DOS Times Vol 26 No 1

Subspeciality-Pediatric Ophthalmology

Isotope Half life Radiation Energy Advantages Disadvantages
Cobalt 60 5.26 years type 1. Long half life
Gamma 1.17,1.33 Mev 2. Good tissue penetration 1. Difficult shielding
2. Less focused target
Iridium 192 74.2 days Gamma 0.38 Mev (avg) 1. Good tissue penetration dose
2. Easily customized 3. High energy, increases
the exposure to
Iodine 125 59.4 days Gamma 27-35 Mev 1. Easy to shield personnel
2. Appropriate tissue penetration Personal exposure higher
Palladium 103 16.99 days Gamma 21 Kev 3. Easily customized than I 125, Pd 103, Ru
Ruthenium-106 371.8 days Beta 3.5 Mev (max) Similar to Iodine 125 106.
1. Easy for shielding Shorter half-life
2. Larger therapeutic window compared to Co 60
3. Steep dose depth gradient
4. Less complications Very short half life
Limited tissue
penetration

The round plaques were used for many delay between the evaluation and regression pattern should be closely
years. The Notch design is extremely the assessment since the doses observed
helpful when the perimeter of the might require a revision in case the Complications
tumor overlaps or lies adjacent to the tumor dimensions change. The most common complication after
optic disc where the plaque provides 2. All surgeries are performed under the plaque brachytherapy is vitreous
a more conformal dosimetry coverage general anesthesia hemorrhage secondary to necrotic
of the tumor base and its surrounding 3. After a localized peritomy and tumor. The hemorrhage usually resolves
margin8. separation of the tenon’s fascia, in about 90% cases and the unresolving
the plaque is placed on the sclera cases might need enucleation.
Manufacture by suturing with nonabsorbable
Currently, the plaques are being sutures through the plaque eyelets 1. Radiation keratopathy.
delivered by BEBIG from Germany. 4. The plaque is inserted and left 2. Radiation cataract (2.5% at 1 year to
The Indian plaques are manufactured in position, keeping the patient
by Bhabha Atomic Research Centre admitted under the standard plaque 54.3% at 10 years) .
(BARC)9 and the modified designs are protocol adhering to the radiation 3. Radiation retinopathy or
under development. safety guidelines of Atomic
Energy Regulatory Board (AERB), papillopathy (15.4% at 1 year to
Surgical Procedure for Plaque Government of India. 21.6% at 5 years).
brachytherapy in Retinoblastoma: 5. The plaque removal is also done 4. Radiation optic neuropathy (8.6%
under general anesthesia. at 1 year to 21.2% at 5 years).
Pre-operative: Regression Patterns after Plaque 5. Radiation Vasculopathy.
Brachytherapy: 6. Scleral necrosis.
1. Evaluation of the tumor by indirect Results
ophthalmoscopy with scleral Different regression patterns have Tumor control of 79% has been
depression for the approximate been observed after the plaque reported in a 5 year follow up study.
distance of the tumor from the brachytherapy. Young patients without associated
optic disc and fovea. seeding showed a good response.
1. Flat scar Tumor recurrence of 4.2% at 1 year
2. Estimation of tumor base, height, 2. Partial or complete fish flesh and 7.1% at 5 years have been reported
width by ultrasonography. in a study. The clinical risk factors for
regression. Cases with such tumor recurrence have been predicted
Surgery as the older patient age and subretinal
1. Reassessment of the tumor size

before starting the surgery. Care
should be taken to minimize any

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 51

Subspeciality-Pediatric Ophthalmology

Figure 1: A,C,E are the fundus images of recurrent Retinoblastoma patients. B,D,F are the 3. American Brachytherapy Society -
fundus images showing excellent response to Plaque Brachytherapy with Ruthenium-106. Ophthalmic Oncology Task Force.
Electronic address: paulfinger@
or vitreous seeds10. of radiotherapy and predicted to occur eyecancer.com; ABS – OOTF Committee.
in patients with large tumors. The American Brachytherapy Society
Conclusion consensus guidelines for plaque
Secondary Plaque brachytherapy References brachytherapy of uveal melanoma
for Retinoblastoma has shown good 1. Shields CL, Shields JA, Cater J, Othman I, and retinoblastoma. Brachytherapy.
results and an eye salvage of about 2014;13(1):1-14.
90%. However, an appropriate case Singh AD, Micaily B. Plaque radiotherapy
selection, accurate tumor dimensions for retinoblastoma: Long-term tumor 4. Raksha Rao, Santosh G Honavar Recent
and a dedicated team approach can control and treatment complications Developments in Retinoblastoma. DJO
greatly improve the chances of eye in 208 tumors. Ophthalmology. 2016;27:50-61.
salvage especially in the refractory 2001;108:2116–21.
cases and one-eyed patients [Figure 2. Stallard HB. Doyne Memorial Lecture, 5. Hernandez JC, Brady LW, Shields CL,
1]. These patients should be closely 1962. The conservative treatment of Shields JA, DePotter P. Conservative
followed to monitor the complications retinoblastoma. Trans Ophthalmol Soc treatment of retinoblastoma. The use of
UK 1962;82:473-535. plaque brachytherapy. Am J Clin Oncol.
1993;16(5):397-401.

6. Schueler AO, Flühs D, Anastassiou G, et
al. Beta-ray brachytherapy with 106Ru
plaques for retinoblastoma. Int J Radiat
Oncol Biol Phys. 2006;65(4):1212-1221.

7. h t t p s : / / w w w . b e b i g . c o m / f i l e a d m i n /
bebig_neu/user_uploads/Products/
Ophthalmic_Brachytherapy/
Ophthalmic_Brochure__Rev._06__
English.pdf

8. https://www.eyephysics.com/PS/PS5/
UserGuide/NotchedPlaques.html

9. Suganeswari G. Update on Intraocular
Oncology, Sci J Med & Vis Res Foun
2015;XXXIII: 88–92.

10. Shields CL, Honavar SG, Shields JA, et al.
Factors predictive of recurrence of retinal
tumors, vitreous seeds, and subretinal
seeds following chemoreduction for
retinoblastoma. Arch Ophthalmol
2002;120:460 – 4.

Corresponding Author:

Dr. Harika Regani
Centre for Sight, Banjara Hills,
Hyderabad, India

52 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

Retinopathy of Prematurity
Screening

Parijat Chandra MD, DNB
Retinopathy of Prematurity Services, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences,
New Delhi, India.

Retinopathy of prematurity (ROP) to develop effective ROP screening Figure 1: RBSK ROP Screening Guidelines.
is an important cause of childhood programme which is the key to prevent
blindness worldwide, and since it ROP blindness. place for ROP screening as the attending
affects many preterm babies, screening paediatrician can monitor the child
is the only way to detect it in time. It is ROP screening guidelines systemically and intervene if required. It
one of the very few ophthalmological Rashtriya Bal Suraksha Karyakram also provides a safe clean environment
diseases where screening programmes (RBSK), Ministry of Health, for the preterm baby. However, in many
have been greatly beneficial to control Government of India, with support ophthalmic hospitals, ROP screening
disease morbidity. of the National Program for Control may be done in a separate dedicated
India is having the highest number of Blindness, released the national area, where appropriate cleanliness
of preterm births in the world, which ROP screening guidelines6 which measures, optimal temperature control
means we have a larger number of are now widely followed across the and emergency equipment is available.
babies to screen1. In addition to that, country. These guidelines serve a useful Typically, a date and time is fixed once
we are in the third epidemic of ROP, purpose for paediatricians as well as a week in the NICU/ROP clinic for ROP
wherein not only smaller preterm ophthalmologist’s and help them to screening.
babies are developing ROP like in run an effective ROP screening program ROP screening procedure
developed countries, but bigger babies (Figure 1). Preparation for Screening - The NICU
are also developing ROP due to poor Whom to screen? All babies <2000g team identifies the babies which need
NICU care, which further increases birthweight (BW) and/or <34 wks ROP screening as per the ROP screening
the burden of ROP screening as a larger gestation age (GA) should be screened. guidelines. If performed in the eye care
number of babies have to be screened2,3. Bigger babies 34-36 wks GA can also be
Not only is there a lack of ROP screening screened if they have notable systemic
programmes across the country, but risk factors for developing ROP.
studies on the knowledge, attitude It is notable that the screening guidelines
and practices of paediatricians across in Asian Region7 are much higher than
the country reveal lack of knowledge American ROP screening guidelines8
and understanding about the disease4. which recommend screening smaller
This lack of infrastructure and trained babies with <30 weeks gestational age
manpower for ROP screening leads to (GA)/<1500 g birth weight (BW).
delayed or late presentation of these When to screen? First screening is
babies with blinding disease. A large advised at 4 weeks/30 days after birth.
number of preterm babies in India ‘Tis din Roshni ke’ is a good motto
continue to go blind with end-stage 5 to remember this. However, in very
ROP due to lack of screening/treatment5. preterm and very low birth weight
It is essential that every NICU should babies (<28 wks GA and <1200g BW)
have an ROP screening programme it can be done as early as 2-3 weeks,
such that all babies under their care are to detect Aggressive Posterior ROP
appropriately screened and blindness (APROP)9, a challenging ROP variant.
can be prevented. Paediatricians need Where to screen? The NICU is the best
to collaborate with ophthalmologists

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 53

Subspeciality-Pediatric Ophthalmology

setting, the processes can be optimized Figure 2: Zones of ROP. dragging etc. A note can be put about
to reduce waiting times10. ROP screening comparison with earlier findings and
is a relatively quick procedure and is done at 1-2 weeks depending on the if disease is progressing or regressing.
can be easily completed within a few zone and stage of the disease as well as The finding should be legibly written
minutes by a trained expert. It is very the post conceptional age of the baby. in detail while avoiding abbreviations.
cost effective and requires only an Earlier follow up may also be required if The next date for follow up should be
indirect ophthalmoscopy, a 28/30D lens, a closer watch on the disease is needed. clearly mentioned on patient records
paediatric speculum, and a paediatric Typically, once treatment is indicated, and explained.
indenter. it should be done without delay.
The Early Treatment ROP (ETROP) Detailed notes help the new/follow up
The child is kept fasting for one hour group12 divided ROP into 2 types to specialist clearly understand the disease
prior to the procedure. Pupillary suggest when treatment is needed. status as many babies get discharged
dilating drops (0.5% tropicamide + Type I ROP – Needs to be treated from NICU and follow up with different
2.5% phenylephrine eye drops) are • Zone I, any stage ROP with plus doctors. In case of nonavailability of the
put half an hour before the procedure, screening specialist, a backup doctor
2 times at 10 minute intervals. Care disease or must be available to perform ROP
should be taken to occlude the puncta • Zone I, stage 3, with or without plus screening, or referral should be done so
to minimize systemic drug absorption, that treatable disease is not missed.
wipe off excess drops, and ensure the disease or
drug is not put repeatedly. • Zone II, stage 2 or 3 ROP, with plus Proper documentation avoids
medicolegal hassles, and it should
Screening steps – Proparacaine disease always be well explained to the
eye drops are instilled for topical Type II ROP – Follow up and treat when parents/guardians so that they clearly
anaesthesia. The sterile paediatric eyelid progresses to type I understand the nature of the disease,
speculum is inserted. First examine the • Zone I, stage 1 or 2 with no plus extent of the disease, and the need for
anterior segment for cornea clarity, regular follow-up/treatment.
occurrence of tunica vasculosa lentis disease or
and extent of pupil dilation. If the • Zone II, stage 3 with no plus disease Digital wide-field screening
pupil does not dilate well, it could be Recording ROP data - It is especially Over the years many wide-field retinal
due to plus disease from severe ROP, important to record all the ROP cameras have become available which
and persistent attempt must be made examination findings in a well- allow ROP specialists to take retinal
to dilate the pupil and examine the prepared proforma. It is essential to photographs and document the
fundus, rather than delaying screening document the date of procedure, post findings. The retinal cameras provide
as disease may worsen. conceptional age, clinical status of exceptional wide field viewing and can
disease (zone, stage, plus disease, clock be performed by a trained technician
Then the posterior pole is examined hours of involvement), as well as other or nurse, and a surgeon is not always
to look for media clarity, plus disease signs of developing traction, pre-retinal/ needed. Further these cameras are
(dilation and tortuosity of posterior pole retinal haemorrhage, retinal/macular
vessels) as well as posterior zone disease
(Figure 2). Thereafter all clock hours of
the retinal periphery can be examined
to look for disease stage in various
quadrants and this is documented as per
the International ROP Classification11.
Scleral indentation is an important
component of retinal examination. It
helps to rotate and stabilise the eye,
indent to examine areas of interest, and
requires an experienced examiner.

Follow up screening - Follow up is
a very important component of ROP
screening, and the baby has to repeatedly
come for multiple visits to detect
disease progression/regression and get
timely treatment. Typically follow up

54 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

portable and they can be positioned in Figure 3: Retcam wide-field imaging opinion. This optimizes resources and
NICU, ROP clinic’s, operation theatres camera system. reduces dependence on eye specialists
etc. to regularly visit. This may totally
The retinal imaging is useful not only warranted ROP as observed in the change the way ROP screening is done
for documentation but also for training Stanford University Network for in the future.
of residents and provides a better way to Diagnosis of Retinopathy of Prematurity
explain to the parents about the disease (SUNDROP) initiative14. It is also In conclusion, since ROP screening is the
of their child. Multiple images across notable that newer technologies like key step for detection of ROP in time, it
various follow-up sessions provide artificial intelligence and deep learning is essential that every NICU must have
a good way to document disease are being used to analyse large data sets a screening program. Obstetricians
progression or regression. of images and predict the development may also be engaged to play a key role
The Retcam (Natus Medical Inc., USA) is of severe ROP, which may also change by counselling mothers undergoing
a popular camera system which involves the screening criteria in the future. preterm labour about the need of eye
a contact procedure wherein the hand Neonatology Led Screening - As there screening after birth to detect ROP.
held camera is placed on the cornea will never be enough eye specialists ROP screening is a simple cost-effective
(Figure 3). The eyes are prior dilated for screening in the community on strategy and is essential to prevent ROP
and a coupling gel like methylcellulose a mass scale, and considering the blindness.
is used. The camera system allows growing burden of ROP screening,
changing focus and brightness to get a new concept of Neonatology led References
the optimal image clarity. Typically, screening is emerging15. The screening 1. Blencowe H, Moxon S, Gilbert C. Update
they allow 130 degrees wide field view is performed using retinal cameras by
and the entire retina can be visualized trained paediatric nurses/technicians, on Blindness Due to Retinopathy of
and documented in a few photos. Video and the eye specialist is consulted Prematurity Globally and in India.
recording, fluorescein angiography, only when there is a need for further Indian Pediatr. 2016 Nov 7;53 Suppl
image database management are some 2:S89–92.
other useful features. 2. Gilbert C. Retinopathy of prematurity:
a global perspective of the epidemics,
ROP Tele-screening population of babies at risk and
A major benefit of wide-field digital implications for control. Early Hum
retinal imaging cameras has been Dev. 2008 Feb;84(2):77–82.
the development of tele-screening 3. Gilbert C, Fielder A, Gordillo L,
programs. This has widely expanded the Quinn G, Semiglia R, Visintin P, et al.
reach of the ROP screening programs Characteristics of infants with severe
to the community at large. The retinal retinopathy of prematurity in countries
cameras maybe located at peripheral with low, moderate, and high levels
centers or in mobile vans and are of development: implications for
operated by trained technicians. The screening programs. Pediatrics. 2005
images are transmitted via the Internet May;115(5):e518-525.
to reading centers or experts located in 4. Patwardhan SD, Azad R, Gogia V,
central eye care facilities who can then Chandra P, Gupta S. Prevailing
opine regarding further follow up or clinical practices regarding screening
treatment. Karnataka Internet Assisted for retinopathy of prematurity
Diagnosis of ROP (KIDROP) model13 in among pediatricians in India: a pilot
India is an example of such a successful survey. Indian J Ophthalmol. 2011
program. With the availability of Dec;59(6):427–30.
cheaper retinal cameras manufactured 5. Azad R, Chandra P, Gangwe A, Kumar
locally like the 3nethra Neo (Forus V. Lack of Screening Underlies Most
Health, India), the popularity of these Stage-5 Retinopathy of Prematurity
programs is set to increase in the among Cases Presenting to a Tertiary
future. Eye Center in India. Indian Pediatr. 2016
Digital screening has showed Nov 7;53 Suppl 2:S103–6.
significantly high specificity and 6. Guidelines for Universal Eye Screening
sensitivity to detect treatment in Newborns Including Retinopathy
of Prematurity [Internet]. Rashtriya bal
swasthya karyakram; 2017 [cited 2020
Jul 1]. Available from: https://www.nhm.
gov.in/images/pdf/programmes/RBSK/
Resource_Documents/Revised_ROP_

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 55

Subspeciality-Pediatric Ophthalmology

Guidelines-Web_Optimized.pdf Retinopathy of Prematurity Clinic: A 14. Wang SK, Callaway NF, Wallenstein
Quality Improvement Project. Indian MB, Henderson MT, Leng T, Moshfeghi
7. Sen P, Wu W-C, Chandra P, Vinekar Pediatr. 2018 Sep 15;55(9):776–9. DM. SUNDROP: six years of screening
A, Manchegowda PT, Bhende P. for retinopathy of prematurity with
Retinopathy of prematurity treatment: 11. International Committee for the telemedicine. Can J Ophthalmol J Can
Asian perspectives. Eye Lond Engl. Classification of Retinopathy of Ophtalmol. 2015 Apr;50(2):101–6.
2020;34(4):632–42. Prematurity. The International
Classification of Retinopathy of 15. Gilbert C, Wormald R, Fielder A, Deorari
8. Fierson WM, American Academy of Prematurity revisited. Arch Ophthalmol. A, Zepeda-Romero LC, Quinn G, et al.
Pediatrics Section on Ophthalmology, 2005 Jul;123(7):991–9. Potential for a paradigm change in the
American Academy of Ophthalmology, detection of retinopathy of prematurity
American Association for Pediatric 12. Good WV, Early Treatment for requiring treatment. Arch Dis Child
Ophthalmology and Strabismus, Retinopathy of Prematurity Fetal Neonatal Ed. 2016 Jan;101(1):6–9.
American Association of Certified Cooperative Group. Final results of
Orthoptists. Screening examination the Early Treatment for Retinopathy Corresponding Author:
of premature infants for retinopathy of Prematurity (ETROP) randomized
of prematurity. Pediatrics. 2013 trial. Trans Am Ophthalmol Soc. Prof. Parijat Chandra MD, DNB
Jan;131(1):189–95. 2004;102:233–48; discussion 248-250. Dr. Rajendra Prasad Centre for Ophthalmic
Sciences, All India Institute of Medical Sciences,
9. Chandra P, Tewari R, Jain S. The 13. Vinekar A, Jayadev C, Mangalesh S, New Delhi, India.
restless retina in aggressive posterior Shetty B, Vidyasagar D. Role of tele-
retinopathy of prematurity: prevention medicine in retinopathy of prematurity
is better than cure. Community Eye screening in rural outreach centers
Health. 2018;31(101):S27–8. in India - a report of 20,214 imaging
sessions in the KIDROP program. Semin
10. Chandra P, Kumawat D, Tewari R, Fetal Neonatal Med. 2015 Oct;20(5):335–
Panyala RR, Sreeshankar SS. Reducing 45.
Waiting-time of Preterm Babies at a

56 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

Laser in Retinopathy of
Prematurity - Lessons to Learn

Abhidnya Surve, Saurabh Verma, Parmanand Kumar, Shorya Vardhan Azad, Rajpal Vohra
Vitreo-Retinal Services, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS , New Delhi, India.

Retinopathy of prematurity (ROP) is a during PPV, laser of avascular retina disease. This regression occurs post laser
preventable retinal disorder occurring is performed in addition to removal of and hence time is required for complete
in preterm babies which can lead to traction. Posterior barrage laser may be effect of laser to set (2-3 weeks).
significant vision loss. Appropriate required at the posterior edge where
neonatal care, timely screening, early there is significant residual traction and Requirements for laser treatment
detection and appropriate treatment of is at risk of progression. This delimits 1. Written informed consent is taken
ROP are important facets. The treatment and prevents progression to retinal
options for ROP include cryotherapy, detachment. from the parents explaining them
laser therapy, pharmacotherapy and the advantages, risk and side-effects
pars plana vitrectomy (PPV) depending In stage 4A ROP where there is a partial of laser.
on the zone, stage and severity(plus) of tractional detachment not involving 2. Low hemoglobin babies are at
disease. Cryotherapy was first described macula, vitrectomy has success rate of risk of recurrent apnea and can
for treatment of threshold ROP but had upto 90% but it carries risk of cataract, desaturate during laser. Hence
various side-effects including severe hemorrhage, glaucoma and retinal hemoglobin > 8 is considered to
post-operative pain, inflammation, detachment2,3. However, studies have be taken up for laser. If < 8, baby
adnexal edema, and exudative retinal shown laser treatment alone can lead to need to be transfused to maintain
detachment. Laser is a well-established regression and decrease in fibrovascular hemoglobin levels.
technique used for various retinal traction in 44.44%-92.85% cases with 3. Child is to be kept fasting for
and vascular disorders. In ROP, laser stage 4A and avoid vitrectomy4,5. The two hours before laser to avoid
treatment in addition to having lesser main goal is to stop progression to any complications related to
side-effects has been time-tested and stage 4B or 5. In stage 4A, cases with regurgitation during laser.
an effective procedure. Laser in ROP is less than 6 clock hours of involvement, 4. Pupils are dilated with 1%
different from that for adults, having a plus disease, less than two clock hour tropicamide – 2.5% phenylephrine
different technique and requirement. involvement, and clear vitreous were topical drops. The drops should
Herein, we have compiled the seen to more likely respond. Further, be instilled, one drop 3 times, 10
indications, technique, and response previously lasered eyes fair better with minutes apart starting half an hour
to laser to help a novice while treating lens sparing vitrectomy as compared prior to the procedure.
ROP. to treatment naïve eyes as it decreases 5. Laser is performed in a neonatal
disease activity and lesser intraoperative intensive care unit (NICU) or
Indication and post-operative bleeding6. pediatric high dependency unit
ETROP has laid guidelines as to which (PHDU) setup where the oxygen
cases require laser and classified them Mechanism of action support, suction apparatus,
as type 1/high-risk ETROP. Those The aim of laser is to ablate the resuscitation equipment and
requiring observation are classified peripheral avascular retina. Ensuing intubation equipment back up is
as type2/low-risk ETROP1. The type 1 chorioretinal scarring converts the available along with a pediatrician
category includes - Zone 1 ROP with hypoxic to anoxic area. This inturn monitoring child’s systemic status
any stage with plus disease or Zone 1 decreases vascular endothelial growth (Figure 1).
ROP with stage 3 without plus or Zone factor (VEGF) produced by the ischemic 6. Special warmers should be available
2 ROP with stage 2-3 with plus. In retina. Once VEGF decreases, the to keep the child warm to avoid
addition, laser is also performed in cases drive for angiogenesis at the border hypothermia.
with APROP in Zone 2 and in Zone 1 of vascularised and avascular retina 7. ECG leads and pulse oximeter is
where injection cannot be given. Also, decreases leading to regression of the

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 57

Subspeciality-Pediatric Ophthalmology

Figure 1: Complete setup (a-d) for laser in a preterm child with temperature regulator (b,c), increased upto 5µg/kg/hour. With
oxygen supply and vacuum for suction (b-red arrow), infusion pump for constant infusion fentanyl, there can be decrease in
of fluids at specific rate (b-green arrow), monitoring equipment (d), bed with walls and the heart rate which needs monitoring.
laser indirect ophthalmoscope system (e). Maintenance intravenous fluids
can be started according to age and
attached to monitor heart rate and general anesthesia (GA). Though weight.
oxygen saturation. The heart rate GA is comfortable for both the
in a child is higher as compared to ophthalmologist and the child, 12. Topical paracaine 0.5% for
adults and maintained at a level non-availability of expert neonatal anesthetic effect, should be started
120-160. The oxygen saturation is anesthesiologists, anesthesia related at least 10 minutes before starting
to be maintained above 95%. Also, risk, morbidity and mortality in the procedure.
one should monitor for any signs of small babies remain a concern.
respiratory distress or lethargy. Systemic risk of developing anemia, 13. A crying baby with moving limbs is
8. The child should be wearing diaper sepsis, pneumonia, respiratory more assuring than a quiet child in
and wrapped in a fresh thick cloth problem are other significant issues. terms of systemic stability.
to keep the baby warm and decrease Considering above, laser under
movements of limbs (Figure 2a). sedation or topical anesthesia is 14. A sterile set of speculum and
9. An intravenous cannula is placed comparatively safer. indentor along with 28 or 30
with stabilization of the limb 11. Fentanyl is sedative of choice with Diopter lens is required (Figure
(Figure 2b). A long tubing with lesser complication as compared 2d,e). Also, the assistant and the
three-way cannula is attached to to ketamine which has higher other staff members around should
it and kept out of the cloth (Figure chance of dissociative anesthesia be wearing protective glasses
2c). This helps in administrating and morphine with risk of CNS during laser (Figure 2f).
drugs and fluids when required. depression. A fentanyl bolus is
10. Laser is done generally under given at the start of the laser at the Technique
topical anesthesia or sedation dose of 1µg/kg followed by infusion
to avoid need and availability of at rate of 1µg/kg/hour. It can be Laser indirect ophthalmoscopy (LIO) is
used to do laser in ROP babies (Figure
1e). The different wavelengths of laser
which have been used are argon green
(514nm) laser, double frequency (df) Nd-
YAG laser (532nm), and diode red laser
(810nm). Different lasers have different
properties and can cause various side
effects. In our setup, we usually use df
Nd-YAG system. The laser parameters
are spot size - 200u, duration - 150-
200ms and interval - 150-300ms. Power
is started around 110-130 mW and then
increased according to the laser reaction.
The laser reaction should be light grey
and not dirty white. The size of laser
burn can be varied depending on the
distance from the eye. The spots should
be nearly confluent that is the distance
between two spots to be kept about
half the spot size. Study comparing the
two laser patterns, less confluent and
confluent, a progression rate of 29.4%
was seen in former as compared to
3.6% in latter.(7) The avascular retina
from the ridge to the ora, 360 degree
is lasered. Importantly in APROP,
presence of avascular loops posterior
to line of vascularized retina harbor
ischemic retina which needs to be
lasered. The total number of laser spots
vary between 1000-4000, depending on

58 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

Figure 2: Baby wrapped in warm cloth (a) to restrict movements. Also note a cotton soaked laser. This allows maximum energy
in dextrose in mouth (a) considered as sucrose analgesia. An IV cannula placed (b) at limb transfer to the treated area and
which is further stabilized with bandage. A long tube with three-way cannula (c) connected avoids dispersion of energy.
to it which has an access outside the cloth wrapped for administration of fluid and drugs. A 5. The more posteriorly the avascular
sterile set of instruments (d) including speculum and indentor required (e). Also, protective retina encroaches, the more is the
glasses (f) to be worn during laser by assisting staff. ischemic stimulus. One should start
with temporal side as the avascular
avascular area needing treatment. The with a sharp focus on retina as any area is larger, more posterior and in
laser should be preferably completed in inclination will not allow effective closer relation to macula.
one sitting if the child is stable as any 6. The posterior area is lasered
delay in the treatment may adversely first after stabilizing the eye
affect the outcome. with indenter, as it allows easier
application of laser with minimum
manipulation. One can complete
the visible area and proceed to mid-
periphery and anterior retina in
that clock hour and then repeat the
same for adjacent clock hours.
7. In case of non-visibility of a
particular area, one can continue
from other end and come back later
to this area from other side.
8. Once eye is indented and laser
initiated, one should try to complete
laser in that visible area as much as
possible. Laser of different areas at
different time can cause skip areas

Important points during laser Figure 3: Adjust eyepiece (a), circle diameter, light required and width of the laser beam (b,c)
to have proper visualization of retina and focus the laser beam on retina for appropriate
1. Sterile pediatric speculum laser reaction. The head should be adjusted and positioned such that laser beam is in straight
(Alphonso type) allows lid to be line through the cornea to the area of focus (d). Indentor (e) is used for stabilization of the eye
separated sufficiently for laser. and indentation of anterior area for visualization of periphery. Post laser congestion and
mild lid edema is common (f).
2. One should be acquainted with
the settings of a LIO, including
adjustment of eye piece, head
adjustment, illumination area,
light intensity, and focusing beam
(Figure 3a).

3. The laser beam, visualized as the
helium neon red light, helps in
focusing the laser on retina. The
beam size can be adjusted for better
focus during laser depending on the
area to be treated. A wider beam for
posterior retina and a sharper one
for anterior retina can help to focus
during laser (Figure 3b,c), especially
in periphery.

4. The beam should fall perpendicular

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 59

Subspeciality-Pediatric Ophthalmology

because of surrounding retinal summit and its posterior slope then progress anteriorly.
edema, hence, laser should proceed difficult. 21. At the end of procedure, the eyelids
from one clock hour to the adjacent 14. Avoid hitting the retinal
one with continuity of laser burns. vasculature and the fibrovascular should be wiped off after removing
proliferation at the ridge with laser speculum and antibiotic instilled.
9. A high intensity burn should be as it can lead to bleeding. The child is kept under monitoring
avoided as it can lead to formation 15. Frequent lubrication of the after the procedure and observed
of iatrogenic break. The setting of eye during the course of laser for any breathing difficulties. Baby
laser is reduced for anterior retina, prevents cornea from drying (but is handed over to the parents only
as it is thin. excess water is to be removed as after the baby is stable and has
it can cause significant reflexes). accepted feed atleast twice.
10. While lasering the periphery, Sometimes cornea edema may 22. Parents need to be explained that lid
laser beam needs to be aligned in a develop as we proceed with laser edema, chemosis, subconjunctival
straight line as steeper peripheral because of either indentation or the hemorrhage, and erythema would
corneal curvature may cause inflammation caused by laser. Use be present for 3-4 days post laser
problems in visualization (Figure of hyperosmolar eyedrops can help to avoid any undue concerns
3d). Also, laser is applied only as in decreasing the same and allow (Figure 3f). Topical antibiotics and
anterior as the ora serrata, avoiding uninterrupted laser. lubricants can be prescribed for one
any area anterior to it as it can 16. Lens used in ROP are 28D or 30D week. In case with severe edema,
lead to severe inflammation. One lens. Hand used for holding 28/30D mild potency steroids would
may keep adjusting the speculum can also help to stabilize the chin decrease the inflammation. These
in direction of the laser to allow and head with other fingers. smaller changes can help one to
maximum separation of lids and 17. For posterior barrage laser, 20D perform complete, effective laser
effective indentation without lens is preferred because its higher with minimum manipulation and
manipulations at other sites. magnification allows better trauma to the eye.
visibility of retinal vessels which
11. In infants, it is important to keep needs to be avoided while lasering. Response of laser
in mind that pars-plana develops Two rows of nearly confluent laser Laser has been the gold standard
post-natal and hence ora serrata spots are applied half-disc diameter of treatment in ROP with disease
lies anteriorly at distance lesser 2.5 posterior to the area of traction. regression seen in 71-100% cases7,9.
to 3 mm posterior to the limbus 18. Dextrose (10%) soaked cotton Zone 1 disease, APROP or severe plus
in children less than 6 months of can be used as a pacifier and is disease need to be followed up closely
age as compared to 4.5-5.5mm in considered as sucrose analgesia. in 3-4 days as compared to one week
adults. Thus while indenting the 19. At the end of laser treatment, the in cases with milder form or zone 2
anterior area for laser, the indenter retina needs to be inspected 360 involvement. Sign such as clearing
is placed about 1-2 mm posterior to degrees for any skip areas. of media, full dilatation of pupil,
the limbus (Figure 3e)8. 20. In cases with APROP, laser may be regression of neovascularization,
difficult because of non-dilating decrease in fibrovascularisation at
12. Manipulation should be kept pupil, presence of media haze, the ridge, clearing of hemorrhages,
to minimum, as excessive corneal haze, vascular loops and decrease in traction, and the decrease
manipulation and indentation can no ridge delineating the vascular in arteriolar tortuosity and venular
lead to conjunctival and corneal and avascular retina. FFA can aid dilatation (plus disease) indicate
edema. in delineating additional avascular response to treatment (Figure 4).
areas within the loops which Although, regression starts early at
13. Indenter can be used need laser for disease regression. around 3-4 days but it takes another 2-3
interchangeably to stabilize the Although, it might be difficult to weeks for the complete effect to come.
globe, rotate the globe, and to laser the anterior retina in a non- Hence, if there are no signs of response
indent anterior retina. However, dilating pupil, pupillary dilatation or there is worsening on follow-up, one
high indentation can cause increase has been seen to improve with should check for skip area and retreat
in intraocular pressure and corneal indentation (due to mechanical if required. Once the disease regresses,
haziness. It may also result in stimulation of nerves). In such follow-up to be maintained every 3-4
undesirable rotation leading to cases it may be prudent to laser the weeks till 50-60 weeks post-conception
further difficulty in laser. Besides, posterior visible retina first and age depending on fundus status. This is
sudden decompression from high shorter as compared to longer follow-
pressure after removing indentor up and risk of late recurrences in cases
can end in pupillary constriction receiving anti-VEGF therapy. After
and hemorrhage. Excessive
indentation makes laser at the

60 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

60-weeks, refraction should be done Figure 4: 30-week-old child with right eye (a-b) and left eye (d-e) Zone II, Stage 2-3 ROP with
under atropine every 6 months to pick plus disease. Posterior pole (a,d) showing plus disease and peripheral area (b,d) showing
up myopic refractive error10. righe with fibrovascular proliferation (arrow). Post laser 2 months showing regression of
disease with laser marks visible (arrow).
Advantages of Laser
photocoagulation to disease progression. Hence, it of ROP, one can achieve better outcomes
1. Lesser incidence of post-operative is important to identify these skip anatomically and functionally.
areas and ablate them. Posterior Although, laser treatment is time tested,
pain, inflammation, adnexal edema, skip areas have greater effect effective, well-established method for
and exudative retinal detachment on disease regression rates than treating ROP, is difficult to perform by
along with better visual outcomes anterior ones. a novice and requires a learning curve.
as compared to cryotherapy11,12. 4. Anatomical injury - Cornea and iris This article aims to help in this aspect.
2. Can be safely performed under may sustain laser burns. Cataract
topical anesthesia and/or sedation can occur in up to 1 % treated cases References
without risk of exposure to GA. and vary from transient lenticular 1. Early Treatment For Retinopathy
3. Laser can be repeated at short opacities to total cataract17,18.
intervals in cases having skip areas Absorption of the laser by persistent Of Prematurity Cooperative Group.
or need of laser augmentation or TVL, severe inflammatory reaction, Revised indications for the treatment
requirement of posterior barrage anterior segment ischemia, uveal of retinopathy of prematurity: results
laser. effusion syndrome or long standing of the early treatment for retinopathy
4. Laser can be easily performed in retinal detachment are various of prematurity randomized trial. Arch
incubator even through its wall13. causes of cataract in a baby with Ophthalmol. 2003;121:1684–94.
This has gained more importance in ROP. 2. Ferrone PJ, Harrison C, Trese MT. Lens
present times of COVID pandemic 5. Anterior segment ischemia, though Clarity after Lens-sparing Vitrectomy in
for performing safe treatment in rare can occur with ablation of long a Pediatric Population. Ophthalmology.
these preterm children. posterior ciliary artery, prolonged 1997;104:273–8.
scleral depression, or inadvertent 3. Wu W-C, Lai C-C, Chen K-J, Hwang Y-S,
Side effects laser of ciliary body area. It was Chen T-L, Chao A-N, et al. Vitrectomy
1. Once avascular retina is lasered, more commonly seen in smaller for Stage 4A Retinopathy of Prematurity
babies and can lead to hypotony, (ROP). Invest Ophthalmol Vis Sci.
the retinal function is lost and this cataract, pupillary membranes 2009;50:3136–3136.
in turn affects the field of vision or phthisis bulbi19,20. Confluent 4. Sukgen EA, Koçluk Y. Treatment for
maintained by the child for life. laser pattern is not found to be a stage 4A retinopathy of prematurity:
2. Myopia of prematurity is known contributing factor. laser and/or ranibizumab. Graefes Arch
to occur because of the anterior With appropriate and timely treatment Clin Exp Ophthalmol. 2017;255:263–9.
segment changes, changes 5. Narnaware SH, Bawankule PK. Role
associated with treatment or that of LASERS in stage 4A retinopathy of
associated with spontaneously prematurity (ROP). Lasers Med Sci. 2020
regressed ROP. Studies have shown
cases undergoing more laser have
higher degree of myopia. In ETROP
study, the prevalence of high
myopia was approximately 35%-
79% respectively with zone1 having
higher prevalence of myopia14. The
underlying mechanism of same is
however still poorly understood.
Anterior chamber changes with
steeper keratometry, greater lens
thickness, shallower anterior
chamber, affected posterior ocular
growth or chronic inflammation
may be the contributing factors15,16.
3. Inadequate laser – Unlasered
avascular areas remain a stimulus
for the VEGF production leading

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 61

Subspeciality-Pediatric Ophthalmology

Sep 3. doi: 10.1007/s10103-020-03130-y. and cryotherapy in prethreshold 17. Christiansen SP, Bradford JD. Cataract
Epub ahead of print. PMID: 32880773. retinopathy of prematurity. Clin in infants treated with argon laser
Experiment Ophthalmol. 2004;32:251– photocoagulation for threshold
6. Gadkari SS, Deshpande M. Variation 4. retinopathy of prematurity. Am J
in the vitreoretinal configuration of Ophthalmol. 1995;119:175–80.
Stage 4 retinopathy of prematurity in 12. Good WV, Early Treatment for
photocoagulated and treatment naive Retinopathy of Prematurity 18. Drack AV, Burke JP, Pulido JS, Keech
eyes undergoing vitrectomy. Indian J Cooperative Group. Final results of RV. Transient punctate lenticular
Ophthalmol. 2017;65:846–52. the Early Treatment for Retinopathy opacities as a complication of argon
of Prematurity (ETROP) randomized laser photoablation in an infant with
7. Banach MJ, Ferrone PJ, Trese MT. trial. Trans Am Ophthalmol Soc. retinopathy of prematurity. Am J
A comparison of dense versus less 2004;102:233–48;248-250. Ophthalmol. 1992;113:583–4.
dense diode laser photocoagulation
patterns for threshold retinopathy 13. Dogra MR, Vinekar A, Viswanathan K, 19. Kaiser RS, Trese MT. Iris atrophy,
of prematurity. Ophthalmology. Sangtam T, Das P, Gupta A, et al. Laser cataracts, and hypotony following
2000;107:324–7; discussion 328. treatment for retinopathy of prematurity peripheral ablation for threshold
through the incubator wall. Ophthalmic retinopathy of prematurity. Arch
8. Hairston RJ, Maguire AM, Vitale S, Surg Lasers Imaging. 2008;39:350–2. Ophthalmol. 2001;119:615–7.
Green WR. Morphometric analysis of
pars plana development in humans. 14. Geloneck MM, Chuang AZ, Clark WL, 20. Gaitan JR, Berrocal AM, Murray TG,
Retina (Philadelphia, Pa). 1997;17:135–8. Hunt MG, Norman AA, Packwood EA, Hess D, Johnson RA, Mavrofrides EC.
et al. Refractive outcomes following Anterior segment ischemia following
9. Tsitsis T, Tasman W, McNamara bevacizumab monotherapy compared laser therapy for threshold retinopathy
JA, Brown G, Vander J. Diode laser with conventional laser treatment: of prematurity. RETINA. 2008;28:S55.
photocoagulation for retinopathy of a randomized clinical trial. JAMA
prematurity. Trans Am Ophthalmol Soc. Ophthalmol. 2014;132:1327–33. Corresponding Author:
1997;95:231–6;237-245.
15. Kaur S, Sukhija J, Katoch D, Sharma M, Dr. Abhidnya Surve
10. Quinn GE, Dobson V, Davitt BV, Wallace Samanta R, Dogra MR. Refractive and Vitreo-Retinal Service,
DK, Hardy RJ, Tung B, et al. Progression ocular biometric profile of children Dr. Rajendra Prasad Centre for Ophthalmic
of myopia and high myopia in the with a history of laser treatment for Sciences, AIIMS , New Delhi, India
Early Treatment for Retinopathy of retinopathy of prematurity. Indian
Prematurity Study: Findings at 4 to Journal of Ophthalmology. 2017;65:835.
6 years of age. Journal of American
Association for Pediatric Ophthalmology 16. Lin H-J, Wei C-C, Chang C-Y, Chen T-H,
and Strabismus {JAAPOS}. 2013;17:124– Hsu Y-A, Hsieh Y-C, et al. Role of Chronic
8. Inflammation in Myopia Progression:
Clinical Evidence and Experimental
11. Azad RV, Pasumala L, Kumar H, Talwar Validation. EBioMedicine. 2016;10:269–
D, Pal R, Paul VK, et al. Prospective 81.
randomized evaluation of diode-laser

62 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

Paediatric Ocular Biometry and
IOL Power Calculation

Mousumi Banerjee, Sudarshan Khokhar
Pediatric Cataracts and Refractive Surgery Services, Dr. Rajendra Prasad Centre for Ophthalmic Sciences. AIIMS, New Delhi, India.

As the axial length of the eye grows in proportion with the lens and cornea, the refraction of a normal infant’s eye stays close to emmetropia.
Gordon and Donzis’s cross sectional biometric study of 148 normal eyes found on average that the axial length increased from 16.8 to 23.6
mm from birth to adulthood, whereas the refraction changed minimally, from +0.4 to – 0.5 D. This is primarily because the power of the
crystalline lens declined from +34.4 to +18.8 D. In contrast, aphakic eyes have a decline in hyperopia of 10D over the same period. As there
is no proportionally changing lens to compensate for the ocular growth; aphakic eyes have a large myopic shift.

Childhood blindness is a public health • Keratometry steeply reduces in without the speculum as there may be
concern across the world due to its deep the first 6 months (-0.40 D/month), erroneous readings due to deforming
impact on psychological, emotional, -0.14 D/month in the next 6 months, effect of the speculum on the globe.
and socioeconomic growth of the and -0.08 D/month in the second
family. Paediatric cataract is a treatable year, reaching the adult range at Axial length can be measured with both
leading cause of childhood blindness. It about 3 years of age6. Thus , mean immersion and applanation A scan,
accounts for 7.4%–15.3% of paediatric corneal power at birth is 51.2D however , immersion method is more
blindness and a significant avertable which reduces to a mean of 43.5D accurate as it avoids compression of the
disability-adjusted life years1,2. by around 3 years of age7. anterior corneal surface. Applanation
An accurate refractive outcome after method gives average 0.27 mm (0.24mm
primary IOL implantation is crucial to • Lenticular power decreases from -0.32 mm) lesser AL measurements
avoid a large myopic shift in later life. a mean of 34 D at birth to 28 D by than immersion technique. Hence,
Numerous studies have found that 6 months, gradually reaching the if immersion scan is not possible, an
IOL formulas for adult cataract surgery adult value of 20 D8. average of ten readings with maximum
are less accurate in children. In adults, anterior chamber depth should be taken
modern theoretical formulas are Biometry into account.
accurate within approximately 0.5 1. Conventional ultrasound biometry
diopters (D); in children various studies Keratometry should be performed
have found mean absolute errors of • Applanation A scan before applanation A scan since
between 1.08 and 1.4 D3,4,5. • Immersion A scan vice versa can lead to erroneous
2. Optical Biometry keratometry measurements.
Concept of emmetropization
Emmetropization involves interplay of • Iol Master 500 (Based on partial IOL formula
3 major variables (axial length , corneal coherence interferometry). Theoretical formulas (SRK-T, Holladay
and lenticular power). 1&2, Hoffer Q and Haigis) preferred
• Mean AL at birth is 16.6 –17.0 mm • Lenstar (based on optical low over regression formula like SRK.
coherence reflectometry). [10] Vasavada et al11 compared the
AL increases rapidly in the first 6 accuracy of modern intraocular
months (0.62 mm/month), then • Swept source IOL master 700. lens power calculation formulae in
has a relatively slower (infantile Paediatric patients are usually not paediatric population and concluded
phase) growth (0.19 mm/month) cooperative for optical biometry, and that in pediatric eyes, SRK/T and the
till 18 months, followed by a slow examination under anesthesia (EUA) Holladay 2 formulae had the least
(juvenile phase) growth (0.10 mm/ is required for calculation of axial predictive error (PE). Personalizing the
month)6. length and keratometry. Keratometry lens formula constant did reduce the
using handheld keratometer (Nidek, PE significantly for all formulae except
Alcon autokeratometer) is performed Hoffer Q. In extremely short eyes (AL
under EUA in paediatric age group.
Keratometry readings should be taken

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 63

Subspeciality-Pediatric Ophthalmology

<20 mm),SRK/T and Holladay 2 gave management of paediatric cataract over 9. McClatchey SK, Parks MM. Myopic shift
the best PE. the years. A comprehensive eye care after cataract removal in childhood. J
approach involving early detection, Pediatr Ophthalmol Strabismus. 1997;
IOL power calculation management and visual rehabilitation 34: 88-95.
An accurate refractive outcome after can help to tackle childhood blindness
primary IOL implantation is crucial to due to cataract with favourable 10. Eibschitz- Tsimhoni, et al. Discrepancies
avoid a large myopic shift in later life. outcomes. between Intraocular Lens Implant
Thus, initial desired refractive outcome Power Prediction Formulas in Pediatric
is therefore hypermetropia. To conclude, words of Ellis17 are worth Patients Ophthalmology,2007;114:383-6.
remembering, “Young children with
• Dahan and Drusedau suggested an IOL in place are a unique clinical 11. Vasavada V, Shah SK, Vasavada VA, et
undercorrection of -20% in <2 years responsibility of an ophthalmologist. al. Comparison of IOL power calculation
and 10% in children between 2 and Long-term follow-up is especially formulae for pediatric eyes. Eye (Lond).
8 years12. important.” 2016;30(9):1242-50.

• Prost suggested undercorrection of References 12. Dahan E, Drusedau MU. Choice of
-20% between 1 and 2 years of age, 1. Gilbert C, Foster A. Childhood blindness lens and dioptric power in pediatric
15% undercorrection between 2 pseudophakia. J Cataract Refract Surg
and 4 years, and 10% between 4 and in the context of VISION 2020 – The 1997;23 Suppl 1:618-23.
8 years of age13. right to sight. Bull World Health Organ
2001;79:227-32. 13. Prost ME. IOL calculations in cataract
• Enyedi suggested postoperative 2. Rahi JS, Sripathi S, Gilbert CE, Foster A. operations in children. Klin Oczna
target refraction to be used for IOL Childhood blindness in India: Causes 2004;106:691-4.
power calculation according to age in 1318 blind school students in nine
(age + postoperative refraction = states. Eye (Lond) 1995;9 (Pt 5):545-50. 14. Enyedi LB, Peterseim MW, Freedman
7)14. 3. Hoffer KJ. The Hoffer Q formula: a SF, Buckley EG. Refractive changes after
comparison of theoretic and regression pediatric intraocular lens implantation.
However, Khokhar et al study on formulas. J Cataract Refract Surg. 1993; Am J Ophthalmol 1998;126:772-81.
biometric changes in Indian paediatric 19: 700-12.
cataract and post operative refractive 4. Andreo LK, Wilson ME, Saunders RA. 15. Khokhar SK, Tomar A, Pillay G,
outcomes noted a lower rate of axial Predictive value of regression and Agarwal E. Biometric changes in Indian
length growth and keratometry theoretical IOL formulas in pediatric pediatric cataract and postoperative
changes in Indian eyes as compared to intraocular lens implantation. J Pediatr refractive status. Indian J Ophthalmol.
western eyes implying the need for less Ophthalmol Strabismus. 1997; 34: 240-3. 2019;67(7):1068-72.
undercorrection in emmetropic IOL 5. Moore DB, Ben Zion I, Neely DE, Plager
power for Indian eyes15. DA, Ofner S, Sprunger DT, et al. Accuracy 16. Ness PJ, Werner L, Maddula S, Davis D,
of biometry in pediatric cataract Zaugg B, Stringham J, et al. Pathology of
Hence, we prefer an undercorrection of extraction with primary intraocular lens 219 human cadaver eyes with 1-piece or
– 20 % for children <3months, 10 % at implantation. J Cataract Refract Surg. 3-piece hydrophobic acrylic intraocular
1 year, 5 % at 2 years and 2 % at 5 years. 2008; 34: 1940-7 lenses: Capsular bag opacification and
6. Capozzi P, Morini C, Piga S, Cuttini sites of square-edged barrier breach. J
Criteria for IOL implantation M, Vadalà P. Corneal curvature and Cataract Refract Surg 2011;37:923-30.
1. AL >17 mm axial length values in children with
2. Corneal diameter >10mm congenital/infantile cataract in the first 17. Bradford GM, Keech RV, Scott WE:
Choice of IOL 42 months of life. Invest Ophthalmol Vis Factors affecting visual outcome after
Hydrophobic acrylic lenses with square Sci 2008;49:4774-8. surgery for bilateral congenital cataracts.
edges inhibits lens epithelial cell (LEC) 7. Gordon RA, Donzis PB Refractive Am J Ophthalmol 1994; 117: 58-64.
migration and posterior capsular development of the human eye. Arch
opacification formation and are hence Opthalmol1985 Jun;103(6):785-9. Corresponding Author:
preferred in pediatric age group16. 8. Larsen JS. The sagittal growth of the eye.
IV. Ultrasonic measurement of the axial Dr. Mousumi Banerjee
Conclusions length of the eye from birth to puberty. Pediatric Cataracts & Refractive Surgery Services,
A paradigm shift has been noted in the Acta Ophthalmol. 1971;49(6):873– 86. Dr Rajendra Prasad Centre for Ophthalmic
Sciences. AIIMS, New Delhi, India.

64 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

Work up in Paediatric Cataract

Lav Kochgaway, Ankita Mitra, Merina Mondal, Sneha Jain
B.B. Eye Foundation V.I.P. Road, Kolkata, India.

Any lens opacification present at fetal vasculature (PFV), lenticonus, fixing point, steady suggests that there
birth or early childhood is defined as or lentiglobus. The incidence varies is no component of nystagmus, and
congenital cataract. The prevalence of from 2.2% to 6.75%6. maintained suggests that there is no
childhood cataract has been reported as • Traumatic cataract- One of the squint. In verbal but preschool children
4.24 per 100001. Congenital cataract has commonest causes of unilateral LEA symbols test, Allen’s picture card,
a significant impact on inflicted patients cataract in children is traumatic7. Lippman’s HOTV test can be done for
because of the low age of its target • Others-In older children the causes assessment of visual acuity. In older
population and subsequent amblyopia of childhood cataract are uveitis, children visual acuity is assessed with
due to stimulus deprivation. It is known steroid induced, radiation and laser Snellen’s chart. Also proper cycloplegic
as a cause of treatable blindness in therapy for ROP. refraction is to be done for every case.
children around the world2. Presentation Other ocular examination should
Mostly the first symptom is a white include pupillary response, ocular
Etiology pupillary reflex noted by the parents. alignment and ocular motility. The slit
• Idiopathic- According to Indian Sometimes strabismus may be the initial lamp examination should be carried
manifestation, especially in unilateral out in each case to evaluate the size,
data most of the unilateral cataract cases. In case of bilateral cataract density, and location of cataract to
and significant number of bilateral nystagmus or poor visual fixation may plan the surgical procedure. Fundus
cataract are idiopathic3. be the presenting complaint. examination should be carried out after
• Hereditary- Most common is pupillary dilatation.
autosomal dominant cataracts with History
incomplete inheritance4. They are A detailed history should be taken from Many of the paediatric cataracts
usually not associated with any the parents to understand whether the are dense and prevents fundus
syndrome with defect in crystallin cataract is congenital, developmental examination. In such cases, USG B scan
and connexin genes. or traumatic in origin. Detailed birth is an important tool in ruling out any
• Cataractassociatedwithsyndromes- history including premature birth, birth posterior segment pathology. Other
Down’s syndrome, Norrie disease, weight, history of oxygen exposure pathologies which may present with
Nance–Horan syndrome. post natally, or any other postnatal white pupillary reflex in the eye include
• Metabolic cataract- Galactosemia: complications must be taken. One retinoblastoma, persistent hyperplastic
It presents as nuclear cataracts but must ascertain if there is any history of primary vitreous (PHPV), Coats’
can also manifest as anterior or maternal drug use or infection during disease, retinopathy of prematurity
posterior subscapular cataract. pregnancy. Parents and siblings must and organized vitreous haemorrhage.
• Infections- Congenital infections be screened to rule out familial causes. The USG B scan is therefore of utmost
such as toxoplasma, rubella, importance to differentiate paediatric
cytomegalovirus, herpes, and Ocular examination cataract from the above mentioned
syphilis (TORCH) are associated A thorough ocular and systemic conditions.
with congenital cataract, with examination is must in every child. It is
rubella being the most common important to quantify the visual acuity Systemic investigations
cause. The incidence of TORCH of the child with cataract as precisely • Most paediatric cataracts are
infections is high in India and up to as possible. In case of preverbal child,
20% of cases may be seropositive5. the fixation and following to light idiopathic and hence do not require
• Preexisting posterior capsular is to be assessed. The type of visual systemic work up.
defect- It is seen in association with fixation is also important. Central • In unilateral cataracts, extensive
posterior polar cataract, persistent fixation suggests that the fovea is the laboratory investigations are not
usually indicated because most of
them are isolated, nonhereditary

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 65

Subspeciality-Pediatric Ophthalmology

and without any systemic and Table 1: Systemic evaluation of paediatric cataract10
genetic abnormality8,9.
• TORCH profile in case of bilateral Unilateral cataract Bilateral cataract
congenital cataract can be ordered
to look for active infection. History- age of onset, family history, History- family history is critical, onset of
In neonates with suspected trauma cataract, developmental milestones
intrauterine infection, IgM titres is
obtained to avoid confusion with Ocular examination- to diagnose Dysmorphic features like midfacial
maternal IgG antibodies that have specific morphological features hypoplasia- consult geneticist
crossed the placenta. of cataract like PHPV, posterior
• VDRL test can be done in suspected lenticonus, anterior polar cataract
syphilis.
• For metabolic disorders fasting Laboratory test- TORCH titre, VDRL Laboratory test-
blood sugar, urine for reducing
substance, and urine amino acids Routine- TORCH titre and VDRL(for
are checked. syphilis) Urine for reducing Substances
• Dysmorphic appearance of the (after milk feeding)
child may require chromosomal
study. Optional- Red cell galactokinase
• All the surgeries are done under (galactokinase deficiency) Urine for
general anaesthesia, so routine amino acids (for Lowe’s syndrome)
blood count, chest x-ray and Calcium and phosphorus (for metabolic
pre anaesthetic check up by disorder)
anaesthetist has to be done.
Paediatric Biometry myopic shift after paediatric cataract Table 2: Suggested age corrections21
Challenges in paediatric biometry surgery13. Axial length increases rapidly Age (years) Undercorrections (%)
in the first 6 months (0.46 mm/month), <1 60-70
• Poor cooperation then has a relatively slower (infantile 1-2 40-60
• Poor fixation phase) growth (0.15 mm/month) till 18 2-3 20-40
• Myopic shift related to normal eye months, followed by a slow (juvenile 3-8 10-20
phase) growth (0.10 mm/month). Error >8 Adult power
growth in axial length measurement affect IOL
• Axial length elongation power calculation the most. It increases the Holladay 2 formulae have the
• Post operative amblyopia to 3.75 D/mm in children as compared least predictive error15.
Most of the children will need biometry to adults (2.5 D/mm). Immersion A-scan • Again according to some studies,
under general anaesthesia on the day gives more accurate estimate of axial there was no significant statically
of cataract surgery. Pediatrics eyes in length than indentation A-scan14. In difference between the 4 formulas
comparison to adults eye have shorter spite of this disadvantage, indentation including Hoffer Q, Holladay,
axial length, steeper cornea with method is more commonly used than SRKII, and SRK/T16,17.
higher keratometry values, and smaller immersion method. So A-scan reading • In fact, any IOL formula can be
anterior chamber depth and myopic with maximum anterior chamber depth used for determining IOL power in
shift will occur with increasing age11. should be taken. children, but more error is expected
than in adults18.
Keratometry- Keratometry values are To minimize errors during biometry- IOL power under correction
obtained using hand held keratometer. IOL power calculation is multifactorial.
Keratometry also steeply reduces in the • Measurement should be taken It depends on the age of presentation,
first 6 months (-0.40 D/month), -0.14 D/ without use of eye speculum. morphology of cataract, visual acuity
month in the next 6 months, and -0.08 at presentation, time of development
D/month in the second year, reaching • To avoid problems associated with of cataract (congenital/developmental),
the adult range at about 3 years of age12. corneal dryness, measurements biometry at presentation, unilateral or
should be taken as soon as bilateral cataract, and refractive status
Axial length- It is difficult to predict possible after induction of general of the fellow eye19. With increasing
the axial length growth and hence the anaesthesia. age there will be myopic shift in
children. So the recommendations are
• BSS should be instilled frequently to
maintain smooth corneal surface.

Intraocular lens power calculation
Formula-

• Few study showed that SRK/T and

66 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

for under correction of IOL power and Figure 1(a–d): Paediatric Cataract. 2012;22(1):37-44.
hyperopic outcomes so the patient 3. Jain IS, Pillai P, Gangwar DN, Gopal
will be hypermetropic postoperatively the Infant Aphakia Treatment Study,
which will shift to emmetropia during primary IOL implantation and contact L, Dhir SP: Congenital cataract:
adulthood. Dahan et al suggested lens did not show a significant difference Etiology and morphology. J Pediatr
undercorrection of 20% in children in terms of final visual acuity in infants Ophthalmol Strabismus 1983; 20:
<2 years and 10% in children between <7 months of age24. It is controversial 238-42.
2 and 8 years20. But this may make the whether to implant an IOL at the time 4. Santana A, Waiswo M. The genetic
child prone to amblyopia and make of cataract surgery in infants, or to leave and molecular basis of congenital
them need corrective glasses or contact the child aphakic with a secondary cataract. Arq Bras Oftalmol
lens with a periodical change in powers. procedure for IOL implantation later 2011;74:136‑42.
So the current recommendation is in childhood25. In children > 2 years age 5. Mahalakshmi B, Therese KL,
postoperative small hyperopia which cararact surgery is followed by primary Devipriya U, Pushpalatha V,
will slowly progress into emmetropia IOL implantation. Margarita S, Madhavan HN, et al.
and finally into small myopia. Conclusion Infectious aetiology of congenital
Pediatric cataract surgery has evolved cataract based on TORCHES
Choice of intraocular lens over the years and has become more safe screening in a tertiary eye hospital
Hydrophobic acrylic lenses with and predictable. Early identification, in Chennai, Tamil Nadu, India.
square edges inhibits lens epithelial proper preoperative work up, and Indian J Med Res 2010;131:559‑64.
cell (LEC) migration and posterior appropriate management have 6. Vasavada AR, Praveen MR,
capsular opacification formation, so it favorable outcomes. Nath V, Dave K. Diagnosis and
is preferred in paediatric age group. Reference management of congenital cataract
1. Wu X, Long E, Lin H, Liu Y. Global with preexisting posterior capsule
Timing of surgery defect. J Cataract Refract Surg
The critical period of eye development prevalence and epidemiological 2004;30:403‑8.
ranges from 2 months to 6 months of characteristics of congenital 7. Khokhar S, Gupta S, Yogi R, Gogia
age and emmetropization of an eye is cataract: a systematic review and V, Agarwal T. Epidemiology and
generally achieved by 9 years of age22. meta analysis. Lancet; 2016:S55. intermediate-term outcomes of
Once visually significant cataract 2. Pi LH, Chen L, Liu Q, et al. Prevalence open-and closed-globe injuries in
(central lental opacity >3mm) is of eye diseases and causes of visual traumatic childhood cataract. Eur J
detected, it should be operated as early impairment in school-aged children
as possible. In unilateral cataract there is in Western China. J Epidemiol.
high risk of amblyopia, so early cataract
surgery by 6 weeks of age is advised23.
Bilateral cataracts should be operated
before 8 weeks of age. In symmetrical
bilateral cases, the second eye should
be operated within one to two week of
the first eye. When there is significant
asymmetry, the denser cataract is
generally operated first. In visually non
significant opacities (blue‑dot cataract
or posterior polar cataract < 3mm), the
child can be kept under close follow up
at regular intervals.

Aphakia vs IOL implantation
In children less than 2 years of
age removal of cataractous lens
(lensectomy) is done followed by
aphakia correction with glasses. The
parents must ensure that the child
wears the prescribed glasses. Contact
lenses are more suitable in children
with unilateral aphakia. According to

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 67

Subspeciality-Pediatric Ophthalmology

Ophthalmol. 2014;24(1):124-130. 15. Vasavada V, Shah SK, Vasavada VA, 22. Atkinson J, Anker S, Bobier W,
Vasavada AR, Trivedi RH, Srivastava Braddick O, Durden K, Nardini M,
8. Lim Z, Rubab S, Chan YH, Levin S, et al. Comparison of IOL power et al. Normal emmetropization in
AV. Pediatric cataract: the calculation formulae for pediatric infants with spectacle correction
Toronto experience-etiology. Am J eyes. Eye (Lond) 2016;30:1242‑50. for hyperopia. Invest Ophthalmol
Ophthalmol. 2010;149(6):887-892. Vis Sci 2000;41:3726-31.
16. Andreo LK, Wilson ME, Saunders
9. Haargaard B, Wohlfahrt J, Fledelius RA. Predictive value of regression 23. Vasavada AR, Nihalani BR. Pediatric
HC, Rosenberg T, Melbye M. A and theoretical IOL formulas cataract surgery. Curr Opin
nationwide Danish study of 1027 in pediatric intraocular lens Ophthalmol 2006;17:54-61.
cases of congenital/infantile implantation. J Pediatr Ophthalmol
cataracts: etiological and clinical Strabismus. 1997;34(4):240-243. 24. Infant Aphakia Treatment Study
classifications. Ophthalmology. Group, Lambert SR, Buckley
2004;111(12):2292-2298. 17. Neely DE, Plager DA, Borger EG, Drews‑Botsch C, DuBois L,
SM, Golub RL. Accuracy of Hartmann E, et al. The infant
10. Paediatric ophthalmology and intraocular lens calculations in aphakia treatment study: Design
strabismus. Kenneth Wright, Peter infants and children undergoing and clinical measures at enrollment.
Spigel. Chapter 27. cataract surgery. J Am Assoc Arch Ophthalmol 2010;128:21-7.
Pediatr Ophthalmol Strabismus.
11. Kekunnaya R, Gupta A, Sachdeva 2005;9(2):160-165. 25. Plager DA, Lynn MJ, Buckley EG,
V, Rao HL, Vaddavalli PK, Prakash Wilson ME, Lambert SR, Infant
VO. Accuracy of intraocular lens 18. Eibschitz-Tsimhoni M, Archer SM, Aphakia Treatment Study Group.
power calculation formulae in Del Monte MA. Intraocular lens Complications, adverse events,
children less than two years. Am J power calculation in children. Surv and additional intraocular surgery
Ophthalmol. 2012;154(1):13-19. Ophthalmol. 2007;52(5):474-482. 1 year after cataract surgery in the
infant Aphakia Treatment Study.
12. Capozzi P, Morini C, Piga S, Cuttini 19. Magli A, Forte R, Carelli R, Ophthalmology. 2011;118 (12):
M, Vadalà P. Corneal curvature Rombetto L, Magli G. Long‑term 2330-2334.
and axial length values in children outcomes of primary intraocular
with congenital/infantile cataract lens implantation for unilateral Corresponding Author:
in the first 42 months of life. Invest congenital cataract. Semin
Ophthalmol Vis Sci 2008;49:4774‑8. Ophthalmol 2016;31:548‑53. Dr. Lav Kochgaway
B.B. Eye Foundation
13. Crouch ER, Crouch ER Jr., Pressman 20. Dahan E, Drusedau MU. Choice of V.I.P. Road, Kolkata, India.
SH. Prospective analysis of pediatric lens and dioptric power in pediatric
pseudophakia: Myopic shift and pseudophakia. J Cataract Refract
postoperative outcomes. J AAPOS Surg 1997;23 Suppl 1:618‑23.
2002;6:277‑82.
21. Ladi JS. Prevention and correction
14. Wilson ME, Trivedi RH. Axial of residual refractive errors after
length measurement techniques in cataract surgery. J Clin Ophthalmol
pediatric eyes with cataract. Saudi J Res 2017;5:45-50.
Ophthalmol 2012;26:13‑7.

68 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

Pearls in Pediatric Cataract

Savleen Kaur MS, Jaspreet Sukhija MS
Advanced Eye Centre, PGIMER, Chandigarh, India.

Management of pediatric cataract has Figure 1: Photograph during examination under anesthesia revealing right eye
shown marked improvement over microphthalmos with cataract due to rubella in a 2 month old child.
the last few years. Newer diagnostic
work ups have evolved. Genetics Classification of congenital cataract.
of the disease has been elucidated
better and surgical techniques have X-linked inheritance have also been are also associated with a pediatric
improved considerably along with described. Cataracts are often associated cataract. These include galactosemia
better technology1-7. There still remains with renal and cerebral disease as in the (oil droplet cataract), G6PD deficiency,
a higher risk of intraoperative and X-linked oculocerebrorenal syndrome hypoglycaemia, hypocalcaemia to
postoperative complications after of Lowe. Child with bilateral cataract name a few. The most common type
pediatric cataract surgery, attributed must be examined by a paediatrician of secondary or complicated cataract
to the peculiarities of a child’s eye and and/or geneticist along with dilated is a result of uveitis occurring in
to the greater inflammatory response examination of the parents. conjunction with arthritis (juvenile
seen in young children after surgery idiopathic arthritis) or because of
which sometimes is coumpunded by Pearl 1: Child with bilateral cataract intermediate or posterior uveitis.
presence of associated ocular infection must be examined by a paediatrician The cataract may be a direct result of
(eg TORCH)8-10. This review overviews and/or geneticist along with dilated inflammation or due to oral steroids
the basics of understanding in pediatric examination of the parents. used to treat the uveitis.
cataract and provides pearls for a Several systemic metabolic diseases Cataracts occurring because of
pediatric cataract surgeon.

Preoperative examination and
evaluation
A thorough preoperative examination
involves establishing the etiology,
carrying out relevant investigations and
preoperative accurate measurements.

Etiology
Childhood cataracts can be classified
depending on the onset, morphology or
etiology (Figure 1).

Hereditary cataracts account for almost
one third portion of bilateral congenital
cataracts. The development of the
human lens requires a tightly regulated
interplay of many genes. Mutations
in these genes affect the structure and
transparency of the lens, which in turn
leads to the development of cataracts.
Most of the isolated congenital cataracts
are inherited in an autosomal dominant
fashion (75%), autosomal recessive and

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 69

Subspeciality-Pediatric Ophthalmology

Figure 2: Traumatic cataract in a 6 year male with Figure 3: Bilateral congenital cataract in a seven-month child on gross Examination.
repaired corneal perforation inferiorly and corneal
sutures in situ.

maternal infection during pregnancy, management. Figure 4: Cataract associated with persistent
mainly include TORCH group of fetal vasculature. Note the vascular network
infections (Figure 1). These include Anterior cataracts on the lens surface.
rubella toxoplasmosis, toxocariasis, and Anterior polar cataracts are often
cytomegalovirus. bilateral, hereditary, and visually congenital cataract is fetal nuclear
insignificant. They mostly present cataract. There is a central white opacity
Overall, the most common etiology as a tiny white dot in the centre of (unilateral or bilateral) between the “Y”
of cataract is idiopathic. But mostly the anterior capsule. These cataracts sutures surrounded by clear cortex. As
idiopathic etiology is a diagnosis of are usually bilateral 1 mm or less in time passes from birth, the surrounding
exclusion. diameter and almost never progress. cortex may become opaquer diffusely
Pyramidal cataracts are named because or in radial extensions called “riders.”
Morphological types of pediatric the anterior opacity resembles a These cataracts are most amblyogenic
cataract pyramid. A more accurate and modern because they are central and hence
description is a likeness to the shape should be removed as soon as possible
The important morphological types of of the chocolate candy called the (Figure 3).
congenital cataract are: Hershey’s Kiss. They are usually fibrous Posterior polar cataracts
and may be associated with an anterior Posterior polar cataracts can be
1. Diffuse/total subcapsular cataract that, when unilateral or bilateral. These cataracts
2. Anterior polar present, often progresses to become should be identified pre-operatively
3. Cortical lamellar visually significant. These pyramidal as much as possible because during
4. Fetal nuclear cataracts are dominant in inheritance surgery, the posterior capsule may
5. Posterior polar and usually present bilaterally. already be ruptured. Hydrodissection
6. Posterior lentiglobus Anterior lenticonus is usually is discouraged because it may result in
7. Posterior (and anterior) subcapsular associated with Alport’s syndrome a large uncontrolled posterior capsular
8. Persistent fetal vasculature and is less common than the posterior tear.
variety of posterior lenticonus.
(Persistent hyperplastic primary
vitreous) Lamellar cataracts
9. Traumatic (Figure 2) Lamellar cataracts involve a layer
10. The other less common ones: (lamellae) of cortex surrounding the
punctate, pulverulent, coraliform, fetal nucleus, peripheral to the “Y”
coronary, floriform, retrodot, sutures. They are almost always bilateral
sunflower, bluedot, and sutural. but are commonly asymmetric. The
Pearl 2: Morphology of the cataract must nucleus is characteristically clear.
be established with as much details as
possible preoperatively. This helps us in Nuclear cataracts
decoding the etiology and deciding the The most common morphology of

70 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

Persistent Hyperplastic Primary Table 1: listing indications of of the child. High-power IOLs result
Vitreous (Persistence of Fetal cataract surgery in a child in a greater myopic shift for the same
Vasculature) PHPV 1. Central cataracts >3 mm in increase in axial length. So, we need to
PHPV is a type of vascularised cataract. diameter (visually significant under correct the IOL power to prevent
The distinct features of this spectrum 2. Dense nuclear cataract unilateral/ a future myopic shift. Most of the
include a membrane on the posterior bilateral surgeons choose hyperopia, with the
lens capsule with blood vessels coursing 3. Cataracts obstructing the expectation of myopic shift with time;
through it (Figure 4). The membrane is examiner's view of the fundus or others choose emmetropia or myopia
often attached to the ciliary processes, preventing refraction of the patient. to help with amblyopia management.
pulling them in toward the center of 4. If the contralateral cataract has The authors prefer moderate hyperopia
the papillary space. A persistent hyaloid been removed and customize the choice of initial
artery may cause haemorrhage if cut 5. Cataracts associated with postoperative refraction based on the
by the vitrector at the time of surgery. strabismus and/or nystagmus child’s age, accounting for the refraction
There is a poor prognosis if posterior 6. Decreased vision in older children: of the opposite eye and whether the
segment is involved and surgical distance and/or near surgery is unilateral or bilateral.
intervention should be selective and
cautious. Pearl 3: Not every child with cataract Two ocular measurements are critical
needs surgery to IOL calculation: axial length (AL) and
Preoperative work up cornea power (K). These can be done
IOL power calculation is one of the while the child is awake in patients
Only a small minority of childhood most crucial step in management of as young as 3 years, by being gentle
cataracts present clinically with pediatric cataract. This occurs mainly and friendly to the patient. AL can be
subjective complaints relating to because of two factors: measurement measured by A-Scan ultrasound done
vision. All new-borns should undergo errors and refractive growth of the using either contact or immersion
a screening which should include an eye. This is because the eye of a child methods. We should be careful that the
evaluation of the red fundus reflexes has not reached the adult dimensions scan is directed toward the macula and
and ophthalmoscopy. Presenting signs yet. The rate of refractive growth for that the tip does not indent the relatively
could be a white reflex, strabismus, children with surgery at age <6 months soft cornea. Handheld keratometry
or nystagmus. Any central opacity is assumed to be -5.4 D; for children can also be practiced in anesthetized
or surrounding cortical distortion >3 with surgery at age 6 months, it is or awake children. Biometry in young
mm can be assumed to be visually assumed to be -3.3 D approximately. children can be performed in the
significant. Slit-lamp examination The predictions for infants with surgery operating room when the child is asleep
clarifies the morphology of the cataract at age < 3 months are theoretical. under general anaesthesia.
and may help determine, along with Variations in refractive growth and
associated findings, the cause and initial ocular measurements will Several studies of children have shown
prognosis. Associated abnormalities significantly affect ultimate refraction larger errors in IOL formula predictions
of the cornea, iris, and pupil should be than are found in adults with almost all
noted. A portable handheld slit lamp adult formulae. The newest formulas
is especially helpful for examining such as SRK-T and Holladay II have
infants and young children. An attempt been shown to be reproducible in most
should be made to visualize the retina,
fovea, and optic disc to estimate the Recommended Postoperative Refractive Goals for Children In (D)
visual potential for the eye and rule out
PHPV. Elevated intraocular pressure Age at Enyedi et al. Plager et al. Trivedi Authors
may accompany congenital cataract Surgery (yr) (1998) (2002) and recommendation
associated with congenital rubella
syndrome or Lowe syndrome. Wilson
(2009)
Indications for cataract surgery
1 +6 +6 +4
The indications for cataract surgery in a
child are listed in (Table 1). 2 +5 +5 +3

Intraocular lens power (IOL) 3 +4 5 +2
calculation
4 +3 4 +4 +1

5 +2 3 +3

6 +1 2 +2

7 1.5 +1

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 71

Subspeciality-Pediatric Ophthalmology

studies. ocular and systemic anomalies and intraocular scissors in addition to
prematurity the vitrector
Pearl 4: IOL power calculation in 4. Setup for pediatric general
children should take into account age anaesthesia is a prerequisite 12. Need for primary posterior capsule
of the patient at the time of surgery, 6. Examination under anaesthesia: management to prevent dense and
measurement errors, refractive growth is necessary sometimes even thick PCO in those too young to
of the eye, fellow eye status, type and to diagnose cataract and for apply an awake YAG laser
site of IOL implantation and target preoperative assessment
refraction. 7. Need for automated keratometer 13. Need for vitrectomy
and A-scan in operating room instrumentation
IOL should generally not be implanted 8. Difficulty in calculating IOL power
if axial length <17 mm and corneal 9. Psychologic issues and preoperative 14. Difficult IOL implantation
diameter <9 mm. The Infantile Aphakia counselling of parents
Treatment Study was a randomized, Intraoperative issues Postoperative Period
multicentre clinical trial of 114 infants
with a unilateral congenital cataract 1. Risks of general anaesthesia 1. Higher risk for opacification of the
comparing primary pseudophakia to 2. Smaller size of the eye visual axis
aphakia. Preliminary results of log 3. Poor dilation of pupil more often
MAR grating visual acuity at 1 year 2. Propensity for increased
of age failed to show a significant associated with pediatric eyes postoperative inflammation
difference between the treated eyes in 4. Low scleral rigidity
the two groups (contact lens group, 5. Relative size of the pars plana: The 3. Compliance with the use of topical
0.80; IOL group, 0.97; P = 0.19)11-13. In postoperative medications difficult
addition, there were more patients pars plana region in the infant eye
with intraoperative complications is incompletely developed, so the 4. Requirement for frequent
(28% versus 11%; P = 0.03), adverse anterior retina lies just behind the correction of residual refractive
events (77% versus 25%; P < 0.0001), pars plicata. error, as it is constantly changing
and additional intraocular surgeries 6. Incision and suturing: As opposed due to growth of the eye
(63% versus 12%; P < 0.0001) in the to adult eyes, a superior tunnel is
IOL group than the contact lens group. preferable in pediatric eyes and is 5. Difficulty in documenting
However, the results of the trial should preferable to suture even a “self- anatomic, refractive, and visual
be weighed with caution. Choice of seal” tunnel incision in children as acuity changes due to poor
implanting an IOL can depend on a lot opposed to adults. compliance- alternative testing
of factors including surgeon experience, 7. Need for high-viscosity viscoelastic methods may be needed
laterality of cataract, parent’s education for capsular management
level, compliance to follow up etc14-17. 8. Difficulty in performing an anterior 6. Examination possibly requiring
capsulorhexis associated with a repeated brief anaesthesia due to
Pediatric cataract surgery highly elastic anterior capsule lack of cooperation with office
There are several ways in which and increased intralenticular and exam
pediatric cataract surgery differs from intravitreal pressure
an adult cataract surgery. 9. Densely formed vitreous and scleral 7. Tendency to develop amblyopia
collapse contributing to vitreous and need for patching
In the preoperative period upthrust giving rise to raised
1. Difficult and often delayed intravitreal and lenticular pressure, 8. Long-term follow-up important but
making anterior and posterior not always easily achieved
diagnosis, Systemic illness capsular management difficult
2. Timing of surgery: In sharp 10. Removal of lens substance rarely Choosing the right IOL
requires phacoemulsification, but
contrast to the treatment of adult the cortex is stickier and gummier IOL implantation in children became
cataracts, the timing of cataract than in adults a common practice in 1990s but
surgery in children is of paramount 11. Posterior capsule plaques are early attempts resulted in frequent
importance. It affects the visual common and may require complications due to poor lens design
result to a much greater extent as well as the inflammation. Continued
than does the surgical technique improvement in designs and better
or method of postoperative optical surgical techniques has resulted in
correction used by the surgeon. IOLs being implanted in children with
3. High incidence of associated increasing frequency. IOLs which are
used in cataract surgery in children
are usually designed to fit to the size
and requirements of adult eye. There
is no IOL which is produced only for
implantations in the child’s eye hence
there is a theoretical increased risk of
PCO/ fibrinous complications/ LEC

72 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

proliferation/glaucoma with almost all Figure 5 (A): Site of IOL implantation in the bag (B): Posterior optic capture of IOL with the
IOLs. optic behind the posterior capsule and haptics in bag.
Changing dynamics of the childs’ eye
make IOL selection critical. The mean Figure 6: Complications of pediatric cataract surgery and IOL implantation. A: Optic got
diameter of the crystalline lens at birth captured in the pupil B: Pigments on IOL surface with pupillary capture.
is 6mm, 8.3mm at 2 years and 9.3mm at
16 years. The size of the capsular bag - preferred in children, a scleral tunnel 4. Insufficient capsular support
7mm at birth, 9.0mm at 2 years and is still sometimes chosen in some
10.2mm at 16 years. When selecting circumstances. Two paracentesis Using high-viscosity cohesive
IOLs for children, we need to find a are performed in front of the limbal ophthalmic viscosurgical device is a
biocompatible material for the pediatric vascular arcade at 2 o’clock and the 10 necessity rather than a choice during
eye with adequate size and power. o’clock positions to help assist bimanual pediatric cataract surgery. Anterior
Hydrophilic acrylic material has the best manoeuvring. The paracentesis should continuous curvilinear capsulorhexis
uveal but less capsular biocompatibility measure approximately 1 mm and run (ACCC), is the goal. Doing a rhexis
so hydrophobic acrylic material with parallel to the iris plane, to facilitate can be difficult in children because of
lower uveal biocompatibility are sealing at the end of surgery. the extreme elasticity of the anterior
considered safest in children. Like capsule, co-existing positive vitreous
adults, in children also biconvex IOLs Pearl 6: Preoperative assessment pressure, and poor dilatation of
are preferable because of better contact must include IOP, Axial length the pupil. Use of higher-viscosity
of edges of posterior capsulotomy and keratometry measurement, ophthalmic viscosurgical devices and
with posterior IOL surface. Foldable measurement of corneal diameters, better microscopes is helpful, but the
IOLs are preferably used in children. establishing lens morphology and creation of an intact as well as well
The average size of currently available posterior segment examination. sized capsulorhexis in infants remains
adult-sized, in-the-bag IOLs is 125-13. mm a challenge. Manual ACCC is best,
and haptics are compressible to some Contraindications of IOL implantation other options include radiothermy
extent and can adjust to the smaller size and vitrectorhexis. Aspiration of lens
of capsular bag. Single piece IOLs are 1. Axial length <17 mm material is next, which does not require
known to have easy implantation and 2. Corneal diameter <9 mm a phaco handpiece. Posterior capsule
good centration with minimal tissue 3. Active uveitis must be opened in children up to the
trauma. Three piece/single piece design
is largely surgeon preference.
In young children, management of the
posterior capsule is more important
than the IOL design or material that
influences PCO. For older children
(without PPC): Square edge design and
material influences incidence of PCO.

Pearl 5: Acrylic hydrophobic foldable
IOL should be the first choice in children.
Closed loop, complex haptic which can
adapt both to capsular shrinking and
capsular growth, prevents decentration
and backward movements of the lens,
ensuring very good stability inside
capsular bag.
Surgical technique
In general, a superior approach allows
the wound to be protected by the brow
and bells phenomenon. Both scleral
and corneal tunnels are described in
children. Although a corneal tunnel is

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 73

Subspeciality-Pediatric Ophthalmology

age of 8 years or left intact if the child obscuration continues to compromise References
is cooperative for slit-lamp examination the outcome of a successful cataract
before cataract surgery. Anterior surgery. Inflammatory precipitates 1. Kaur S, Sukhija J, Ram J. Comparison of
vitrectomy is advisable till 5 years on the IOL, posterior synechia, and posterior optic capture of intraocular
because anterior hyaloid phase also inflammatory cyclitic membranes are lens without vitrectomy vs endocapsular
proliferates. But vitrectomy becomes all signs of increased inflammation after implantation with anterior vitrectomy
optional above 5 years and in conditions pediatric cataract surgery (Figure 6). The in congenital cataract surgery: A
where posterior optic capture is done. frequecy of topical steroids (even up to randomized prospective study. Indian J
Primary posterior capsulorhexis is done putting one drop every hour) needs to Ophthalmol. 2020 Jan;68:84-88.
manually or by vitrectorhexis. It can be be tailored in children following catract
done before or after IOL implantation. surgery19. Amblyopia is often a big 2. Yangzes S, Kaur S, Gupta PC, Sharma M,
The size should be 1–1.5 mm smaller barrier to a successful visual outcome in Jinagal J, Singh J, Ram J. Intraocular lens
than the IOL optic. the management of pediatric cataracts. implantation in children with unilateral
Phacoaspiration with complete Visual outcomes in pediatric cataract congenital cataract in the first 4 years of
rhexis IOL overlap, adequately sized surgery are greatly influenced by the age life. Eur J Ophthalmol. 2019 May;29:304-
posterior capsulotomy combined with of surgery because of the co-existence of 308.
anterior vitrectomy in the hands of deprivation amblyopia. A critical period
an experienced surgeon can cause a exists for congenital cataracts, and 3. Sukhija J, Kaur S. Central corneal
considerable decrease in the amount of hence rapid removal and rehabilitation thickness and intraocular pressure
visual axis obscuration18. is recommended, especially in cases changes after congenital cataract surgery
with unilateral cataracts. with intraocular lens implantation in
Pearl 7: In young children, management children younger than 2 years. J Cataract
of the posterior capsule has a greater Pearl 9: Postoperative topical steroids Refract Surg. 2017 May;43:662-666.
influence on PCO than the IOL design or and dilators are as important as the
material surgery itself in cases of pediatric 4. Sukhija J, Kaur S, Ram J, Yangzes S,
The site of IOL implantation can be in cataract. Madan S, Jinagal J. Outcome of various
the bag, in ciliary sulcus or captured Hydrophobic Acrylic Intraocular
through the capsule (anteriorly or Parents of children with pediatric Lens Implantations in Children with
posteriorly) (Figure 5). Bag in the lens cataract should be explained about congenital Cataract. Eur J Ophthalmol.
IOL has also been described. Multifocal the benefits of the surgery as well as 2017 Nov 8;27:711-715.
IOLs have been described in older the risks involved. Only surgery is
children. Since the average age of not the end of treatment but giving 5. Sukhija J, Kaur S, Ram J. Outcome of
IOL implantation has reduced from the child an optimal correction primary intraocular lens implantation
7 years to 2 years in the last decade, along with amblyopia therapy is of in infants: Complications and rates of
many surgeons prefer primary IOL prime importance. The child’s eye additional surgery. J Cataract Refract
implantation. is a growing visual system so there Surg. 2016 Jul;42:1060-5.
In contrast to adult cataract surgery, is always an anticipated growth and
incisions must be sutured in children. hence myopic shift and an ever-lurking 6. Sukhija J, Kaur S, Pandav SS, Kaushik
Vitrectomy collapses the sclera, and low possibility of IOL exchange. There is S, Raj S, Ram J. Cataract surgery in
scleral rigidity results in fish-mouthing still no consensus of IOL implantation children with buphthalmos. Clin Exp
of the wound hence incisions are leaky. in children less than 7 months of Ophthalmol. 2016 Nov;44:739-740.
Synthetic absorbable or non-absorbable age. Hence IOL implantation must
10-0 sutures are preferred. be explained with benefits involved. 7. Sukhija J, Kaur S, Ram J. Outcome
Pearl 8: In contrast to adult cataract Visual axis opacification and glaucoma of a New Acrylic Intraocular Lens
surgery, incisions must be sutured in are the major complications post- Implantation in Pediatric Cataract. J
children. surgery20. Despite a successful surgery, Pediatr Ophthalmol Strabismus. 2015
Postoperative management these children require a a long follow Nov-Dec;52:371-6.
Although cataract surgical techniques up.
hasevolvedovertheyears,postoperative 8. Sukhija J, Ram J, Gupta N, Sawhney
inflammation and visual axis Pearl 10: The parents play a more A, Kaur S. Long-term results after
proactive role in the postoperative visual primary intraocular lens implantation
rehabilitation in a child after cataract in children operated less than 2 years
surgery. Preoperative counselling of the of age for congenital cataract. Indian J
parents for the journey ahead is of prime Ophthalmol. 2014 Dec;62:1132-5.
importance.
9. Sukhija J, Ram J, Kaur S. Complications
in the first 5 years following cataract
surgery in infants with and without
intraocular lens implantation in the
infant aphakia treatment study. Am J
Ophthalmol. 2014 Dec;158:1360-1.

10. Infant Aphakia Treatment Study Group,
Lambert SR, Lynn MJ, Hartmann EE,
DuBois L, Drews-Botsch C, Freedman
SF, Plager DA, Buckley EG, Wilson

74 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

ME. Comparison of contact lens and and Optotype Visual Acuity in Children Age Using the Toddler Aphakia and
intraocular lens correction of monocular Using Data From the Infant Aphakia Pseudophakia Treatment Study Registry.
aphakia during infancy: a randomized Treatment Study: A Secondary Analysis Ophthalmology. 2020 Apr;127:501-510.
clinical trial of HOTV optotype acuity of a Randomized Clinical Trial. JAMA 19. Sukhija J, Kaur S, Ram J. Minimizing
at age 4.5 years and clinical findings at Ophthalmol. 2016 Aug 1;134:863-9. inflammation after congenital cataract
age 5 years. JAMA Ophthalmol. 2014 surgery. J Cataract Refract Surg. 2014
Jun;132:676-82. 14. Trivedi RH, Wilson ME. Selecting Jun;40:1056-7
intraocular lens power in children. 20. Freedman SF, Lynn MJ, Beck AD,
11. Lambert SR, Cotsonis G, DuBois L, Eyenet Pearls [online]. www.aao.org/ Bothun ED, Örge FH, Lambert SR; Infant
Wilson ME, Plager DA, Buckley EG, publications/eyenet/200601/pearls.cfm. Aphakia Treatment Study Group.
McClatchey SK; Infant Aphakia Accessed on December 17, 2011. Glaucoma-Related Adverse Events
Treatment Study Group. Comparison of in the First 5 Years After Unilateral
the rate of refractive growth in aphakic 15. Bluestein E.C., Wilson M.E., Wang Cataract Removal in the Infant Aphakia
eyes versus pseudophakic eyes in the X.H. et al: Dimensions of the pediatric Treatment Study. JAMA Ophthalmol.
Infant Aphakia Treatment Study. J crystalline lens: implications for 2015 Aug;133(8):907-14.
Cataract Refract Surg. 2016 Dec;42:1768- intraocular lenses in children. J Pediatr
1773 Ophthalmol Strabismus. 1996; 33, 18-20. Corresponding Author:

12. Infant Aphakia Treatment Study Group, 16. Enyedi LB, Peterseim MW, Freedman SF, Dr. Jaspreet Sukhija MS
Lambert SR, Buckley EG, Drews-Botsch et al. Refractive changes after pediatric Advanced Eye Centre, PGIMER,
C, DuBois L, Hartmann EE, Lynn MJ, intraocular lens implantation. Am J Chandigarh, India.
Plager DA, Wilson ME. A randomized Ophthalmol 1998;126:772–781.
clinical trial comparing contact lens
with intraocular lens correction of 17. Plager DA, Kipfer H, Sprunger DT,
monocular aphakia during infancy: et al. Refractive change in pediatric
grating acuity and adverse events at pseudophakia: 6-year follow-up. J
age 1 year. Arch Ophthalmol. 2010 Cataract Refract Surg 2002;28:810–815.
Jul;128(7):810-8
18. Bothun ED, Wilson ME, Vanderveen
13. Drews-Botsch C, Celano M, Cotsonis DK, Plager DA, Freedman SF, Trivedi
G, Hartmann EE, Lambert SR; Infant RH, Traboulsi EI, Anderson JS, Loh AR,
Aphakia Treatment Study Group. Yen KG, Weil NC, Morrison D, Lambert
Association Between Occlusion Therapy SR. Outcomes of Bilateral Cataracts
Removed in Infants 1 to 7 Months of

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 75

Subspeciality-Pediatric Ophthalmology

Update on Pediatric Cataract / The
Conundrum of Pediatric Cataract

Parikshit Gogate MS
Community Eye Care Foundation, Dr.Gogate’s Eye Clinic, Pune, Maharashtra, India.

Indian ophthalmology is blessed during the perinatal period (upto 4 operatively. Its best to have the child
with great cataract surgeons thanks weeks of age) has great anesthesia risk. sleep to as late as possible till it’s time
to the focus on cataract surgery in Surgery in the first 6 weekswas found to to transfer the child to the operating
residency and the sheer number of be associated with increased incidence room. Having a parent around while
cataract surgeries done1. This leads to of secondary glaucoma3. There is a inducing anesthesia makes the child
many ophthalmologists to think that refractive shift post-surgery. This is more comfortable, and the process
pediatric cataracts are just soft adult greater in infancy, which has led to smoother and easier. A favorite toy or
cataracts, easier to perform. Nothing many ophthalmologists questioning blanket, along with the parents, should
could be farther from truth. the wisdom of implanting an intra- be kept near the child when she comes
ocular lens implant in infant cataracts out of anesthesia.
Children’s eyes are not just small adult (congenital and developmental)4.
eyes. They are different. The sclera While doing an A-scan we must
has less rigidity, the anterior capsule Pre-operative work up: consider the factors affecting axial
is thickerand more elastic, corneal growth after cataract surgery.
curvature and axial length keep on There is often a considerable delay
changing and the eye is more prone to between a child being detected for Factors affecting axial growth after
inflammation2. As the equator of the cataract and actually undergoing cataract surgery:
crystalline lens keeps on producing new cataract surgery5,6. This may vary
cells, incidence of secondary cataract from anywhere between 6 months to 1. Normal physiological growth
and posterior capsular opacification is half a decade. Fear of surgery, fear of 2. Age at time of surgery
nearly 100%. anesthesia, reluctance on part of health 3. Amount of visual deprivation
care givers, cost and distance to the 4. Aphakia or psuedophakia
The growth anddevelopment in a tertiary center are some of the barriers7. 5. Laterality: Unilateral cataract eyes
newborn’s eye.
Basic investigations like hemogram, shorter initially, grow more later
• Axial length in newborn: 16.6 to 17 urine routine and TORCH titre 6. Genetics
mm. (wherever available) should be done. Of this, age is the most important
Cardiac fitness especially in children variable. Children below 2 years of age
• Mean keratometric power: 51.2 with Congenital Rubella Syndrome and at time of surgery with or without IOL
Diopters. those with Marfan’s Syndrome is a must. implantation have greater myopic shift
History of seizures and anti-epileptic and variance then older children.
• Refractive changes primarily seen medication is a must. A pediatrician
due to increase in axial length. fitness and pre-anesthesia visit by the Measurements of axial length in
anesthesia team is of great help. A child uniocularpseudophakic/ aphakic eye
• Maximum axial growth by 2 years. usually needs 4 hours of nil by mouth. and un-operated fellow eye showed
• Lens and total eye power decreases If the surgery is scheduled at 9 am, the no difference in change in axial length
mother should be told to have an alarm between 2 eyes.
by 10 diopters in 1st year. at 4:30 am and breast feed the infant.
• Further decrease by 3-4 diopters The surgery should be scheduled early The expected myopic shift after cataract
in the morning to have a shorter fasting surgery is summarized in the (Table 1)8-
from 2- 10 years age. duration and also allow the anesthesia 10:
There is still debate about when and and pediatric team a longer time during
how to operate cataracts in infants. the day to manage the child post- There is a conundrum, should we choose
Earlier,even a one day old child would be
operated. Now the thinking is to operate
a child after 6 weeksof age. Surgery

76 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

for Emmetropia NOW Vs Emmetropia Infant aphakiatreatment study14: that SRK II formula was better suited
in ADULT (at 6-8 years of age). for pediatric IOL power calculation. 17
1. 4-6 weeks age: +8 diopters. This was seconded by a series of 128
Residual refractive error: Choosing 2. 6 weeks to 6 months: +6 diopters. eyes from Hyderabad where SRK II
adult IOL power in children <2 years There is no study that demonstrates a was found to be better for IOL power
visual advantage of one approach over calculation in children18.
With expected growth and myopic other.
shift, the childis expected to have good Intra-operative considerations
unaided vision in adulthood as she A-scan Biometry in children has unique In pediatric cataract surgery, we worry
becomes emmetropic by then. But this problems like: about the sphere, not the cylinder. As
means a significant hypermetropia the child’s refractive status is going to
now.Near vision is severely affected i. Axial length and keratometry are change, multifocals do not offer any
due to lack of accommodation and it difficult to measure. advantage. There would be further
may cause dense amblyopia in case of loss of contrast sensitivity. Torics are
anisometropia. ii. Need for general anesthesia for rarely indicated. But children tolerate
evaluation and anesthesia related progressive spectacles very well. As the
Choosing emmetropia post operatively complications. child would need sspectacles, its best
in <2years age to counsel parents about this before
iii. Less reproducible. surgery. We have to understand that
This makes for easier amblyopia iv. Less predictable with lower surgery is just one important step in a
management in early post-operative series of steps needed to improve the
period, especially for child with poor accuracy compared to adults. child’s vision.
compliance and follow ups.With v. Lack of fixation and centration.
myopic shift and ocular growth, use of vi. Absence of an IOL calculation As there is nearly 100% posterior
contact lens and refractive surgery or capsular opacification (PCO) in children,
piggyback IOLmay be resorted to later formula specifically for pediatric primary posterior capsulotomy (PPC)
to correct the residual error. eyes . is indicated till 6-8years of ageand in
A- scan biometry: Applanation technique all mentally challenged/ syndromic
Enyedi et al. had suggested rule of 7 indents the globe resulting in under children, after which the child becomes
(Age in years + residual diopters=7)to estimation (1D error). Immersion old enough to sit at a Nd:YAG LASER
get near emmetropia in teens11: technique is preferred. Keeping the for a capsulotomy. Only PPC does not
probe perpendicular is important but help as the thick vitreous base allows
1. 1 year old: +6 diopters there is no way of ensuring that the new lens cells to migrate over itas a
2. 2 year: +5 diopters child is fixing on the centre of the probe scaffoldand obscure the visual axis.
3. 3 year: +4 diopters since the child is under anesthesia15. So PPC is to be coupled with anterior
4. 4 year: +3 diopters vitrectomy (AV) till age of at least 6
5. 5 year: +2 diopters For keratometry, hand held auto years. So having an automated vitrector
6. 6 years: +1 diopters keratometer is used in operating room is a must! The PPC +AV can be done
7. 7 years: plano as soon as the child is induced, without before or after PCIOL implantation.
8 years and above: -1 to -2 diopters using a speculum.BSS can be instilled to But after may be safer as at times an
maintain smooth corneal surface to get unfolding IOL may extend the posterior
Dahan and Drusedau suggested accurate keratometry. Optical biometry capsulotomy. So ideally the surgery
under-correction of 20% of the A-scan (IOL Master), Partial coherence should be done using a phacomachine,
reading in children< 2years and under- Interferometry (PCI) and I- Trace are though SICS is an alternative. In the
correction of 10% in children 2- 8 being used in older and co-operative first decade of life, suturing of main port
years12. children. and side-ports should be done, a suture
for each millimeter of opening. Single
Prost suggested13: But all power calculation formulae are suture for side port and a crossed one for
derived from studies on adult eyes. Eye the 2.8 mm incision.
• Under-correction of 20% between 1 of the child changes considerably post
and 2 years. operatively with significant refractive Presently sevoflurane inhalation
change, further compounding the anesthesia is popularly used. It may be
• Under-correction of 15% between issue. A comparative study conducted used with endotracheal intubation or
2-4 years. by Vasvada et. al (117 eyes) in children with a laryngeal mask. In slightly older
younger than 2 years age, demonstrated children and remote places, ketamine
• Under-correction of 10% between that SRK/T and Holladay 2 gave the least
4-8 years. prediction error16. Another series of 461
eyes by Lee BJ et al from Korea showed

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 77

Subspeciality-Pediatric Ophthalmology

and peribulbar block are also an assessment, refraction and amblyopia • Older children
alternative. In the second decade of life, treatment ensure good outcome. A six-
many children co-operate for surgery monthly follow-up is needed in the first • No post-operative uveitis
under local (and vocal) anesthesia, decade and an annual follow-up in the
peribulbar, sub-tenon or topical second decade of life. Posterior capsular • Regular follow-up
depending on patient and operating opacification (PCO), amblyopia and
surgeon’s comfort. secondary glaucoma are the factors • Binocular Cataracts
affecting good outcome.[24, 25] Regular
There is a debate as to how early intra-ocular pressure measurement is Recent studies have vouched for
to implant an intra-ocular lens? needed. the safety and efficacy of anterior
Generally, 8-12 months is an accepted vitrectomy28. Precision pulse
norm though some institutes implant vitrectomy has been used for pediatric
even at 6 months for unilateral cataract surgery29. Optic Capture of the
cataracts19,20. For bilateral congenital IOL and bag-in the lens are novel ways
or developmental cataracts, 24-30 In a study looking at the long-term of reducing PCO Incidence30-31.
months of age is a good time as children follow-up after cataract surgery,
tolerate aphakic correction well. Since congenital cataracts had the least PCO Traumatic cataract
phacoemulsification has become while developmental the most, as Trauma in children can cause cataract.
common, the trend is to implant PPC+AV was restricted to <6 years of There is usually less delay in children
foldable acrylic IOLs instead of the age. getting operated for traumatic cataract
very safe PMMA lenses. Hydrophobic than congenital or developmental
acrylic IOLs have an advantage of being Older children and longer follow-up cataracts32. If there is no co-existing
less prone to pigment dispersion and were associated with more PCO. damage to the cornea and retina,
posterior capsular opacification. The the visual outcomes of traumatic
most important thing is to ensure that Post-operatively, it’s not just vision but cataracts are better than congenital
the IOL is in the bag as that considerably stereopsis that is also important. [24, 26] or developmental cataracts33. This
reduces the chances of inflammation. A In the Miraj Pediatric Cataract Study, for may be because the visual system has
peripheral iridectomy may be done in bilateral congenital and developmental developed in a child who had trauma
cases with PPC + AV. While choosing the cataracts, 48 (37.2%) had binocular and the cataract surgery restores
implant power, there is a conundrum, stereoacuity<480 sec of arc by TNO the child’s vision. There is a male
whether to go for ‘emmetropia now’, test. One had 60 sec of arc stereopsis, preponderance in traumatic cataracts
i.e. put in the power that has come on 17 (13.2%) had 240 arc, 30 (23.9%) had as boys are more likely to engage in
A-scan, easy for amblyopia treatment binocular stereoacuity of 480 sec of arc outdoor activities than girls and are
now, but would need spectacles later; or stereopsis. perhaps less careful! The challenges
‘emmetropia in teens’, i.e. put in lower of anesthesia, IOL power calculation,
power than that has come on A-scan, For unilateral congenital or intra-operative PPC+AV and regular
difficult amblyopia treatment now, but developmental cataracts, 11 (21.6%) post-operative management are similar
no spectacles needed later in adulthood. had binocular single vision <480 to other cataracts in children. To this
After the first 8 weeks, the child should seconds of arc on TNO test (one was 60 may be added problems like managing
be followed up every 3 months till she is sec, one 120, 4 were 240 and another five with a corneal tear, iridodialysis and
2 years of age. A six-monthly follow-up were 480)26. retinal detachment.
in the first decade and an annual one in
the second decade of life is very helpful. For a child, it’s not just distance, but Public health aspect of pediatric
A secondary Nd:YAG LASER should near vision that is equally important. cataract.
be performed earlier as the capsule is The child needs good near vision for
easier to break when it thinner rather tasks of daily living like eating and Pediatric cataract is the commonest
than waiting for it to become fibrosed. playing and for his /her educational cause of treatable blindness in
purposes. Spectacles for near would be children34,35. After refraction and
Post-operative care, amblyopia needed for the child as the facility for amblyopia management, it forms the
treatment accommodation is lost during surgery27. significant workload of all pediatric
ophthalmology departments. There are
Surgery is just one crucial step in a series Visual acuity after pediatric cataract barriers in performing a sight restoring
of steps needed to restore the child’s surgery is better when - surgery on a child. The cost of pediatric
vision. Post-operative care is extremely cataract surgery also significantly
important21-23. A regular follow-up for • Pre-operative vision is better higher than that in an adult36.
the first six months with visual acuity
• Developmental rather than
congenital cataract

• Use of phacoemulsification, Piggyback IOLs are an alternative in
automated vitrectors older children to correct the residual
refractive error (mostly myopia) as it can

78 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

be a significant error impairing unaided primary intraocular lens implantation immersion techniques in pediatric eyes
vision. Piggy back IOLs implanted in infants: Complications and rates of with cataract. Ophthalmology 2011
in the sulcus can provide adequate additional surgery. J Cataract Refractive Mar;118(3):498-502.
power in highly hyperopia and myopic Surg2016 Jul;42(7):1060-5.
error. IOL exchange is another option, 16. Vasavada V, Shah SK, Vasavada VA,
but it’s extremely difficult in a child 5. Gogate P, Khandekar R, Shrishrimal M, Vasavada AR, Trivedi RH, Srivastava S,
as it poses risk for capsular rupture Dole KD, Taras S, Kulkarni SR, Ranade Vasavada SA. Comparison of IOL power
and zonulardamage. The rrefractive S, Deshpande M.Cataracts with delayed calculation formulae for pediatric eyes.
formula used for piggyback IOL are presentation- Are they worth operating Eye 2016 Sep;30(9):1242-50.
calculated purely by child’s refraction. upon? Ophthalm Epidemiology 2010;
No knowledge of the power of primary 17(1): 25-33. 17. Lee BJ et.al. Predictability of formulae
implant or the cause of refractive error for intraocular lens power calculation
is necessary. The lens implantation 6. Mwende J, Bronsard A, Mosha M, according to the age of implantation
in the ciliary sulcus is an efficient and Bowman R, Geneau R, Courtright in paediatric cataract. Br J Ophthalmol
relatively easy method. The Holladay’s P. Delay in presentation to hospital 2019 Jan;103(1):106-111.
formula for Piggyback IOLs is: For for surgery for congenital and
myopic eyes (One for one formula), IOL developmental cataract in Tanzania. Br J 18. Kekuniya R, et. al. Accuracy of
power=1 Diopter of desired spherical Ophthalmol2005;89:1478-82. intraocular lens power calculation
equivalent formulae in children less than two years.
7. Gogate P, Parbhoo D, RamsonP, BudhooR, Am J Ophthalmology 2012 Jul;154(1):13-
For hyperopia refractive error: IOL ØverlandL, MkhizeN, Naidoo K, Levine 19.
power= 1.5 diopter of desired spherical S, du Bryn A, Benjamin L. Surgery for
equivalent Sight: Outcomes of congenital and 19. Outcomes of Unilateral Cataracts in
developmental cataracts operated in Infants and Toddlers 7 to 24 Months of
Gills Nomograms were developed Durban, South Africa. Eye 2016; 30: 406- Age: Toddler Aphakia and Pseudophakia
using Holladays formulae. The surgeon 12. Study (TAPS). Ophthalmology 2019.
should know the axial length to use the
nomogram. 8. Plager DA, Lynn MJ, Buckley EG, 20. Sukhija J, Kaur S, Ram J, Yangzes s,
Wilson ME, Lambert SR, Infant Aphakia Madan S, Jinagal J. Outcome of various
Acknowledgement: Dr. Nikhil Rishikeshi, Treatment Study Group. Complications Hydrophobic Acrylic Intraocular
Head of Pediatric Ophthalmology in 1st five years following cataract Lens Implantations in Children with
Department, H.V. Desai Eye Hospital, surgery in infants with and without Congenital Cataract. Eu J Ophthalmol
Pune, Dr. Supriya Phadke of Community intraocular implant in the infant 2017; 27(6):711-15.
Eye Care Foundation, Pune and Dr. Prasad aphakia treatment study group. Am J
Walimbe, pediatric ophthalmologist, Pune Ophthalmol 2014; 158 (5) : 892-898. 21. Gogate P, Patil S, Kulkarni A, Mahadik
for their valuable inputs. A, Tamboli R, Mane R, Borah RR, Rao
9. Crouch ER, Crouch ER Jr. pressman GV. Barriers to follow-up for pediatric
References SH. Prospective analysis of pediatric cataract surgery in Maharashtra, India.
1. Gogate PM, Biswas P, Natarajan S, pseudophakia : myopic shift and post How regular follow-up is important
operative outcomes. J AAPOS 2002 for good outcome. The Miraj Pediatric
Ramamurthy D, Bhattacharya D, Golnik Oct;6(5):277-82. Cataract study II. Ind J Ophthalmol 2014
K. Residency Evaluation and Adherence 62(3):327-32.
Design Study: Young ophthalmologists’ 10. Wilson ME, Trivedi RH. Axial length
perception of their residency programs measurement techniques in pediatric 22. Gogate P, Gilbert C. Clinical and cost
I: Clinical and surgical skills. Ind J eyes with cataract Saudi J Ophthalmol impact of a pediatric cataract follow-up
Ophthalmol 2017 Jun;65(6):452-460. 2012;26(1):13-7. program in Western Nepal and adjacent
2. Gogate P, Muhit M. Blindness and Indian States. J AAPOS 2014; 19 (1): 94.
cataract in children in developing 11. Enyedi LB,Peterseim MW, Freedman
countries. Journal of Community Eye SF, Buckley EG refractive changes after 23. Congdon NG, Ruiz S, Suzuki M, Herrera
Health. 2009; 22(69):4-5. pediatric intra-ocular lens implantation. V. Determinants of pediatric cataract
3. Traboulsi El, Freedman SF, Wilson ME, Am J Ophthalmol. program outcomes and follow up in a
Edmondson LE, Logan SA, Cheeseman large series in Mexico. J Cataract Refract
EW, Shortridge E, Trivedi RH. Cataract 12. Dahan E, DrusedauMU.Choice Surgy2007;33: 1775-1780.
morphology and risk of glaucoma of lens and dioptric power in
after cataract surgery in infants with pediatricpseudophakia.JCataract Refract 24. Gogate PM, Sahasrabudhe M, Shah M,
unilateral congenital cataract. J Cataract Surg1997;23:618-23. Patil S, Kulkarni AN, Trivedi R, Bhasa
Refract Surg 2017;43(12):1611-12. D, Tamboli R, Mane R. Long term
4. Sukhija J, Kaur S, Ram J. Outcome of 13. Prost ME. IOL power calculation Outcomes of Bilateral Congenital and
in cataract operations in children. Developmental Cataracts Operated
KlinOczna2004;106:691-4. in Maharashtra, India. Miraj Pediatric
Cataract Study III. Ind J Ophthalmol
14. Bothun ED, Lynn MJ, Christiansen SP, 2014; 62(2): 186-195.
Neely DE, Vanderveen DK, Kruger SJ,
Lambert SR; Infant Aphakia Treatment 25. Khanna R, Foster A, Krishnaiah
Study.J AAPOS. 2016 Feb;20(1):49-53. S, MehetaMK,Gogate P M. Visual
Outcomes of bilateral Congenital and
15. Trivedi RH, Wilson ME. Axial length Developmental Cataracts in Young
measurements by contact and

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 79

Subspeciality-Pediatric Ophthalmology

Children in South India and Causes of to manual capsulorhexis in pediatric impairment and blindness in infants:
Poor Outcome. Ind J Ophthalmol 2013; cataract. BMJ Open Ophthalmol2019; Causes and opportunities for control.
62 (2): 65-70. 4(1):e000255. Middle East Afr J Ophthalmol Apr-Jun
2011;18:109-114.
26. Gogate P, Patil S, Kulkarni A, 30. Kaur S, Sukhija J, Ram J. Comparison of 35. Gogate P, Kalua K, Courtright P.
Sahasrabudhe M, Shah M, Mahadik A, posterior optic capture of intraocular Childhood blindness in developing
Sane S, Bhasa D. Unilateral congenital lens without vitrectomy vs endocapsular countries- Time for a reassessment?
and developmental cataracts in implantation with anterior vitrectomy PLoS2009 ;6(12):e1000177.
children in India: How useful were in congenital cataract surgery: A 36. Gogate P, Dole K, Ranade S, Deshpande
long term outcomes of surgery. Miraj randomized prospective study. Ind J M. Cost of pediatric cataract in
Pediatric Cataract Study 4. Asia Pac J Ophthalmol 2020 Jan;68(1):84-88. Maharashtra, India. Int J Ophthalmology
Ophthalmol2015; 4(6): 376-80. 2010; 10(7): 1248-52.
31. Kokhar SK, Tomar A, Pillay G, Agrawal
27. Tripathi S, Rishikeshi N, Tripathi S, E. Biometric changes in Indian Corresponding Author:
Kaduskar-Aney A, Taras S, Deshpande pediatric cataract and postoperative
M. Evaluation of visual outcomes refractive status. Ind J Ophthalmol Dr. Parikshit Gogate MS
after pediatric cataract surgery in a 2019;67(7):1068-72. Community Eye Care Foundation,
Tertiary Eye Care Hospital in Western Dr.Gogate’s Eye Clinic, Pune,
Maharashtra. J ClinOphthalmol Res 32. Gogate P, Sahasrabudhe M, Shah M, Patil Maharashtra, India.
2016;4:13-8. S, Kulkarni AN. Causes, epidemiology
and long-term outcome of traumatic
28. Cao K, Wang J, Zhang J, Yusufu M, cataracts in children in rural India. Ind J
Jin S, Hou S, et .al. Efficacy and safety Ophthalmol 2012; 60(5):481-4.
of vitrectomy for congenital cataract
surgery: a systematic review and meta- 33. Mboni C, Gogate P, PhiriA, Seneadza
analysis based on randomized and A, RamsonP, Manolakos-TsehisiH,
controlled trials. ActaOphthalmologica Musonda L, Benjamin L, Øverland L.
2019;97(3):233-39. Outcome of pediatric cataract surgery
in Copper belt province of Zambia. J
29. Chougale P, Warkad V, Badakere Pediatric Ophthalmol Strabismus 2016;
A, Kekuniya R.Precision pulse 53(5): 311-7.
capsulotomy: An automated alternative
34. Gogate P, Gilbert C, Zin A. Severe visual

80 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

Pediatric Optic Neuritis: Current
Concepts in Diagnosis and
Management

1Divya Natarajan, 2Mohan Kannam, 3Ramesh Kekunnaya, 2Virender Sachdeva
1. Consultant, Child Sight Institute, Miriam Hyman Children’s Eye Care Centre, L.V. Prasad Eye Institute, Bhubaneshwar.
2. Consultant, Child Sight Institute, Nimmagadda Prasad Children’s Eye Care Centre, L.V. Prasad Eye Institute, Visakhapatnam.
3. Consultant and Head, Child Sight Institute, Jasti V Ramanamma Children’s Eye Care Centre, L.V. Prasad Eye Institute, Hyderabad.

Optic neuritis (ON) in children is presentations. These differences are findings, and outcomes in pediatric
relatively uncommon but is one of most pronounced in younger (pre- optic neuritis in the light of current
the most common conditions seen pubertal) children (Table 1). In recent available literature.
in pediatric neuro-ophthalmology years, widespread availability of the
clinics. Although pediatric optic neuroimaging and serological testing Subtypes of optic neuritis
neuritis clinically presents with a triad has enabled greater understanding Morphological Subtypes
of vision loss, pain on eye movements and prospective outcome studies in Optic neuritis in children can be
and dyschromatopsia like in adults, pediatric optic neuritis. This article subclassified based on the fundus
there are significant differences in the attempts to provide an overview of the appearance into the following three
etiological considerations and clinical clinical presentations, neuroimaging types (Figure 1):

Table 1: Difference between Adult and Pediatric Optic neuritis

Characteristics Pediatric optic neuritis Adult optic neuritis

Gender distribution Males=Females (pre-pubertal)1 Females>Males
Females>Males (post pubertal)2

Associated Prodromal Prodromal illness often present Prodromal illness often absent
illness

Laterality Frequently bilateral Typically, unilateral, bilateral is uncommon and atypical

Etiology Frequently post infectious or post Clinically isolated syndrome or part of systemic
vaccination; Associated systemic demyelinating disease: MS, NMOSD followed by clinically
disease: ADEM, MOG optic neuritis isolated syndrome, ADEM. MOG optic neuritis
followed by MS, NMO

Pain on Eye movements Painless in nearly half (50%)3 Pain on eye movements in nearly 90% in Western data5
and 25 to 50% in Indian population6

Vision loss at Severe visual impairment (70% to Moderate vision loss
presentation 85%)

Dyschromatopsia Dyschromatopsia in 50%7 Dyschromatopsia in 90%8

(Impaired Color vision)

Fundus findings Frequently Papillitis (73%)9 Frequently Retrobulbar (67%)4

Visual field defect Central or Centro-caecal field defect Generalized depression most common11
pattern most common10

Visual outcomes Good visual prognosis with Good recovery, however, as more often reported to
spontaneous improvement in most have systemic etiology visual prognosis depends on the
cases underlying etiology

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 81

Subspeciality-Pediatric Ophthalmology

Figure 1: Morphological subtypes of optic neuritis: (a) Papillitis with disc edema, (b) (Box 1).
Retrobulbar neuritis with normal appearing discs and (c) Neuroretinitis showing disc
edema with associated macular edema and exudates. Since bilateral disc edema is the most
common presentation, we always
Box 1: Classical presentation of a child with optic neuritis need to consider secondary causes of
yy Acute or subacute onset (1-3 weeks) disc edema with vision in differential
yy Prodromal illness (fever, headache, vomiting) diagnosis in children (Box 2).
yy Bilateral involvement
yy Severe visual impairment Following section describes the
yy Papillitis presentation of optic neuritis in various
yy Spontaneous recovery clinical settings:
yy Good visual prognosis
yy Pain on eye movements is variable seen in about 50% of children Post infectious and post vaccination
optic neuritis:
Box 2: Differential diagnosis for disc edema in children
yy Acute expansion of an intracranial tumor e.g. Chiasmal glioma, posterior fossa Post-infectious and post-vaccination
optic neuritis is one of the most
tumor, neuroblastoma, etc. common presentations of optic neuritis
yy Papilledema due to idiopathic intracranial hypertension, cerebral sinus venous in children. The presumed mechanism
of optic neuritis is demyelination
thrombosis, or meningitis secondary to an autoimmune reaction
yy Leber’s hereditary optic neuropathy to the precipitating infectious agent or
yy Infiltration of the optic nerve in leukaemia, lymphoma, or inflammatory vaccination.

diseases Etiology: Common causes of post-
infectious optic neuritis include
Most commonly, Papillitis (associated infectious, post vaccination, or may measles, mumps, chickenpox, rubella,
with optic disc edema, hyperaemia, and occur in association with systemic pertussis, herpes simplex, herpes zoster,
blurred margins), demyelinating conditions like acute adenovirus, Lyme disease, brucellosis,
Retrobulbar neuritis (with a normal disseminated encephalomyelitis cat-scratch disease, cryptococcus and
fundus appearance) and (ADEM), multiple sclerosis (MS) and pneumonia due to Mycoplasma13,14.
Neuroretinitis (associated with disc neuromyelitis optica spectrum disorder Vaccinations that are most frequently
edema, peripapillary RNFL edema, hard (NMOSD)12,13. associated with development of
exudates often extending to the macula, pediatric optic neuritis include measles,
peripapillary full thickness or outer Clinical presentation mumps, rubella, influenza, rabies, and
retinal lesions). Patients with neuro- Optic neuritis in children most smallpox.
retinitis usually have an infectious commonly presents acutely with
etiology and are not associated with bilateral, painless vision loss. Visual Presentation: There can be 3 modes of
demyelination. impairment is severe (<20/200) in up to presentation: Postinfectious, para-
Etiological subtypes: Optic neuritis 70%-85% of the cases. Papillitis is the infectious and subclinical.
in children may be idiopathic, post most common presentation, followed
by retrobulbar neuritis. The typical Post-infectious: This is the most common
clinical features are summarized in form. Onset is usually acute to subacute
(few days to 3 weeks) following onset
of infection or vaccination. Majority
of the patients tend to have a post-
infectious optic neuritis and there are
no concurrent symptoms. However,
there may be prior history of fever,
lymphadenopathy, and weight loss.
In these patients’ presentation is like a
typical case of papillitis or retrobulbar
neuritis.

Para-infectious Optic neuritis: Few patients
might have optic nerve involvement
concurrent with a systemic or

82 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

patients with neurological symptoms,
seizures, and abnormal neuroimaging.
MRI is normal in isolated cases apart
from the involvement of optic nerves.
In patients with para-infectious optic
neuritis, evidence of sinusitis might be
noted.

Outcomes: Visual recovery is good in

majority of these patients. Existing
literature suggests that 85% to 90% of
the cases regain visual acuity greater
than 20/30 at one-year follow-up.
While most recover spontaneously, in
Figure 2: MRI orbit (a) axial section showing right optic nerve enhancement (yellow arrow) atypical cases, bilateral vision loss, one-
on contrast suggestive of optic neuritis; (b) coronal section showing swollen and thickened eyed patients, or concerned patients
right optic nerve (yellow arrow head) with concurrent bilateral maxillary sinusitis (yellow intravenous methylprednisolone
asterisk) suggestive of para-infectious optic neuritis. (IVMP) can be given to hasten the
recovery. In practice, majority of the
Box 3: Points to remember about post-infectious/post-vaccination optic patients, do opt for high dose IV steroids.
neuritis
yy Common presentation Pediatric Multiple sclerosis (MS)
yy Majority present with papillitis/ retrobulbar neuritis Multiple sclerosis is a demyelinating
yy Onset few weeks following infection/ vaccination disease of the central nervous system
yy Few may have a para-infectious course that characteristically follows a
yy Good outcome, monophasic, and recurrences are uncommon. remitting-relapsing course. There is a
female preponderance and MRI shows
Box 4: Clinical features that may suggest possible MS in a child with optic lesions that show dissemination in time
neuritis and space.
yy Older age at presentation (Post-pubertal)
yy Recurrent optic neuritis Etiology: Multiple sclerosis is an
yy Associated neurological symptoms involving long white matter tracts such as inflammatory and demyelinating
disease where antibodies against myelin
limb weakness, paraesthesia, imbalance. are released causing neuronal cell death
yy Presence of periventricular white matter lesions, centrum semiovale, and axonal degeneration15.
Presentation: Unlike in adults, optic
subcortical white matter, which show dissemination in time and space. neuritis in children is less frequently
associated with multiple sclerosis. Prior
local infection (Figure 2). Common high contrast visual acuity is generally retrospective studies have reported the
systemic infections include Lyme good, there might be impairment of low risk of MS after the first episode of ON
disease, Brucellosis, etc. In these cases, contrast visual acuity, subnormal color ranges from 7% to 39% in children16.
concurrent systemic and neurological vision and mild disc changes. Many of Factors associated with highest risk
symptoms such as headache, seizures these patients might have associated of conversion to Clinically definite
and meningismus, when associated sinusitis. multiple sclerosis (CDMS) are older
with meningoencephalitis. They age (>11 years), presence of MRI lesions
usually present with vision loss, Investigations: Serological and systemic at presentation, bilateral sequential
manifest bilateral disc edema or investigations are usually not needed or recurrent ON and presence of
neuroretinitis-like picture. in patients with postinfectious oligoclonal bands on CSF analysis17,18.
Subclinical: Another presentation could optic neuritis. However, it is usually Optic neuritis in MS is frequently
be a subclinical optic neuritis where mandatory in patients with para- recurrent which is diagnosed by disc
the patients develop mild vision loss infectious optic neuritis. Investigations pallor in the affected or fellow eye.
following episode of viral illness or are guided by the presenting features, Other presentations can be seizures
chronic sinusitis. These patients might and clinical presentations such as and ataxia (focal supratentorial
have only mild blurred vision and might associated peripapillary retinal lesions. syndrome), weakness, imbalance, and
even be detected accidentally. While Lumbar puncture and cerebrospinal cranial neuropathy (focal brainstem
(CSF) analysis might be needed in

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 83

Subspeciality-Pediatric Ophthalmology

Figure 3: MRI Brain (a) axial section and (b) sagittal section, showing multiple hyperintense progressive course of the neurological
lesions (yellow circles) ranging from subcortical to deep white matter predominantly in deficit in MS with debilitating
periventricular region. However, these might also be seen in the subcortical white matter, neurological disease. Fortunately such
centrum semiovale, long white matter tracts, spinal cord, and cerebellum. cases are relatively uncommon in
pediatric MS.
Figure 4: Fundus photo of the RE (a) showing normal appearing disc and LE (b) showing
disc pallor (yellow arrow) in a patient with sequential optic neuritis in NMO. Neuromyelitis Optica Spectrum
Disorder (NMO-SD):
or cerebellar syndrome), or partial white matter lesions, predominantly
myelopathy19. in the periventricular white matter Neuromyelitis Optica classically
Investigations: The diagnosis of Pediatric region (Figure 3). Lesions in MS show refers to the demyelinating disease
MS, like that in adults, is based on dissemination in time and space. characterized by optic neuritis, and
the McDonald criteria or Swanton Though not an absolute criterion for severe transverse myelitis (Devic’s
criteria but the positive predictive diagnosis, CSF shows the presence of disease). However, over the last decade
value is lower for children less than 11 oligoclonal bands in about 85% of cases. or so, widespread availability of
years20,21. One study showed that after Outcome: ON is commonly recurrent in antibodies against aquaporin channels
a first demyelinating clinical episode MS. 85% of cases follow a remitting- and understanding of neuroimaging
(Clinically isolated syndrome), children relapsing course. Usually visual findings has changed the diagnostic
younger than 7 years were more likely recovery is good after the first episode criteria for neuromyelitis optica (NMO).
to have ADEM while those older than and even in cases of recurrence, but Currently the term neuromyelitis
12 years were more likely to develop might be incomplete in recurrent optica spectrum disorders (NMOSD)
CDMS22. MRI brain characteristically cases. Recurrences are usually spaced refers to disease entities that present
shows asymmetric but well-defined out. Further, 15% show a primary with clinical features of optic neuritis,
transverse myelitis, brainstem
involvement and cerebral signs23,24. It
was further stratified on the basis of
aquaporin 4 (AQP4-IgG) positivity in
the 2015 revised consensus diagnostic
criteria for NMO-SD. Although a
detailed description of the disease is
beyond the scope of this article, we
describe some salient differences in the
pathogenesis of NMO and MS.

Etiology: NMO is primarily an
astrocytopathy and autoantibodies
are directed against aquaporin-4 water
channels on astrocytes foot plates.
These aquaporin channels are expressed
closely in relation to optic nerves, as
well as grey and white matter of the CNS
(hippocampus, neocortex, cerebellum
and circumventricular structures),
ependymal cells of the ventricles, pia
mater, posterior optic nerve, retina and
inner ear25. Further damage mediated
by these autoantibodies leads to more
profound and often permanent damage
secondary to necrosis.

Presentation: Optic neuritis may be
the presenting feature in 50% to 75%
cases in pediatric NMO26. Optic neuritis
in these children is usually bilateral
(sequential, (Figure 4) or simultaneous),
and often associated with profound

84 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

vision loss. The reported risk of Box 5: Characteristic features that suggest diagnosis of Neuromyelitis
developing NMO after an episode of Optica
ON in children ranges from 1% to yy Bilateral involvement with profound vision loss.
7%. However, in children, isolated ON yy Transverse myelitis.
frequently presents without transverse yy Prolonged vomiting, hiccups (area postrema syndrome).
myelitis27,28. Even when present, spinal yy Hearing loss, facial weakness, trigeminal neuralgia, diplopia, ptosis, and
involvement is less specific and can
occur in MS and ADEM as well. nystagmus.
yy Symptomatic narcolepsy (acute diencephalic syndrome).
NMO associated neurological yy Longitudinally extensive optic neuritis with chiasmal involvement on MRI
symptoms are summarized in (Box 5).
brain.
Investigations: While seropositivity for yy Longitudinally extensive transverse myelitis (LETM) involving more than 3
aquaporin antibodies plays a major
role in diagnosis of NMO, reported contiguous segments of spinal cord.
sensitivity of the test in children ranges
from 67% to 80% as compared to 73% Figure 5: MRI orbit (a) axial section post contrast showing bilateral enhancement up to
in adults29,30. Neuroimaging of Brain and chiasm (yellow arrows) suggestive of atypical optic neuritis frequently seen in NMO and
spine plays a major role in establishing MRI brain sagittal section (b) showing area large brainstem lesion extending in lower
diagnosis of NMO irrespective of the medulla and upper cervical cord (yellow arrow) suggestive of NMO.
seropositivity for Aquaporin antibodies
(Figure 5). describe the characteristic cerebellar ataxia, brainstem syndromes, ill-defined lesions and typically
MRI lesions of NMO. myelitis, and rarely myeloradiculopathy do not show any dissemination in
and extrapyramidal syndromes. time (enhance at the same time, i.e.
Outcome: NMO in children is reported they are of the same age) (Figure 6).
to be more frequent and disabling in Investigations: Neuroimaging often Periventricular hyperintensities are
children, with greater preponderance shows multiple white matter lesions less frequent compared to MS. Lesions
of brain MRI lesions. Visual recovery which might be round to oval, large and on MRI show near-complete resolution
in pediatric NMO is very poor with enhance on gadolinium administration. with time. Even rare recurrences of
an average time from disease onset However, unlike lesions in MS, these optic neuritis, new lesions usually
to severe vision loss (<20/200) of 1.3 tend to be confluent, have perilesional do not appear in the CNS. (Table 2)
years31. edema, and might occur in areas other summarizes the difference between
than periventricular area. ADEM and MS on neuroimaging33.
Acute demyelinating CSF analysis shows mild to moderate
encephalomyelitis (ADEM) MRI shows T2 and fluid attenuation pleocytosis34. Oligoclonal bands may
ADEM is an acute demyelinating inversion recovery (FLAIR) be present transiently but unlike MS
syndrome with encephalopathy that hyperintense lesions in predominantly disappear with time.
presents predominantly in children Outcome: The disease is characteristically
and young adults with acute onset the white matter of the cerebrum, monophasic, though rare cases of
neurological symptoms. cerebellum, brainstem, and spinal cord recurrences have been reported.ADEM
and occasionally in the deep grey matter
Etiology: Classically most patients occur of thalamus and basal ganglia. These are
following infections, usually viral, usually diffuse, large (1-2 cm), bilateral,
or post vaccination due to an auto-
immune response against myelin or
other self-antigens32. However, in some
cases this history may not be elicitable.

Presentation: Classical presentation
is with fever, confusion, headache,
meningismus, seizures, vomiting,
abdominal pain, and encephalopathy.
Prodromal illness may precede
neurological symptoms. Optic nerve
involvement is common and bilateral.
There may also be associated altered
mental status, pyramidal signs,

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 85

Subspeciality-Pediatric Ophthalmology

Figure 6: MRI Brain axial cuts showing multiple diffusely ill-defined, confluent lesions associated splinter haemorrhage
with perilesional edema and enhancement on contrast suggestive of Acute demyelinating in the peripapillary area. (Figure 7)
encephalomyelitis (ADEM). Presence of confluent lesions with perilesional edema is highly Patients with MOG optic neuritis have
suggestive of ADEM. features that suggest widespread CNS
involvement as in ADEM. However,
these patients may have associated
myelitis, encephalomyelitis, and
are believed to be part of spectrum
of widespread CNS demyelinating
disease, Myelin Oligodendrocyte
Glycoprotein associated Optic Neuritis,
Encephalomyelitis and Myelitis
(MONEM)38. Box 7 summarizes clinical
features of optic neuritis seen in MOG.
Investigations: Anti-MOG antibodies are
more common in the Pediatric age group
but less specific as they may be positive
in several clinical syndromes including
multiphasic ADEM, recurrent ON, TM,
ADEM followed by ON and NMO-IgG
seronegative NMO-SD39,40. CSF shows

Table 2: Clinical and radiological differences between ADEM and MRI

Acute demyelinating Multiple sclerosis
encephalomyelitis

Age More frequent in children Relatively more frequent in adults

Prodromal symptoms Often present Less frequent, might have other neurological symptoms

History of recent Frequent Rare
vaccination/ febrile illness

Recurrences Uncommon Frequent

Neuroimaging Characteristics33

Distribution of lesions Bilateral, extensive Scattered, asymmetric

White matter lesions Confluent and ill-defined Well-defined Periventricular preponderance
Subcortical and periventricular

Corpus callosum lesions Rare Common

Grey matter lesions Thalamic and basal ganglia Uncommon
involvement common

Edema and mass effect May be present Absent

Follow-up MRI No new lesions New lesions – dissemination in space and time

does not require long term treatment Box 6: Points to remember about ADEM
and visual recovery is usually good. yy Associated with multiple neurological symptoms.
MOG-IgG associated optic neuritis: yy Prior viral illness like prodrome might precede vision loss.
Although described only a few years yy Multiple, confluent lesions on MRI with significant perilesional edema.
ago, this entity is becoming more yy Monophasic course, and usually prognosis is good.
conspicuous in patients especially
children with optic neuritis. Males are Etiology: This demyelinating along with myelin basic protein (MBP)
affected more in contrast to NMO and disease primarily affects myelin and proteolipid protein, resulting
the Caucasian race is especially prone35. oligodendrocyte (MOG) which is a in inflammation and destruction of
component of the CNS myelin sheath myelin36,37. In comparison to NMO,

86 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

Figure 7: Fundus photos depicting bilateral disc edema, peripapillary retinal nerve fibre mild to moderate pleocytosis and high
layer obscuration and few peripapillary haemorrhages (yellow arrows) in a patient with levels of MBP41. Neuroimaging in MOG
MOG associated ON. shows with bilateral, longitudinally
extensive optic nerve involvement
Figure 8: MRI orbits (a) axial T2 -weighted images section showing longitudinally extensive but sparing the chiasma (Figure 8
bilateral optic nerve involvement 9yellow arrows) but sparing of the chiasma (yellow and Box 7). Usually these patients
asterisk); coronal (post contrast) showing significant perineural enhancement (yellow also have associated Perineuritis (i.e.
arrows)compared to optic nerve parenchyma and (c) MRI brain FLAIR (fluid attenuation inflammation of the optic nerve sheath
inversion recovery) axial section showing hyperintense lesion along the visual pathway in addition to the involvement of the
yellow arrows) suggestive of MOG optic neuritis Note again the lesions are larger than the optic nerve itself)
typical lesions in multiple sclerosis and can involve deeper CNS structures.
Outcome: Current understanding of this
Box 7: Clinical and neuroimaging features that characterize optic neuritis condition, shows that patients with
in MOG antibody associated Optic Neuritis MOG- Optic neuritis show recurrences
yy Bilateral involvement with profound vision loss. similar to NMO, however there is better
yy Evidence of prior optic nerve involvement: disc pallor in same or fellow eye. recovery and lesser neuro-axonal retinal
yy Associated splinter haemorrhage in the peripapillary area. damage as compared to NMO. Most
yy Good recovery but frequent recurrences and steroid dependant course. patients do need long term steroids and
yy Associated constitutional/ prodromal symptoms (nausea, vomiting, abdominal immune-suppressive agents to prevent
relapses for a long time, and might need
pain, hiccups). anti-epileptics to control seizures if
yy Neurological symptoms such as ataxia, limb weakness, seizures, etc. associated.
yy Longitudinally extensive optic neuritis but sparing of posterior optic nerves
yy Perineuritis (involvement of the optic nerve sheath). Recommended work up in
yy Diffuse widespread demyelinating lesions in the subcortical, deep white pediatric optic neuritis
Goals of workup of a child with optic
matter, grey matter and brainstem. neuritis are as follows:
yy Longitudinally extensive lesions in the lower spinal cord especially conus
• Characterize and monitor visual
medullaris. function: Ocular investigations
such as Visual fields, VEP and OCT
astrocytes are spared. or sequential involvement with
Presentation: Optic neuritis in MOG profound vision loss. Most cases • Confirm diagnosis of Optic neuritis:
usually shows bilateral simultaneous present with papillitis with MRI orbits with contrast

• Establish diagnosis of associated
demyelinating disease: MRI brain
with contrast and MRI spine in cases
of recurrent disease/ Neurological
symptoms.

• Serological workup to identify
biomarkers and characterize the
disease

• Ancillary workup: CSF analysis.
Ocular investigations

Visual fields: Wherever feasible they
should be obtained in patients with
both eyes at presentation and at
regular follow-ups for monitoring
recovery. Most common patterns in
acute presentation include generalized
depression, central / centro-cecal
scotoma, arcuate/other optic nerve
kind of field defects. However, it may be
difficult to get the visual filed test done

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 87

Subspeciality-Pediatric Ophthalmology

Figure 9: Distinctive imaging features of optic neuritis, myelitis, and brain lesions in AQP4- in very young, uncooperative and in
IgG–positive NMOSD patients versus MOG-IgG–positive NMOSD patients and patients children with severe visual loss.
with multiple sclerosis. The lesions are shown in dark red or light red. A, Optic nerve: AQP4-
IgG–positive patients preferentially present with long-length, bilateral, and posterior optic Visual evoked potentials (VEPs): VEP is
nerve involvement with chiasmatic extension. MOG-IgG–positive patients usually exhibit classically reported to show prolonged
long-length, bilateral, and anterior optic nerve involvement, with intraorbital optic nerve latency with marked preservation of the
swelling and usually with perineural gadolinium enhancement (light red straight lines amplitudes. However, these findings are
around the optic nerves). Multiple sclerosis patients classically have unilateral and short- not pathognomic but quite suggestive
length optic neuritis. B, Spinal cord: AQP4-IgG–positive patients have more LETM that is of optic neuritis. Therefore, VEP in
centrally or both centrally and peripherally located and involves more than 50% of the cord can be used to corroborate rather than
area, predominantly in the cervicothoracic region. MOG-IgG–positive patients usually have confirm the diagnosis of optic neuritis.
medullary conus and thoracolumbar spinal cord involvement with an atypical appearance In children, VEP shows reduced
on axial views but commonly centrally or both centrally and peripherally located. amplitudes in 83% cases of acute ON
Multiple sclerosis patients have longitudinal short-length spinal cord lesions, particularly and prolonged latency in 45% to 65%
in the cervical segment, which are peripherally distributed on axial images in the dorsal up to 12 months after onset44. Often VEP
and lateral areas. C, Brain: AQP4-IgG–positive patients typically have periventricular and visual field might suggest further
and circumventricular involvement and involvement of the corticospinal tracts (focal or fellow eye involvement in patients with
associated with vasogenic edema, demonstrating a trident-shaped appearance). MOG- unilateral vision loss and symptoms in
IgG–positive patients more often have basal ganglia, thalamic, and infratentorial lesions. one eye only.
Multiple sclerosis patients typically have ovoid white matter lesions that are distributed
in the periventricular regions (Dawson fingers), corpus callosum, callosal-septal interface, Optical coherence tomography (OCT): OCT
cortical/juxtacortical areas, and infratentorial regions with involvement of the intrapontine of the retinal nerve fibre layer (RNFL)
trigeminal nerve. (Reproduced with permission from Dutra BG, da Rocha AJ, Nunes RH, and macular ganglion cell analysis
Maia ACM Júnior. Neuromyelitis Optica Spectrum Disorders: Spectrum of MR Imaging (MGCL) can be done to look for the
Findings and Their Differential Diagnosis. Radiographics. 2018 Jan-Feb;38(1):169-193. structural damage. At presentation, it
might show increased RNFL thickness
in patients with papillitis, however,
over next few weeks thinning of the
retinal nerve fibre layer and ganglion
cell layer sets in. This can further assist
in prognosis and give subtle clues to
the possible association, e.g. in patients
with NMO and MOG optic neuritis
there is usually more marked thinning
of the RNFL and MGCL even after the
first episode of optic neuritis.

Systemic investigations

Magnetic resonance imaging of the
brain and orbits with and without
gadolinium contrast to look for optic
nerve inflammation is warranted in
every case. MRI of the orbits and brain
is the most important investigation
and gives clues to the diagnosis of
optic neuritis and associated systemic
disease. Further it rules out other CNS
inflammatory diseases, intracranial
neoplasm especially with acute
extension, helps prognosticate and
stratify risk of future development of
MS.

Figure 9 shows the radiological
difference appreciated on Neuroimaging

88 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

Table 3: Comparison between different demyelinating conditions associated with pediatric optic neuritis

ADEM MS associated NMO associated MOG associated

Pathology Autoantibodies against Autoantibodies Autoantibodies Autoantibodies against
myelin and other self- against myelin against astrocytes oligodendrocytes
antigens

Clinical onset and Predominantly Remitting-relapsing Relapsing course in Relapses occur but reduce
course monophasic. course in 85%. up to 90% in frequency with long
Recurrences very rare. term treatment

Mean age at onset <10 years 29 years 39 years Third decade

Gender predominance No gender predilection F:M=2:1 F:M=9:1 F:M=2.8:143

Constitutional Common Uncommon Uncommon Common
symptoms

Optic neuritis Bilateral papillitis Unilateral Sequential or Bilateral papillitis
features (most retrobulbar. simultaneous
common ON is the presenting bilateral retrobulbar.
presentation) sign in 40% ON is the presenting
sign in 50%-75%

Associated Meningismus, Non- Transverse myelitis, Area postrema syndrome
neurological features encephalopathy, encephalopathic Area postrema (nausea, vomiting,
seizures, fever, CNS clinical event syndrome (nausea, hiccough),
headache (paresis, paralysis, vomiting, hiccough), Limb weakness, ataxia,
tremors, ataxia, Symptomatic seizures
bladder, and bowel narcolepsy
disturbances)

MRI features Bilateral, extensive, Patchy Longitudinally Longitudinally
ill-defined lesions in hyperintensities of extensive optic nerve extensive optic nerve
the subcortical and optic nerve of T2 hyperintensities hyperintensities but
periventricular white sparing of posterior optic
matter. with gadolinium with chiasmal nerves.
Thalamic and basal involvement.
ganglia lesions. enhancement Longitudinally Perineuritis.
No new lesions Scattered,
well-defined extensive transverse Diffuse widespread
periventricular white myelitis (LETM) demyelinating lesions in
the subcortical, deep white
matter lesions. involving more
Grey matter rarely than 3 contiguous matter, grey matter and
involved segments of cervical brainstem.
Lesions disseminated or thoracic spinal Longitudinally extensive
in time and space cord lesions in the lower spinal
cord especially conus

medullaris.

CSF analysis Mild to moderate Mild pleocytosis. Moderate to Moderate to prominent
pleocytosis. pleocytosis
Oligoclonal bands, if Oligoclonal bands prominent
present, are transient present in 85% cases pleocytosis

Treatment (apart Not required Interferon beta, Azathioprine, Long term oral steroids,
from short term Glatiramer acetate, Rituximab, MMF Rituximab, Azathioprine,
steroids or IVIG/ Rituximab, MMF
PLEX) natalizumab.

Prognosis Good visual outcome Poor visual prognosis Good visual prognosis

ADEM, acute demyelinating encephalomyelitis; MS, multiple sclerosis; NMO, neuromyelitis optica; MOG, myelin oligodendrocyte; F, female; M, male,
ON, optic neuritis; CNS, central nervous system; MRI, magnetic resonance imaging; CSF, cerebrospinal fluid; IVIG, intravenous immunoglobulin; PLEX,
plasma exchange; MMF, mycophenolate mofetil.

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 89

Subspeciality-Pediatric Ophthalmology

Table 4: Drugs used for long term immunosuppression in pediatric optic neuritis

Drugs Route Dosage Details

Prednisone Oral 1-2 mg/kg/day Overlap with other immunosuppressors for 3-6
months

Azathioprine Oral 2-3 mg/kg/day in 2 doses Target dose guided by Absolute lymphocyte count
and Mean corpuscular volume; monitor liver
function

Mycophenolate Oral 1500–3000 mg/day in 2 doses Target dose guided by ALC and blood
mofetil concentration (1–2 μg/mL)

Rituximab Intravenous 1000 mg given twice, 14 days apart. First-line therapy in NMO-SD; CD19+ B/CD20+B
Repeat every 6 months or based on cells as a marker
re-emergence of CD19 B cells

in three common demyelinating by an oral corticosteroid taper46. mofetil and rituximab. However,
disease: multiple sclerosis (MS), Retrospective study by Jayakody et al Cyclophosphamide and mitoxantrone
neuromyelitis optic (NMO), and MOG- showed no difference in relapse rate are not recommended in children due
optic neuritis. between short (2 weeks) vs prolonged to severe side effects like haemorrhagic
(>2 weeks) course of oral steroids in cystitis, secondary malignancy, and
Magnetic resonance imaging of the the relapse rate or visual outcome in infertility, while mitoxantrone carries a
spine might be needed in patients with children47. risk of cardiotoxicity and leukemia.
recurrent optic neuritis, neurological
signs and in patients with high Intravenous Immunoglobulin (IVIG) All these immunomodulatory drugs
suspicion of NMO/ MOG optic neuritis and Plasma Exchange (PLEX) or should have an overlapped with oral
or Multiple sclerosis. plasmapheresis: Both IVIG and PLEX steroids for about 3-6 months until
have been used in patients with poor they reach their maximum therapeutic
CSF analysis: CSF analysis is not response to corticosteroids especially effect. All of the below drugs can be
routinely recommended usually in conditions like NMO48,49. Side effect used as long-term therapy and there are
but might be indicated in following include headache, myalgia, fever, and no studies showing definite preference
situations: allergic reactions. Plasmapheresis of one drug over other and their
eliminates the pathogenic antibodies, efficacy. However, Rituximab holds
• Multiple sclerosis without other from circulation and has higher greater promise even in patients having
confirmatory features to look for therapeutic efficacy than IV recurrences on other agents. Choice
oligoclonal bands. corticosteroids. Its early use as first-line of drugs depend on drug availability,
therapy following attack is a predictor cost, affordability, disease course and
• Patients with high suspicion for of better remission. experience of the treating physician.
atypical etiology. Immunosuppressive therapy is safe
Long Term treatment to avoid to continue for disease-free 3-year
• Recurrent Optic neuritis in NMO/ relapses period before stopping to avoid relapse
MOG but not meeting diagnostic especially in pediatric population
criteria. The greatest challenge is the long-
term management of these patients Prognosis
Serology: NMO for suspected cases of who are more prone to relapses. Long
NMO-SD; and MOG for suspected MOG term immunosuppression is required Most studies have reported good visual
optic neuritis. in all cases of recurrent optic neuritis outcomes (visual acuity better than
I.e. usually associated with Multiple 20/40) in 70%-85% of children (Table
Treatment guidelines sclerosis, NMOSD and MOG optic 5).
Acute episodes neuritis. While most commonly
used agents for management The extent and mean time to visual
Intravenous high dose Methyl of MS are interferons, Tysabri, recovery depends on the presenting
prednisolone: Treatment of acute phase Fingolimod, and Dimethyl fumarate, visual acuity, presence of underlying
of optic neuritis is extrapolated from immunomodulatory agents used neurologic disease (such as MS or
adult ONTT trials. Treating children for NMOSD and MOG optic neuritis NMOSD), and whether corticosteroid
with 3 days of intravenous methyl- are azathioprine, mycophenolate treatment was given.
prednisolone (16 mg/kg/d), for first
attack and for 5 days for recurrent
attacks are recommended followed

90 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Subspeciality-Pediatric Ophthalmology

Even in cases of complete recovery of 2. Absoud M, Cummins C, Desai N, et 15. Söderström M, Link H, Sun JB,
visual acuity, sequelae of optic neuritis al. Childhood optic neuritis clinical Fredrikson S, Wang ZY, Huang WX.
like reduced low contrast visual acuity, features and outcome. Arch Dis Child. Autoimmune T cell repertoire in optic
reduced colour vision, persistent 2011;96(9):860-862. neuritis and multiple sclerosis: T cells
pupillary defect, thinning of the retinal recognising multiple myelin proteins
nerve fibre and ganglion cell layer 3. Wan MJ, Adebona O, Benson LA, are accumulated in cerebrospinal
persist. Gorman MP, Heidary G. Visual fluid. J Neurol Neurosurg Psychiatry.
Close follow-up is required in children outcomes in pediatric optic neuritis. Am 1994;57(5):544-551.
(4 weeks, 3 months, and 6 months) J Ophthalmol. 2014;158(3):503-7.e2.
after optic neuritis to look for an 16. Kim YM, Kim HY, Cho MJ, et al. Optic
underlying neurological disorder and 4. Beck RW, Cleary PA, Anderson MM Jr, Neuritis in Korean Children: Low Risk of
a repeat MRI in 3 months if the initial et al. A randomized, controlled trial of Subsequent Multiple Sclerosis. Pediatr
MRI was abnormal. Early intervention corticosteroids in the treatment of acute Neurol. 2015;53(3):221-225.
and appropriate long-term therapy can optic neuritis. The Optic Neuritis Study
reduce recurrences and disability in Group. N Engl J Med. 1992;326(9):581- 17. Waldman AT, Stull LB, Galetta SL, Balcer
these children. 588. LJ, Liu GT. Pediatric optic neuritis and
RBN, retrobulbar neuritis; VA, visual risk of multiple sclerosis: meta-analysis
acuity; ON, optic neuritis; ADEM, acute 5. The clinical profile of optic neuritis. of observational studies. J AAPOS.
demyelinating encephalomyelitis; MS, Experience of the Optic Neuritis 2011;15(5):441-446.
multiple sclerosis; NMO, neuromyelitis Treatment Trial. Optic Neuritis
optica; MOG, myelin oligodendrocyte; Study Group. Arch Ophthalmol. 18. Heussinger N, Kontopantelis E, Gburek-
MRI, magnetic resonance imaging; 1991;109(12):1673-1678. Augustat J, et al. Oligoclonal bands
PEDIG, pediatric eye disease investigator predict multiple sclerosis in children
group. 6. Saxena R, Phuljhele S, Menon V, with optic neuritis. Ann Neurol.
Gadaginamath S, Sinha A, Sharma P. 2015;77(6):1076-1082.
Conclusion Clinical profile and short-term outcomes
Most cases in children present acutely of optic neuritis patients in India. Indian 19. Thompson AJ, Banwell BL, Barkhof F,
or sub-acutely after a prodromal illness J Ophthalmol. 2014;62(3):265-267 et al. Diagnosis of multiple sclerosis:
with bilateral papillitis causing severe 2017 revisions of the McDonald criteria.
visual impairment. Most frequently 7. Tekavcic-Pompe M, Stirn-Kranjc B, Lancet Neurol. 2018;17(2):162-173.
patients have postinfectious, post- Brecelj J. Optic neuritis in children--
vaccination etiology or clinically clinical and electrophysiological follow- 20. Borchert M, Liu GT, Pineles S, Waldman
isolated syndrome, however, recent up. Doc Ophthalmol. 2003;107(3):261- AT. Pediatric Optic Neuritis: What Is
literature suggests that rise in the 270. New. J Neuroophthalmol. 2017;37 Suppl
detection of associated systemic diseases. 1(Suppl 1):S14-S22.
Visual recovery is usually excellent in 8. Abou Zeid N, Bhatti MT. Acute
most children; however, recurrences inflammatory demyelinating optic 21. Polman CH, Reingold SC, Banwell B, et al.
are frequent in NMOSD, MOG optic neuritis: evidence-based visual Diagnostic criteria for multiple sclerosis:
neuritis and MS Careful attention to the and neurological considerations. 2010 revisions to the McDonald criteria.
associated symptoms, fundus findings Neurologist. 2008;14(4):207-223. Ann Neurol. 2011;69(2):292-302.
and neuroimaging findings often gives
clues to the underlying etiology. Most 9. Morales DS, Siatkowski RM, Howard 22. Mikaeloff Y, Suissa S, Vallée L, et al. First
patients need neuroimaging and further CW, Warman R. Optic neuritis episode of acute CNS inflammatory
work up based on neuroimaging. in children. J Pediatr Ophthalmol demyelination in childhood: prognostic
However, optic neuritis associated with Strabismus. 2000;37(5):254-259. factors for multiple sclerosis and
demyelinating conditions requires disability. J Pediatr. 2004;144(2):246-252.
long term immunosuppression to 10. KENNEDY C, CARROLL FD. Optic
prevent recurrences and neurological neuritis in children. Arch Ophthalmol. 23. Wingerchuk DM, Banwell B, Bennett
complications. 1960;63:747-755. JL, et al. International consensus
diagnostic criteria for neuromyelitis
References 11. Beck RW, Trobe JD. What we have Optica spectrum disorders. Neurology.
1. Boomer JA, Siatkowski RM. Optic learned from the Optic Neuritis 2015;85(2):177-189.
Treatment Trial. Ophthalmology.
neuritis in adults and children. Semin 1995;102(10):1504-1508. 24. Chitnis T, Ness J, Krupp L, et al.
Ophthalmol. 2003;18(4):174-180. Clinical features of neuromyelitis
12. Chang MY, Pineles SL. Pediatric optica in children: US Network of
Optic Neuritis. Semin Pediatr Neurol. Pediatric MS Centers report. Neurology.
2017;24(2):122-128. 2016;86(3):245-252.

13. El-Dairi MA, Ghasia F, Bhatti MT. 25. Graber DJ, Levy M, Kerr D, Wade WF.
Pediatric optic neuritis. Int Ophthalmol Neuromyelitis Optica pathogenesis
Clin. 2012;52(3):29-xii. and aquaporin 4. J Neuroinflammation.
2008;5:22. Published 2008 May 29.
14. Rappoport D, Goldenberg-Cohen N,
Luckman J, Leiba H. Parainfectious 26. Tenembaum S, Chitnis T, Nakashima
optic neuritis: manifestations in I, et al. Neuromyelitis Optica spectrum
children vs adults. J Neuroophthalmol. disorders in children and adolescents.
2014;34(2):122-129. Neurology. 2016;87(9 Suppl 2):S59-S66.

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 91

Subspeciality-Pediatric Ophthalmology

27. Hino-Fukuyo N, Haginoya K, Nakashima 38. Dos Passos GR, Oliveira LM, da Costa of pediatric optic neuritis. Curr Treat
I, et al. Clinical features, and long-term BK, et al. MOG-IgG-Associated Optic Options Neurol. 2012;14(1):93-102.
outcome of a group of Japanese children Neuritis, Encephalitis, and Myelitis:
with inflammatory central nervous Lessons Learned from Neuromyelitis 47. Jayakody H, Bonthius DJ, Longmuir R,
system disorders and seropositivity to Optica Spectrum Disorder. Front Neurol. Joshi C. Pediatric optic neuritis: does
myelin-oligodendrocyte glycoprotein 2018;9:217. Published 2018 Apr 4. a prolonged course of steroids reduce
antibodies. Brain Dev. 2015;37(9):849- relapses? A preliminary study. Pediatr
852. 39. Peschl P, Bradl M, Höftberger R, Berger Neurol. 2014;51(5):721-725.
T, Reindl M. Myelin Oligodendrocyte
28. Nagaishi A, Takagi M, Umemura A, et al. Glycoprotein: Deciphering a Target in 48. Tenembaum S, Chitnis T, Nakashima
Clinical features of neuromyelitis Optica Inflammatory Demyelinating Diseases. I, et al. Neuromyelitis Optica spectrum
in a large Japanese cohort: comparison Front Immunol. 2017;8:529. Published disorders in children and adolescents.
between phenotypes. J Neurol 2017 May 8. Neurology. 2016;87:59-66.
Neurosurg Psychiatry. 2011;82(12):1360-
1364. 40. Hacohen Y, Wong YY, Lechner C, et 49. Brenton JN, Banwell BL. Therapeutic
al. Disease Course and Treatment Approach to the Management of
Responses in Children with Relapsing
29. Peña JA, Ravelo ME, Mora-La Cruz E, Myelin Oligodendrocyte Glycoprotein Pediatric Demyelinating Disease:
Montiel-Nava C. NMO in pediatric Antibody-Associated Disease. JAMA Multiple Sclerosis and Acute
patients: brain involvement and Neurol. 2018;75(4):478-487.
clinical expression. Arq Neuropsiquiatr. Disseminated Encephalomyelitis.
2011;69(1):34-38. 41. Nakajima H, Motomura M, Tanaka K, et Neurotherapeutics. 2016;13(1):84-95.
al. Antibodies to myelin oligodendrocyte
30. Wingerchuk DM, Lennon VA, glycoprotein in idiopathic optic neuritis. 50. Wilejto M, Shroff M, Buncic JR, Kennedy
Lucchinetti CF, Pittock SJ, Weinshenker BMJ Open. 2015;5(4):e007766. Published J, Goia C, Banwell B. The clinical
BG. The spectrum of neuromyelitis 2015 Apr 2. features, MRI findings, and outcome of
optica. Lancet Neurol. 2007;6(9):805-815. optic neuritis in children. Neurology.
42. Pache F, Zimmermann H, Mikolajczak 2006;67(2):258-262.
J, et al. MOG-IgG in NMO and related
31. Collongues N, Marignier R, Zéphir H, et disorders: a multicenter study of 51. Khadse R, Ravindran M, Pawar N,
al. Long-term follow-up of neuromyelitis 50 patients. Part 4: Afferent visual Maharajan P, Rengappa R. Clinical
optica with a pediatric onset. Neurology. system damage after optic neuritis profile, and neuroimaging in pediatric
2010;75(12):1084-1088. in MOG-IgG-seropositive versus optic neuritis in Indian population:
AQP4-IgG-seropositive patients. J A case series. Indian J Ophthalmol.
32. Alexander M, Murthy JM. Acute Neuroinflammation. 2016;13(1):282. 2017;65(3):242-245.
Published 2016 Nov 1.
disseminated encephalomyelitis: 52. Ambika S, Padmalakshmi K,
Treatment guidelines. Ann Indian Acad 43. Jarius S, Ruprecht K, Kleiter I, et al. Venkatraman V, Noronha OV. Visual
MOG-IgG in NMO and related disorders: Outcomes and Clinical Manifestations
Neurol. 2011;14(Suppl 1):S60-S64. a multicenter study of 50 patients. Part of Pediatric Optic Neuritis in Indian
2: Epidemiology, clinical presentation, Population: An Institutional Study. J
33. Panicker JN. Acute disseminated radiological and laboratory features, Neuroophthalmol. 2018;38(4):462-465.
encephalomyelitis. Annals of Indian treatment responses, and long-term
Academy of Neurology. 2007 Jul outcome. J Neuroinflammation. 53. Writing Committee for the Pediatric
1;10(3):137. 2016;13(1):280. Published 2016 Sep 27. Eye Disease Investigator Group (PEDIG),
Pineles SL, Repka MX, et al. Assessment
34. Chatterjee A, Datta S. Recurrent acute 44. Kriss A, Francis DA, Cuendet F, et of Pediatric Optic Neuritis Visual Acuity
disseminated encephalomyelitis: A al. Recovery after optic neuritis Outcomes at 6 Months [published online
favourable outcome among recurrent in childhood. J Neurol Neurosurg ahead of print, 2020 Oct 15]. JAMA
brain diseases in pediatric patient. J Psychiatry. 1988;51(10):1253-1258. Ophthalmol. 2020;e204231.
Pediatr Neurosci. 2016;11(3):241-243.
45. Dutra BG, da Rocha AJ, Nunes RH,
35. Sato DK, Callegaro D, Lana-Peixoto Maia ACM Júnior. Neuromyelitis Corresponding Author:
MA, et al. Distinction between MOG Optica Spectrum Disorders: Spectrum
antibody-positive and AQP4 antibody- of MR Imaging Findings and Their
positive NMO spectrum disorders. Differential Diagnosis. Radiographics.
Neurology. 2014;82(6):474-481. 2018 Jan-Feb;38(1):169-193. doi:
10.1148/rg.2018170141. Erratum in:
36. Johns TG, Bernard CC. The structure Radiographics. 2018 Mar-Apr;38(2):662.
and function of myelin oligodendrocyte PMID: 29320331.
glycoprotein. J Neurochem. 1999;72(1):1-
9. 46. Bonhomme GR, Mitchell EB. Treatment

37. Mayer MC, Meinl E. Glycoproteins Dr. Virender Sachdeva
as targets of autoantibodies in CNS Child Sight Institute, Nimmagadda Prasad
inflammation: MOG and more. Ther Children’s Eye Care Centre, L.V. Prasad Eye
Adv Neurol Disord. 2012;5(3):147-159. Institute, Visakhapatnam, India.

92 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Monthly Meeting Update

Hard Cataract: Decrease and
Conquer – Technique with a
Difference

1N.Z. Farooqui MS, 1J.S. Bhalla MS, 2Kiran Bhanot MS, 1Sunita MS, 1Pooja Kothari, 1­Karuna Dhepe
1. Department of Ophthalmology, D.D.U. Hospital, New Delhi, India.
2. Department of Ophthalmology, G.G.S.G. Hospital, New Delhi, India.

Abstract: This is a description of a technique with a difference from the conventional nucleofractis techniques commonly in
use where the surgeon aims for complete fragmentation of the nucleus. In the current technique an incomplete fragmentation
leaves the posterior leathery central plate intact thereby separating the hard endonuclear core from the epinucleus. The
central nucleus volume is initially decreased by central crater which assist in easy fragmentation as well. The presence of
epinuclear plate protecting the posterior capsule throughout the procedure offers safer and more effective completion of the
phacoemulcification procedure in the hard nuclear cataract by this technique.

Virtually every step of cataract surgery Figure 1 Figure 2
is more difficult in the setting of a
mature, brunescent nucleus (Figure 1). capsule (Figure 2). inadvertently placed over the
B) Do gentle hydrodissection to anterior capsule causing zonular
To appreciate how Phacoemulsification dialysis and also the capsular edge
techniques can improve our safety prevent capsular block. Elevating is less likely to be nicked by the
margin with challenging hard cataract bulky brunescent nucleus is more phaco tip.
cases, it is important to understand likely to seal the capsulorrhexis Preventing Incisional wound burns:
what causes these complications and to from below causing capsular
conceptualize different techniques. lenticular block. Any additional The thermal damage to the incision due
fluid will distend and rupture the to excessive heat production during
There are three main concerns to posterior capsule. phacoemulsification is prevented
enhance and improve safety margin C) Performing a large capsulorrhexis by Phaco-power modulation1 of the
with challenging hard cataract cases. prevents capsular – lenticular longitudinal U/S modality. Either
block and makes it easy to reach hyperpulse mode is used with reduced
1. Thermal damage to the incision the periphery of the nucleus for duty cycle or reduced burst width in
due to excessive heat production. horizontal chop. It further reduces burst mode. The rotational U/S modality
the chances of the chopper being of Ozil – IP (Alcon) and Ellips (AMO)
2. Endothelial cell trauma. used in continuous mode generates
3. Posterior Capsular rupture. lesser heat and also prevents Phaco tip
To maximize the surgical outcome in
hard brunescent cataract:

A) Stain anterior capsule with trypan
blue for proper visualization.
Poor red reflex impedes proper
visualization of capsulorrhexis
edge. Stained capsule edge is less
likely to nick it with the Ultrasound
Phaco tip or misplace a chopping
instrument on top of the anterior

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 93

Monthly Meeting Update

blockage.

Endothelial trauma: Endothelial cell
loss in brunescent nucleus is more
likely because of the:

a) IncreasedU/Spowerandphacotime2 Figure 3: Intraoperative OCT picture showing large swirling fragments coming into contact
utilized for phacoemulsification with central cornea.
due to increased density and
volume of nuclear material. The iii) The added rigidity and size of the
thermal injury due to increased nucleus more directly transfers
phaco power is in the vicinity of instrument related forces to the
incision and not in the corneal capsule and zonules.
centre.
iv) Vacuum level is maximized to
b) Shallow anterior chamber. improve holding power, thereby
increasing the risk of post occlusion
c) More manipulation and surge.
manoeuvres are required for
fragmentation. v) Sharp edges of the brunescent
fragments.
d) Excessive particulate turbulence Figure 4: Large Brunescent nuclear
within the anterior chamber fragment removed under Viscoelastic vi) And the greater capsular laxity
due to small swirling fragments injected behind the nuclear fragment. caused by associated weak zonules.
hitting the centre of the cornea
(Figure 3) are the most significant Figure 5A: Horizontal Chop; B: Vertical Protecting the Posterior Capsule
influence on the endothelial cell Chop. Creating an artificial epinucleus. The
loss. The microscopic nuclear techniques, phaco power modulation of viscoadaptive agent injected between
“shrapnel” emanating from the longitudinal U/S modality or rotational posterior capsule and nucleus elevates
phaco tip mechanically bombards U/S modality, torsional and ellips for the posterior nuclear plate from
the endothelium. This is more phacoemulsification further reduces the posterior capsule, stabilized the
likely responsible for the greater the endothelial loss intraoperatively. nucleus, thereby reducing tumbling of
endothelium cell trauma and Posterior Capsular Rupture (PCR) the nuclear fragments.
may be more relevant than the PCR is more likely to occur because:
contribution of phaco energy or i) Less protective epinucleus Post-occlusion surge is of particular
changes in the biomechanical concern as the final sharp edged nuclear
milieu during surgery3. to cushion the movement of fragments are being removed. The post-
endonucleus. occlusion surge permits the posterior
e) Corneal endothelial cell loss was ii) Thinner posterior capsule stretch capsule to trampoline forward because
higher in bevel down position of due to enlarged nucleus. of lax or deficient zonules and thin
phaco tip4. or absent epinucleus. Thus, the last
brunescent nuclear fragment removed
Endothelial cell loss can be minimized under viscoelastic injected behind the
by the use maximally retentive nuclear fragment and decreased pre-set
Ohthalmic Viscosurgical Device (OVD) vacuum (Figure 4).
which maximizes anterior chamber
depth. The dispersive, retentive Chopping Technique Classification
viscoadaptive device (Viscoat) is more Conventionally Chang divided
likely to be retained as a protective chopping into 2 general categories6:
layer against endothelium. The
cohesive viscoadaptive devices such a) Horizontal / Peripheral Chopping
as High molecular wt Hyaluronic acid Technique (Figure 5A): Instrument
is often flushed within seconds. The tips move toward each others in the
repeatedly recoating the endothelium horizontal plane during the chop.
with dispersive OVD during the Nagahara technique is the classical
phacoemulsification procedure is of example.
paramount importance5 in reducing the
endothelial loss. Reduction of the phaco
energy used by the use of phaco chop

94 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Monthly Meeting Update

b) Central / Vertical Chopping Technique Figure 6: Tightly Wedged chopped Figure 7: Central Crater.
(Figure 5B): The instruments tips fragments like jigsaw pieces.
move towards each other in the Splitting of the Nucleus (Figure 8)
vertical plane during the chop. portion of the nucleus frees an additional In conventional chopping (Figure
central space for manoeuvring to be 8 A&B) the aim is for complete
The conventional chopping techniques able to separate the remaining hard fragmentation of the lens by cracking
though popular but remains a challenge endonucleus core without the jamming the radial suture line. Whereas in the
in certain cases with hard brunescent of the nuclear fragments. Decrease and Conquer technique the
nucleus . The complete fragmentation is Decrease and Conquer Technique9 aim is for separation of the loosely
not easy because of posterior “ leathery The Decrease and Conquer technique adhesive lamellated structure thereby
quality” in hard nucleus prevents with the added modification of isolating the endonucleus (Figure 8 C).
fragment separation7. debulking of the nucleus by forming a Using the sharp vertical chopper
central crater, offsets various limitations (Figure 9A) splitting of the nucleus
The separated nuclear piece in both the of the conventional nucleofractis is initiated. However, in this step the
conventional techniques is hard with techniques. completer fragmentation of the nucleus
pointed ends and this coupled with The basic technique involves debulking is not required. The separation of the
minimal and / no epinucleus can result the nucleus by central crater formation, epinucleus from the nucleus core is
in PCR during its manoeuvring during nuclear splitting circumferential and enough to proceed to the next step.
phacoemulsifiction. separating the epinucleus from the (Figure 9B,9C,9D).
hard endonucleus core. The nucleus splitting is carried out all
In Peripheral Chopping technique The isolated hard endonuclear core is around the circumference.
the horizontally directed path of the phacoemulsified, thereby decreasing Phacoemulsification of Central
chopper may not be deep enough to the volume of the central nucleus and Hard Nucleus: On completing the
severe the leathery posterior plate of an subsequently conquering the remaining circumference splitting into 8
ultra brunescent nucleus. part of the lens. fragments, the unfragmented central
hard endonucleus is separated and
Further there will be insufficient space lifted up with the phacotip (Figure 10
in the peripheral bag for the chopper A,B). The isolated nuclear core is then
due to the absence of an epinuclear
shell.

In ‘Direct chop’ of Nagahar and ‘Quick
Chop’ technique of Dilman , the
chopped fragments remain tightly
wedged like jigsaw pieces which makes
it difficult to separate (Figure 6).

So the preferred technique in hard
cataract is to make a central crater8
(Figure 7) approximately 5 mm before
the proceeding for the splitting of the
remaining ultra brunescent nucleus.
The debulking of the hard central

Figure 8 DOS Times - Volume 26, Number 1, July-August 2020 95

www.dosonline.org/dos-times

Monthly Meeting Update (C) (D)
(A) (B)

Figure 9 (B) (C) (D)
(A) (B)

Figure 10
(A)

Figure 11

phacoemulsified (Figure 10C,D). The nuclear material is removed from dense brunescent nucleus as it
Removal of the remaining nuclear the epinuclear plate circumferentially is performed along the loosely
material in the Epinuclear Shell (Figure leaving only thinned epinuclear plate adhesive lamellated zone instead
11A,B). The remaining nuclear material which is gently held and lifted up with of the radial suture plane which
usually stay within the epinuclear shell. vacuum applied at the phacotip and is often densely packed at the
The nuclear material is held piece by removed. posterior epinucleus.
piece with the phaco tip, separating iii) The cleavage plane being different,
it from the epinucelar plate, pushing Advantages of Decrease and it is free from entanglement with
it back into position simultaneously, Conquer leathery posterior bridged nucleus
using the other hand instrument i) It does not require complete fibers.
(usually an elongated sinsky hook). iv) Minimizes the lateral movement
fragmentation. required for separation of the
ii) Separation is quite easy even in

96 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Monthly Meeting Update

nuclear fragment thereby reducing 4. Amir Faramazi, Mohammad Ali 8. VanathiM, Vajpayee RB, Tandon R,
the mechanical stress to the Javadi, Farid Karimian, Mohammad Titiyal JS, Gupta V. Crater-and-chop
zonules. Reza Jafarinasab, Alireza Baradaran- technique for phacoemulsification of
Rafii, Farba Jafari, Mehdi Yaseri. hard cataracts. J Cataract Refract Surg
References Corneal endothelial cell loss during 2001;27:659-661.
phacoemulsification: Bevel-up versus
1. Fishkind W, Bakewell B, Donnenfield bevel-down phaco tip. J Cataract Refract 9. Hong Kyun Kim. Decrease and conquer:
ED, Rose AD, Watkins LA, Olson RJ. Surg 2011; 37:1971-1976. Phacoemulsification technique for hard
Comparative clinical trial of ultrasound nucleus cataracts. J Cataract Refract Surg
phacoemulsification with and without 5. Storr-Paulsen A, No rregaard JC, Fanik 2009;35:1665-1670.
the WhiteStar system. J Cataract Refract G, Tarnhoj. The influence of viscoelastic
Surg 2006; 32:45-49. substances on the corneal endothelial Corresponding Author:
cell population during cataract surgery:
2. WalkowT,AndersN,KlebeS.Endothelial a prospective study of cohesive and Dr. N.Z. Farooqui MS
cell loss after phacoemulsification: dispersive viscoelastic. Acta Ophthamol Department of Ophthalmology,
relation to preoperative and Scan 2007;85:183-187. D.D.U. Hospital, New Delhi, India.
intraoperative parameters. J Cataract
Refract Surg 2000; 26:727-732. 6. Phacodynamics : Mastering the Toos
and Techniques of Phacoemulsification
3. Sahand Amir-Asgari, Nino Himschall, Surgery. Barry S. Siebel . 4th Edition.
Oliver Findl. Using continuous
intraoperative optical coherence 7. Gimbel HV. Principles of nuclear
tomography to classify swirling lens phacoemulsification. In: Steinert RF,
fragments during cataract surgery ed. Cataract Surgery; Techniques,
and to predict their impact on corneal Complications and Management 2nd ed.
endothelial cell damage. J Cataract Phiadelphia. PA, Saunders 2004 154-181.
Refract Surg 2016;42:1029-1036.

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 97

Subspeciality-Cornea

Missed DOS Times Copy
If you have missed your copy of DOS Times
Please Contact: Secretary DOS: Dr. Namrata Sharma

Room No. 479, 4th Floor, Dr. Rajendra Prasad Centre for Ophthalmic Sciences,
All India Institute of Medical Sciences, New Delhi - 110029.

Ph.: 91-11-20863791  E-mail: [email protected],  Website: www.dosonline.org

Dear All,
Kindly submit your research work for publication to [email protected] or
submission.dostimes.org.in for the DOS Times.
You can submit your article in following categories.

Expert Corner Surgical Technique
What’s New Photoessay
Subspecialities DOS Quiz
Tearsheet
Cornea Monthly Meeting Update
Lens/Cataract Beyond Ophthalmology
Oculoplasty Career Opportunities
Glaucoma Appliances
Retina
Refractive Surgery
Community Ophthalmology

Systemic Diseases
PG Corner

Dr. (Prof.) Namrata Sharma
Secretary - Delhi Ophthalmological Society

Room No. 479, 4th Floor,
Dr. Rajendra Prasad Centre for Ophthalmic Sciences,

All India Institute of Medical Sciences,
New Delhi - 110029

98 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times

Tearsheet

Work of a case of Squint

1Ankur Sinha MD, 2Digvijay Singh MD
1. Max Vision Eye Care Centre, Jaipur, India.
2. Noble Eye Care Gururgam, India.

2. Preliminary examination and history taking 1. Rule out psuedo-strabismus - A simple Hirshberg
test may help (for very young children), older children
a) Presenting complains – Asthenopia, Diplopia, Squint can be evaluated in detail
a) Pseudoesotropia
b) Age of onset – early onset, long duration, constant a. Epicanthus /Telecanthus /Euryblepharon
angle (poor prognosis); old photos b) Negative angle kappa
a. Pseudoexotropia
c) Pattern of deviation – intermittent/constant/ b. Hyperteloria /Positive angle kappa
same/alternating c) Pseudohypotropia
a. Ptosis
d) Precipitating factor – fever/ trauma/ diarrhea etc
a) Binocularity and Stereopsis (common methods are
e) Pre-natal, Peri-natal and Developmental history listed below, any/all of them may be used)
a. Worth-four dot test - For distance binocularity
f) Treatment history – Refraction/ glasses/ occlusion/ b. Bagolini’s glasses - For near (least
surgery dissociating)
c. Lang’s Two pencil test - Gross stereopsis
3. Assessment of Sensory system d. TNO/ Titmus fly test - quantify near
stereopsis
c) Visual acuity and Refraction e. Frisby Davis - quantify distance steroacuity

a. Use Age specific visual acuity charts like Teller’s b) Sensory anomalies to look for/ rule out
acuity, Cardiff acuity, HOTV etc. For preverbal if a. Amblyopia / Suppression /Low Stereopsis
unequal resentment of closure of either eye -
poor vision in opposite eye. b. Eccentric Fixation/Anomalous retinal

b. Prescribe full Hypermetropic correction & correspondence
appropriate myopic correction (do not overcorrect
myopia)

d) Fixation -Fixation star of direct ophthalmoscope can be
used to asses fixation while funds examination

a. Central- good prognosis for amblyopia (if any)
treatment

b. Eccentric – poor prognosis for treatment of
Amblyopia and regression of squint

4. Assessment of Motor System
a. Check head posture for Head tilt right or left, Face turn right or left, Chin up or down

b) Ask for old picture to ascertain it’s presence since early childhood
a. Causes - Incomitant squint / A-V pattern/ Nystagmus/ One eyed persons/ Refractive errors

c) Detection of squint

a. Use Accommodative targets (Toys and Mobiles are good target for children)

b. Height of the charts should be at eye level, else error in assessment of deviation can occur in case of pattern
strabismus.

c. Hirschberg corneal reflex Test/ corneal light reflex test - Use a light source from approximately 2 feet and shine it
at midline (say center of forehead), ask patient to focus their gaze on it, Observe the reflection of light off the
cornea, appears as a pin-point white light near the center of the pupil

d. Cover test: To pick up tropias (true squint) - Cover is placed briefly over the eye which appears to fixate, Should
be done for distance and near, Detects manifest strabismus, Prerequisites - ability to fixate the target/ Central
fixation /No gross motility defect

e. Cover-uncover test - For latent strabismus (Heterophoria)/To see for the movement of the eye under cover after
the disruption of fusion

f. Assess and control Accommodation - use age specific cycloplegia (it is preferred to use atropine for children
less than 5 years of age, Homatropine for older children) and Use proper refractive correction

g. Measurement of angle of Deviation using PBCT – ( prism bars/ loose prisms used - apex towards deviation or
Synoptophore

h. Variation for distance and near devotion by more than 10 PD difference may points towards -Convergence
excess / High AC over A ratio/ Convergence insufficiency/ True or Pseudo Divergence excess

i. Variation in fixing right vs fixing left - may point towards incomitance. check of paralysis or old surgery
j. Measurement in up and down gaze ( to note A/V phenomenon), it is advisable to measure the deviation in 25

degrees up and 35 degrees down gaze to bring out pattern strabismus

i. A difference in up and down gaze by 15 PD for V and 10 PD for A pattern is considered significant

k. Note Ocular rotations (both ductions and versions), ocular movements and 9 gazes

www.dosonline.org/dos-times DOS Times - Volume 26, Number 1, July-August 2020 99

Tearsheet

100 DOS Times - Volume 26, Number 1, July-August 2020 www.dosonline.org/dos-times