73 (30),
2011 non- months AcrySof
(90)
comparative
has been corroborated by demonstrating reasonably high10. Clearly, there are cataracts. It would have been useful if the
some undetermined factors, particularly authors had clarified whether there was
increased biocompatibility and improved in infants, which may lead to visual axis any difference in between the two groups
opacification.
IOL design of Acrylic foldable IOLs. (IOL vs. Aphakia group), with respect to
We believe that certain aspects of
Further, placement of in-the-bag has methodology, practiced in this study, presence of residual lens matter, capsular
could have affected the outcomes. In a
been shown to be associated with lesser previous study, similar visual outcomes contraction rates, posterior synechiae,
postoperative inflammation and PCO and complication rates were reported
rates. Table 1 reveals that the information with the limbal and pars plana approaches IOL deposits or IOL displacement in their
on type of fixation of IOLs either in sulcus for doing PPC in children (mean patient series. All of these can directly influence
or bag, either has been missed or the exact age: 6.3 years ± 2SD). But the latter the rate of AEs and the need for AIS in
number of cases with each type of fixation approach would be more challenging in
has not been mentioned. Moreover, the infants in view of inadequately developed the postoperative period. Additionally,
pars plana. Moreover, the limbal
presence of associated ocular anamolies, approach for PPC also obviates the need the authors’ postoperative protocol
for raising a conjunctival flap. This may
like persistent fetal vasculature, in almost seem more relevant, especially in view included instilling topical steroids four
half of the studies could have influenced of the need for conjunctival preservation
the VAO rates. Hence, the differential for any future glaucoma filtering surgery times a day, which does not seem to
reporting of visual axis opacification in these patients. Use of a vitrector for
and re-operation rates in various studies cortical clean up, as has been used in this corroborate with the amount of expected
study, may be challenging in congenital postoperative inflammation in infants. In
could partly be explained by the above- our experience, topical steroids instilled
mentioned factors. It is worth noting that 8-10 times/day and tapered over 6-8
in the recent study by IATS group, despite weeks provides satisfactory outcomes.
following meticulous surgical protocols The authors’ view that “surgery to
based on current knowledge, the re- remove the re-proliferated lens epithelial
cells is straightforward” appears to be
operation rates in the IOL group were technically true, but the psychological
trauma that the parents and the young
40 DOS Times - July-August 2018 Monga S. Pediatric Cataract Surgery: What Roles do Study Methodology...
Perspective
Table 2: Studies on Primary IOL implantation in Children less than 2 years of age: Results
Authors, Year of Outcome Measures Conclusion Reoperation
Publication Rates
Lambert SR etal4,1999 Adverse events, Myopic Shift Increased adverse events, large myopic shift 72%
Lambert SR5,2001 Grating Visual acuity at Improved visual outcome compared to CL 83%
conclusion of study
Plager D etal6, 2002 Complication rate VAO rate=80%; Higher reoperation rates 80%
Trivedi RH7,2004 37.9%
Visual Axis Opacification(VAO) VAO rate= 37.9%, Greater VAO with associated
ocular Anamolies
Lundvall A8,2006 Complications and visual VAO rate=67%; Better visual outcomes in BL 70%
results cases compared to UL cases
Ashworth JL9,2007 Refractive Outcome Satisfactory mean refractive outcomes, but wide -
Ram J etal10,2007 Safety profile of IOLs range of errors
VAO rate 13.3%, No major refractive surprises 28%
IATS11, 2010 Grating visual acuity at 1 year Similar visual outcome in IOL and CL ; VAO rate 63%
Gupta A etal12, 2011 of age, Adverse events 72%, Higher reoperation rates in IOL group NA
Safety profile of IOLs VAO rate (6.7%); adverse events comparable in
age group < 6 months and beyond
child undergo with an additional surgery Going forward, the safety profile and as likely to overcorrect as undercorrect.
cannot be overlooked. The limited better visual outcomes of primary IOL This may be explained by the higher
data available from other retrospective in young children needs to validated by proportion of short eyes (AL < 22 mm; 69
studies, evaluating the safety profiles of randomized controlled trials, spread eyes) in their series, and because Hoffer Q
primary IOL implantation in infantile across different countries. was formulated for shorter eyes.
subset of patients, revealed much less re-
operation rates. IOL power calculation and A potential source of error in IOL
Myopic Shift power selection in children than adults
An attempt has been made to is inaccuracy of axial length and/or
document the visual outcomes of primary Other issues that have received keratometry power measurements. Most
IOL implantation in monocular aphakia attention, in literature, are the need studies have either used applanation or
in infancy, first by a pilot study and then for accuracy in post-operative target immersion technique for axial length
by a randomized clinical trial by the refraction and trend of emmetropisation calculation under general anesthesia.
Infant Aphakia treatment study group. of refractive errors. The need for Applanation technique is thought
The results of the latter study, at 1 year of pediatric IOL calculation formulae has to artificially reduce the AL and can
age, showed that there was no significant been felt for long time, primarily because contribute to the significant amount
difference between the median visual all available formulas were derived from of additional error, especially for high
acuity in the treated eyes with IOL and considerations regarding the adult eye. powered IOLs. Additionally, most studies
the CL group after cataract surgery Unlike adults, pediatric eyes undergo except one failed to mention whether
during the first 6 months of life. This rapid growth and significant refractive they tried to minimize the inter-observer
was in contrast to the pilot study, where changes in the early years. Moreover, variability, by deploying single observer
visual outcomes in the IOL group were in most pediatric cases, the desired for biometry and refractive error
found to be better than the CL group. The postoperative refraction is different measurements.
authors of IATS study reason that the from Plano. All these factors add to the
discrepancy could probably be explained complexity of the IOL power calculations With the trend towards implanting
by the fact that the subjects in the pilot in children. IOLs in infants with shorter ALs (Table
study were comparatively older at time 1), there is a felt need to understand the
of evaluation of grating visual acuity and Various IOL formulae designed for accuracy and the differences between
that all children could not be assessed adult eyes are being used to predict IOL prediction formulas at the lower extremes
for visual acuity. Moreover, in the IATS power in pediatric eyes, which have of axial length and higher keratometry
study, standardized protocols for testing shown varying degrees of accuracy14,15. values. While in few studies, the age
visual acuity, by a masked examiner, Neely et al found that the SRK II stratification of younger children was not
were followed for testing all the included regression formula gave the least amount done, in others an attempt was made to
subjects. of variability, whereas the Hoffer Q gave dichotomize children according to the
the greatest amount, particularly among age at the time of surgery. As most of the
Hence, although IOL implantation the youngest group of children with AL ocular growth occurs during the first 2
has the advantage of providing at least a <19 mm. In a recent study, Nihalini B R years of life, most studies have taken the
partial optical correction at all times, this etal concluded for eyes with significant same as the cut off for grouping children.
advantage must be balanced against the deviation in prediction error (>0.5 D), In two major studies ,the number of eyes
reported high complication rate in the there was usually an undercorrection, in < 2 year age group constituted about
eyes of infants undergoing such surgery. except with Hoffer Q, which was almost one-fifth of the total, the percentages
Monga S. Pediatric Cataract Surgery: What Roles do Study Methodology... www. dos-times.org 41
Perspective
being 22.7% in the study by Neely etal and reporting of myopic shifts, in various children, depending on the population
16.2% in the study by Nihalini B R etal15. studies has been the variable timing of
In other studies, the cut off for ‘younger the initial refraction and the length of studied and the length of follow-up.
children’ varies between 12 months, 18 follow up period. The initial refraction
months and 36 months18. This disparity may vary from 2 weeks to 12 weeks, and Initial reports by Chandler, Phelps and
and fewer number of cases, limits our the mean follow up has ranged from 1 to Arafat drew attention to this problem20.
understanding about refractive outcomes 7 years17. This has led to disagreement on
in the younger, especially the infantile age the amount of myopic shift, in different Magnusson etal studied swedish children
group. However, these studies concur that largely retrospective studies. Further the
there is a greater variability of refractive heterogeneity of study groups, in terms of born with cataracts and found a 12%
outcomes, post cataract surgery, in age groups and number of eyes included,
younger children compared to the older may be a determining factor. incidence of aphakic glaucoma (mean
children. In the work by Eibschitz et al16, follow up 9.6 years)21. This figure is
an analytical comparison of predicted It has been reported that probably more reliable since the study
implant power using in the pediatric pseudophakic eyes have tendency to was conducted within the confines of a
range of axial length and keratometry grow longer than the fellow phakic eyes. fixed geographic location with centralized
values was performed. Significant Interestingly, in pseudophakic eyes data collection. In the Infant Aphakia
differences in intraocular lens power apart from the normal process of axial
prediction were found among the Hoffer elongation with age, there can possibly treatment study, preliminary results
Q, Holladay I, and SRK II formulas. This be factors like secondary glaucoma or
explains the higher degree of prediction amblyopic visual deprivation which showed that glaucoma developed in 5%
error that was documented in the age could influence the amount of myopic
group <2 years, in two recent studies. shift. Further, there could be other eyes in the contact lens group and 12%
undetermined reasons, including genetic
It has been reported there is a factors that could contribute to the eyes in the IOL group. But the follow up
trend towards larger postoperative myopic shift. In the study by Greiner etal,
prediction errors in younger children, the axial elongation in pseudophakic eyes was too short to derive a true incidence.
compared to adults. The refractive was reduced in comparison to the phakic
outcome of pediatric patients depends eye. It was worth noting that all eyes in Various factors have been postulated
on the assumed anterior chamber depth this study had sulcus fixation of IOLs, to influence the risk of developing
(ACD), effective lens position of IOL and compared to in-the-bag implantation postoperative glaucoma viz, age at
the effects of axial displacement of IOL. in most other studies. However, the
The effect is even more pronounced in exact mechanism of sulcus fixation detection of cataract, age at cataract
pediatric eyes as these short eyes require contributing to the myopic shift needs
higher power IOLs. The standardized to evaluated. Further, an interesting surgery, cataract surgery procedure,
assumptions about ACD and vaulting proposition was made by Nischal KK etal,
characteristics of an IOL within the bag who wondered whether the reduction primary intraocular lens implantation,
may not be accurate for pediatric eyes of peripheral visual input by anterior significant postoperative uveitis, and
owing to shallow anterior chambers and capsular fibrosis in pseudophakic eyes microphthalmia22. However, such
the particular postoperative dynamics of might explain the refractive and axial
posterior capsule contraction, vitreous length change following pediatric cataract information is available from reports of
pressure, haptic angulation, effects of surgery. It is possible that with improved
primary posterior capsulotomy and understanding of IOL power calculation selected individual case series, which may
vitrectomy on lens position and, later, the and prediction of post-operative
sometimes significant re-proliferation refraction in younger children improved be subject to bias and confounding. The
of lens material. So it is imperative results may be achieved in future studies.
that studies, evaluating IOL calculation data, procured from British Congenital
formulae in children, should include Glaucoma
information on these parameters so as Cataract Study, was an attempt to derive
facilitate better comparison between Secondary glaucoma is one of the results from population based research23.
them. most vexing problems after congenital Its finding suggested that detection of
cataract surgery. It can emerge years later cataract was the only significant factor
It has been documented that axial and can jeopardize vision. Some earlier associated with the development of
elongation of the globe in a pseudophakic studies had studied children and eyes with
eye leads to myopic shift, akin to what a co-existence of conditions associated glaucoma after surgery for congenital
happens in normally developing eyes13. with cataracts that will also develop
Younger children are more prone to glaucoma, such as Lowe’s syndrome, cataract. It is imperative to believe that
have larger and more unpredictable aniridia, and trauma19. However, more
myopic shifts. Further, the myopic shifts useful information could be attained only early detection of cataract would directly
have been shown to be variable and after studying “uncomplicated” cases of
no consistent correlations with pre- surgical aphakia. There is a wide variation translate to early age of cataract surgery.
operative axial length or IOL power have in the reported incidence of glaucoma,
been found. after congenital cataract extraction. Literature review points out to a
It ranges between 6% and 58.7% of
One basic discrepancy, in the bimodal pattern of onset for aphakic
glaucoma. The first onset peak is
noticed within the first weeks to months
following cataract surgery. This early-
onset glaucoma is frequently associated
with pupillary block, shallowing of the
anterior chamber, and angle closure.
It is well known that smaller eyes and
eyes with reduced corneal diameter are
more prone to develop angle closure
in postoperative period. However, the
unavailability of data about preoperative
gonioscopic findings, in cases undergoing
pediatric cataract extraction, limits our
understanding about the pre-existing
state of the angle and its predisposition
to insult in the postoperative period.
Residual lens material contributes to the
development of glaucoma by forming
Elschnig pearls that may cause pupillary
block and induce inflammatory adhesions
in the angle and at the pupil edge which
can cause pupillary block and angle
42 DOS Times - July-August 2018 Monga S. Pediatric Cataract Surgery: What Roles do Study Methodology...
Perspective
closure. It has been observed that sulcus- facility of outflow of the eyes. Clearly, lensectomy, and posterior chamber
fixation of the IOL is a risk factor for the there are some undetermined genetic and lens implantation in children: Limbal
development of pupillary block. But, it is versus pars plana. J Cataract Refract Surg
notable that most studies reporting the mechanical factors that may contribute 1999;25:768–75.
incidence of glaucoma following pediatric 12. Gupta A, Kekunnaya R, Ramappa M,
primary IOL implantation have failed to the development of glaucoma after Vaddavalli PK . Safety profile of primary
to specify the differentiation of in-the- intraocular lens implantation in children
bag versus sulcus fixation. The possible pediatric cataract surgery. below 2 years of age. Br J Ophthalmol 2011;
mechanism of glaucoma can occur due to 95:477-480.
the pupillary block leading to extensive On one hand, early cataract surgery 13. Ram J, Brar GS, Kaushik S etal. Primary
synechial closure or the forward intraocular lens implantation in the first
displacement of iris tissue by the haptics has been advocated to prevent amblyopia two years of life: safety profile and visual
of sulcus fixated IOL causing crowding of results. Indian J Ophthalmol 2007;55:185-
structures in the angle. and on the other hand, the possibility of 9.
14. Eibschitz-Tsimhoni M, Archer S M and
The age of onset of delayed-onset, increased complication rate after early Del Monte M A. Intraocular Lens Power
open-angle glaucoma may occur years Calculation in Children. Surv Ophthalmol
following the cataract. Because of the cataract surgery looms large. This leads 2007;52:474-82.
late onset occurring five or more years 15. Neely D E, Plager D A, Borger S M and
following the cataract surgery, it is to the dilemma, for which there are no Golub R L. Accuracy of Intraocular Lens
not likely that inflammation, use of Calculations in Infants and Children
postoperative corticosteroids, or any clear answers. At present, the clinician Undergoing Cataract Surgery. J AAPOS
other portion of the cataract surgery is 2005; 9:160-65.
the cause of this problem. Asrani etal, in may be best advised to do a balancing 16. Nihalani B R, VanderVeen D K. Comparison
a meta-analysis of 377 eyes with primary of Intraocular Lens Power Calculation
lens implantation, found only one eye act of performing early cataract surgery, Formulae in Pediatric Eyes. Ophthalmology
with open angle glaucoma24. They have 2010; 117:1493–1499.
suggested that when a primary IOL avoiding the neonatal period, followed 17. Eibschitz-Tsimhoni M, Tsimhoni O, Archer S
is used, there is a reduced incidence M and Del Monte M A. Effect of axial length
of delayed-onset glaucoma. However, by a careful surveillance, to detect and keratometry measurement error on
it is worth noting that implants are intraocular lens implant power prediction
infrequently placed in eyes with micro- postoperative glaucoma. The latter must formulas in pediatric patients. J AAPOS
cornea and the surgery in this series was 2008; 12:173-76.
relatively delayed (mean age at surgery: continue for long term basis. Probably 18. Nischal K K, Solebo L,Russell-Eggitt
5.06 years). Hence, two important risk I. Paediatric IOL implantation and
factors, micro-cornea and early age of future prospective studies, in which postoperative refractive state:what role do
surgery, which could have influenced the study methodology and surgical technique
results otherwise, were conspicuous by children would be randomized to cataract play? Br J Ophthalmol 2010;94:529-31.
their absence. 19. Griener ED, Dahan E, Lambert SR. Effect
surgery at different ages within the of age at time of cataract surgery on
Although it is clear that pediatric subsequent axial length growth in infant
cataract surgery places the eye at risk for critical period, would enlighten us about eyes. J Cataract Refract Surg 1999;25:1209-
glaucoma, the exact mechanism remains 13.
elusive. The volume of the lens and its the issue of optimum timing. 20. Russell-Eggitt I, Zamiri P. Review of
dynamic role in accommodation are aphakic glaucoma after surgery for
more prominent in younger eyes. Surgical References congenital cataract. J Cataract Refract Surg
removal of the lens early in life can alter 1997;23:664-8.
normal development of the filtration 1. Lambert SR, Drack AV. Infantile Cataracts. 21. Phelps CD, Arafat NI. Open-angle glaucoma
angle. Morphologic studies of developing Surv Ophthalmol 1996:40:427-458. following surgery for congenital cataracts.
eyes of children have shown that the Arch Ophthalmol 1977;95:1985-7.
angle recess of the iridocorneal angle is 2. Vasavada A R, Praveen M R, Tassignon M J, 22. Magnusson G, Abrahamsson M, Sjostrand
expected to move toward the periphery, Shah S Ketal. Posterior capsule management J. Glaucoma following cataract surgery:
exposing the scleral spur and the ciliary in congenital cataract surgery. J Cataract an 18-year longitudinal follow-up. Acta
body band25. It has been speculated that Refract Surg 2011; 37:173–193. Opthalmol Scand 2000;78:65-70.
the absence of the lens early in life alters 23. Rabiah P. Frequency and predictors of
or causes an arrest in development of 3. Lambert S R, Buckley E G, Plager D A, glaucoma after pediatric cataract surgery.
the filtration angle, or it may be a lack Medow N B and Wilson M E. Unilateral Am J Ophthalmol 2004; 137:30-7.
of accommodation and pull of the ciliary Intraocular Lens Implantation During the 24. Chak M, Rahi J S.Incidence of and Factors
muscles on the trabecular meshwork First Six Months of Life. J AAPOS 1999; Associated with Glaucoma after Surgery
that in some way permits the meshwork 3:344-9. for Congenital Cataract: Findings from
to become compact and dysfunctional/ the British Congenital Cataract Study.
It may be that some chemical substance 4. Lambert SR, Lynn M, Drews-Botsch C etal. Ophthalmology 2008; 115:1013–18.
may diffuse from the posterior eye into A comparison of grating acuity, strabismus 25. Asrani SG, Wilensky JT. Glaucoma after
the anterior chamber and change the and reoperation outcomes among children congenital cataract surgery. Ophthalmology
with aphakia and pseudophakia after 1995;102:863-7.
unilateral cataract surgery during the first 26. Mori M, Keech RV, ScottWE. Glaucoma and
six months of life. J AAPOS 2001;5:70-75. ocular hypertension in pediatric patients
with cataracts. J AAPOS 1997; 1:98-101
5. Plager D A, Yang S,Neely D, Sprunger D
and Sondhi N. Complications in the First Correspondence to:
Year Following Cataract Surgery With and Dr. Sumit Monga
Without IOL in Infants and Older Children. Strabismus and Neuro-Ophthalmology Service,
J AAPOS 2002;6:9-14. Centre for Sight Group of Eye Hospitals,
Delhi NCR, India
6. Trivedi R H, Wilson M E, Jr, Bartholomew L
R, Lal G and Peterseim M M. Opacification
of the Visual Axis After Cataract Surgery
and Single Acrylic Intraocular Lens
Implantation in the First Year of Life. J
AAPOS 2004;8:156-164.
7. Lundvall A, Zetterstrom C. Primary
intraocular lens implantation in infants:
Complications and visual results. J Cataract
Refract Surg 2006;32:1672–77.
8. J L Ashworth, A P Maino, S Biswas, et
al. Refractive outcomes after primary
intraocular lens implantation in infants. Br
J Ophthalmol 2007;91: 596-99.
9. Ram J, Brar GS, Kaushik S, Sukhija J etal.
Primary intraocular lens implantation in
the first two years of life: safety profile and
visual results. Indian J Ophthalmol. 2007;
55:185-9.
10. The Infant Aphakia Treatment Study Group.
A Randomized Clinical Trial Comparing
Contact Lens with Intraocular Lens
Correction of Monocular Aphakia During
Infancy. Arch Ophthalmol. 2010;128:810-
818.
11. Ahmadieh H, Javadi MA, Ahmady M etal.
Primary capsulectomy, anterior vitrectomy,
Monga S. Pediatric Cataract Surgery: What Roles do Study Methodology... www. dos-times.org 43
Recent Trends and Advances
Childhood Glaucoma- The CGRN
Classification
Dr. Deepika Dhingra, Dr. Savleen Kaur, Dr. Sushmita Kaushik
Advanced Eye Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India
Childhood glaucoma classification began with 3. Cup disc asymmetry of ≥ 0.2 when the optic discs are
the term buphthalmos, or ox-eyed, which was similar size, or focal rim thinning.
used to describe the secondary effect of elevated 4. Corneal findings: Haab’s striae or diameter ≥ 11 mm in
newborn, > 12 mm in child < 1 year, or > 13 mm at any age.
intraocular pressure (IOP) on the elastic infantile
5. Progressive myopia, myopic shift, or an increase in ocular
eye1. Buphthalmos was subdivided into simple dimensions out of keeping with normal growth.
buphthalmos due to a primary mechanism, now 6. Reproducible visual field defect consistent with
glaucomatous optic neuropathy with no other observable
known as primary congenital glaucoma, and buphthalmos reason for defect.
Glaucoma suspect: No IOP related damage; at least 1criteria
associated with other developmental anomalies. The more
complex anatomic classification was given by Hoskins, which required for diagnosis:
divided the developmental abnormalities into trabecular 1. IOP > 21 mm Hg on 2 separate occasions.
meshwork, iris, and/or cornea2. The Shaffer-Weiss classification 2. Suspicious optic disc appearance for glaucoma, i.e.,
introduced the categories of isolated congenital (infantile)
increased cup disc ratio for size of optic disc.
glaucoma, glaucomas associated with congenital anomalies, 3. Suspicious visual field for glaucoma.
4. Increased corneal diameter or axial length in the setting of
and acquired glaucoma3. Walton proposed an exhaustive
normal IOP.
listing of all disorders known to be associated with childhood
CGRN CLASSIFICATION OF CHILDHOOD GLAUCOMA
glaucoma4.
Unlike the adult definition of glaucoma which focuses Primary childhood glaucoma
on the pathology of the optic nerve to make the diagnosis, IA. Primary Congenital Glaucoma (PCG) (Figure 1)
subdividing glaucoma in children is more difficult because the 1. Isolated angle anomalies (+/ - mild congenital iris
optic nerve can be difficult to evaluate properly in the presence
of corneal opacity. anomalies)
2. Meets glaucoma definition (usually with ocular
The definition of childhood glaucoma is IOP-related
damage to the eye, rather than being based solely on optic-nerve enlargement)
3. Subcategories based on age of onset
criteria as suggested by an expert panel of glaucoma specialists a. Neonatal or newborn onset (0-1 month)
b. Infantile onset (>1-24 months)
in the world. The childhood glaucoma research network (CGRN) c. Juvenile onset or late-recognized (>2 years)
composed of clinicians and scientists who specialize in treating
children with glaucoma, has provided definitions of childhood
glaucoma, glaucoma suspect, and a new classification system of
pediatric glaucomas5.
According to CGRN classification,
Childhood age is based on national criteria:
• US: younger than 18 years
• EU, UK, UNICEF: 16 years or younger
DEFINITIONS OF GLAUCOMA AND
GLAUCOMA SUSPECT
Glaucoma: IOP related damage to Figure 1: Primary Congenital Glaucoma. A-C: Newborn glaucoma; A: Central Haab’s striae; B:
the eye; at least 2 criteria required for Persistent corneal opacity in a rubella positive neonate after IOP control; C: Bilateral large eye
diagnosis: with right eye central corneal opacity and optical iridectomy; D,E: Infantile Glaucoma
1. IOP > 21 mm Hg; however, investigator D: Bilateral large eyes with clearer cornea; E: Bilateral glaucoma with left eye larger in size than
right eye and haab striae in left eye; F: Late onset PCG presented at 5 years of age with bilateral
discretion is required if there is data of large cornea (L>R) and advanced cupping in left eye.
examination under anesthesia alone
due to variable effects of anesthesia on
all methods of IOP assessment.
2. Optic disc cupping: progressive
increase in cup-disc ratio.
44 DOS Times - July-August 2018 Kaushik S. et al. Childhood Glaucoma the CGRN Classification
Recent Trends and Advances
2. No congenital ocular anomalies or
syndromes
3. Open angle (normal appearance)
4. Meets glaucoma definition
Figure 2: Juvenile Open Angle Glaucoma. A: Anterior segment photograph with deep anterior Secondary childhood glaucoma
chamber; B: Gonioscopy showing high iris insertion with iris processes.
A: Glaucoma Associated with Non-
Figure 3: Secondary Glaucoma associated with non-acquired ocular anomalies. A: Posterior
embryotoxon in Axenfeld anomaly; B: Aniridia with peripheral corneal haze due to limbal stem Acquired Ocular Anomalies (Figure 3)
cell deficiency; C: Retroillumination in a case of aniridia; D: Microspherophakia with anteriorly
subluxated lens; E: Axenfeld-Rieger anomaly with multiple iris holes and corectopia; E: A case of Includes conditions of predominantly
Peters anomaly with bilateral central corneal opacity and enlarged cornea; F: Bilateral enlarged
cornea with right eye primary congenital glaucoma and left eye Peters anomaly; G: One of ocular anomalies present at birth which
the most severe forms of Peters anomaly with lens touching the descemet’s membrane defect
visible on Ultrasound Biomicroscopy; H: Bilateral microcornea; I: Peters anomaly with right eye may or may not be associated with
keratoplasty and left eye central corneal opacity.
systemic signs and meets glaucoma
4. Spontaneously arrested cases with IB. Juvenile Open Angle Glaucoma definition
(JOAG) (Figure 2)
normal IOP but typical signs of PCG Includes:
may be classified as PCG 1. No ocular enlargement • Axenfeld Rieger anomaly (syndrome
if systemic associations)
• Peters anomaly (Syndrome if
systemic associations)
• Ectropion uveae
• Congenital iris hypoplasia
• Aniridia
• Persistent fetal vasculature/PFV (if
glaucoma present before cataract
surgery)
• Oculodermal melanocytosis (nevus
of Ota)
• Posterior polymorphous dystrophy
• Microphthalmos, Microcornea
• Ectopia lentis
• Simple ectopia lentis (no systemic
associations, possible Type 1 FBN
mutation).
B: Glaucoma Associated with
Non-Acquired Systemic Disease or
Syndrome (Figure 4)
Includes conditions predominantly
of systemic disease present at birth which
may be associated with ocular signs and
meets the definition of glaucoma; namely
• Chromosomal disorders such as
Trisomy 21 (Down syndrome)
• Connective tissue disorders
Figure 4: Secondary Glaucoma associated with non-acquired Figure 5: Secondary acquired glaucoma. (A-C): A: case of vernal keratoconjunctivitis
systemic anomalies. A: Sturge-Weber syndrome with unilateral with steroid induced cataract; B: pseudophakia in other eye; C: and advanced
port-wine stain and glaucoma; B: Sturge-Weber syndrome optic nerve head cupping; D: Uveitic patient with anterior and posterior synechiae
with bilateral port-wine stain and glaucoma; C: Episcleral with glaucoma controlled by glaucoma drainage device surgery; E: Uveitic patient
hemangioma in a patient of Sturge-Weber syndrome; D: with iris-bombe; F: Traumatic hyphaema.
Neurofibromatosis with S-shaped lid deformity and ipsilateral
glaucoma.
Kaushik S. et al. Childhood Glaucoma the CGRN Classification www. dos-times.org 45
Recent Trends and Advances
RECEFNigTuTrReE6N:DGSlaucoma secondary to cataract surgery. A: Unilateral glaucoma after cataract surgery • Angle closure glaucoma (< 50%
in right eye; B: Iris-fixated intraocular lens (IOL) in a case of microcornea; C: Iris-fixated IOL in a open or acute angle closure).
case of microcornea with corneal decompensation and glaucoma; D: Iris fixated IOL with shallow
Includes:
Figuarnete7riosrhcohwasmabneraalngdorgitlhaumcofomrac. lassification of childhood glaucoma • Uveitis
• Trauma (hyphema, angle recession,
ectopia lentis)
• Steroid induced
• Tumors (benign/malignant, ocular/
orbital)
• Retinopathy of Prematurity
• Prior ocular surgery other than
cataract surgery.
D. Glaucoma Following Congenital
Cataract Surgery (Figure 6).
Meets glaucoma definition after
cataract surgery performed.
Excludes acquired cataract or
cataract in the setting of a syndrome with
a known glaucoma relationship, such
as Lowe syndrome, congenital rubella
syndrome, aniridia, or persistent fetal
vasculature.
SUGGESTED READING
1. Duke-Elder, Stewart. “Part 2. Congenital
deformities”. System of Ophthalmology.
Vol. III. Duke-Elder, Stewart (ed.).
London: Kimpton, 1964.
2. Hoskins HD Jr, Shaffer RN, Hetherington
J. Anatomical classification of the
developmental glaucomas. Arch
Ophthalmol. 1984;102:1331-6.
3. Shaffer, R., Weiss, D. Congenital and
Pediatric Glaucomas. Saint Louis:
Mosby, 1970.
4. Yeung HH, Walton DS. Clinical
classification of childhood glaucomas.
Arch Ophthalmol. 2010 Jun;128:680-4.
5. Beck A, Chang TC, Freedman S.
Definition, classification, differential
diagnosis. In: Weinreb RN, Grajewski
A, Papadopoulos M, Grigg J, Freedman
S, editors. Childhood Glaucoma.
Amsterdam: Kugler Publications; 2013.
pp. 3–10.
Figure 7: Shows an algorithm for classification of childhood glaucoma
Figure 7: An algorithm for classification of childhood glaucoma
• Marfan syndrome • Rubinstein-Taybi Correspondence to:
• Weill-Marchesani syndrome • Congenital rubella Dr. Sushmita Kaushik
• Stickler syndrome Glaucoma Services, Advanced Eye Centre
• Metabolic disorders C. Glaucoma Associated with Department of Ophthalmology,
• Homocystinuria PGIMER, Chandigarh, India
• Lowe syndrome Acquired Conditions (Figure 5)
• Mucopolysaccharidoses 1. Meets glaucoma definition after the
• Phacomatoses
• Neurofibromatosis (NF-1, NF-2) acquired condition is recognized
• Sturge-Weber syndrome
• Klippel-Trenaunay-Weber syndrome (except congenital cataract surgery)
2. Acquired conditions
3. Based on gonioscopy results:
• Open angle glaucoma (>50%
open) or
Dhingra et al. Childhood glaucoma classification Kaushik S. et al. Childhood Glaucoma the CGRN Classification
46 DOS Times - July-August 2018
Recent Trends and Advances
Intra-vitreal Anti-vascular Endothelial
Growth Factor Drugs for Treatment of
Retinopathy of Prematurity
Dr. Bruttendu Moharana MS, Dr. Savleen Kaur MS, Dr. Mangat Ram Dogra MS
Advanced Eye Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India
Retinopathy of prematurity (ROP) is a proliferative Phase I occurs from birth to 30-31 weeks of gestation. The
vitreoretinopathy in preterm infants. It is one of metabolically active but nonperfused immature retina becomes
the major avoidable causes of childhood blindness hypoxic and stimulates over-production of VEGF. This marks
affecting countries of all economic strata. ROP phase II which begins around 31-32 weeks of gestation. Excess
causes lifelong irreversible significant visual loss VEGF leads to formation of abnormal new vessels, fibrovascular
in the infants. It is well established that low birth proliferation and later tractional retinal detachment. Fetal IGF
weight and small gestational age are good clinical predictive 1 (insulin like growth factors) and various other angiogenic
factors for development of ROP1. Other risk factors include cytokines also play a role in pathogenesis of ROP4.
high level of oxygen supplementation, hypercapnia, anaemia,
chronic lung disease, blood transfusion, sepsis, intraventricular GOLD STANDARD TREATMENT OF ROP
haemorrhage, multiple birth etc.2
ROP has become a major public health problem in middle Treatment is primarily aimed at ablating the avascular,
income country like India. Increasing number of neonatal hypoxic retina and converting it into anoxic retina and thereby
intensive care units (NICU) and special new-born care unit preventing excess VEGF secretion and the complications
(SNCU) has led to survival of more and more very low birth that follows4. Cryotherapy and laser photocoagulation to the
weight (VLBW) infants. But suboptimal neonatal care, lack of avascular retina is the traditional treatment modality with
awareness, screening and treatment programme has caused laser treatment being the gold standard (Figure 1)5. In ETROP
the absolute number of infants with ROP to rise3. study6, laser ablation of peripheral retinal significantly reduced
progression of the disease, although the visual outcome was
PATHOGENESIS OF ROP poor especially in zone I disease. Also, there is permanent loss
of peripheral visual field and development of high myopia later
ROP is a two-phase disease. Phase I is characterised by in life7. Progression to retinal detachment occurred in 12%
delayed retinal vascular growth due to hyperoxia. Neonatal of eyes in the ETROP study even with adequate peripheral
hyperoxia results in apoptosis and capillary regression. This ablation. This may be related to either the individual skills of
interrupt normal vascularisation and leads to retinal ischemia. the surgeon who applies laser treatment, or vitreal VEGF levels
Figure 1: Male neonate, 1000gm birth weight, 28-week gestational age presents at 34-week PMA. BE APROP with plus disease in posterior zone II
(image on the left). BE laser photocoagulation done. Marked regression of ROP at 4-week after laser (image on the right).
Moharana B. et al. Intra-vitreal anti-vascular endothelial growth factor drugs... www. dos-times.org 47
Recent Trends and Advances
Figure 2A: Figure on left - female child, 900gm birth weight, 27-week gestational age, presented at 33 weeks PMA, BE ZONE I APROP received anti
VEGF (lucentis) and then LIO after poor response.
Figure 2B: Left- Recurrence of Disease which progressed to stage IVB in a week.
which cannot be reduced by retinal laser these drugs decrease VEGF levels both of anti-VEGF drugs used as monotherapy
photocoagulation. Although VEGF is in the retina and vitreous.8 Various anti- or along with cryotherapy or laser
mainly secreted in the avascular retina, VEGF drugs in use are pegaptanib sodium photocoagulation.
vitreal macrophages as a second source (Macugen), ranibizumab (Lucentis),
of VEGF may be responsible for the lack bevacizumab (Avastin) and aflibercept TRIALS USING ANTI VEGFS IN ROP
of the effectiveness of laser therapy for (Eylea). Pegaptanib sodium, ranibizumab
ROP8. and aflibercept are approved by the US Use of pegaptanib along with
Food and Drug Administration (FDA) conventional laser (combined treatment)
ANTI-VEGF THERAPY IN ROP for intraocular use in the treatment has significant favourable outcome
of adult ocular neovascular diseases. compared to only cryotherapy or laser
VEGF is a potent mitogen for vascular Bevacizumab has not yet been approved photocoagulation. In patients with stage
endothelial cells and necessary for the by FDA for the same. 3 ROP in zone I and posterior zone II,
physiological angiogenesis. It is regulated the combined modality of treatment
by tissue hypoxia. It also promotes However, the safety and efficacy of showed faster regression of plus
pathological angiogenesis. Blocking the these drugs in preterm neonates is yet disease and development of peripheral
action of VEGF is expected to reduce uncertain. There is lack of consensus retinal blood vessels and lower rate
the neovascular activity associated with on criteria, dosage, frequency of of recurrence of neovascularisation.
ROP9. Treatment with cryotherapy or administration and follow-up schedule. Pegaptanib stimulated development of
laser photocoagulation indirectly reduces VEGF is essential for organ development peripheral retinal blood vessels rather
VEGF levels by ablating the peripheral of neonates. There is no conclusive data than preventing it10. Although, it is well
avascular hypoxic retina and converting on any late systemic complications established that anti-VEGF agents impede
it into anoxic retina. On the other and neuro-developmental issues after peripheral retinal vascular development
hand, anti-VEGF agents don’t have the treatment with anti-VEGF drugs. In this leading to persistent peripheral avascular
disadvantage of tissue destruction. Also, review, we discuss the efficacy and safety retina and late recurrence of the disease.
48 DOS Times - July-August 2018 Moharana B. et al. Intra-vitreal anti-vascular endothelial growth factor drugs...
Recent Trends and Advances
Though recurrence rate was lower, the inhibits all isoforms of VEGF-A may to anterior segment development that is
provide longer duration of intraocular present with intravitreal bevacizumab
interval from treatment to recurrence effect17. No randomised study is available but minimal or absent following laser
was significantly higher in eyes receiving to compare the efficacy of ranibizumab or ablation of peripheral retina.
bevacizumab with aflibercept.
combined treatment. This highlights the Neurodevelopmental delay is
DISEASE RECURRENCE another area of concern while treating
need for long term follow-up in patients ROP with anti-VEGF. Rien et al27
Recurrence occurs much later after studied sixty-one patients who were
who receive anti-VEGF therapy. No local anti-VEGF treatment than conventional treated with bevacizumab. At two
laser photocoagulation. Time interval years after treatment, no difference
or systemic complications were noted. from anti-VEGF injection and disease on neurodevelopment for those who
recurrence varies with different received only bevacizumab versus only
BEAT-ROP (Bevacizumab Eliminates studies. Hwang et al18 reported 1-year laser treatment were found. Those infants
recurrence free interval after with either who required rescue therapy with laser
the Angiogenic Threat of ROP)11 is a bevacizumab or ranibizumab. Castellano or bevacizumab injection after initial
et al19 in a small case series showed unsuccessful treatment showed some
prospective, controlled, randomized, 3-year recurrence free interval after detrimental neurodevelopmental effects.
stratified, multicentre trial to assess single ranibizumab injection. However The reason for this is not investigated
multiple other case reports and series in the study. It could be due to a greater
intravitreal bevacizumab monotherapy have reported early and late recurrence number of procedures requiring more
after anti-VEGF treatment20,21,22. Wong sedation or anaesthesia. Morin et al28 in
for zone I or zone II posterior stage et al16 reported reactivation after an observational study found higher odds
ranibizumab injection between 5 to 7 of severe neurodevelopmental disabilities
3+ (i.e., stage 3 with plus disease) weeks after injection whereas recurrence in infants treated with bevacizumab. In
free interval of 9 to 10 months with contrast, two-year follow-up of patients
retinopathy of prematurity. Infants were bevacizumab injection. Chan et al23 enrolled in BEAT-ROP didn’t show any
reported reactivation between 4 to 8 adverse neurodevelopmental effect
randomly assigned to receive intravitreal weeks after treatment with ranibizumab. in the bevacizumab arm. There were
Hu et al24 have reported recurrence at 35 no significant differences between the
bevacizumab (0.625 mg in 0.025 ml of weeks post-injection of bevacizumab. bevacizumab and laser therapy groups
in weight, length, head circumference,
solution) or conventional laser therapy. SAFETY OF ANTI-VEGF AGENTS cerebral palsy, or Bayley scores29. There
are two potential explanations for the
Intravitreal bevacizumab monotherapy, VEGF plays many positive roles difference. First, in the non-randomised
related to neural, vascular, and lung observational study28 the sicker infants
as compared with conventional laser development. The long-term systemic would preferentially be treated with
complications of anti-VEGF agents are bevacizumab. Second, the observational
therapy, in infants with stage 3+ not known25. BEAT-ROP suggests that, study was much larger and thus had
bevacizumab being a large molecule greater power to identify differences.
retinopathy of prematurity showed a cannot penetrate the intact retina or Although the BEAT-ROP study did not
significant benefit for zone I but not zone escape the eye except in very small identify any concerning trends toward
amounts. The study was not large enough worse systemic outcomes among
II disease. At 54 week of postmenstrual to assess if bevacizumab injection infants treated with bevacizumab, it
results in significant mortality and was not adequately powered to identify
age (PMA), the rate of recurrence for zone morbidity. There were five deaths in the significant differences.
I disease was significantly higher with bevacizumab arm of the study. Though
statistically not significant, it is of concern CONCLUSION
conventional laser therapy than with that four out of these five deaths were
related to respiratory problem. Other 1. Anti-VEGF agents have been claimed
intravitreal bevacizumab. There was no studies have shown that bevacizumab has to have multiple advantages over
significant difference in recurrence rate detectable concentration in the serum of conventional laser photocoagulation
the neonate after intra-vitreal injection26. for treatment of ROP. These are:
in posterior zone II disease. Development Bevacizumab has longer serum half-
life than ranibizumab. So theoretically, A. They are less destructive than
of peripheral retinal vessels continued ranibizumab is a safer option for the laser treatment as they don’t
neonates. ablate the peripheral retina.
after treatment with intravitreal
High myopia after laser B. There is no permanent loss of
bevacizumab, but conventional laser photocoagulation is a well-known peripheral field.
complication. In BEAT-ROP study high
therapy led to permanent destruction of myopia was found in eyes that received C. There is less chance of
laser treatment than compared to development of myopia.
the peripheral retina. APROP regression the eyes that received intravitreal
rate was significantly better after bevacizumab. The difference is related D. Anti-VEGF drugs are easily
accessible and easier and faster
bevacizumab monotherapy compared to administer compared to laser
photocoagulation.
with laser photocoagulation at follow-up
of 80 weeks12.
The use of intravitreal ranibizumab
for the treatment of ROP has been
described in several case reports and
series with variable results. Intravitreal
ranibizumab induced rapid, complete
regression of high-risk posterior ROP
with continued growth of peripheral
retina. The potential for recurrent ROP
requires vigilant monitoring. Subsequent
peripheral laser for ROP recurrences
may spare the posterior retina from the
destruction caused by photocoagulation13.
Both bevacizumab and
ranibizumab have similar responses
in neovascularization and plus disease
regression, and none of the eyes had
recurrence of ROP after an initial good
response. After one-year follow-up,
there was no recurrence of disease if
there was initial good response to either
drug. Multiple other studies show higher
recurrence rate in group treated with
ranibizumab15,16. Aflibercept, which
Moharana B. et al. Intra-vitreal anti-vascular endothelial growth factor drugs... www. dos-times.org 49
Recent Trends and Advances
E. The short duration of treatment Prematurity Study: structural findings ranibizumab as salvage therapy in an
is highly important if laser at age 2-years. Br J Ophthalmol. 2006;
photocoagulation cannot be 90: 1378–1382. extremely low-birth-weight infant with
performed in very sick neonates. 7. Geloneck MM et al BEAT-ROP
It also can be used in eyes with Cooperative Group. Refractive outcomes rush type retinopathy of prematurity.
small pupil or hazy media. following bevacizumab monotherapy
compared with conventional laser Oman J Ophthalmol. 2012;5:184-186.
2. The possible local complications treatment: a randomized clinical trial.
related to anti-VEGF injection are JAMA Ophthalmol 2014; 132: 1327-33. 21. Mota A et al. Combination of
lens injury, retinal detachment, and 8. Naug H, Browning J, Gole GA, Gobe G.
infection. The concerns are related Vitreal macrophages express VEGF165 intravitreal ranibizumab and laser
to the systemic safety issues of anti- in oxygen-induced retinopathy. Clin
VEGF, which are yet to be studied in Experiment Ophthalmol. 2000;28:48– photocoagulation for aggressive
randomised controlled trials with a 52
large cohort. Studies are needed to 9. McColm JR, Geisen P, Hartnett ME. VEGF posterior retinopathy of prematurity.
establish safe yet effective dose of isoforms and their expression after a
anti-VEGF. single episode of hypoxia or repeated Case Rep Ophthalmol. 2012 Jan;3:136-
fluctuations between hyperoxia and
3. Late recurrence of disease is major hypoxia: relevance to clinical ROP. Mol 141
area of concern with anti VEGF Vis. 2004;10:512–520
therapy when used alone. More 10. Autrata R et al.Intravitreal pegaptanib 22. Jang SY, Choi KS, Lee SJ.Delayed-onset
studies are needed to determine an combined with diode laser therapy for
appropriate follow-up schedule after stage 3+ retinopathy of prematurity retinal detachment after an intravitreal
anti-VEGF treatment. in zone I and posterior zone II.Eur J
Ophthalmol. 2012;22:687-94 injection of ranibizumab for zone
4. Insufficient data precludes strong 11. Mintz-Hittner HA, Kennedy KA, Chuang
conclusion favouring monotherapy AZ, BEAT-ROP Cooperative Group 1 plus retinopathy of prematurity.J
of anti-VEGF agents or combination Efficacy of intravitreal bevacizumab for
therapy with laser photocoagulation stage 3+ retinopathy of prematurity. N Aapos. 2010;14:457-459
in preterm infants with ROP25. Engl J Med. 2011;364:603–615
However, these drugs could be used 12. Nicoara SD et al. Regression Rates 23. Chan JT et al.Risk of recurrence of
as a rescue treatment in sick infants Following the Treatment of Aggressive
or eyes with non-dilating pupil or Posterior Retinopathy of Prematurity retinopathy of prematurity after initial
poor media after informing the with Bevacizumab Versus Laser: 8-Year
caretakers all risks associated with Retrospective Analysis. Med Sci Monit intravitreal ranibizumab therapy.Sci
anti-VEGF drugs. 2016;22: 1192–1209
13. Baumal CR, Goldgerg RA, Fein Rep 2016;6:27082
REFERENCES JG.Primary intravitreal ranibizumab for
high-risk retinopathy of prematurity. 24. Hu Q et al. Recurrence of Retinopathy
1. Early treatment for retinopathy Ophthalmic surg lasers imaging retina
of prematurity cooperative group. 2015;46:432-438 of Prematurity in Zone II Stage 3+
Revised indications for the treatment 14. Chen SN et al.Intravitreal anti-vascular
of retinopathy of prematurity: results endothelial growth factor treatment after Ranibizumab Treatment: A
of the early treatment for retinopathy for retinopathy of prematurity:
of prematurity randomized trial. Arch comparison between ranibizumab and Retrospective Study J Ophthalmol.
Ophthalmol 2003; 121: 1684-94. bevacizumab.Retina 2015;35:667-674
15. Erol MK et al.Comparision of 2017;2017:5078565. doi:
2. Chattopadhyay MP, Pradhan A, Singh R, intravitreal ranibizumab and
Datta S. Incidence and risk factors for bevacizumab treatment for retinopathy 10.1155/2017/5078565. Epub 2017
retinopathy of prematurity in neonates. of prematurity.Arq Bras Oftalmol
Indian Pediatr 2015; 52: 157-8. 2015;78:340-343 Apr 9.
16. Wong RK, Hubschman S, Tsui
3. Dogra MR, Katoch D. Retinopathy of I.Reactivation of retinopathy of 25. Sankar MJ, Sankar J, Chandra P.
prematurity: an emerging and evolving prematurity after ranibizumab
challenge. Indian J Ophthalmol 2017; treatment.Retina 2015;35:675-680 Anti-vascular endothelial growth
65: 782-784. 17. Stewart MW. Clinical and differential
utility of VEGF inhibitors in wet age- factor (VEGF) drugs for treatment of
4. Smith LE et al. Pathogenesis of related macular degeneration: focus
retinopathy of prematurity. Growth on aflibercept. Clin Ophthalmol. retinopathy of prematurity.Cochrane
hormone IGF Res 2004; 14: Suppl A: 2012;6:1175–1186
S140-144. 18. Hwang CK, Hubbard GB, Hutchinson Database Syst Rev. 2018;1:CD009734.
AK, Lambert SR.Outcome after
5. Ng EY, Connolly BP, McNamara JA, intravitreal bevacizumab versus laser doi: 10.1002/14651858.CD009734.
Regillo CD, Vander JF, Tasman W. A photocoagulation for retinopathy of
comparison of laser photocoagulation prematurity: a five year retrospective pub3.
with cryotherapy for threshold analysis.Ophthalomol 2015;122:1008-
retinopathy of prematurity at 10 years: 1015 26. Sato T, Wada K, Arahori H, Kuno
part 1. Visual function and structural 19. Castellanos MA, Schwartz S, Garcia-
outcome. Ophthalmology. 2002; 109: Aguirre G, Quiroz-Mercado H.short- N, Imoto K, Iwahashi-Shima C,
928–934. term outcome after intravitreal
ranibizumab injections for the Kusaka S. Serum concentrations of
6. The Early Treatment for Retinopathy treatment of retinopathy of prematurity.
of Prematurity Cooperative Group. The Br J Ophthalmol 2013;97:816-819 bevacizumab (avastin) and vascular
Early Treatment for Retinopathy Of 20. Lin CJ, Chen SN, Hwang JF.Intravitreal
endothelial growth factor in infants
with retinopathy of prematurity. Am J
Ophthalmol. 2012;153(2):327–333
27. Lien R et al. Neurodevelopmental
Outcomes in Infants with Retinopathy
of Prematurity and Bevacizumab
Treatment. PLoS One. 2016 Jan
27;11(1):e0148019. doi: 10.1371/
journal.pone.0148019
28. Morin J et al. Neurodevelopmental
Outcomes Following Bevacizumab
Injections for Retinopathy of
Prematurity. Pediatrics. 2016;137(4).
pii: e20153218.
29. Kennedy KA, Mintz-Hittner HA;
BEAT-ROP Cooperative Group.
Medical and developmental outcomes
of bevacizumab versus laser for
retinopathy of prematurity.J Aapos
2018;22:61-65.
Correspondence to:
Dr. Bruttendu Moharana
Advanced Eye Centre
Department of Ophthalmology,
PGIMER, Chandigarh, India
50 DOS Times - July-August 2018 Moharana B. et al. Intra-vitreal anti-vascular endothelial growth factor drugs...
Recent Trends and Advances
Pharmacotherapy in Amblyopia - New Drugs
Dr. Samreen Khanam MS, Dr. Savleen Kaur MS
Advanced Eye Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India
Amblyopia is classically described as unilateral or was restored to normal acuity levels by administration of
bilateral reduction in the best corrected visual acetylcholinesterase inhibitors. Thus, based on the laboratory
acuity due to form vision deprivation and/or evidence one can potentially accomplish effective amblyopia
abnormal binocular interaction in the absence therapy even after the ‘‘critical period’’ has passed.
of any identifiable organic pathology. Visual
neurodevelopment is immature at birth and Pharmacological agents
appropriate integration of binocular information at the cortical
level requires adequate visual stimulation during the critical Several different pharmacological agents have been
period of development. Critical period of visual development is identified to have clinical application in the treatment of
the period of cortical brain plasticity and extends upto 7-8 years amblyopia. Penalisation with atropine is the most common of
of age. Conditions that interfere with binocular sensory fusion all these. We are describing four new drugs whose effects are
such as significant refractive error, anisometropia, stimulus relatively promising.
deprivation and ocular misalignment cause abnormal visual
development resulting in amblyopia. The standard treatment Dopamine
regimen for unilateral amblyopia is based on eliminating
the competitive advantage of the dominant eye by providing Dopamine is a neurotransmitter that has been evaluated
appropriate refractive correction and occlusion therapy or for its ability to enhance cortical plasticity. Dopamine is active
penalization. However, maximum response to treatment is seen in the retina and the cortex but does not cross the blood–brain
during the critical period and, if left untreated past this period barrier. A catecholamine precursor to dopamine, known as
of cortical plasticity, amblyopia can result in permanently levodopa, does cross the blood– brain barrier and is converted
impaired vision. to dopamine in the brain. Levodopa, in combination with a
peripheral decarboxylase inhibitor –Carbidopa, has been used
Changes in excitatory-inhibitory balance regulate the in various human studies on amblyopia. Carbidopa inhibits the
plasticity of neural networks. A number of current researches peripheral conversion of levodopa to dopamine and therefore
aim at evolving strategies to modulate brain plasticity in increases the availability of levodopa in the central nervous
adulthood as well. system. Levodopa-Carbidopa is usually administered in a
4:1 ratio. Most studies advocate a low dose of 1.5 to 2 mg/kg
Cortical brain plasticity and its modulation levodopa for a 3-7 week period4.
Duffy et al1 conducted animal experiments with bicuculline, Yang et al5 have shown that older amblyopic children
a GABA receptor blocker and suggested that a reduction of GABA treated with carbidopa-levodopa had visual cortical activation.
may be a crucial step for restoration of plasticity in adulthood. Levodopa administration has also been shown to improve
GABA neurons express parvalbumin and become enwrapped pattern VEP and decrease fixation point scotomas6,7. The
by perineuronal nets. It has been seen that maturation of these Pediatric Eye Disease Investigator Group (PEDIG) evaluated
parvalbumin cells corresponds to the closing of the critical the role of levodopa in amblyopia and found that despite initial
period. Techniques that promote parvalbumin cell function improvement in visual acuity, there may be partial regression
or delay perineuronal net formation can push plasticity onset after stopping the medication8.
into adulthood. The involvement of perineuronal nets was
demonstrated by Pizzorusso et al2, when an injection of the Citicoline
enzyme chondroitinase into the visual cortex of adult rats
with amblyopia restored the critical period. Chondroitinase Citicoline is a molecule that prevents neurological cell
dissolved the extracellular matrix and destroyed perineuronal damage by maintaining the anatomical and functional integrity
nets to reactivate ocular plasticity. After patching a rat’s good of cell membrane. It has been used in cases of ischaemic and
eye, the researchers witnessed the recovery of normal vision. traumatic insults to the neuronal tissue and also as an adjunct to
Cortical circuitries for both the left and right eyes began firing levodopa in Parkinson’s disease. Citicoline improves membrane
together, just as they are during the early phase of childhood ATPase activity and modulates the turnover of catecholamine
development. The Hensch lab3 has demonstrated that brain and acetylcholine. Oral administration of citicoline combined
plasticity is modulated by cholinergic transmission, and that with occlusion therapy seems to show that there are more
it may be possible to reactivate plasticity by manipulating stable effects of this medication on the results of occlusion
cholinergic pathways. In adult mice with amblyopia, vision therapy compare to occlusion therapy alone. And in patients
beyond the critical period of visual plasticity, citicoline showed
visual acuity improvement, although the improvement was not
Khanam S. et al. Pharmacotherapy in Amblyopia - New Drugs www. dos-times.org 51
Recent Trends and Advances
Table 1: A brief review of drugs used for Amblyopia
Drug Mechanism Dosage/Duration Adverse Effects Remarks
Levodopa - Promising in older
carbidopa Levodopa is a precursor of Various doses of Lowers body children
combination dopamine, and easily crosses levodopa have been temperature, headache,
therapy blood–brain barrier. Carbidopa tried for different nausea, dry mouth, and The drug is well
is a peripheral decarboxylase durations: single-dose, abdominal cramps tolerated with no
Fluoxetine inhibitor which prevents 1-week, 3-week, and significant systemic
peripheral conversion of levodopa 7-weeks Some studies cholinergic effects.
Citicholine to dopamine, thereby increasing used lower doses – 1.5
the availability of levodopa in mg/kg/day and 30 mg/
the central nervous system. The day of levodopa – while
drug significantly increases the others used higher ones
density of NGF-immunoreactive – 6–13 mg/kg/day.
cells and elevate the expression Levodopa is usually
of endogenous NGF in the visual administered along
cortex. with carbidopa in a 4:1
dose ratio, either in the
form of oral tablets or
as oral suspension
It acts by altering the cortical Fluoxetine can have
various side effects
expression of various heat shock such as irritability,
proteins and neurofilaments behavioral changes,
which are important for synaptic restlessness, and
agitation. This drug
functions should be prescribed
with caution in patients
with impaired liver and
renal function, in case
of diabetes and bipolar
disorders.
Citicoline, once absorbed, 1000 mg intramuscular Some patients can
crosses the blood–brain barrier administration, have side effects such
and gets incorporated into the daily for 15 days) in as insomnia, headache,
cell membrane phospholipids. experimental setting. diarrhoea, low or high
It has been shown to increase blood pressure, and
the levels of norepinephrine nausea.
and dopamine levels in CNS.
It has been shown to inhibit
apoptosis in neurodegenerative
models, thereby potentiating
neuroplasticity.
sustained after discontinuation of the This drug is currently being used Vetencourt et al13 to reactivate cortical
medication9,10. for the treatment of Alzheimer’s disease, plasticity in amblyopic rats, promoting
and for control of cognitive impairment full recovery of visual function. However,
Donepezil in dementia, schizophrenia, Down’s there have been no clinical studies
syndrome,andautismspectrumdisorders. involving fluoxetine in amblyopia.
The neurotransmitter Acetylcholine The ability of Donepezil to enhance Fluoxetine can have systemic side effects
(Ach) is involved in many cognitive perceptual learning and modulate neural such as irritability, behavioural changes,
functions, including attention and plasticity makes it a potentially promising and agitation and mandates caution in
learning. The role of Ach in facilitating drug for the treatment of amblyopia even patients with liver and kidney disease.
plasticity has been demonstrated in in adults. Studies evaluating the benefit
animal models by increasing the level of Donepezil in amblyopia are currently Conclusion
of Ach in the cortex while the animals underway12.
were passively exposed to a stimulus. While occlusion therapy remains the
Donepezil is a centrally acting reversible Fluoxetine primary weapon, our armamentarium for
acetylcholinesterase inhibitor that has treating amblyopia is expected to expand
been postulated to exert its treatment Fluoxetine is a selective serotonin in the near future with the availability
effect by enhancing cholinergic function, reuptake inhibitor which increases of newer drugs and techniques for
and increasing the level of Ach in the extracellular serotonin and noradrenalin neuromodulation. The amblyopic patients
brain11. levels. It has been shown by Maya who cross the age of cortical plasticity are
52 DOS Times - July-August 2018 Khanam S. et al. Pharmacotherapy in Amblyopia - New Drugs
Recent Trends and Advances
expected to benefit the most. Regression 2017;7:62-69. Indian J Ophthalmol 2014;62:124–29.
of effect after stoppage of therapy and 5. Yang CI, Yan ML, Huang JC. Functional 11. Rokem A, Silver M.A. Cholinergic
occlusion amblyopia in younger patients
(≤8 years) remain matters of concern. MRI of amblyopia before and after enhancement augments magnitude
Further studies are therefore needed to levodopa. Neurosci Lett 2003;339:49– and specificity of visual perceptual
evaluate the full efficacy and side effect 52. learning in healthy humans. Curr Biol.
profile of these agents. 6. Den D, Long S, Mai G, Wu DZ, Chen G. 2010;20:1723.
Effect of levodopa on visual evoked 12. Treatment of residual amblyopia
References potential in amblyopia. Yan Ke Xue Bao. with donepezil. Phase 1 clinical
1997;13:18-5. trial. ClinicalTrials.gov identifier:
1. Duffy FH, Burchfiel JL, Conway JL. 7. Gottlob I, Wizov SS, Reinecke RD. NCT01584076.
Bicuculline reversal of deprivation Visual acuities and scotomas after 3 13. Maya Vetencourt JF, Sale A, Viegi A, et al.
amblyopia in the cat. Nature 1976;260: weeks’ levodopa administration in The antidepressant fluoxetine restores
256–7. adult amblyopia. Graefes Arch Clin Exp plasticity in the adult visual cortex.
Ophthalmol. 1995;233:407–13. Science 2008;320:385–88.
2. Pizzorusso T, Medini P, Landi S, et al. 8. PEDIG. A pilot study of levodopa dosage
Structural and functional recovery as treatment for residual amblyopia Correspondence to:
from early monocular deprivation in children 8 to 518 years old. Arch Dr. Samreen Khanam
in adult rats. Proc Natl Acad Sci USA Ophthalmol 2010;128:1215–17. Advanced Eye Centre
2006;103:8517–22. 9. Campos EC, Schiavi C, Benedetti P, et Department of Ophthalmology,
al. Effect of citicoline on visual acuity PGIMER, Chandigarh, India
3. Morishita H, Miwa JM, Heintz N, Hensch in amblyopia: preliminary results.
TK. Lynx1, a cholinergic brake, limits Graefes Arch Clin Exp Ophthalomol.
plasticity in adult visual cortex. Science. 1995;233:307–312.
2010;330:1238–40. 10. Pawar PV, Mumbare SS, Patil MS,
Ramakrishnan S. Effectiveness of the
4. Singh A, Nagpal R, Mittal SK, Bahuguna addition of citicoline to patching in the
C, Kumar P. Pharmacological therapy treatment of amblyopia around visual
for amblyopia. Taiwan J Ophthalmol. maturity: a randomized controlled trial.
Khanam S. et al. Pharmacotherapy in Amblyopia - New Drugs www. dos-times.org 53
Case Reports
Phacomatosis Pigmentovascularis with Klippel
Trenaunay Weber Syndrome – Case Report
with Review of Literature
Dr. Shailja Tibrewal MS, Dr. Regina Lalramhluni MS, Dr. Rolli Khurana MS, Dr. Suma Ganesh MS, DNB
Pediatric Ophthalmology and Strabismus Services, Dr. Shroff Charity Eye Hospital, New Delhi, India
A12-year-old female child presented to Pediatric Figure 2: Pigmented nevi on trunk.
Ophthalmology clinic of Dr. Shroff charity eye
hospital with complaints of diminution of vision
in right eye noticed since past 15 days. The
parents had noted larger size of the right eyeball
since birth. The patient had a prior ophthalmic
consultation 15 days back and was started on antiglaucoma eye
drops in the right eye. She was born of non-consanguineous
marriage and the birth history was unremarkable.
On examination, her weight was 31 kgs and height was
137 cms which was slightly below average for her age. She
was observed to have pinkish capillary venous malformations
consistent with port wine stain on face (Figure 1). Examination
of the rest of the skin revealed pigmented nevi on trunk (Figure
2) and limbs (Figure 3). Additionally she had a right sided hemi-
hypertrophy of the lower limbs (Figure 4). There was no history
of similar illness in the family.
On ocular examination, her vision in right eye was PL+
with accurate PR and left eye was 6/6. She had right eye
exotropia of 20 degree (40 Prism Diopters), right sided RAPD,
buphthalmos (Figure 5) and ocular melanocytosis (Figure 6).
Her left eye anterior examination was within normal limit.
Fundus examination showed total glaucomatous optic atrophy
of right eye. The cup-disc ratio of left eye was 0.5:1. The
Figure 3: Pigmented nevi on lower limbs.
Figure 1: Port wine stain on face. intraocular pressure in right eye was 54mmHg and left eye
was 18mmHg. Based on the above findings she was diagnosed
to have congenital glaucoma in the right eye in association
with phacomatosis pigmentovascularis and Klippel Trenaunay
Weber syndrome. She was started on antiglaucoma medications
Tibrewal S. at al. Phacomatosis Pigmentovascularis... www. dos-times.org 55
Case Reports
Figure 4: Right sided hemihypertrophy of Figure 5: Photograph showing right eye buphthalmos.
lower limbs.
for the right eye and Humphrey flameus and aberrant
visual field was ordered for the
mongolian spots or blue
left eye.
nevus); Phacomatosis
Spilorosea (coexistence of
Discussion nevus spilus and telangiectatic
Phacomatosis Pigmento- nevus); Phacomatosis
vascularis (PPV) is a Cesiomarmorata (association
neurocutaneous syndrome of aberrant Mongolian spots
which was first described by Ota or blue nevus with cutis
in 1947 as “an association of a marmorata telangiectatica
widespread vascular nevus with congenita) and Phacomatosis
an extensive pigmentary nevus”1.
Pigmentovascularis of non-
It is a rare disease which presents classifiable type9. The most
at birth. Approximately 222 cases common type is type II a/b or
phacomatosis cesioflammea
have been published worldwide
(75% - 86%)10,11.
so far.2,3, It is found almost
Various systemic
exclusively in Asians, Africans
associations of PPV have been
and Hispanics and female to male
reported namely oculodermal
ratio is 1.34 :1. Figure 6: Ocular Melanocytosis.
melanocytosis (ODM),
The vascular nevus in
Sturge Weber syndrome
PPV is a cutaneous vascular It is defined as the occurrence of two
(SWS) or Klippel Trenaunay Weber
malformation composed of ectatic genetically different clones of cells
syndrome (KTW) or a combination of
capillaries and postcapillary venules within a region of normal cells leading
in dermis similar to Nevus flammeus to mosaicism. It explains the coexistence these12,13. Features of KTW syndrome
or portwine stain. It is seen involving of dermal melanocytosis and nevus that were present in our patient was
the face and/or body. Histopathological nevus flammeus and hemihypertrophy
examination reveals increased vessel flammeus.
Hasegawa and Yasuhara classified of soft tissues and bone. However the
density but no apparent proliferation of PPV into four types according to the type patient lacked any venous or lymphatic
vessels. Though it changes in colour with of pigmentary lesion associated with malformations or AV shunts.
ageing, it usually does not disappear. nevus flammeus and later a fifth type Although there can be a variety
The pigmented nevi in PPV consist was described, characterized by cutis of ocular manifestation in PPV like
of dermal melanocytosis in the form of marmorata telangiectatica and dermal melanosis oculi, iris mamillations, iris
mongolian spots, nevus of ota and/or melanocytosis. Each type was further hamartomas, prominent vessels in sclera,
blue nevus. It may also fade with ageing subdivided according to the presence of chronic oedema in the cornea, pigmentary
but rarely disappear4,5. cutaneous-only manifestations (subtype alterations in retina and pigmentary
The pathogenesis of PPV is believed a) or if associated with a systemic cataract, the most sight threatening one
to be due to an abnormality in the manifestations (subtype b), the majority is that of glaucoma. Rarely choroidal
development of melanocytic nevus cells of which are ocular, vascular, skeletal and melanomas have been reported to develop
and vasomotor neural cells derived from neurological8. in intraocular melanocytosis14,15. Hence
the neural crest. The genetic mechanism In 2005, Happle proposed a new a periodic detailed retinal evaluation is
has been explained by the theory simplified classification utilizing recommended. The incidence of glaucoma
in PPV is not known. The first detailed
of didymosis (twin spotting) which descriptive terms: Phacomatosis
results from somatic recombination6,7. Cesioflamea (association of the nevus report in ophthalmology literature was
56 DOS Times - July-August 2018 Tibrewal S. at al. Phacomatosis Pigmentovascularis...
by Teekhasaenee C et al in 199716. The report of 25 cases. Pediatr Dermatol Case Reports
pathogenesis of glaucoma in PPV can
be threefold; (1) anomaly of trabecular 2001;18:70. Trenaunay syndrome with Nevus of Ota
meshwork (2) increased pigmentation if and Ito. Arch Derm 102:640–645.
angle and thickened iris processes and 4. Hasegawa Y, Yasuhara M. Phakomatosis 13. Noriega-Sanchez A, Markland ON,
(3) increased episcleral pressure due to Herndon JH. 1972. Oculocutaneous
venous malformation. pigmentovascularis type VIa. Arch melanosis associated with the Sturge-
Weber syndrome. Neurology 22:256–
The occurrence of both extensive Dermatol 1985;121:651-5. 262.
melanocytosis and episcleral venous 14. Shields CL, Kligman BE, Suriano M,
malformation of the globe strongly 5. Mandt N et al. Phakomatosis Viloria V, Iturralde JC, Shields MV,
predispose to congenital glaucoma. Say EA, Shields JA. Phacomatosis
Milder forms may present later in life with pigmentovascularis type IIa. J Am Acad pigmentovascularis of cesioflammea
raised IOP. A detailed ocular examination type in 7 patients: combination of
immediately after birth, and periodically Dermatol1999; 40: 318-321. ocular pigmentation (melanocytosis or
thereafter, is required to detect glaucoma melanosis) and nevus flammeus with
and prevent its progression. Medical 6. Danarti R, Happle R. Paradominant risk for melanoma. Arch Ophthalmol.
management of glaucoma in PPV may be 2011;129:746-50.
unsuccessful at times warranting surgical inheritance of twin spotting: 15. Zhou H, Han J, Mao R, Chen M.
control of the intraocular pressure. Choroidal melanoma in phacomatosis
phacomatosis pigmentovascularis pigmentovascularis cesioflammea.
References Indian J Dermatol Venereol Leprol.
as a further possible example. Eur J 2016;82:339-42.
1. Ota M, Kawamura T, Ito N. Phakomatosis 16. Teekhasaenee C, Ritch R. Glaucoma
pigmentovascularis. Jpn J Dermatol Dermatol. 2003;13:612. in phakomatosis pigmentovascularis.
1947;52:1–31. 7. Moutray T, Napier M, Shafiq A, Fryer A, Ophthalmology 1997;104:150-7.
2. Fernández-Guarino M, Boixeda P, Rankin S, Willoughby CE. Monozygotic Correspondence to:
de Las Heras E, Aboin S, García- Dr. Shailja Tibrewal
Millán C, Olasolo PJ. Phakomatosis twins discordant for phacomatosis Pediatric Ophthalmology & Strabismus Services,
pigmentovascularis: Clinical findings in Dr. Shroff Charity Eye Hospital, New Delhi, India
15 patients and review of the literature. pigmentovascularis: evidence for the
J Am Acad Dermatol. 2008;58:88-93.
concept of twin spotting. Am J Med
3. Cordisco MR, Campo A, Castro C,
Bottegal F, Bocian M, Persico S, Pierini Genet A. 2010;152A:718-20.
AM. Phakomatosis pigmentovascularis:
8. Hasegawa Y, Yasuhara M. A variant of
phacomatosis pigmentovascularis. Skin
Res 1979;21:178-86.
9. Happle R. Phakomatosis
pigmentovascularis revisited
and reclassified. Arch Dermatol
2005;141:385-8.
10. Fernández-Guarino M, Boixeda P,
de Las Heras E, Aboin S, García-
Millán C, Olasolo PJ. Phakomatosis
pigmentovascularis: Clinical findings in
15 patients and review of the literature.
J Am Acad Dermatol. 2008;58:88-93.
11. Cordisco MR, Campo A, Castro C,
Bottegal F, Bocian M, Persico S, Pierini
AM. Phakomatosis pigmentovascularis:
report of 25 cases. Pediatr Dermatol
2001;18:70.
12. Furukawa T, Igata A, Toyokura Y, Ikeda
S. 1970. Sturge-Weber and Klippel-
Tibrewal S. at al. Phacomatosis Pigmentovascularis... www. dos-times.org 57
Case Reports
Inferior Rectus Loss and its Management
Following Trauma
Dr. Prachi Agashe DNB, ICO (UK), FPOS
Aravind Eye Hospital, Madurai, India
Abstract: A 15 year old boy presented with complaints of diplopia since 1 year following trauma to the right eye with a handle of bicycle
1 year back. He gave history of some mass being excised after which he manifested with a large hypertropia. His clinical features were
suggestive of a lost inferior rectus in the right eye with limited infraduction. He was managed with an `inverse Knapp procedure’ in the
right eye where full tendon width of the medial and lateral recti were transposed to the inferior rectus insertion. One year later the patient
was orthophoric in primary position but had diplopia in downgaze which was addressed by doing a Faden procedure of the inferior rectus
and posterior two third of superior oblique tenectomy of the left eye. Two years after presentation patient was orthophoric in primary
and reading position and had 4 PD of hypertropia in extreme downgaze.
Alost inferior rectus muscle is an uncommon Figure 1
problem reported after squint surgeries, trauma
or retinal detachment surgeries1. The resulting rectus insertion and the muscle was not found. With the FDT for
hypertropia with absence of infraduction superior rectus being negative an` inverse Knapp`s procedure’
causes diplopia in primary position and was performed with full tendons of the medial and lateral recti
downgaze which are gazes utilized in our daily being transposed near the inferior rectus insertion along the
activities. We report a case of severed inferior rectus managed spiral of Tillaux. Three months post operatively the hypertropia
by inverse Knapp`s procedure on that eye followed by Faden significantly reduced and one year post operatively the patient
of inferior rectus and posterior two third of superior oblique was orthophoric in primary position. The patient had diplopia
tenectomy (PTSO) of the normal eye to achieve binocular single in down gaze with limitation of depression in the right eye for
vision in primary position and downgaze. which the patient underwent Faden procedure on the inferior
rectus of the left eye. A 5-0 non absorbable polyester suture
Case Report was passed 14 mm from the insertion along the medial and
lateral border of the muscle fixing it to the sclera. This was
A 15 year old boy presented with complaints of diplopia accompanied with PTSO to further limit depression of the
since 1 year following penetrating trauma to the right eye by normal eye.
bicycle handle.
Two years post operatively the patient was orthophoric for
He was operated on that eye with some mass being excised, near and distance as well as in reading position but had a small
details of which were unavailable following which he developed hypertropia of 4 PD in extreme downgaze which the patient
hypertropia. was able to fuse with prisms (Figure 2).
His unaided visual acuity was 6/6 in both the eyes. Anterior Discussion
segment examination revealed wide palpebral aperture with
lower lid retraction of the right eye with inferior conjunctival Extraocular muscle transection or detachment following
scarring. surgery or trauma is uncommon. Various strategies have been
Hirschberg test revealed hypertropia of 15 degrees of the Agashe P. Inferior Rectus Loss and its Management Following Trauma
right eye. Ocular motility examination was remarkable with
severe limitation of depression in the right eye more marked
in abduction with consequent overaction of the left superior
oblique (Figure 1). Prism cover test measured a primary
deviation of 40 prism dioptres (PD) of right hypertropia for
near and distance with exotropia of 12 PD. The hypertropia
measured 18 PD in upgaze and was more than 50 PD in
downgaze. The hypertropia measured equal on either tilt.
Forced duction test (FDT) revealed no restriction to passive
depression and the forced generation test demonstrated no tug
on active infraduction.
CT scan showed focal atrophy of the inferior rectus near
the insertion while the distal portion was thinned out.
The patient underwent surgical correction of squint.
Surgical exploration showed fat infiltrated near the inferior
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