dos_jul_2017

Perspective

SR and overaction of contralateral SO and overaction of the inferior oblique in OTR any lesion. Patient was followed up for
resultant hypertropia (HT) simulating as opposed to oblique muscle palsies. recovery as large number of skews may
SO palsy. A lesion selectively involving Associated 3rd, 4th and 6th nerve palsies recover with time, however significant
PSC produces hypotropia and extorsion could coexist congealing the clinical recovery did not take place. Surgery in
of ipsilateral eye, may simulate IO palsy. picture. Similarly, asymmetrical visual such cases is congealed as weakening
A lesion involving both ASC and PSC inputs to two eyes in infancy can lead to an inferior oblique for example may add
pathways will result in accentuated breakdown in binocularity and lead to to intorsion and worsen his symptoms,
vertical (Skew), conjugate torsion dissociated vertical deviations (DVD), surgery on the vertical recti is only viable
and head tilt to the same direction primary oblique muscle overaction option.
constituting complete OTR. OTRs can be and pursuit asymmetry in infantile
thus quite variable complete/ incomplete, strabismus. See-saw nystagmus and DVD The Pitch Plane, The Laterally
asymmetrical/ symmetrical, comitant or can be conceptualised as inverse OTRs. Alternating Skew deviations-Manifests as
incomitant, tonic / phasic, physiologic / Other conditions must be distinguished reversing HT in lateral gaze positions. The
pathologic. by attendant clinical clues. abducting eye is usually higher but reverse
may also be true. The forward body pitch
Etiologies The Roll Plane, the Ocular tilt is sub served by ASC and backward
reaction- The lesion could be anywhere body pitch by PSC, the corresponding
Skew deviations share diverse along vertical VOR pathways, Fellow otolithic pathways are segregated in
travellers may include internuclear the brainstem. A selective damage to
etiologies from most benign to most Ophthalmoplegia (paresis), pretectal bilateral ASC pathways will result in the
syndrome, ataxia, vertigo, vertical upbeat dominance of PSC pathways and result
malignant including tuberculomas, / downbeat nystagmus etc. in bilateral abducting HT, A pattern and
intorsion whereas a dominance of ASC
stroke, infarction, tumors, trauma, Illustrative Case Report pathways will catapult into adducting HT,
V pattern and extorsion, head tilt test will
hydrocephalus, Arnold Chiari A 50-year-old man with history be negative in symmetrical injury. There
of atrial myxoma, presented with left may be a primary position HT if damage
malformations, epilepsy and drugs. The ischemic INO, left HT and right head is asymmetrical.
tilt. Objective ocular torsion revealed
list is exhaustive. conjugate intorsion of left eye, extorsion
of right eye, constituting classical
Symptoms OTR (Figure 2). Patient complained of Illustrative Case Report
oscillopsia, vertigo and occasional vertical
Skew deviations rarely cause and horizontal diplopia. Three step test A 20-year-old girl presented with
symptoms by themselves since there is was positive for left superior oblique palsy
tilt in the subjective visual vertical (SVV), suggesting selective damage to otolithic occasional vertigo and vertical horizontal
which patient is not aware. There could pathways corresponding to right ASC.
be symptoms due to fellow travellers. The vertical tropia and conjugate torsion diplopia, she betrayed bilateral abducting
Rarely symptoms like vertical/ horizontal improved significantly in supine position
diplopia/ disequilibrium, oscillopsia, alluding to the diagnosis of an OTR rather HT, PP HT of left eye, downbeat nystagmus,
ataxia may be experienced. a superior oblique palsy. An ischemic
micro- infarct along the left MLF was bilateral intorsion and A pattern. MRI
suspected, however MRI did not reveal
Differential diagnosis brain showed large tuberculoma at

Incomitant OTRs can be confused the level of the pons having bilateral
with superior / inferior oblique palsies,
inferior division 3rd nerve palsy, ocular involvement. She showed good response
myasthenia gravis, thyroid eye disease,
mitochondrial cytopathies, chronic to anti-tubercular treatment. (Figure 3).
progressive external ophthalmoplegia,
the list is long. Vertical deviation and Acute Acquired Comitant
conjugate torsion disappear in supine
position in OTR but not in oblique muscle Esotropia (AACE) accompanying
palsies and upright / supine test should
be performed in all such cases suspected serious neurological disease – Posterior
of harbouring a skew. An incomitant
OTR due to selective damage to otolithic Figure 2: Montage of 9-gaze clinical photographs of a 50 year old man with complete right
pathways corresponding to contralateral OTR with left hypertropia, head tilt to right and conjugate fundus torsion from an ischemic INO
ASC may produce a superior oblique palsy involving left MLF.
(SOP) sporting a positive Bielschowsky
test, meeting the diagnostic criteria
for SOP. Head tilt test is positive as the
vestibule on the opposite side (hypotropic
side) is damaged, leading to worsening
of HT on ipsilateral tilt to hypertropic
side. A similar damage to ipsilateral PSC
pathways can result in inferior oblique
palsy. The hypertropic eye is intorted
due to overaction of the superior oblique
and hypotropic eye is extorted due to

58 DOS Times - July-August 2017 P.K. Pandey. Skew Deviations

Perspective

Figure 3: Montage of 9-gaze clinical photographs of a 20 year old girl with asymmetrical neuroimaging and neurology opinion.
horizontal gaze palsy, A pattern and abducting hypertropia A, bilateral intorsion B, secondary to Wait for resolution. Surgical options
CNS tuberculoma C. different with variable outcomes as
hypertropic eye is intorted and hypotropic
Figure 4: Montage of 9-gaze clinical photographs of a 12 year old boy with acute acquired eye extorted. Muscle sequalae may not
comitant esotropia A, with conjugate torsion (extorsion OD, intorsion OS) B, and multiple CNS be prominent. Better understanding
tuberculomas on neuroimaging C. of skew deviations may enhance our
understanding of acquired strabismus
fossa lesions such as Arnold Chiari acute onset horizontal diplopia, isolated and give us insight as to when to order
malformations and vermal tumors in AACE with V pattern and conjugate neuroimaging and seek neurology
particular may cause AACE as otolithic torsion, there was no papilloedema, opinion. Ophthalmologist may be the first
pathways sub serving horizontal rather MRI brain revealed disseminated CNS one to have a close encounter with a skew
vertical vergence are affected. The tuberculomas. Patient responded to as initial presentation and thus be held
esotropia is greater in distance and motor antitubercular therapy and esotropia responsible for a missed diagnosis and
fusion is grossly compromised, some may as well as conjugate torsion resolved / or botched up treatment. Skew should
also have associated divergence palsy. completely with treatment (Figure 4). be kept in the differential diagnosis of
This horizontal form of skew deviation acquired strabismus or motility disorder.
may have its physiological substrate in Skews may simulate comitant/
translational VOR. Associated fellow incomitant strabismus including SOP/ References
travellers like nystagmus, ataxia, IOP/ AACE. Mostly acquired, skews
papilloedema should be carefully looked share diverse etiology from most benign 1. Brandt T. Dieterich M: Skew deviation
for and neuro imaging ordered, lest a life- to most malignant. Look for signs of with ocular torsion, a vestibular
threatening condition be missed. CNS involvement, include evaluation of brainstem sign of topographic
subjective / objective torsion & head diagnostic value, Ann. Neurol. 1993;33:
Illustrative Case Report position dependent changes as 4th & 5th 528-34.
step in motility assessment. SUMMARY -
A 12-year-old boy presented with Keep low threshold for suspicion, order 2. Donahue SP, Levin PJ, Hamed LM: Tonic
Ocular Tilt reaction simulating superior
oblique palsy, diagnostic confusion
with 3 step test. Arch. Ophthalmol.
1999; 117:347-52.

3. Donahue SP, Levin PJ, Mohney B et al,
Skew Deviation and inferior oblique
palsy, Am J. Ophthalmol.132,751-6,
2001.

4. Keane JR, Ocular skew deviations, an
analysis of 100 cases, Arch. Neurol.
1975; 32:185-90.

5. Keane JR, Alternating skew deviations
in 47 patients, Neurology, 1985;35:725-
8.

6. Brodsky MC, Donahue SP, Vaphiades M,
Brandt T, Skew Deviations Revisited,
Surv. Ophthalmol, 2006;31:105-128.

Correspondence to:
Dr. P.K. Pandey,
Professor Ophthalmology,
Guru Nanak Eye Centre,
Maharaja Ranjit Singh Marg,
New Delhi, India.

P.K. Pandey. Skew Deviations www. dos-times.org 59

Recent Trends and Advances

Pediatric Cataract: Evaluation and
Management

Manisha Mishra, Anju Rastogi, Shweta Dhiman
Guru Nanak Eye Center, New Delhi

Summary: Management of congenital and childhood cataracts remains a challenge. Increased intraoperative difficulties, low
scleral rigidity, high vitreous pressure, propensity for increased postoperative inflammation, changing refractive state of the
eye, very high incidence of visual axis opacification and a tendency to develop amblyopia, all add to the difficulty in achieving a
good visual outcome in the pediatric patient.

Pediatric cataract is an important cause of intraocular pressure have to be measured with a tonopen
vision impairment and blindness because of or Perkins hand held applanation tonometer.
the impact on child’s development, education, c) Dilated fundus examination
future work opportunities as well as quality of d) Ultrasound B Scan if fundus is not visible
life1,2. Management of congenital and childhood e) Ultrasound A Scan
cataracts remains a challenge. Low scleral rigidity, f) Presence of strabismus and nystagmus
increased elasticity of the anterior capsule, and high vitreous 3. LABORATORY INVESTIGATIONS- It is important in
pressure are among the major obstacles that interfere with cases of bilateral cataract
the highly demanding cataract surgery in children. Increased a) Hemoglobin
postoperative inflammation, a changing refractive state, higher b) TORCH
re-surgery rate and an inherent risk of amblyopia makes the c) Plasma calcium/ phosphorus/ phosphatase
management further challenging. d) Urine reducing substance
SYSTEMIC EVALUATION-A detailed systemic evaluation
PRE-OPERATIVE EVALUATION is to be done as congenital cataract can be associated with
galactosemia, Lowe’s syndrome, Alport syndrome, Down’s
1. HISTORY - The first complaint is often leucocoria, syndrome etc.
strabismus, nystagmus, inability to recognize mother or closing
of eyes in bright light. A detailed history is taken that includes TIMING OF CATARACT SURGERY3-7
asking about
• Age of onset and duration of symptoms a) In visually significant opacities –
• Antenatal history including that of any drug intake and • Unilateral cataracts should be operated before 6

fever with rash weeks of age
• Perinatal history of low birth weight, pre-term delivery is • Bilateral cataracts should be operated before 8 weeks

important as bilateral cataracts are more common in these of age
patients • In bilateral symmetric cataracts second eye should be
• Family history of congenital cataract and consanguity
• History of any trauma or previous surgery operated within 1 week of the first eye
2. OCULAR EXAMINATION- b) When there is significant asymmetry, the worse eye is
a) Vision: -
i. Preverbal children- vision can be assessed in the operated first
c) In visually non-significant opacities
following ways • Surgery should be avoided for 1-4 years due to
• Fixation pattern
• Optokinetic nystagmus difficult pre-operative and post-operative assessment
• Preferential looking tests like Tellers acuity test and management.

and Cardiff acuity test PROGNOSTIC FACTORS
ii. Verbal children - vision can be assessed in the
Pediatric cataract causes decreased vision and interferes
following ways with the normal development of the child. Later age of onset,
• Landoff C test early cataract surgery, choice of aphakic correction, good
• HOTV Test parental motivation and amblyopia management are of utmost
• Tumbling E test importance in achieving good outcomes.
• Allen’s test
• Snellen’s letter chart A number of factors may influence the surgical outcome
b) Anterior segment biomicroscopy- done to assess the size, adversely which include presence of a unilateral cataract (as
they are detected at a later age), nystagmus, strabismus, any
location, density of the opacity. Corneal diameters and associated ocular defects like microphthalmos, persistent fetal
vasculature and poor parental compliance.
Mishra M et al. Pediatric Cataract
www. dos-times.org 61

Recent Trends and Advances

IOL POWER CALCUATION8 KERATOMETRY Table 1: Post-operative refractive
goal according to age at surgery24
Calculation of accurate IOL In a young child keratometric values
(Intraocular Lens) power is a major are obtained under general anaesthesia Age In Years Post Operative
hurdle for long term care of patients using a hand held autokeratometer. Refraction Goal
undergoing pediatric cataract surgery. Keratometry steeply reduces in the first
Accuracy of the calculated IOL power 6 months, i.e., −0.40 D/month, −0.14 D/ 1 +6
is affected by the short eyes and the month in the next 6 months, and −0.08 D/
steep keratometric values. Selecting a month in the 2nd year. Corneal curvature 2 +5
fixed-power lens for implantation into reaches the adult range at about 3
an eye that is still growing requires a years of age. Keratometry readings 3 +4
complicated decision-making process. without speculum are preferred though
Implantation of an IOL at the emmetropic technically difficult, as keratometry with 4 +3
power risks a significant myopia at ocular the speculum is known to deform the
maturity. However, if we aim for early globe and give an unreliable reading15,16. 5 +2
hyperopia with the expectation that the
eye will become emmetropic during ACCURACY OF IOL CALCUATION 6 +1
adulthood, then this may predispose the FORMULAE17-22
child to amblyopia. Both approaches have 7 Emmetropic
pros and cons; the best solution probably The Hoffer Q and Holladay 2
lies in finding a compromise. formulae were reported to be more need for axial length measurement and
accurate for shorter adult eyes. The keratometry25.
AXIAL LENGTH MEASUREMNT9 accuracy of different IOL calculation
formulae in children has been previously CURRENT SURGICAL TECHNIQUE
A small difference in axial length studied. Neely et al.21 used SRK II, SRK/T,
measurement may result in clinically Hoffer Q and Holladay 1 to recalculate Pediatric cataract surgery is
significant residual refractive error. the IOL power for 101 patients who had evolving with advances in microsurgical
For example, a 0.1 mm difference in undergone surgery at mean age of 4.8 techniques. Currently there is an
axial length may result in 0.25–0.75 D years and found that the mean prediction increasing trend toward IOL implantation
difference in IOL calculation. In shorter error was 0.3 ± 1.5 D. There was no in children with evidence of better visual
eyes of children, the error could be significant difference in the predictability outcomes in infants managed with IOL
magnified to 14 D/mm. Axial length of the lens power between these four implantation. The aim of the surgical
can be measured using ultrasound or formulae and the newer theoretical technique is to provide a long-term clear
optical biometry. A-scan ultrasound formula did not out-perform the older visual axis by preventing development of
biometry is the conventional method for regression formulae. In this study, there VAO or secondary membrane.
measurement of axial length in children. was greater variability in shorter eyes
Ultrasound can be performed using (less than 19 mm), and in children less Lensectomy and vitrectomy: This
applanation or immersion techniques. than 2 years old. The least variable was was introduced in 1975 and can be done
The applanation technique may introduce the SRK II and the greatest was Hoffer Q. by 2 routes. The pars plana route was
a measurement error in recorded axial Another study found that Hoffer Q was advocated by Peyman in 1978 while
length (shorter axial length obtained) more predictable than the other formulae limbal route was described by Calhoun
by the slight indentation of the corneal especially in young children (<2 years and Harley in 1980. This method leaves
surface and the lack of fixation in a patient old) and in shorter eyes (<22 mm). lack of adequate capsular support for
under anesthesia. With the immersion PCIOL implantation. It can be done
technique, care must be taken to ensure Dahan, et al have suggested under in cases where IOL implantation is
that the ultrasound beam is perpendicular correcting biometry reading by 10% in contraindicated like microcornea and
to the retina by ensuring that the retinal children between 2 to 8 years. For children microphthalmos, uveitis, glaucoma,
spike is displayed as a straight, steeply younger than 2 years, perform biometry aniridia and persistent hyperplastic
rising echo spike. A recent prospective and under-correct by 20% or use the primary vitreous26.
study on 50 cataractous eyes of 50 axial length only. IOL power suggested for
children (mean age, 3.87 ± 3.72 years) by 21 mm is (22.00D), 20 mm (24.00D), 19 Lens aspiration with IOL
Trivedi and Wilson found that the axial mm (26.00D), 18 mm (27.00D) and for 17 implantation: Current surgical steps are
length was shorter in eyes with contact mm axial length 28.00D23. described under the following heads:
ultrasonography compared to immersion
ultrasonography (21.36 ± 3.04 mm Eneydi et al recommended post Wound construction: Tunneled
vs. 21.63 ± 3.09 mm respectively; P < op goal as described in the following clear corneal incisions have replaced
0.001)10-13. (Table-1). limbal incisions. The preferred location
is superior rather than temporal. Brown
Partial coherence interferometry INTRA OPERATIVE APHAKIC et al recommended tight suturing in
for axial length measurement has been REFRACTION pediatric patients as vitrectomy done in
shown to be very accurate but requires pediatric patients collapses the sclera
patient cooperation, and thus, may not be Intra operative aphakic refraction making the already elastic sclera even
a viable option in infants, young children, can be used to calculate the IOL power. less rigid27.
and uncooperative children14. Spherical equivalent from the aphakic
refraction can singularly predict the Increased intravitreal pressure:
emmetropic IOL power without the The ophthalmic visco surgical devices
such as 1.4% sodium hyaluronate allows
the surgeon to neutralize the positive
vitreous pressure. A bolus of thiopentone
and hyperventilation helps reduce the
intravitreal pressure.

Anterior capsular management:
The anterior capsule in children is more

62 DOS Times - July-August 2017 Mishra M et al. Pediatric Cataract

Recent Trends and Advances

elastic and has more tensile strength. A the two groups. But alarmingly adverse is to remove the central anterior vitreous
cohesive OVD is recommended to facilitate events like membrane proliferation into without removing the peripheral or
capsulorhexis and prevent its peripheral the visual axis, corectopia were almost posterior vitreous.
extension. Capsular staining aids in better 10 times more common in infants with
visualization and also makes it less elastic IOL implantation compared to aphakic VAO is the most common
in children. An adequate size and location infants36. complication after pediatric cataract
of the ACCC is of utmost importance in surgery with or without IOL implantation.
long term centration of the IOL28. Monocular cataracts in infants, The aim of the surgical technique is
where only way of visual rehabilitation to provide a long term clear axis by
Gimbel and Neuhann in 1992 is contact lens, is often problematic in preventing development of visual axis
introduced the technique of continuous developing countries because of poor opacification43-46.
curvilinear capsulorhexis which is now hygiene, socioeconomic factors and non-
the gold standard for use in pediatric availability of contact lenses in smaller Traditionally, forceps have been
cataract patients. Ideally, the technique towns. So, these infants will probably do used to perform PCCC. Manual posterior
provides for any size of smooth, circular, better with IOL. The following factors capsulorhexis remains the gold standard
capsular opening with a strong capsular need to be considered while selecting an because it yields a smooth, round edge
rim that resists tearing even when IOL for implantation in pediatric eye. and also resists capsule tearing. It has a
stretched during lens material removal or long learning curve and achieving the
IOL implantation29. Material of the IOL: In terms of the appropriate sized opening still remains a
type of IOL and the safety profile, foldable challenge.
Vitrectorhexis is an alternative to hydrophobic single piece acrylic IOL
manual ACCC. Dr. M. Edward Wilson material is preferred37. Vitrectorhexis is an alternative to
demonstrated the use of a vitrectorhexis manual PCCC. The PCCC and LAV may
to create the opening in the anterior Size of Haptics: One of major be performed before or after in-the-bag
capsule in a case of infantile cataract. problems in choosing an IOL for a child placement of the IOL. If performed before
While a manual capsulorhexis is typically relates to the changing eye size during placement of the IOL, care must be taken
used in pediatric cataract patients more growth. The average size of currently when placing the lens in the capsular
than a year old, in very young infants, a available adult-sized, in-the-bag IOLs is bag and not to extend the posterior
vitrector provides improved control and 12mm. However, for children <2 years the capsulotomy. Performing posterior
a smooth rhexis30,31. “ideal” size of IOL should be 10-11mm. capsulotomy after implantation of IOL
The haptics should also be able to adapt ensures in-the-bag IOL fixation47,48.
Radiofrequency diathermy capsu- to capsular shrinking as well as capsular
lotomy, developed by Kloti and colleagues growth. The desirable size of posterior
in 1984, has been used as an alternative capsulorhexis is 3-3.5 mm.
to CCC for cataract surgery in children32. IOL Design and Material: Square-edge
single piece hydrophobic acrylic IOLs of Posterior capsulotomy and anterior
The Fugo blade has also been optic size 5.75-6.25mm is considered vitrectomy can be done using the anterior
recently introduced as a plasma knife ideal in pediatric age group. If the optic limbal approach or the pars plana
that can be used to perform an anterior is too small dysphotoptic phenomenon approach.
capsulotomy33,34. can be produced because of distribution 1. Pars plana approach: The unique
of light around the optic edge in mesopic
Hydrodissection and lens conditions. anatomy of the pediatric eyes
matter aspiration: Multiquadrant requires modification of the surgical
hydrodissection (at least three quadrants) IOL Filter: Implantation of IOL in technique. A major anatomic
is the preferred method, following which children leaves retina exposed to harmful constraint is imposed by the relative
bimanual lens aspiration is completed to effects of blue light, therefore a blue filter size of the pars plana. In newborn
remove the lens matter35. IOL would be more beneficial. eyes, the pars plana region is
incompletely developed so that the
Intraocular lens implantation: So the ideal IOL for children will be anterior retina lies just behind the
Whether IOL implantation should be hydrophobic acrylic, one piece, biconvex pars plicata. To avoid iatrogenic
done as a primary procedure has been with 5.75-6.25 mm optic diameter with retinal breaks entry incision are
debated by pediatric ophthalmologists closed loop haptic38-42. made through or anterior to the
for several years. The infant aphakia region of pars plicata. The distance
treatment study (IATS) was designed PMMA lenses are not commonly of the sclerotomy from the limbus
to answer this question. This was a used now due the need for a longer depends on the age of the patient.
prospective randomized multi-centric incision and hence greater post-operative 2. Limbal approach: Limbal based
trial comparing infants (<7 months) who astigmatism which can be amblyogenic approach is preferred by anterior
underwent cataract surgery for unilateral in children. However, they are useful in segment surgeons. It avoids the
cataract with either IOL implantation or certain scenarios like in post-traumatic adverse effect of pars plana approach
were left aphakic and were fitted with a cataract, for optic capture of the IOL in on the growth of a young eye and
contact lens. The main outcome variable PCCC and are also used in developing avoids retina related complications49.
was visual acuity at 1 year and 4 ½ years nations due to financial constraints. Other approaches to prevent
of age. The investigators also looked posterior capsular opacification: In
at complications, re-surgery rates and Posterior capsular management and the year 1994, Gimbel H V described
strabismus and stereopsis and compliance anterior vitrectomy: Anterior vitreous that posterior capsulorhexis with optic
to occlusion. IATS found that there was acts as a scaffold for proliferation of the capture may be done for secondary
no difference in visual acuity at either lens epithelial cells thus causing VAO. membrane formation eliminating the
1 year of age or at 4 ½ years between Thus, posterior capsulotomy (PCCC) need for vitrectomy. However, other
and limited anterior vitrectomy (LAV) is
recommended. The goal of a vitrectomy

Mishra M et al. Pediatric Cataract www. dos-times.org 63

Recent Trends and Advances

studies have shown that optic capture 1. Age at cataract surgery: The (33%). It was concluded that multifocal
performed alone (without vitrectomy) closer to birth the implantation is IOL implantation is a viable alternative
does not eliminate the risk for VAO even performed, the more marked the to monofocal pseudophakia in this age
though it was associated with better undercorrection. group. However, we do not recommend
centration of the IOL. In order to perform multifocal IOL implantation in children
optic capture, a well centered PCCC of 2. Status of the fellow eye: It is important due to reduced contrast sensitivity,
adequate size is a pre-requisite, which is to determine the refractive status problems like glare, need for proper
technically demanding50. of the fellow eye to minimize the centration, changing refractive status of
aniseikonia. the eye and inaccuracies in biometry55.
Small gauge vitrectomy in pediatric
cataract surgery: In recent years, there 3. Expected compliance: It is better to POST OPERATIVE VISUAL
has been a continuous refinement in leave less hypermetropic if the child REHABILITATION
the field of vitrectomy devices with and/or family is not expected to be
introduction of small gauge vitrectomy compliant. 1. Spectacles have the advantage
systems. Fujii et al introduced the first Anterior capsular management: of low cost and that they can be
commercially available transconjunctival, conveniently changed.
sutureless 25-gauge vitrectomy system manual CCC or vitrectorhexis?? A well
(Bausch and Lomb, Rochester, NY, USA). performed manual CCC provides a However, they cause problems like
This system is based on microcannulae capsular opening with strong capsular image magnification, spherical
for three-port pars plana vitrectomy. rim which resists radial extension. aberration, field restriction and ring
The cannula is inserted through the However, manual CCC is especially scotoma
conjunctiva and sclera with a sharp difficult to perform in infants (<1 year of
trocar. During insertion of the cannula, age) because the capsule is highly elastic. 2. Contact lens when compared to
the conjunctiva is displaced to misalign Here vitrectorhexis is a good option spectacles minimize spherical
the scleral and conjunctival openings. as owing to the highly elastic capsule, aberrations and provide better
The main purpose of the cannula is to the edge remains regular and resists binocular vision. However, they have
maintain the misalignment of these radial tearing. In the older children, the the disadvantage of higher cost, lens
openings to facilitate self-sealing closure capsulotomy edge of a vitrectorhexis is loss, intolerance to contact lens,
of wounds after cannula removal. The scalloped and never as regular as a CCC. difficulty in insertion in children and
main advantage of beveled incision infectious keratitis.
is prevention of wound leakage. The Posterior capsular management
smaller instrumentation does not require in young children: In children younger AMBLYOPIA MANAGEMENT56
conjunctival peritomy or suturing of than 5 years, PCCC with anterior
sclerotomies. The advantages of small- vitrectomy is mandatory. Posterior CCC This is one of the most critical
gauge PPV include reduction in operative without anterior vitrectomy is done in steps in post-operative management of
time, inflammation, reduced astigmatism, children between 5 to 8 years. In older a young child for better visual outcome.
patient discomfort and recovery time51-54 children, maintaining intact posterior This is achieved by occlusion therapy.
capsules is advised. Subsequent Nd: YAG The amount of patching required is
CONTROVERSIES IN THE capsulotomy should be considered in the dependent on age.
MANAGEMENT OF PEDIATRIC presence of PCO.
CATARACT Beyond 1 year of age the amount
Posterior capsular management:
Primary IOL implantation: Primary manual CCC or vitrectorhexis? Manual Table 2: Schedule for occlusion
IOL implantation is the recommended PCCC has a long learning curve and according to age
practice in children older than 2 years. In achieving the appropriate sized opening
children below 2 years it is controversial. still remains a challenge. Vitrectorhexis Age In Months Patching Required
However, with advancements in after IOL implantation is an easy to learn
microsurgical techniques, more and alternative to manual PCCC in pediatric 0-1 Month No Patching
more surgeons are implanting lenses in cataract surgery. It is more predictable Required
younger infants. and reproducible, with a short learning
curve and lesser surgical time. 1-2 Months 1-2Hours/Day
IOL power calculation formulae:
Hoffer Q and Holladay 2 are more Timing of anterior vitrectomy: LAV 2-4 2-3 Hours/Day
predictable than the other formulae can be done before or after placement
especially in young children (<2 years of the IOL in the bag. Vitrectomy done 4-6 Up To 50% of
old) and in shorter eyes (<22 mm). after IOL implantation ensures in-the-bag Waking Hours
placement of the IOL while doing it before
Post-operative refractive goal: may increase the chances of decentration 6-12 Up To 80% of
Immediate postoperative refractive of the IOL. Waking Hours
error and its correction are important
factors in preventing amblyopia that may Multifocal IOL in pediatric patients: of occlusion of the amblyopic eye can be
seriously affect the visual outcome of an Jacobi et al. studied pediatric patients increased with the general rule-patching
excellent surgery. Most of the authors aged 2-14 years with multifocal IOL of amblyopic eye and normal as a ratio of
prefer to aim for an initial postoperative implantation with more than 1 year of age. Careful supervision and monitoring
undercorrection (hyperopia) that varies follow-up. They found that only 22% is mandatory.
with the following factors. children reported permanent use of an
additional near correction. The remaining COMPLICATIONS OF PEDIATRIC
children were either using distance CATRACT SURGERY
correction only (44%) or no glasses at all
1. Visual axis opacification: It is the
most common complication of
infantile cataract surgery and

64 DOS Times - July-August 2017 Mishra M et al. Pediatric Cataract

Recent Trends and Advances

IOL implantation. Linnola et al, care workers and the public will help in Ophthalmol.2012; 26(1):13-17
hypothesized that without an IOL 11. Trivedi R.H., Wilson M.E. Prediction
implantation the capsular edges early detection and timely treatment of
fuse better, making it difficult for error after pediatric cataract surgery
any proliferated cortex to reach the pediatric cataract in the future. Surgical with intraocular lens implantation:
visual axis whereas in eyes with IOL, Contact versus immersion A-scan
the capsule may not seal well to the management of pediatric cataracts biometry. J Cataract Refract Surg.
IOL. This may allow some new lens 2011;37:501–505.
cortex to reach the central visual is different from adult cataracts. The 12. Trivedi R.H., Wilson M.E. Axial
axis. The first 6 months of post- length measurements by contact and
operative period is a high-risk period reduced scleral rigidity, elastic lens immersion techniques in pediatric
for development of VAO57,58. eyes with cataract. Ophthalmology.
2. Post-operative inflammation: it capsules and positive vitreous pressure 2011;118:498–502.
is a common sequela in children. 13. Ben-Zion I., Neely D.E., Plager D.A.,
Uveitis may cause a fibrous make surgical manipulations more Ofner S., Sprunger D.T., Roberts
membrane formation, pigment difficult. The high rates of posterior G.J. Accuracy of IOL calculations in
deposition and posterior synechiae. capsular opacifications make PCCC and children: a comparison of immersion
Primary IOL implantation is risk anterior vitrectomy mandatory in the versus contact A-scan biometery. J
factor for increased post-operative AAPOS. 2008;12:440–444.
inflammation. younger age group. Ocular growth makes 14. Lenhart P.D., Hutchinson A.K., Lynn
3. Posterior synechiae: The incidence selection of IOL power a difficult choice. M.J., Lambert S.R. Partial coherence
was higher when implanted early in However, outcomes have improved interferometry versus immersion
life. ultrasonography for axial length
4. IOL Decentration: Capsular bag greatly in the last few decades and with measurement in children. J Cataract
placement of IOL is mandatory Refract Surg. 2010;36:2100–2104.
to reduce this complication. better microsurgical instrumentation, 15. Trivedi RH, Wilson ME, Peterseim MM,
Asymmetrical fixation bag-sulcus is Lal G Axial length and keratometry in
to be avoided in order to minimize techniques and improved understanding eyes with pediatric cataract. Annual
IOL decentration. American Society of Cataract and
5. Glaucoma: The incidence of of pediatric eye growth. Refractive Surgeons Symposium on
glaucoma following pediatric Cataract, IOL, and Refractive Surgery
cataract surgery varies from 3% to REFERENCES April 12-16, 2003 San Francisco, CA
32%. Glaucoma occurring soon after 16. Noonan CPMackenzie JChandna A
surgery is usually due to pupillary 1. Foster A, Gilbert C, Rahi J. Epidemiology Repeatability of the hand-held Nidek
block or peripheral anterior of cataract in childhood: a global auto-keratometer in children. J AAPOS
synechiae formation while open- perspective. J Cataract Refract Surg. 1998;2 (3) 186- 187
angle glaucoma may occur late. 1997;23 Suppl 1:601–4. 17. Zetterström C: Intraocular lens
The most common type of glaucoma implantation in the pediatric eye. J
noted after pediatric cataract surgery is 2. Rahi JS, Gilbert CE, Foster A, Minassian Cataract Refract surg 1997; 23: 559-
open angle glaucoma which is diagnosed D. Measuring the burden of childhood 600.
years after the surgery. Lifelong blindness. Br J Ophthalmol. 1999 18. Gavin E.A., Hammond C.J. Intraocular
monitoring of intra ocular pressure is Apr;83(4):387–8. lens power calculation in short eyes.
recommended for early detection of Eye (Lond) 2008;22:935–938.
glaucoma59-61. 3. Hubel DH, Wiesel TN. The period of 19. Andreo L.K., Wilson M.E., Saunders
6. Cystoid macular edema: It is a rare susceptibility to the physiological R.A. Predictive value of regression and
complication as children have a effects of unilateral eye closure in theoretical IOL formulas in pediatric
healthy retinal vasculature than kittens. J Physiol. 1970; 206(2):419- intraocular lens implantation. J
adults. 436. Pediatr Ophthalmol Strabismus.
7. Retinal detachment: The incidence 1997;34(4):240–243.
of retinal detachment following 4. Birch EE, Stager DR. The critical 20. Neely D.E., Plager D.A., Borger S.M.,
cataract surgery has been reported period for surgical treatment of dense Golub R.L. Accuracy of intraocular lens
between 1 to 1.5%. Retinal congenital unilateral cataract. Invest calculations in infants and children
detachments are usually a late Ophthalmol Vis Sci. 1996; 37(8):1532- undergoing cataract surgery. J AAPOS.
complication of pediatric cataract 1538. 2005;9:160–165.
surgery. The significant risk 21. Nihalani B.R., VanderVeen D.K.
factors for an occurrence of retinal 5. Lambert SR, Lynn MJ, Reeves R, Comparison of intraocular lens power
detachment are high myopia and Plager DA, Buckley EG, Wilson ME. Is calculation formulae in pediatric eyes.
repeated surgeries62. there a latent period for the surgical Ophthalmology. 2010;117:1493–1499.
treatment of children with dense 22. Tromans C., Haigh P.M., Biswas S.,
Conclusions bilateral congenital cataracts? J AAPOS. Lloyd I.C. Accuracy of intraocular lens
2006;10(1):30-36. power calculation in pediatric cataract
Vision screening programs and surgery. Br J Ophthalmol. 2001.
improved education of primary health 6. Birch EE, Cheng C, Stager DR Jr, 23. Dahan E, Drusedau MU: Choice of
Weakley DR Jr, Stager DR Sr. The critical lens and dioptric power in pediatric
period for surgical treatment of dense pseudophakia. J Cataract Refract Surg
congenital bilateral cataracts. J AAPOS. 1997; 23 (Suppl): 618-23.
2008; 13:67-71. 24. Enyedi LB, Peterseim MW, Freedman
SF, et al: Refractive changes after
7. Dave H, Phoenix V, Becker ER, Lambert pediatric intraocular lens implantation.
SR. Simultaneous vs sequential bilateral Am J Ophthalmol 1998; 126: 772-81.
cataract surgery for infants with 25. Hug T.Use of the aphakic refraction
congenital cataracts: Visual outcomes, in intraocular lens (IOL) power
adverse events, and economic costs. calculations for secondary IOLs in
Arch Ophthalmol. 2010; 128(8):1050- pediatric patients.J Pediatr Ophthalmol
1054. Strabismus. 2004; 209-11.
26. Peyman GA, Raichand M, Oesterele C,
8. Wilson ME, Apple DJ, Bluestein EC,
Wang XH: Intraocular lenses for
pediatric implantation: biomaterials,
designs, sizing. J Cataract Refract Surg
1994;20:584-591

9. Sinskey RM, Stoppel JO, Amin PA:
Ocular axial length changes in a
pediatric patient with aphakia and
pseudophakia. J Cataract Refract Surg
1993;19:787-788

10. Wilson ME, Trivedi RH. Axial
length measurement techniques in
pediatric eyes with cataract. Saudi J

Mishra M et al. Pediatric Cataract www. dos-times.org 65

Recent Trends and Advances

Goldberg MF.Pars Plicata Lensectomy pediatric cataract surgery. J Cataract A new 25-gauge instrument system
and Vitrectomy in the Management of Refract Surg. 2003;29:1579–1584. for transconjunctival sutureless
Congenital Cataracts. Ophthalmology. 39. Vasavada AR, Trivedi RH, Nath VC. vitrectomy surgery. Ophthalmology.
1981;88(5); 437-9. Visual axis opacification after Arcysof 2002 Oct;109(10):1807–1812;
27. Basti S, Krishnamachary M, Gupta intraocular lens implantation in 52. Xie L, Huang Y. Pars plana capsulectomy
S. Results of sutureless wound children. J Cataract Refract Surg. and vitrectomy for posterior capsular
construction in children undergoing 2004;30:1073–1081. opacification in pseudophakic children.
cataract extraction. J Pediatr 40. Küchle M, Lausen B, Gusek-Schneider J Pediatr Ophthalmol Strabismus. 2008
Ophthalmol Strabismus. 1996 GC. Results and complications of Dec;45(6):362–5.
Feb;33(1):52–4. hydrophobic acrylic vs PMMA posterior 53. Chee KYH, Lam GC. Management
28. Wilson ME. Anterior lens capsule chamber lenses in children under 17 of congenital cataract in children
management in pediatric cataract years of age. Graefes Arch Clin Exp younger than 1 year using a 25-gauge
surgery. Trans Am Ophthalmol Soc. Ophthalmol. 2003;241:637–641. vitrectomy system. J Cataract Refract
2004;102:391–422. 41. Wilson ME, Elliot L, Johnson B, Surg. 2009 Apr;35(4):720–4.
29. Gimbel HV, Neuhann T. Development, Peterseim MM, Rah S, Werner L, et 54. Kim MJ, Park KH, Hwang JM, Yu HG, Yu
advantages, and methods of the al. AcrySof acrylic intraocular lens YS, Chung H. The safety and efficacy of
continuous circular capsulorhexis implantation in children: clinical transconjunctival sutureless 23-gauge
technique. J Cataract Refract Surg. indications of biocompatibility. J vitrectomy. Korean J Ophthalmol. 2007
1990;16:31–37 AAPOS. 2001;5:377–380. Dec;21(4):201–7.
30. Wilson ME, Bluestein EC, Wang XH, et 42. Vasavada A, Chauhan H. Intraocular 55. Jacobi PC, Dietlein TS, Konen
al. Comparison of mechanized anterior lens implantation in infants with W. Multifocal intraocular lens
capsulectomy and manual continuous congenital cataracts. J Cataract Refract implantation in pediatric cataract
capsulorhexis in pediatric eyes. J Surg. 1994;20:592–598 surgery. Ophthalmology 2001; 108:
Cataract Refract Surg. 1994;20:602– 43. Koening SB, Ruttum MS. Management 1375-80.
606. of the posterior capsule during 56. Greenwald MJ, Parks MM. Treatment
31. Wilson ME, Saunders RA, Roberts EL, et pediatric intraocular lens implantation. of amblyopia. In: Duane TD, Jaeger
al. Mechanized anterior capsulectomy Am J Ophthalmol. 1993; 116: 656-7. 75. EA. Clinical Ophthalmology, Annual
as an alternative to manual 44. Buckley EG, Klombers LA, Seaber Revision. Philadelphia, JB Lippincott,
capsulorhexis in children undergoing JH, Scalise-Gordy A, Minzter R. 1986, vol 1, ch 11, pp 1-9.
intraocular lens implantation. J Pediatr Management of the posterior capsule 57. Khaja WA, Verma M, Shoss BL, Yen KG.
Ophthalmol Strabismus. 1996;33:237– during pediatric intraocular lens Visual axis opacification in children.
240. implantation. Am J Ophthalmol. 1993; Ophthalmology. 2011; 118: 224-5.
32. Kloti R. Bipolar wet-field diathermy 115: 722-8. 76. 58. Atkinson CS, Hiles DA: Treatment
in microsurgery. Klin Monatsbl 45. Vasavada AR, Praveen MR, Tassignon of secondary posterior capsular
Augenheilkd. 1984;184:442–444. MJ, Shah SK, et al. Posterior capsule membranes with the Nd: YAG laser in a
33. Morgan JF, Ellingham RB, Young RD, management in congenital cataract pediatric population. Am J Ophthalmol
et al. The mechanical properties of surgery. J Cataract Refract Surg. 2011; 1994; 118: 496-501.
the human lens capsule following 37: 173-93. 77. 59. Asrani SG, Wilensky JT: Glaucoma
capsulorhexis or radiofrequency 46. Ram J, Brar GS, Kaushik S, Gupta A, after congenital cataract surgery.
diathermy capsulotomy. Arch Gupta A. Role of posterior capsulotomy Ophthalmology 1995; 102: 863-7.
Ophthalmol. 1996;114:1110–1115. with vitrectomy and intraocular 60. Chrousos GA, Parks MM, O’Neill JF:
34. Singh D. Use of the Fugo blade in lens design and material in reducing Incidence of chronic glaucoma, retinal
complicated cases. J Cataract Refract posterior capsule opacification after detachment and secondary membrane
Surg. 2002;28:573–574. pediatric cataract surgery. J Cataract surgery in pediatric aphakic patients.
35. Vasavada AR1, Trivedi RH, Apple DJ, Refract Surg. 2003; 29: 1579-84. Ophthalmology 1984; 91: 1238-41.
Ram J, Werner L.Randomized, clinical 47. Lav Kochgaway, Partha Biswas, Ajoy 61. Mills MD, Robb RM: Glaucoma
trial of multiquadrant hydrodissection Paul, Sourav Sinha, Rupak Biswas, following childhood cataract surgery:
in pediatric cataract surgery. Am J Puspen Maity, andSumita Banerjee. J Pediatr Ophthalmol Strabismus 1994;
Ophthalmol. 2003 Jan;135(1):84-8. Vitrectorhexis versus forceps posterior 31: 355-60.
36. Infant Aphakia Treatment Study Group, capsulorhexis in pediatric cataract 62. Kanski JJ, Elkington AR, Daniel R:
Lambert SR, Buckley EG, Drews-Botsch surgery. Indian J Ophthalmol. 2013 Jul; Retinal detachment after congenital
C, DuBois L, Hartmann EE, et al. A 61(7): 361–364. cataract surgery. Br J Ophthalmol 1974;
randomized clinical trial comparing 48. Hazirolan DO, Altiparmak UE, Aslan 58: 92-5. 107.
contact lens with intraocular lens BS, Duman S. Vitrectorhexis versus
correction of monocular aphakia forceps capsulorhexis for anterior and Correspondence to:
during infancy: grating acuity and posterior capsulotomy in congenital Dr. Manisha Mishra,
adverse events at age 1 year. Arch cataract surgery. J Pediatr Ophthalmol Guru Nanak Eye Center,
Ophthalmol. 2010 Jul;128(7):810–8 Strabismus. 2009;46:104–7. New Delhi
37. Wilson ME Jr, Trvedi RH, Buckley EG, 49. Pearson RV, Aylward GW, Marsh RJ.
Granet DB, Lambert SR, Plager DA, et Ocutome lensectomy: results and
al. ASCRS white paper. Hydrophobic complications. Br J Ophthalmol. 1991
acrylic intraocular lenses in children. J Aug;75(8):482–6.
Cataract Refract Surg. 2007;33:1966– 50. Gimbel HV. Posterior capsulorhexis
1973 with optic capture in pediatric
38. Ram J, Brar GS, Kaushik S, Gupta A, cataract and intraocular lens surgery.
Gupta A. Role of posterior capsulotomy Ophthalmology.1996; 103: 1871-5.
with vitrectomy and intraocular 51. Fujii GY, De Juan E, Humayun MS,
lens design and material in reducing Pieramici DJ, Chang TS, Awh C, et al.
posterior capsule opacification after

66 DOS Times - July-August 2017 Mishra M et al. Pediatric Cataract

Recent Trends and Advances

Intraocular Lens Power Calculation for
Pediatric Cataract

Shubhangi Bhave MS, Rohini Khurana Juneja MS
Drishti Eye Clinic & Squint Centre, Nagpur, India.

Abstract: Intraocular Lens (IOL) power calculation of a small eye in a growing child is quiet challenging. To implant an IOL
of fixed power into an eye that is still growing is difficult. An optimum IOL power needs to be considered, which best benefits
the child’s eye at that time as well as in future as the child grows. The younger the child at the time of surgery, more difficult
becomes the task. During initial days of IOL implantation for paediatric cataract surgery, adult IOL powers were used, leading
to hyperopia, which in turn caused or aggravated amblyopia.

Intraocular Lens (IOL) power calculation of a small eye in by 8 weeks of age, a decrease in lens power is believed to
a growing child is quiet challenging. To implant an IOL of compensate for the increase in axial length of the infant eye
fixed power into an eye that is still growing is difficult. during the first year of life3.
An optimum IOL power needs to be considered, which
best benefits the child’s eye at that time as well as in IOL Power Calculation
future as the child grows. The younger the child at the
time of surgery, more difficult becomes the task. During initial Biometry: A-scan ultrasound and keratometry
days of IOL implantation for paediatric cataract surgery, adult measurements can be very difficult or unattainable especially
IOL powers were used, leading to hyperopia, which in turn in very young children in the OPD. Examinations under
caused or aggravated amblyopia. anaesthesia should be preferably done.

Anatomic Considerations Axial length measurement can be done either by immersion
or contact method. We prefer the immersion method as the tip
The Human eye undergoes rapid growth during first year does not indent the cornea so there are less chances of error
of life. in the readings. It also depends on the ultrasound velocity
• During the first 6 weeks of life the cornea flattens from a settings. Axial length of 25mm is best measured with 1550m/
sec and 20mm with 1560m/sec. Accurate measurement may
mean of 51D to 44D. Keratometry becomes stable by 12 to be obtained by setting an average velocity of 1532m/sec
18 months1 (Figure 1). and then correcting for axial length by adding Corrected AL
• The axial length increases from a mean of 17mm at birth Factor (CALF) of 0.32 to it. Repeated measurements are taken
to 20mm by 1 year of age, 22mm at 3 years and a presumed until three equal readings are obtained. Errors in axial length
endpoint of 23mm at 13 years2 (Figure 2). measurement are the most significant errors in IOL power
• The lens power decreases from 34D at birth to 28D by 6 calculations accounting to 2.5D/mm. However, this error jumps
months of age. It drops by 10D in first year and then by to 3.75D/mm in very short eyes (20mm)
3-4D from 2-10 years.
Since the corneal curvature assumes its adult dimensions

Figure 1: Change in keratometry with age Figure 2: Change in axial length with age

Bhave et al. IOL Power Calculation for Pediatric Cataract www. dos-times.org 67

Recent Trends and Advances

Keratometry readings should be undercorrection is to be done at what left more hyperopic in the beginning to
taken with hand held auto keratometer age to reduce the myopic shift in future is reduce the later myopic shift7.
under anaesthesia, whenever possible. calculated as shown in (Table 1).
Eyelid speculum should not be used as Site of IOL implantation: The power
it alters the curvature. The K-readings in On the basis of percentage - Dahan of the IOL which was initially intended
the newborn are ignored and replaced by suggested that final aim of the refraction for capsular bag placement should be
the average adult reading of 44D. should be an under correction of 20% in reduced by 1D to 2D if it needs to be
infants and 10% in toddlers6 or on the placed in the sulcus.
IOL power calculation formula is an basis of axial length alone (Table 2).
unresolved issue in paediatric cataracts. IOL power: Higher the IOL power
The Holladay formula is considered Status of fellow eye: More hyperopia more is the under correction needed. For
most accurate for eyes with axial length can be left when surgery is done bilaterally example, at the age of 6 months, if one
between 22 and 26 mm. The Hoffer Q child has an emmetropic power of 50D
formula is considered most accurate Table 1: IOL Power on the and another child has emmetropic power
for short eyes (<24.5mm). The SRK/T basis of age of 40D, the first child will need a higher
formula is considered optimal for long residual refraction.
eyes (>26mm)4,5. Age Residual Refraction
Key points
Important points to be kept in <1year +10D to +7mm • Target hypermetropia with treatable
mind include the velocity that needs
to be used for specific eyes (phakic/ 1-2years +6D amblyopia to avoid myopic shift in
aphakic/ pseudophakic), the A-constant children
for the specific IOL to be used, and the 2-4 years +5D • Careful measurements to avoid
characteristics of a good A-scan tracing errors in keratometry and axial
with a spike from each layer of eye. 4-5 years +4D length calculations.

Target Postoperative 5-6 years +3D References
Refraction
6-7 years +2D 1. Gordon RA, Donzis PB: Refractive
Ideally one should aim for a development of thehuman eye. Arch
refractive state that would cause minimal 7-8 years +1.5D Ophthalmol. 1985;103:785-9.
amblyopia in childhood (due to high
hypermetropia), while inducing least 8-10 years +1D 2. Manzitti E, Gamio S, Damel A, Benozzi
refractive error (myopia) in adulthood. J: Eye length incongenital cataracts, in
There is no common opinion regarding the 10-14 years +0.5D Cotlier E (ed): Congenital Cataracts.
ideal postoperative refraction in infants Austin, TX, R.G. Landes Company, 1994,
and children after IOL implantation. >14 years +0D pp. 251-9.
• Initial Emmetropia after the surgery
Table 2: IOL power on the basis of 3. Taylor David Pediatric Ophthalmology
will remove the need of spectacle axial length Third edition 2005, chapter 2, page 36.
correction and chances of amblyopia.
However, the patient will eventually Axial length IOL power 4. Sanders DR, Retzlaff JA Comparison
develop high myopia in adulthood. of the SRK/T formula and other
• Initial high hypermetropia would 17mm 28D theoretical and regression formulas. J
compensate for the myopic shift Cataract Refract Surg 1990; 16:341.
in the adulthood but will leave the 18mm 27D
child amblyopic in the early phase 5. Neely DE, Plager DA, Borger SM
with immediate spectacle correction 19mm 26D Accuracy of intraocular lens
along with the amblyopia therapy. calculations in infants and children
• Slight initial under correction will 20mm 24D undergoing cataract surgery. J AAPOS.
make the child slightly hyperopic 2005; 9:160-5.
during childhood with easily 21mm 22D
treatable myopia during adulthood 6. Dahen E, Drusedau MU. Choices of
(this is what most surgeons try to as the chances of amblyopia would be lens and dioptric power in pediatric
achieve). lesser even in cases of poor compliance pseudophakia. J Cataract Refract Surg.
with glasses. In case of contralateral 1997; 23.
Factors Affecting Target pseudophakia, the refractive status of the
Postoperative Refraction other eye has to be kept in mind. 7. Mutti DO, Zadnik K. The utility of
three predictors of childhood myopia:
Age at the time of surgery Visual Acuity: In dense amblyopia it a Bayesian analysis. Vision Res.
is better to leave less hyperopia or even 1995;35:1345–52.
Lesser is the age of the child at emmetropia to help vision recovery by
the time of cataract surgery, more will occlusion therapy. Correspondence to:
be the under correction needed. What Dr. Shubhangi Sudhir Bhave,
Expected compliance: It is better Consultant Ophthalmologist & Paediatric
to leave less refractive error if the child Ophthalmologist
and/or family are expected to comply Drishti Eye Clinic & Squint Centre,
poorly with glasses, contact lenses and Nagpur, Maharashtra, India.
amblyopia therapy.

Parent’s refractive error: It has
been noted that 30 to 40% of children
become myopic if both the parents have
myopia, while the percentage reduces to
20-25% in others. Such children can be

68 DOS Times - July-August 2017 Bhave et al. IOL Power Calculation for Pediatric Cataract

Recent Trends and Advances

Pediatric Cataract in Difficult Situations

Sudarshan Khokhar MD, Ganesh Pillay MD, Mrittika Sen MBBS
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India

Abstract: Pediatric cataract can sometimes put us in a spot of bother, managing these difficult situations wisely with proper
technique can lay the premonitions to rest. The early identification with few tell-tale signs can help us in confidently tackling
the condition during surgery.

Difficult situations Management: The anterior capsulorhexis can be difficult
Pre-existing Posterior capsular defect due to the partially absorbed nature of the lens. Preferably,
anterior continuous curvilinear capsulorhexis should be
Posterior capsular defect can not only be seen in traumatic attempted under high molecular weight viscocohesive
cataracts but can also be a pre-existing defect in association substance either using Utrata forceps or 23G intravitreal
with posterior polar cataract, persistent fetal vasculature, forceps. This is the most important step as the likelihood of
lenticonus or lentiglobus. The incidence varies from 2.2- placing lens in sulcus is high in such cases. Hydro dissection and
6.75%1,2 and has higher unilateral association3. The defect delineation should be avoided. The bottle height/ intraocular
may be due to the developmentally weak posterior capsule pressure is kept low and preferably, vitrectomy cutter is kept in
or traction of regressing hyaloid artery. Once the defect is irrigation-aspiration cut (I-A cut) mode and the lens matter is
there, the fluid vitreous starts hydrating the lens and with aspirated keeping vacuum at 400 mmHg, aspiration flow rate at
accommodation, there is egress of the lens material into the 50 cc/min and cut rate at 100 cpm (cuts per minute). Anterior
Berger’s space. Posterior capsular defect may present to us with vitrectomy is invariably required to clean the anterior vitreous.
differential opacification i.e. whiter at center than at periphery The placement of Intra-ocular lens in the bag can be difficult as
and parent often noticing a white dot initially, which has there is high risk of extension of the posterior capsular defect
increased in size gradually. The other tell-tale sign we should but can be achieved in long standing cases where fibrosis of the
look for, is membranous looking cataract with flattening of margin of defect is present. The three-piece lens can be placed
anterior capsule (deep anterior chamber), white dots4 and fish in sulcus and posterior optic capture may be attempted in an
tail sign (due to lens matter in Berger’s space)5. The diagnosis adequately sized anterior capsulorhexis.
can almost be established with ultrasound biomicroscopy using
35MHz probe though sometimes it might be difficult in early Juvenile idiopathic Arthritis associated Uveitis (JIA)
stages where it just looks like a posterior subcapsular cataract
(Figure 1A-D). Pediatric cataract is most common in Juvenile idiopathic
arthritis associated uveitis (71%)6. The cataract formation
Figure 1A): Preoperative clinical picture not able to identify pre- is mainly due to severe intra-ocular inflammation and use of
existing posterior capsular defect. 1B): Ultrasound biomicroscopy topical steroid. These patients generally present with posterior
picture showing pre-existing posterior capsular defect. 1C): Posterior subcapsular cataract associated with posterior synechiae, iris
capsulorhexis. 1D): Intraocular lens placed in bag. bombe and even peripheral anterior synechiae7. Long standing
cases presenting with band shaped keratopathy and hypotony,
generally have a poor visual prognosis8 (Figure 2A-D).

Management: The preoperative evaluation should ensure
there is no anterior chamber activity i.e. child is in remission
at least for a period of 3 months9. Hence slit lamp examination
to look for cells (<1+) and flare along with fundus examination
is of utmost importance. The pupils are often non-dilating and
ultrasound is required for posterior segment evaluation. The
ultrasound biomicroscopy (UBM) helps us to see the extent of
the pupillary membrane, which sometimes can be mistaken
for cataract. The child should be started on topical steroid 1%
prednisolone acetate 1 week prior and oral steroids 1mg/kg at
least 3 days prior to surgery10. The role of immunosuppressive
agents has been documented to be of benefit in these patients
and lower dose methotrexate can be started in consultation
with the rheumatologist11. Intra-operatively, removal of
pupillary membrane should be done with intra-vitreal forceps
before staining with dye. The pupil can be released of posterior
synechiae using cannula of OVD (Ophthalmic Viscosurgical
device) and simultaneously injecting the viscodispersive agent.

Khokhar et al. Pediatric Cataract Surgery in Difficult Situations www. dos-times.org 69

Recent Trends and Advances

Figure 2A): Band shaped keratopathy with total cataract in chronic Figure 3A): Spherophakia with iris defect. 3B): Anteriorly dislocated
uveitis. 2B): Poor glow in case of chronic uveitis. 2C): Ultrasound Spherophakic lens 3C) Intralenticular aspiration. 3D): Aphakia in a
biomicroscopy showing anterior and posterior subcapsular cataract. case of Spherophakia. 3E): Anterior chamber intraocular lens (ACIOL)
2D): Hydrophobic acrylic lens implanted in the bag. with peripheral iridectomy (PI) in a case of Spherophakia.

If the pupillary dilation is insufficient Spherophakia Persistent fetal vasculature
iris hooks or Malyugin ring can be used.
Preferably iris manipulation should be Spherophakia was first described by Persistent fetal vasculature (PFV)
kept to minimum to avoid post-operative Hartridge in 1886, it is due the defective is the term coined by ‘Goldberg’. The
reaction and break in blood aqueous lens is supplied by hyaloid artery in
barrier. The anterior capsule may development of the lens zonules which cloquet’s canal and when this persists
become fibrous requiring use of intra- beyond 7 month of gestation it results
vitreal scissor to make an opening and results in increased length, weakness, in persistent fetal vasculature23. This
completion of the rhexis. The anterior generally presents as eccentric posterior
capsulorhexis should be on the larger absence and non-attachment of posterior fibrovascular plaque hence initially was
side just covering the IOL margin to zonules to the ciliary processes17. Hence, called as “Persistent Hyperplastic Primary
avoid iris forming posterior synechiae in zonules do not exert sufficient pressure Vitreous (PHPV)”. This sometimes may be
optical zone. The posterior capsulorhexis to flatten the developing lens resulting in associated with tunica vasculosa lentis,
and anterior vitrectomy is preferred even fetal spherical conformation. The arrest of iridohyaloid blood vessels or persistent
in children older than 8 years as there pupillary membrane. The presence of
is high risk of visual axis opacification lens development usually occurs between microphthalmos and elongated ciliary
(55-100%)12,13. The decision to put the fifth and sixth month of intrauterine body is pathognomonic of persistent
intraocular lens is debatable but should life18. It usually presents in first or second fetal vasculature although it can rarely
be tried as these patients are often decade of life with progressive myopia, present in normal and buphthalmic
intolerable to contact lens because of the eyes24. The patients with anterior PFV
formation of band shaped keratopathy. angle closure glaucoma due to pupillary generally have good visual prognosis,
The results have improved with modern in contrast to patients with posterior
minimally invasive techniques and block, anteriorly or posteriorly dislocated PFV in whom visual potential is often
foldable intraocular lens. The heparin lens and subluxation19. Spherophakia limited by coexisting retinal and optic
coated hydrophobic acrylic foldable nerve abnormalities. If the persistent
IOL is preferred. The need to remove all is usually associated with systemic fetal vasculature is not obscuring the
the viscoelastic substance needs to be visual axis during the first year of life, the
reemphasized as these patients are also disorders such as Weill-Marchesani’s’ prognosis for patient’s vision is good, if
prone to glaucoma (23-100%)14 in post- surgery and treatment for amblyopia of
operative period. Intracameral injection syndrome, Marfan’s syndrome, homocys- the affected eye is done at the earliest
of 0.1cc (4mg) triamcinolone acetonide (Figure 4A-D).
reduces the post-operative fibrinous tinemia, Alport’s syndrome and
reaction. The tapering of steroid should Klinefelter’s syndrome20 (Figure 3A-E). Management: Ultrasound shows
be slow and should be done over a period a stalk connecting the optic nerve head
of 8 to 12 weeks along with short acting Management: The acute angle to the posterior capsule of lens but
mydriatic and cycloplegic (tropicamide closure glaucoma secondary to pupillary this finding can sometimes be missed.
1% with 2.5% phenylephrine TDS) Colour Doppler imaging is an informative
to avoid synechiae formation15 Long block is worsened by miotics and relieved screening and diagnostic tool that shows
term follow up is generally required by cycloplegics also known as ‘Inverse characteristic flow patterns in PFV. There
to look for cystoid macular edema and glaucoma’. On ultrasound biomicroscopy may be risk for the development of
need for secondary procedures like vitreous haemorrhage during and even
membranectomy and YAG capsulotomy (UBM), shallow anterior chamber, steep after surgical repair. UBM preoperatively
(56-100%)16. might help us identify elongated ciliary
anterior lens curvature, iridolenticular processes and posterior plaque or
defects. Intra-operatively, presence of
contact, elongated zonules, increased

distance between the lens equator and
the ciliary processes can be seen21.

Intralenticular bimanual irrigation and

aspiration using low bottle height and

vitrectomy cutter kept in irrigation-

aspiration cut (I-A cut) mode, the lens

matter is aspirated keeping vacuum
at 400 mmHg, aspiration flow rate at
50 cc/min and cut rate at 100 cpm
(cuts per minute)22. Anterior chamber
Intraocular lens (ACIOL) or Scleral fixated
intraocular lens (SFIOL) with or without

trabeculectomy can be done.

70 DOS Times - July-August 2017 Khokhar et al. Pediatric Cataract Surgery in Difficult Situations

Recent Trends and Advances

Figure 4A): Salmon Patch sign. 4B): Persistent fetal Figure 5A): Ectopia lentis et Pupillae. 5B): Subluxation opposite to iris atrophy. 5C):
vasculature with choroidal coloboma. 4C): Diathermy Intra-lenticular lens aspiration. 5D): 5.2 mm entry for anterior chamber intraocular
and cutting of anterior stalk. 4D): Intraocular Lens (IOL) lens (ACIOL). 5E): ACIOL placed under air. 5F): Pupilloplasty done with suture.
placed in the bag.

‘salmon patch sign’ i.e., eccentric pink hue of a poorly dilating pupil (Figure 5A). evaluation and intra-operative decisions
is suggestive of active vasculature within in difficult cases can make the surgical
the PFV25. The posterior capsule is fibrotic Other ocular features that may outcomes excellent.
and tenacious. Fugo plasma blade can be
used to avoid intra-operative bleeding by be associated include megalocornea, References
using pulses of plasma that are generated
around the tip to cut and cauterize persistent pupillary membrane, polycoria, 1. Vasavada AR, Praveen MR, Nath V,
tissue without extensive collateral tissue et al. Diagnosis and management of
damage26. The diathermy can also be used axial myopia, cataract, glaucoma, retinal congenital cataract with preexisting
to cauterize the bleeders before cutting posterior capsule defect. J Cataract
with intra-vitreal scissors or vitrectomy detachment, retinitis pigmentosa, Refract Surg 2004;30:403–408.
cutter. Generally, more extensive anterior
vitrectomy is done to avoid visual retrobulbar cyst, coloboma of iris, lens 2. Wilson ME, Trivedi RH. Intraocular
axis opacification. Intra-ocular lens lens implantation in pediatric eyes
implantation though difficult should be and choroid, anterior uveitis, phacolytic with posterior lentiglobus. Trans Am
tried in unilateral cataract to decrease the Ophthalmol Soc 2006;104:176–182.
chance of developing amblyopia. glaucoma and displacement of lens in the
3. Crouch ER Jr, Parks MM. Management
Ectopia lentis et pupillae anterior or posterior chamber30. of posterior lenticonus complicated by
unilateral cataract. Am J Ophthalmol
Hereditary ectopia lentis can be The pathomechanism of this 1978;85:503–508.
classified as 1) Ectopia lentis with
generalized malformations or systemic condition is controversial. Several 4. Vasavada AR, Praveen MR, Dholakia
diseases; 2) Ectopia lentis not associated SA, et al. Preexisting posterior capsule
with systemic problems and 3) Ectopia theories have been suggested including defect progressing to white mature
lentis et pupillae not associated with cataract. J AAPOS 2007;11:192–94.
systemic problems. Of all the cases mechanical interference of zonular
of ectopia lentis without systemic 5. Cheng KP, Hiles DA, Biglan AW, et al.
abnormalities, 81-93% are cases of development due to persistent remnants Management of posterior lenticonus.
simple ectopia lentis while 7-19% also J Pediatr Ophthalmol Strabismus
have an eccentric pupil, a condition of tunica vasculosa lentis31. Occurrence 1991;28:143–49.
known as ectopia lentis et pupillae27,28.
The lens and the pupil are usually of skeletal mesodermal abnormalities, 6. Tugal-Tutkun I, Havrlikova K, Power
displaced in the opposite directions as WJ, et al. Changing patterns in
described by Towne. It is usually bilateral like in Marfan’s syndrome, suggest a uveitis of childhood. Ophthalmology
and asymmetric although unilateral cases 1996;103:375–83.
have also been reported. These patients mesodermal origin of ectopia lentis.
generally present in the second decade 7. BenEzra D, Cohen E. Cataract surgery
of life. The pupil is usually small, slit like Presence of iris stromal hypoplasia and in children with chronic uveitis.
and poorly dilating. In 40% patients, Ophthalmology 2000;107:1255–1260.
the pupil is found to be normal while persistent pupillary membrane in many
60% have limitation of dilatation. Trans- 8. Terrada C, Julian K, Cassoux N, et
illumination may be positive in about 60% patients also suggests a mesodermal al. Cataract surgery with primary
of the cases29. The lens subluxation may intraocular lens implantation in
be difficult to appreciate in the presence dysgenesis. Luebbers proposed the children with uveitis: long-term
outcomes. J Cataract Refract Surg
neuroectodermal origin of this condition 2011;37:1977–83.

based on iris trans-illumination defects 9. Holland GN, Stiehm ER. Special
considerations in the evaluation and
due to maldevelopment of pigmentary management of uveitis in children. Am
J Ophthalmol 2003;135:867–78.
epithelium of iris and absence or
10. Quinones K, Cervantes-Castaneda RA,
hypoplasia of dilator pupillae muscle Hynes AY, et al. Outcomes of cataract
surgery in children with chronic uveitis.
resulting in corectopia and poor J Cataract Refract Surg 2009;35:725–
31.
dilatation32.
11. Sijssens KM, Rothova A, Van De
Management: Pre-operatively,

dilated fundus exam, ultrasound and

specular count are essential. Due to the

poorly dilating pupil, there might be a

need for iris hooks intra-operatively. After

doing the intra-lenticular lens aspiration

and anterior vitrectomy, pupilloplasty

is done to center on the intraocular lens

(IOL) (Figure 5A-5F). Then choice can be

made between an iris claw lens, anterior
chamber lens or a scleral fixated IOL.

Conclusion: Children under general

anaesthesia need to be tackled with care.
The proper identification, pre-operative

Khokhar et al. Pediatric Cataract Surgery in Difficult Situations www. dos-times.org 71

Recent Trends and Advances

Vijver DA, et al. Risk factors for the related secondary glaucoma--a distinct hyperplastic primary vitreous. Middle
development of cataract requiring phenotype caused by recessive LTBP2 East Afr J Ophthalmol. 2013;20: 217-
surgery in uveitis associated with mutations. Molec. Vis. 2011;17: 2570- 220.
juvenile idiopathic arthritis. Am J 9. 27. Nelson LB, Maumenee IH. Ectopia
Ophthalmol 2007;144:574–79. 19. Asaoka R, Kato M et al. Chronic angle lentis. Surv Ophthalmol 1982;27:143–
12. Probst LE, Holland EJ. Intraocular lens closure glaucoma secondary to frail 160.
implantation in patients with juvenile zonular fibres and spherophakia. Acta 28. Townes PL. Ectopia lentis et pupillae.
rheumatoid arthritis. Am J Ophthalmol Ophthalmol Scand. 2003;81: 533–5. Arch Ophthalmol. 1976;94:1126-8.
1996;122:161–170. 20. Traboulsi EI, Apte SB. Ectopia lentis 29. Pagon Roberta A, Spaeth George L.
13. Urban B, Bakunowicz-Lazarczyk and associated systemic disease.In: Congenital malformations of the eye. In:
A. Cataract surgery with primary Traboulsi EI, ed. Genetic Diseases of the Tasman W, Jaeger EA, editors. Duane’s
intraocular lens implantation in Eye. Oxford University Press, New York: Foundations of Clinical Ophthalmology.
children suffering from chronic uveitis. 2012. Philadelphia: Lippincott-Raven; 1995.
Klin Oczna 2010;112:111–114. 21. Bitar MS, Farooq AV, Abbasian v. 1. p.29.
14. Lundvall A, Zetterstrom C. Cataract J Challenges in Diagnosing 30. Cross HE. Ectopia lentis et pupillae. Am
extraction and intraocular lens Microspherophakia in a Pediatric J Ophthalmol. 1979; 88 (3 Pt 1): 381-4
implantation in children with uveitis. Patient. JSM Ophthalmol 2016;4(1): 31. Byles DB, Nischal KK, Cheng H. Ectopia
Br J Ophthalmol 2000;84:791–93. 1040. lentis et pupillae. A hypothesis revisited.
15. Li J, Heinz C, Zurek-Imhoff B, et 22. Khokhar SK, Pillay G, Agarwal E, Ophthalmology. 1998;105(7):1331-
al. Intraoperative intraocular Mahabir M. Innovations in pediatric 1336.
triamcinolone injection prophylaxis for cataract. Indian J Ophthalmology 32. Luebbers J A, Goldberg MF, Herbst R, et
post-cataract surgery fibrin formation 2017;65:210-216. al: Iris trans-illumination and variable
in uveitis associated with juvenile 23. Goldberg MF. Persistent fetal expression in ectopia lentis et pupillae.
idiopathic arthritis. J Cataract Refract vasculature (PFV): an integrated Am J Ophthalmol, 1977; 83:647-56.
Surg 2006;32:1535–39. interpretation of signs and symptoms
16. Sijssens KM, Los LI, Rothova A, et al. associated with persistent hyperplastic Correspondence to:
Long-term ocular complications in primary vitreous (PHPV). LIV Edward Dr. Sudarshan Khokhar,
aphakic versus pseudophakic eyes Jackson Memorial Lecture [review]. Am Professor of Ophthalmology
of children with juvenile idiopathic J Ophthalmol 1997; 124:587– 626. Dr. R.P. Centre for Ophthalmic Sciences,
arthritis-associated uveitis. Br J 24. Reese AB. Persistence and hyperplasia All India Institute of Medical Sciences,
Ophthalmol 2010;94:1145–49. of primary vitreous; retrolental New Delhi, India.
17. Romano PE, Kerr NC, Hope GM. Bilateral fibroplasia-two entities. Arch
ametropic functional amblyopia in Ophthalmol 1949; 41:527–52.
genetic ectopia lentis: its relation to the 25. Khokhar SK, Gupta S, et al. Salmon
amount of subluxation, an indicator for pink patch sign: Diagnosing persistent
early surgical management. Binocul Vis fetal vasculature. Oman Journal of
Strabismus Q. 2002; 17:235-41. Ophthalmology 9(1): 68,2016.
18. Khan, A. O., Aldahmesh, M. A., Alkuraya, 26. Sinha R, Bali SJ, et al. Results of Cataract
F. S. Congenital megalocornea with surgery and plasma ablation posterior
zonular weakness and childhood lens- capsulotomy in anterior persistent

72 DOS Times - July-August 2017 Khokhar et al. Pediatric Cataract Surgery in Difficult Situations

Recent Trends and Advances

What is New in Treatment of Amblyopia?

Surabhi Shalini1, Subhash Dadeya2

1. East and North Hertfordshire NHS trust, UK, 2. Guru Nanak Eye Center, New Delhi.

Abstract: Amblyopia is the most common cause of monocular vision loss in children and population under 40 years, with an
estimated prevalence of 1–5%. Amblyopia is caused by a prolonged period of abnormal retinal stimulation due to strabismus
(ocular misalignment), anisometropia (refractive imbalance), or both (combined) and leads to functional deficits, including
reduced contrast sensitivity, poor spatial localization, poor stereovision, and foveal crowding. The present treatments for
amblyopia are predominately monocular, aiming to improve the vision in the amblyopic eye. Recent evidence shows that
amblyopes possess binocular cortical mechanisms for both threshold and suprathreshold stimuli. Hence, there is an ongoing
search for binocular stimulation methods. The purpose is to present stimuli of varying contrast to either eye leading to a
concomitant improvement in monocular acuity of the amblyopic eye with the reduction in suppression and strengthening of
binocular fusion.

Pathophysiology • Make the patient use the amblyopic eye
Various treatment modalities have been described for
Amblyopia is the most common cause of monocular
vision loss in children and population under 40 years, with an amblyopia. These are: Refractive correction, Occlusion therapy,
estimated prevalence of 1–5%1-4. It has initially been described Penalization, Pharmacotherapy, Home vision therapy, Refractive
as “amblyopia ex anopsia” meaning amblyopia from non- Surgery, Pleoptics, CAM stimulator, Red filter, Acupuncture,
seeing. The mechanism of retinal and cortical interaction varies Transcranial magnetic brain stimulation, Television games,
in different amblyogenic condition. In strabismus, the foveae in Mobile games, Omega fatty acids and Smart glasses. However,
the two eyes are directed towards different visual objects. As none of them is perfect.
the foveae have a common visual direction, it provokes visual
confusion, retinal rivalry, diplopia and eventually, a decrease REFRACTIVE CORRECTION
of visual acuity of the deviated eye (strabismic amblyopia).
However, it is visual confusion and not double vision that is An essential component in amblyopia treatment is to
amblyogenic. In anisometropia, the foveae receive images from provide clear foveal image to the amblyopic eye. Refractive
the same visual object; however, the images from the more correction alone improves visual acuity in one third of patients
ametropic eye are out of focus (anisometropic amblyopia). with anisometropic amblyopia and about two-thirds in
As the details are clearly focused in the better eye, there is no ametropic amblyopes5-7. Hence, it is mandatory to carry out
stimulus to the further effort of accommodation to produce cycloplegic refraction in every case of amblyopia before starting
clear images in the worse eye. any adjunctive treatment.

Critical period is a time in early life, during which the visual OCCLUSION
system shows lability of deprivation and ability for reversal of
the effect of deprivation. This critical period begins at about 4 Occlusion is considered the gold standard for amblyopia
months of age, probably passes its peak by 2 years and is well treatment. There is no shortcut or substitute for occlusion
down by 5 years and thereafter undergoes a slow decline to in amblyopia therapy till date. However, variability exists in
cease by about 12 years of age. The time of onset of the critical number of hours prescribed to treat amblyopic patients. The
period is not uniform for all visual functions, which explains the success rate of occlusion therapy varies from 30%-92% in
varied defects seen in amblyopes. various reports. The variation in success rate is due to various
factors like patient selection, treatment duration, age, definition
Treatment of amblyopia used in study and type of amblyopia.

It is crucial to detect and treat amblyopia as early as Different authors have recommended different amount of
possible. Amblyopia can be treated effectively in the first patching.
decade of life, greater success being achieved when therapeutic
measures are instituted at the earliest as the success rates of A recent study by Scott et al8 demonstrated that full time
amblyopia treatment may decline with increasing age. However, occlusion provided excellent visual acuity results. 88% of the
all children should be considered for treatment of amblyopia patients, who achieved a visual acuity of 20/20 at the end of
regardless of age. treatment, maintained that level after an average follow up of
15.5 years. This has been supported by Dorey et al9 and Cleary10.
GOALS OF AMBLYOPIA THERAPY Study done at our centre by Shalini et al. in children aged 4-12
• Present a clear retinal image to the amblyopic eye by years showed 6 hours of occlusion shows improvement in
visual acuity in moderate and severe amblyopia. The means
eliminating causes of visual deprivation and correcting improvement over the baseline visual acuity was 2.2 (SD-1.23).
visually significant refractive errors
Hug11compared full time occlusion with part time occlusion
on 3-7 year children. A higher percentage of those treated with

Surabhi et al. What is New in Treatment of Amblyopia www. dos-times.org 73

Recent Trends and Advances

full time achieved 20/30 VA in amblyopic 20/100). It was concluded that both kg of levodopa –carbidopa were found
eye than part time occlusion patients over the treatments were well tolerated and to be well tolerated and efficacious at
a shorter duration of treatment. the effect of each treatment seemed
consistent. The Atropine Group had temporarily improving visual acuity in
Recent recommendations of PEDIG statistically significant higher degree of
support part time occlusion and rate it acceptability. Patching has a potential amblyopic eyes of children17.
similar in efficacy to full time occlusion as advantage of a more rapid improvement
ATS212-13. in visual acuity and possibly a slightly Study by Dadeya et al18 evaluated
better acuity outcome, whereas atropine
ATS 2B concluded that in moderate has the potential advantage of easier the role of levodopa/carbidopa in the
amblyopia, prescribing two hours of daily administration and lower cost. Atropine
patching produces an improvement in or patching for a six months period treatment of amblyopia and concluded
visual acuity that is of similar magnitude produced a similar improvement in
to the improvement produced by amblyopia two years after treatment. that there was more than two lines
prescribing six hours of daily patching in Improvement from baseline to 6 months
children 3 to less than 7 years of age. was 3.16 lines in the patching group and improvement in visual acuity, especially
2.84 in atropine group.
ATS 2A concluded that for severe in children younger than eight years of
amblyopia, prescribing six hours of daily Since compliance is the most
patching produces an improvement in important determinant to success in age.
visual acuity that is of similar magnitude amblyopia therapy, clinical experience
to the improvement produced by has found that atropine penalization has ATS 1419 was done to study the
prescribing full-time daily patching in a high acceptability. The PEDIG assessed
children 3 to less than 7 years of age. the acceptance of both types of treatment dosage of levodopa as a treatment of
(occlusion and penalization) by means
Another problem with full time of a questionnaire. Although atropine residual amblyopia in children 8-18 years
occlusion, particularly in severe was better accepted; the difference in
amblyopia is the functional debilitation acceptance was small. It has the advantage old. The study intervention consisted
that it causes, especially early in over occlusion by providing a wider
treatment. Poor vision during occlusion visual field for both eyes, which may have of continuing 2 hours of daily patching
of the sound eye presents a significant safety and other functional implications.
deterrence to good compliance. The child There is also evidence that it improves plus the addition of levodopa in one of
may require additional supervision to the ability to see with both eyes.
avoid accidental injury. Occlusion also two doses randomly assigned with equal
causes fusion disruption and increase in The PEDIG compared daily atropine
angle of deviation.Other than cosmetic to weekend atropine for moderate probability (0.51 or 0.76 mg/kg/tid,
unacceptability, allergic skin rash may amblyopia in a randomized clinical trial
also hamper patient willingness of putting and the study concluded that weekend referred to as lower dose and higher dose,
the patch. This can be minimized with use atropine provides an improvement in
of skin cream to irritated areas14. All these visual acuity of a magnitude similar to respectively). The lower dose had been
adverse effects are seen in both full time that of improvement provided by daily
and part time occlusion, but are more atropine in treating amblyopia in the 3 -7 used in most prior studies. The study
pronounced with full time occlusion. years age Group14.
medication was administered for 8 weeks
Conclusions - in this study atropine
penalisation has been shown to be with one additional week for tapering
as effective as occlusion therapy in
the treatment of amblyopia. Patient of treatment. Levodopa was prepared in
acceptance of atropine penalisation was
superior to that for occlusion therapy capsules combined with carbidopa 0.17
as was shown by the compliance
rate. Atropine treatment was also mg/kg/tid. Carbidopa was combined
advantageous in that compliance could
be readily checked by inspection. with levodopa to reduce side-effects

Pharmacological therapy associated with levodopa alone. The

Most recently, PEDIG performed a mean improvement in amblyopic eye
randomized, placebo-controlled study
of levodopa in treatment of amblyopia visual acuity from baseline to the 9-week
.They concluded that the combination
of levodopa- carbidopa and occlusion primary outcome visit was +4 (±4) letters
improves visual function more than
levodopa- carbidopa alone in amblyopic in the 16 subjects in the lower dose Group
children16. Average dose levels of
0.95/0.24 mg/kg and 1.94/0.49 mg/ and +6 (±6) letters in the 17 subjects in

the higher dose Group (mean difference
between Groups = −2 letters).

ATS 1720 compared the efficacy and

safety of oral levodopa and patching

versus oral placebo and patching at 18

weeks, after 16 weeks of treatment for

PENALIZATION amblyopia in children 7 to <13 years old

In view of the difficulties and it concluded that children 7 to 12

encountered during occlusion therapy years of age with residual amblyopia after

and the occasional complication of patching therapy, oral levodopa while

occlusion amblyopia, alternative methods continuing to patch 2 hours daily does

of treatment of amblyopia have been not produce a clinically or statistically

explored. Classically, penalization has meaningful improvement in VA compared

been used as a second treatment when with placebo and patching

occlusion was not complied with, or Citicholine (CDP Choline)

for post-occlusion as a maintenance is an essential intermediate for

treatment phosphatidylcholine synthesis. It
increases cerebral blood flow. It also
Indications for penalization are:
shows neural restorative effect, via
1. Moderate amblyopia in
actionon dopaminergic pathway of
uncooperative patient
central nervous systems.
2. Anisometropic amblyopia

3. Maintenance therapy NEAR VISUAL ACTIVITIES

4. Occlusion failure Near visual activities are often
prescribed during patching for amblyopia
5. Occlusion nystagmus based on the assumption that those
ATS 115 was initiated to address activities stimulate the visual system.
In the recent times their role again has
whether occlusion or atropine

penalization is best initial treatment

for moderate amblyopia (20/40 -

74 DOS Times - July-August 2017 Surabhi et al. What is New in Treatment of Amblyopia

Recent Trends and Advances

come to forefront with the Pediatric Eye ROLE OF REFRACTIVE SURGERY attentive game play (noughts and crosses)
Disease Investigator Group incorporating was the CAM treatment28 (Campbell et al).
near visual activities into the prescribed LASIK is an alternative method for Its beneficial effects were later isolated to
treatment regimes. Authors have put correcting high myopic and hyperopic the short term nature of the occlusion
forth the emerging role of television/ anisometropia. Recent trends show that and the attentive game play (Mitchell,
video games and mobile games for near refractive surgery is effective and safe Howell, & Keith). There is no doubt that
vision activity as an adjunct to amblyopia alternate treatment modality in desperate perceptual learning combined with short-
therapy, as these are extremely popular cases of conventional therapy failure term patching is much better than longer-
with children and easily available, as well. cases of anisometropic amblyopia25,26. term patching with passive stimulation
in terms of improving monocular acuity
Park et al21 from Korea in a ACUPUNCTURE IN AMBLYOPIA (Li et al.)29, however its usefulness for
retrospective study published in 2008 re-establishing binocular vision and
highlighted the importance of near Acupuncture is a potentially useful stereopsis is less clear. A number of
visual activities with part time patching complementary treatment modality that hybrid-binocular approaches have been
(6 hours) in treating children (mean may provide sustainable adjunctive effect suggested, which are all directed to
age 4.86 years) with anisometropic, to refractive correction for anisometropic recovering monocular function under
strabismic or combined amblyopia (both amblyopia in young children. binocular viewing. The aim is to involve
moderate and severe). They noted 86% the fixing eye in recovery of vision
success rate with visual acuity improving Although the treatment effect of through intensive training/detection
from baseline by an average of 3.2±2.5 acupuncture appears promising, the of targets presented exclusively to the
lines (0.33±0.26 log MAR), in a follow-up mechanism underlying its success as a amblyopic eye. The iBit system30 (Cleary
period of 1.62±1.20 years. treatment for amblyopia remains unclear. et al.), the ‘‘Push–Pull’’ (Ooiemail et al.)31.
Acupuncture at vision-related acupoints
A randomized control trial i.e. ATS may modulate the activity of the visual As binocular game play was shown
622 the investigators did not notice any cortex. Moreover, acupuncture has been to be superior to monocular game play in
difference in visual acuity improvement shown to be effective in increasing visual acuity and stereopsis improvement,
between children who performed blood flow to the cerebral and ocular researchers began to develop dichoptic
common near activities and those who vasculatures (including the choroid), games.
performed distance activities during stimulating the expression of retinal
patching treatment for amblyopia. This nerve growth factors and leading to Hess et al32 examined the potential
finding was in contrast to the results metabolic changes in the central nervous of treating amblyopic adults using mobile
of the previous randomized pilot study system. game. In this study dichoptic mobile
and also to several case series reporting game Tetris was used on IPod to treat 14
the effect of near activities or activities TRANSCRANIAL MAGNETIC BRAIN amblyopic patients aged 13 to 50 years
involving eye-hand coordination in STIMULATION were examined. The dichoptic video
treatment of amblyopia21,22,23. game treatment was conducted at home
Transcranial magnetic stimulation and visual function assessed before and
Role of television/ video games (TMS) is a non-invasive method for after treatment.
stimulating parts of the brain by use of
Television has always been a very weak electric current that are induced On this basis they concluded that the
popular mode of entertainment, having into the tissue by use of rapidly changing home-based dichoptic iPod mobile game
being regarded by many as notorious magnetic currents. TMS produces effects approach represents a viable treatment
for engaging children and adults alike in that last slightly longer than the actual for adults with amblyopia.
spending idle time in front of it. In spite time of stimulation.
of that, its popularity refuses to die down. Birch et al.33 also did similar study to
As a matter of fact, it has in the past, MOBILE GAMES find out the effectiveness of a novel home-
found utility in administering amblyopia based binocular amblyopia treatment.
therapy. It has been shown that loss of They treated children (4–12 years of
binocularity is one of the defining features age) who wore anaglyphic glasses to play
In 1977, Saladin and Bohman24 of amblyopia (McKee, Levi, & Movshon)27 binocular games on an iPadplatform for
noted that orthoptic therapy often therefore, the focus of research in 4 h/w for 4 weeks. The first 25 children
entails the repetitive usage of detailed this area has shifted from monocular were assigned to sham games and then
and complicated training procedures interventions that involve patching of 50 children to binocular games. They
and amblyopia and antisuppression the fellow eye to approaches that directly concluded that Binocular iPad treatment
therapy in particular may require weeks target binocular visual function and as rapidly improved visual acuity, and visual
or months of training, so as to maintain the primary therapeutic step. This has led acuity was stable for at least 3 months
a high level of patient interest and to increased interest in the development following the cessation of treatment.
cooperation throughout the training of amblyopia treatments that directly
procedure. They reported an anaglyphic address binocular dysfunction by These Games achieve their
T.V. ping pong antisuppression trainer. promoting binocular vision and reducing therapeutic effect by presenting a
inhibitory interactions within the visual different image to each eye, thus
In study conducted by Dadeya et al it cortex. rewarding the patient when both eyes
was concluded that the television games work together to win the game. For
along with full time patching, are effective The first attempt to provide the instance, in the Stereoblock game, some
in the treatment of amblyopia. combination of short-term occlusion (20 of the blocks seen by the amblyopic eye
min), controlled visual stimulation and are in high contrast, while other blocks

Surabhi et al. What is New in Treatment of Amblyopia www. dos-times.org 75

Recent Trends and Advances

in lower contrast are seen by the healthy

eye.A pilot study done at our centre was
done to evaluate the efficacy of 2 hours
of occlusion along with mobile game

exercises as a form of near visual activity

in the treatment of amblyopia.

Role of omega fatty acids Binocular treatment of amblyopia by mobile and iPad games

Amblyopia can be considered the debilitation, cosmetic blemish, allergic quality of life and atropine drops can
result of a lack of normal plasticity. Visual skin rash, recurrence, poor compliance, cause light sensitivity and disorientation.
cortical dominance by the better eye psychological problems, ocular deviation Even the patient who has done occlusion
leads to correspondent visual deprivation due to disruption of fusion, and visual therapy with good compliance may
of the representations related to the decline. Penalization also suffers from improve visual acuity in only 27-
eye with worse acuity. Knowledge of potential side effects due to systemic 32% of cases; leading to suppression,
neuroplasticity and the factors that absorption of drug, allergic reactions, stereo blindness and a deficient depth
control the opening and closure of critical and the fact that active inhibition is not perception in the rest.
periods will lead to new therapeutic eliminated.
strategies which may allow for greater NEWER STRATEGIES
recovery of visual functions in both A study of moderate and severe
children and adults with amblyopia. amblyopia treatment found approximately Recent studies have shown that
25% of patients under age seven had a amblyopia is a binocular problem caused
Omega fatty acids are important recurrence of amblyopia within the first by active suppression that converts a
structural components of membrane year of stopping treatment, and children structurally intact binocular system into a
lipids in central nervous system. There ages seven to 12 had a 7% chance of functionally monocular system. Evidence
are two long chains polyunsaturated recurrence (worsening of two lines of suggests that focusing on binocular
fatty acids, DHA and ARA. During third visual acuity)36,37. This recurrence is more treatments for amblyopia may prove to
trimester of human foetal development common in patients with severe amblyopia be beneficial in both improving vision as
there is tenfold increase in brain size who went from six hours of patching well as possibly improving binocularity.
which is accompanied by 30 fold increase per day to no patching. Additionally, These treatments are based on three
in DHA content and a 15 fold increase in patients with a history of successfully principles:
ARA content34. treated amblyopia need continued close • Monocular perceptual learning (PL),
monitoring for a recurrence of amblyopia. • Monocular videogame play (VGP)
Several randomized trials are To help prevent this recurrence, patients
conducted and found specific benefit should be weaned off patching therapy. and
of omega fatty acids supplementation Residual amblyopia is another treatment • Dichoptic PL/VGP.
for retinal maturation, visual acuity challenge, considering vision does not
development, or cognitive development35. improve sufficiently with one treatment A dichoptic treatment presents a
for some patients. stimulus to each eye separately and the
Preliminary results from a study in brain is forced to integrate the images into
England show that school performance Lacunae in existing amblyopia a single perception. In these treatment
improved among a group of students therapy modalities; under binocular conditions,
receiving omega-3 fatty acids. In an the signal strength coming into the
Australian study, 396 children between Besides the fact that these treatment patient’s good eye is reduced enough so
the ages 6 and 12 who were given a modalities have dominated the pediatric that it cannot suppress the amblyopic eye.
drink with omega-3 fatty acids and ophthalmologist world for ages, patient The result is binocular perception in a
other nutrients (iron, zinc, folic acid compliance is poor because of the many patient with otherwise deep suppression
and vitamins A, B6, B12 and C) showed negative side effects of patching and of the amblyopic eye. Over time, the
higher scores on tests measuring verbal drops. Furthermore, patching can cause viewing conditions are changed and the
intelligence, reading, learning and many psychosocial problems when trying image seen by the good eye is suppressed
memory after six months and one year to force a child to comply with wearing less until both eyes see approximately
than a control group of students who their eye patch. Kids don’t want to wear the same image. There are many popular
did not receive the nutritional drink. their eye patch because it impacts their softwares that are developing now-a-
This study was also conducted with 394 days promoting binocular vision therapy
children in Indonesia. The results showed
higher test scores for boys and girls in
Australia, but only for girls in Indonesia.
However a randomized controlled study
from our centre did not find any added
advantage of Omega Fatty acids.

Disadvantages of current treatment
modalities

Occlusion therapy is not free
from side effects. Disadvantages can
be: occlusion amblyopia, functional

76 DOS Times - July-August 2017 Surabhi et al. What is New in Treatment of Amblyopia

Recent Trends and Advances

with 3D gaming and at the same time acting like a digital patch that flickers on 19. Repka MX, Kraker RT, Beck RW, et. al., Pediatric
stimulating perceptual learning. These and off. Amblyz™ occlusion glasses were Eye Disease Investigator Group. Pilot study of
are believed to treat amblyopia not only levodopa dose as treatment for residual amblyopia
in children but also in adults. used for 4 hours daily in a study, where in children aged 8 years to younger than 18 years.
Arch Ophthalmol 2010;128(9):1215-7
Goal of newer treatment strategies the lens over the eye with better vision
20. Pediatric Eye Disease Investigator Group. A
• Perceptual learning switched from clear to opaque every randomized trial of levodopa as treatment
• Visual processing development for residual amblyopia in older children.
• Oculomotor therapy 30 seconds. (Presented at AAO but not Ophthalmology 2015;122:874-81.
• Eye-hand coordination training
published). 21. Park KS, Chang YH, Na KD,et al. Outcomes of 6 Hour
‘Perceptual learning’ approaches Part-time Occlusion Treatment Combined with
involving monocular training on gameplay KEY POINTS Near Activities for Unilateral Amblyopia. Korean J
or psychophysical tasks have proven Ophthalmol. 2008; 22(1):26-31
effective in this regard. Softwares based • Conventional occlusion is still the
on dichoptic PL also have been found to treatment of choice. Although part 22. Holmes JM, Lyon DW, Strauber SF,et al. A
improve stereoacuity. Besides perceptual Randomized Trial of Near versus Distance Activities
learning; antisuppression exercises time occlusion is gaining acceptance while Patching for Amblyopia in Children 3 to < 7
and interactive binocular therapies years old. Ophthalmology. 2008; 115(11):2071–
have found similar improvements in after ATS reports. 2078.
amblyopes. • Penalization should be considered as
23. Ghanem AA, Moad AI, Nematallah EH,et al. Laser in
Hess and colleagues have developed an alternate line of therapy. situ keratolileusis for treated myopic anisometropic
antisuppression therapy, which uses • Near Vision exercises are beneficial amblyopia in children. Saudi J Ophthalmol 2010;
games (with elements split across the 24:3-8.
eyes to promote binocularity) as a in treatment of amblyopia.
mean of treating amblyopia in adults • Binocular treatment is the future of 24. Saladin JJ, Bohman CE. Anaglyphic T.V. ping pong
and children. After 2 to 6 weeks of antisuppression trainer. J AmOptom Assoc. 1977
treatment, visual acuity improved by an amblyopia management. Jul;48(7):929-32
average of approximately 0.15 logMAR
and stereopsis was measurable in REFERENCES 25. Tamer Adel Rafai, Olfat Ahmed Hassanin. Evaluation
approximately 45% of participants38,39. Of Improvement Of Best Corrected Visual Acuity
1. Arnold R. Amblyopia risk factor prevalence. Journal Following Lasik Treatment in Anisometropic
Other promising approaches for of Pediatric Ophthalmology and Strabismus. Amblyopia. Australian J Basic & Appl Sci 2011;
adult patients include the application 2013;50(4):213-7. 5(11):23-29.
of safe, non-invasive brain stimulation
techniques to the visual cortex and the 2. Tarczy-Hornoch K, Cotter S, Borchert M, et al. The 26. Kumari E, Dadeya S. Efficacy of LASIK surgery
combination of patching with Fluoxetine Multi-Ethnic Pediatric Eye Disease Study Group. in anisometropic amblyopia in adults. Thesis
(Prozac), which has previously been Prevalence and causes of visual impairment in submitted to Delhi University in 2009.
found to reverse amblyopia in adult asian and non-Hispanic white preschool children.
animals. This research not only aims to Ophthalmology. 2013;120(6):1220-6. 27. Levi DM, Li RW. Perceptual learning as a potential
improve vision, but is also designed to treatment for amblyopia: A mini-review. Vision Res.
provide new insights into the mechanisms 3. Chen X, Fu Z, Yu J, et al. Prevalence of amblyopia and 2009 Oct 29;49(21):2535–49.
that control brain plasticity. This means strabismus in eastern China: results of screening
that studying the visual system could lead preschool children aged 36-72 months. British 28. Campbell FW, Hess RF, Watson PG and Banks R.
to the development of new treatment Journal of Ophthalmology. 2015;0:1-5. Preliminary results of a physiologically based
techniques that could be applied to a treatment of amblyopia. Br J Ophthalmol 1978; 62:
range of different brain disorders. 4. Pai A, Rose K, Leone J, et al. Amblyopia prevalence 748- 55.
and risk factors in Australian preschool children.
Current occlusion therapy, although Ophthalmology. 2012;119(1):138-44. 29. Li, R.W., K.G., et al. ”Perceptuallearning
efficient in recovering the monocular improvesvisualperformanceinjuvenile amblyopia.”
visual acuity of the amblyopic eye, 5. Wallace D, Chandler D, Beck R, et al. Treatment of Investigative ophthalmology & visualscience
prevents the two eyes from working bilateral refractive amblyopia in children 3 to <10 2005;46:3161-3168.
together. Published reports indicate that years of age. American Journal of Ophthalmology.
the learning (or improving) potential is 2007;144(4):487-96. 30. Herbison N, Cobb S, Gregson R, et al. Interactive
still present in treated amblyopes, who binocular treatment (I-BiT) for amblyopia: results
have normal monocular visual acuity 6. Cotter S, Edwards A, Wallace D, Beck R. Treatment of of a pilot study of 3D shutter glasses system. Eye
and that additional treatment might be anisometropic amblyopia in children with refractive Lond Engl. 2013;27:1077–83.
necessary. The current research aims at correction. Ophthalmology. 2006;113(6):895-903.
stimulating binocular interaction. 31. Ooi TL, Su YR, Natale DM,et al. A push-pull treatment
7. Cotter S, Foster N, Holmes J, et al. Optical for strengthening the “lazy eye” in amblyopia. Curr
Programmable electronic glasses treatment of strabismic and combined strabismic- Biol CB. 2013 Apr 22;23(8):R309-310. 149.
anisometropic amblyopia. Ophthalmology.
The lenses are liquid crystal display 2012;119(1):150-8. 32. Hess RF, Babu RJ, Clavagnier S,et al. The iPod
(LCD), they can also be programmed to binocular home-based treatment for amblyopia
turn opaque, occluding vision in the left 8. Scott WE, Kutschke PJ, Keech RV,et al. Amblyopia in adults: efficacy and compliance, Clinical and
or right eye for different time intervals, treatment outcomes. JAAPOS. 2005; 9:107-11. experimental optometry 2014; 97: 389–398

9. Dorey SE, Adams GG, Lee JP,et al. Intensive occlusion 33. Birch, E. E., Li, et al. (2014).Binocular iPad treatment
therapy for amblyopia. Br J Ophthalmol. 2001; for amblyopia in preschool children. Journal of
85(3):310-3 American Association for Pediatric Ophthalmology
and Strabismus (JAAPOS), 18(4), e1–e2
10. Cleary M. Efficacy of occlusion for strabismic
amblyopia: Can an optimal duration be defined? Br 34. Davis-Bruno K, Tassinari MS. Essential fatty acid
J Ophthalmol. 2000; 84(6):572-8. supplementation of DHA and ARA and effects on
neurodevelopment across animal species: a review
11. Hug T. Full time occlusion compared to part time of the literature. Birth Defects Res B Dev Reprod
occlusion for treatment of amblyopia. Optometry. Toxicol. 2011 Jun;92(3):240-50.
2004; 75(4):241-4.
35. Jun B, Mukherjee PK, Asatryan A, Kautzmann MA,
12. Repka MX, Beck RW, Holmes JM, et al. Pediatric Eye Heap J, Gordon WC, Bhattacharjee S, Yang R, Petasis
Disease Investigator Group. A randomized trial NA, Bazan NG. Elovanoids are novel cell-specific
of patching regimens for treatment of moderate lipid mediators necessary for neuroprotective
amblyopia in children. Arch Ophthalmol. 2003; signaling for photoreceptor cell integrity. Sci Rep.
121(5):603-11 2017 Jul 13;7(1):5279.

13. Kraker RT, Beck RW, Birch EE, et al. Pediatric Eye 36. Holmes J, Beck R, Kraker R. Risk of amblyopia
Disease Investigator Group. A randomized trial recurrence after cessation of treatment. J AAPOS.
of patching regimens for treatment of severe 2004;8(5):420-8.
amblyopia in children. Ophthalmology. 2003;
110(11):2075-87. 37. Hertle R, Scheiman M, Beck R, et al. Stability of visual
acuity improvement following discontinuation of
14. Pediatric Eye Disease Investigator Group. A amblyopia treatment in children 7 to 12 years old.
randomized trial of atropine regimens for treatment Archives of Ophthalmology. 2007;125(5).
of moderate amblyopia in children. Ophthalmology
2004; 111:2076-85 38. Li SL, Jost RM, Morale SE, et al. A binocular
iPad treatment for amblyopic children. Eye.
15. Foley-Nolan A, McCann A, O‟Keefe M. Atropine 2014;28:1246–1253.
penalisation versus occlusion as the primary
treatment for amblyopia. Br J Ophthalmol. 1997; Correspondence to:
81:54–7 Dr. Surabhi Shalini,
East and North Hertfordshire NHS Trust
16. Duffy FH, Burchfield JL and Snodgrass SR. The
pharmacology of amblyopia. Ophthalmology 1978;
85: 189-195.

17. Domemici L, Cellerino A and Maffei L. Monocular
deprivation effects in the rat visual cortex and
lateral geniculate nucleus are prevented by nerve
growth factor (NGF) II. Lateral geniculate nucleus.
Proc R Soc Lond Series B 1993; 25: 25-31.

18. Dadeya S, Vats P, Malik KP. Levodopa/carbidopa in
the treatment of amblyopia. J Pediatr Ophthalmol
Strabismus. 2009 Mar-Apr; 46(2):87-90

Surabhi et al. What is New in Treatment of Amblyopia www. dos-times.org 77

Recent Trends and Advances

Do you want to be a Pediatric Ophthalmologist?

Take Control of your Future: Scope of Pediatric Ophthalmology

Savleen Kaur MS
Advanced Eye Center, Post Graduate Institute of Medical Education and Research, Chandigarh, India

As I walked into my first paediatric clinic posting few selective people pursuing it. Most of the times, a pediatric
in my first year of residency; the chaotic crying ophthalmologist is dependent on adult cataract surgery or
noises and several screaming kids made me other realms due to work restraints or for livelihood. How
want to cry. Ten minutes into it and I told my many pediatric ophthalmologists you know who practice just
colleague “I can’t do this. I can never have that on pediatric eye diseases? Doing pediatric surgery will make
much patience”. I reminded myself most of the you an equally good and sometimes a better adult cataract
days, “It’s just a day; this shall also pass”. I restricted myself to a surgeon. In the 3D world that we are heading into; strabismus
long history taking and I kept trying to make the mothers realise surgery is becoming functionally more necessary than being
that their kid was a monster whom they could not control. I only cosmetic. Retinopathy of prematurity is an ever-increasing
kept yelling “You have to make them stop crying like you do at epidemic and in a country like India, parents choose the girl
home”. I copied down retinoscopy values like a donkey; writing child to rather go blind than be enucleated for retinoblastoma.
‘uncooperative’ most of the time in my strabismus examination. Pediatric ophthalmologists are very few in proportion to
I nodded deceptively in agreement every time my seniors cataract and refractive surgeons. So, what are the tricks of
increased or decreased the spectacle prescription. the trade? Imagine the most difficult thing you had to do as a
fellow in ophthalmology. Applanation tonometry or gonioscopy
And then I met this kid. A four-year-old I operated for a in the first year, retinoscopy or indirect ophthalmoscopy? May
bilateral total white cataract. He kept howling preoperatively; be a tough task in life like riding a bike or car, reconciling with
before induction of anesthesia and postoperatively. Till I an offended colleague or raising your own child? Could you
removed his postoperative dressing. I took it out and he overcome that? If yes, how difficult will it be to make a kid talk
stopped crying. He looked at me and stared at me for a good to you. If I have learnt something from my teachers, it is that
two minutes. And then he looked at his mother, looked around intelligence is nothing but an infinite capacity for hard work. If
the room and smiled. He could see. It dawned on me that this you believe in yourself, you can do it. Believe me, as a pediatric
kid, like many others is going to do everything I say. I had the ophthalmologist; you get instant gratification and good
power to shape his life and his future. prognostic cases, make the kids laugh; the parents indebted

Today after only a few years into this speciality I realise not Get rid of your white coat sometimes if needed or decorate it
only did I change the kids’ future; I also changed mine. with a patch

Choosing paediatric ophthalmology demands a lot of Use as many different toys as you can each time/ have kids’
patience and stamina with a holistic approach of the child’s videos on mobile
visual system. It can be boring for some; with no lucrative
treatment options and minimal changes in paradigms. Do not Examine the child in the mother’s lap in a usual chair rather
choose this branch because someone said so. But if you don’t than examining chair
know what it is all about, how will you even decide that you
don’t want to do it? I myself have a very limited experience in Shake the child’s hand before starting examination (with a
the field mostly under an excellent direction and guidance by candy perhaps)
my teachers. My objective behind this piece of writing is to give Build a relationship with the child first-he/she will let you
you an insight on the work life of a pediatric ophthalmologist. examine at ease

A paediatric ophthalmologist is not only about strabismus Try examining only one kid at a time-their attention breaks
or spectacles. It’s a whole lot of ophthalmology shrunken in one easily
small clinic. You need to know the basics of optics, refractive
errors, spectacle prescriptions, normal visual and growth Keep your walls coloured and as less equipment in the room
milestones in a child; genetic disorders and a lot of paediatrics as possible; don’t make kids listen to other kids crying while
too. One needs to be aware of a vast era of abnormalities ranging dilation
from allergic eye diseases; to epiphora; strabismus; paediatric
cataract; glaucoma; uveitis; retinoblastoma and retinopathy Learn to make funny noises!
of prematurity to name a few. So, don’t restrict yourself to just
what you are taught and what you see people doing. Expand Expand the visual acuity charts you have
and make your own realm.
Learn not to shout-even when not in clinic
It is a branch that is ever increasing in demand, with only
Educate the mother and trust her- she can be a better doctor
78 DOS Times - July-August 2017 than you!

Kaur. Scope of Pediatric Ophthalmology

Recent Trends and Advances

and most of all-you get to laugh and to Speciality Societies / Conference avenues
have fun. Strabismus and pediatric ophthalmology society of India [www.sposiindia.org]
American Association for Pediatric Ophthalmology and Strabismus [www.aapos.org]
Even while being a resident if you International Strabismus Association. http://www.isahome.org/
have thoughts like “I am not good with Global pediatric ophthalmology congress [www.pediatricophthalmology.
kids” doesn’t mean you cannot pursue a conferenceseries.com]
career in pediatric ophthalmology. You
can be a pediatric ophthalmologist even Fellowship opportunities
if you do not have much passion and http://education.lvpei.org/education_lvpei/ophthalmology/pediatriclong.php
patience towards kids. This is something http://www.sankaranethralaya.org
that can be trained. Also, it is said there http://www.aravind.org
is no good borne-surgical hand, most International
of things come with mere training and Commonwealth fellowships-http://cehc.lshtm.ac.uk/clinical-fellows/
perseverance. The teacher of teachers, http://www.utovs.com/education/fellowships/paediatrics-strabismus
Prof Amod Gupta; taught us all the http://soevision.org/fellowship-platform/
most important quality needed to be a https://www.willseye.org/fellowship-pediatric-ophthalmology-for-foreign-medical-
physician- that is ‘empathy’. I tried to graduates
remember it on every clinic day. That’s all International council of Ophthalmology fellowships including fellowship for ROP
it takes to be a pediatric ophthalmologist http://www.icoph.org/refocusing_education/fellowships.html
too.
Must read journals
I am no expert, we are all learners Journal of the American Association of pediatric ophthalmology and strabismus
here. I am not promising you any good Journal of pediatric ophthalmology and strabismus
money to begin with. But I can promise Indian journal of pediatric ophthalmology and strabismus
you gratification if you choose this career Web education sources
with a change in your own personality. https://www.aao.org/clinical-education/pediatric-ophthalmology-education-center
You may have to make the medical http://www.eophtha.com
fraternity appreciate and perceive http://eyewiki.aao.org/Category:Pediatric_Ophthalmology/Strabismus
the burden of childhood ophthalmic Cybersight organization: provides online courses, live lectures (webinars), and
problems. May be you must alert consultation. Pediatric ophthalmology is an integral part of this website. https://
everyone to the fact that a child must be cybersight.org/
screened early for something as simple as Eyerounds by University of Iowa http://webeye.ophth.uiowa.edu/eyeforum/index.
a refractive error to a malignant condition htm
like retinoblastoma. May be you will have Canadian neuroophthalmology group: Neuro-ophthalmology is intimated to the
to increase awareness amongst parents pediatric ophthalmology. http://www.neuroophthalmology.ca/
or travel miles to tell kids they have * Paid sources;
problems. Maybe you have to become American Academy one network. https://www.aao.org/international/programs/
more of a social worker and counsellor about-one-network
sometimes because even making them Uptodate.com. e.g. https://www.uptodate.com/contents/congenital-nasolacrimal-
wear spectacles could be a social stigma. duct-obstruction-dacryostenosis-and-dacryocystocele
You know, 153 million people can be Facebook, telegram, and Yahoo groups and virtual universities
cured of their blindness worldwide by Telegram of free ophthalmic downloading;
wearing glasses alone. Facebook; e.g. Piece of ophthalmology videos
Yahoo group: e.g. FRCophthyahoogroup- Asian ophthalmology yahoo group.
The first step towards getting Virtual university for postgraduate teaching e.g. Muthusammy
somewhere is to decide you’re not going
to stay where you are. Correspondence to:
Dr. Savleen Kaur,
” —John Pierpont “J.P.” Morgan Senior Research Associate,
So, what does being a pediatric PGIMER, Chandigarh
ophthalmologist mean? To me, being
a pediatric ophthalmologist means
being a teacher, a counselor, a guide,
an empathetic doctor and a brilliant
surgeon! A touch of humanity, a pinch of
compassion, a bit of patience and loads
of hard work- is this not why we became
doctors in the first place?

Dedicated to all my teachers

Acknowledgment (For education
material): Ghada Zein El-Abedin Rajab
MD. Lecturer of Ophthalmology, Menoufia,
University Hospitals, Egypt.

Kaur. Scope of Pediatric Ophthalmology www. dos-times.org 79

Techniques

Botulinum Toxin in Strabismus

T.S. Surendran MS, Supraja Kasturirangan
Sankara Nethralaya, Chennai, Tamil Nadu, India.

Abstract: C. botulinum elaborates eight antigenically distinguishable exotoxins (A, B, C1, C2, D, E, F and G). Type A is the most
potent toxin, followed by types B and F toxin. The uses of botulinum in strabismus are manifold and highlighted in this article.

INTRODUCTION c. 3rd nerve palsy
2. Childhood strabismus-
C. botulinum elaborates eight antigenically distinguishable a. Infantile esotropia
exotoxins (A, B, C1, C2, D, E, F and G). Type A is the most potent b. Accommodative esotropia
toxin, followed by types B and F toxin. Types A, B and E are c. Cyclic esotropia
commonly associated with systemic botulism in humans. d. Intermittent exotropia
e. Cerebral palsy
All botulinum neurotoxins are produced as relatively 3. Adult strabismus-
inactive, single polypeptide chains with a molecular mass a. Strabismus already operated multiple times
of about 150 kDa with a high degree of amino acid sequence b. Post retinal detachment strabismus
homology among the toxin types. The polypeptide chain c. Thyroid ophthalmopathy
consists of a heavy (H) chain and a light (L) chain of roughly d. Post-operative adjustment
100 and 50 kDa respectively, linked by a disulphide bond. e. Intrinsic muscle disorders
The botulinum toxin neurotoxin complex is also associated f. Nystagmus
with various other nontoxic proteins, which may also have g. Post cataract strabismus surgery
hemagglutinating properties1,2. 4. Any strabismus for which the patient is not fit for

MECHANISM OF ACTION anesthesia due to any systemic illness
5. When multiple muscles need to be operated on in a single
All the serotypes interfere with neural transmission by
blocking the release of acetylcholine, which is the principal eye, one of the muscles can be given injection of botulinum
neurotransmitter at the neuromuscular junction. Intramuscular toxin to prevent anterior segment ischemia.
administration of botulinum toxin acts at the neuromuscular
junction to cause muscle paralysis by inhibiting the release CONTRAINDICATIONS
of acetylcholine from presynaptic motor neurons. Botulinum
toxins act at four different sites in the body: The neuromuscular 1. Restrictive strabismus when the muscle is scarred -severe
junction, autonomic ganglia, postganglionic parasympathetic restrictions caused by scarred inelastic muscles favour
nerve endings and postganglionic sympathetic nerve endings surgery, because botulinum toxin depends on reciprocal
that release acetylcholine. The heavy (H) chain of the toxin agonist-antagonist readjustments of length-tension
binds selectively and irreversibly to high affinity receptors at elasticity, scarring restricts this readjustment.
the presynaptic surface of cholinergic neurons, and the toxin-
receptor complex is taken up into the cell by endocytosis. The 2. Contraindicated in the presence of infection at the
disulphide bond between the two chains is cleaved and the toxin proposed injection site(s)
escapes into the cytoplasm. The light (L) chain interact with
different proteins (synaptosomal associated protein (SNAP) 3. In individuals with known hypersensitivity to any
25, vesicle associated membrane protein and syntaxin) in the botulinum toxin preparation or to any of the components
nerve terminals to prevent fusion of acetylcholine vesicles in the formulation.
with the cell membrane. The affected nerve terminals do not
degenerate, but the blockage of neurotransmitter release is TECHNIQUE-
irreversible. Function can be recovered by the sprouting of
nerve terminals and formation of new synaptic contacts; this It can be done under-
usually takes two to three months. The toxin requires 24-72 w EMG guidance
hours to take effect, reflecting the time necessary to disrupt w Direct visualisation of the muscle by making a snip incision
the synaptosomal process. In very rare circumstances, some
individuals may require as many as five days for the full effect to on the conjunctiva overlying the muscle
be observed. Peaking at about 10 days, the effect of botulinum
toxin lasts for nearly 8-12 weeks. EMG guided Botulinum toxin injection-
1. Informed consent- mention about over/undercorrection,
INDICATIONS3,4,5,6
spatial disorientation, possible diplopia, repeat injections
1. Paralytic strabismus- 2. Equipment needed-
a. 6th nerve palsy a. Amplifier
b. 4th nerve palsy b. Needle with intact tip and good coating of teflon
3. Anaesthesia-
80 DOS Times - July-August 2017 a. Topical with proparacaine in a minimum of a drop

a minute for 3 doses + vasoconstrictor drop (E.g.
Epinephrine 1%) to blanch the conjunctival blood

Surendran et al. Botulinum for Strabismus

Techniques

Dosage of botulinum2 when the injected muscle itself receives a
strongly weakening effect.
Horizontal strabismus 2.5 units
Under 25 PD 2.5-5 units Injection not into the muscle.
Over 25 PD Disorientation of the patient. Past-
pointing and spatial disorientation occur
MR injection for LR palsy 1.0-2.0 units with paralysis of a fixing eye. When this
Early 2.5 units is a problem, that eye should be covered.
Later in conjunction with Adaptation to the paralysis typically
Surgery 2.5 units occurs after the third or fourth day.
Later for partially or fully Diplopia. Frequently an eye is turned
Healed palsy with mr by the injection temporarily to a position
Contracture where no suppression occurs. Occlusion
of the eye is Indicated.
Vertical muscles 5.0 units
Inferior rectus (IR) for thyroid 2.5 units COMPLICATIONS NOT
IR for concomitant deviation 2.0 units ENCOUNTERED
Superior rectus 2.5 units
Inferior Oblique 1. Penetration of the sclera. With
visual guidance of the needle tip
Children with infantile esotropia or exotropia (bilateral injections) 2.5 units sub-conjunctivally and exterior to
the muscle to a position at least
Weak muscles- myasthenia, external ophthalmoplegia, aberrant 1.0-2.0 units posterior to the equator, and EMG
degeneration, cerebral palsy guidance of the needle thereafter,
this complication should never occur.
Retrobulbar injection for nystagmus 25.0 units
2. Serious retrobulbar hemorrhage.
Courtesy - Clinical Strabismus Management, principles and surgical techniques, Arthue Since the needle should be exterior
L Rosenbaum, Alvina Pauline Santiago, Chapter 32 to the muscle cone or within the
muscle, and never within the muscle
vessels subconjunctival While listening to the EMG signals, cone, this should never occur.
advance the needle tip towards the area
injection of 0.1-0.4 ml of giving off the loudest sound. When a References
crackling sharp EMG signal is heard,
lidocaine inject the fluid slowly. It is a good sign 1. Chemodenervation of Extraocular
if the EMG sound diminishes with the Muscles – Botulinum Toxin. In: Gunter K.
b. Sedation- Diazepam or a similar injection, indicating that the solution von Noorden, Emilio C. Campos: Binocular
pushed the nearby muscle fibres away Vision and Ocular Motility: Theory and
drug given one hour prior in from the tip. Leave the tip there for 15-30 Management of Strabismus, Sixth Edition.
seconds until the pressure of the solution Ophthalmology Books & Manuals (Cyber
apprehensive patients diminishes. Withdraw the needle slowly Sight); pages 559–565.
while the patient maintains primary gaze.
c. Short GA for children. Make a note of how reliable the EMG 2. Clinical strabismus Management,
response and injection were, for future principles and surgical techniques, Arthue
4. Procedure- reference. L Rosenbaum, Alvina Pauline Santiago,
Chapter 32, Page 423.
Placement in the muscle is crucial, In our experience dosage can also
be calculated depending on the angle of 3. Scott AB. Botulinum toxin injection of eye
centred halfway back in the muscle. deviation - For example 2 units of Botox muscles to correct strabismus. Trans Am
for 20 PD of deviation, 3 units for 30 PD of Ophthalmol Soc. 1981;79:734-70
Aspirate the volume of the proposed deviation and so on.
4. Jones A, Jain S. Botulinum toxin: a novel
dose plus an additional 0.1 to 0.2 ml into COMPLICATIONS treatment for pediatric cyclic esotropia. J
AAPOS. 2014;18:614-5
the syringe. Then the injection electrode Corneal irritation
is firmly attached and the excess is 5. Merino P, Rojas P, Gómez de Liaño P,
Sub-conjunctival hemorrhage from Franco Iglesias G. Spasm of the near
injected through the needle electrode to needle insertion. reflex. Treatment with botulinum toxin.
Arch Soc Esp Oftalmol. 2015;90:244-6.
check for patency and spot a leak at the Discomfort at time of injection.
Prolonged discomfort lasting a few 6. Flores-Reyes EM, Castillo-López MG,
syringe / needle junction. hours. This is usually associated with a Toledo-Silva R, Vargas-Ortega J, Murillo-
needle touching the orbital periosteum Correa CE, Aguilar-Ruiz A. Botulinum
Attach a ground lead to the patient or encountering scar tissue from a toxin type A as treatment of partially
prior operation. There is no really good accommodative esotropia. Arch Soc Esp
at the forehead for the medial rectus treatment except avoidance and analgesic Oftalmol. 2016;91:120-4.
medication.
(MR) and laterally for the Lateral rectus Effect of the drug on adjacent Correspondence to:
muscles. The levator appears to be Dr. T.S.Surendran,
(LR). Wipe this area beforehand with especially susceptible. These effects on Sankara Nethralaya,
adjacent muscles typically occur only Chennai, Tamil Nadu, India.
alcohol to ensure adhesion of the usual

electrocardiographic leads.
Turn on the amplifier to half the

volume and test the connections by

touching the needle tip to the conjunctiva:

it should make a loud “tick”.

Have the patient gaze at a target away
from the field of action of the muscle.

Insert the needle electrode, its

bevel facing the muscle, through the

conjunctiva 8-10 mm from the limbus

and, avoiding the large vessels, push it

5-6 mm posteriorly, keeping to the orbital

wall side of the muscle, away from the

globe. Slowly move the gaze target to the

primary position to activate the target

muscle.

Surendran et al. Botulinum for Strabismus www. dos-times.org 81

Techniques

Hang-Back Muscle Recession

Kamlesh MS, Yashpal Goel MS, Atul Kumar Sahu MS
Guru Nanak Eye Center, Maulana Azad Medical College, New Delhi, India.

Abstract: The goal of the strabismus surgery is to achieve both motor alignment and sensory improvement with minimum
number of procedures. Weakening procedure on the recti constitute an important surgical modality for correcting strabismus.
Although many surgical techniques are available for weakening of recti muscle, the need for a safe, easy and effective surgical
procedure led surgeons to experiment with synthetic absorbable sutures. These high tensile strength absorbable sutures form
the basis of suspension recession techniques. Hang back muscle recession technique and its various modifications are now
being extensively used because of the multiple advantages that they offer along with increased safety and a lower complication
rate

Earlier myotomies were performed to weaken the Figure 1: This figure compares the muscle position in (i) conventional
extraocular muscles. These efforts were generally recession, (ii) Hang-back muscle recession and (iii) Anchored hangback
unsuccessful and surgeons were frustrated by the muscle recession. (Courtesy: Macleod et al.).
random retraction and reattachment of the cut
muscles to the globe. In search of more predictable Synthetic absorbable sutures such as vicryl are not completely
procedures, Jameson1 in 1922 introduced the
technique on which all conventional recessions are based. The absorbed until 90 days after intramuscular implantation. Firm
conventional recession technique involved direct suturing of
the muscle to the sclera posterior to their insertion at the site muscle scleral union occurs within 1-2 months after surgery.
of recession. This procedure allowed accurate grading of the
surgery permitting a definite understanding of the location of Advantages of hangback muscle recession are as follows:
reattachment of the recessed muscle. However, this technique • Reduced risk of scleral perforation.
alone neither guarantees precise muscle reattachment nor • Easy intraoperative adjustment in case of over action
perfect ocular alignment. Below are the problems associated
with conventional muscle recession surgery: (spring-back balance test).
a) Inadvertent perforation of sclera resulting in retinal • Convenient site for scleral suturing.
detachment, endophthalmitis or phthisis bulbi. • Less chance of snaring tenon’s capsule.
b) No provision for customization. • Simplified technique - good for residents and general
c) Problematic in large recession, in high myopes, and infants.
d) More chances of tenons capsule snaring in large recessions. ophthalmologists.
• Avoid excessive manipulations of the eye.
Technique • Integral part of Adjustable suture.

Gobin in 1960s first used the term, loop recession2. He Hang-back muscle recession also formed an integral part of
used two 5-0 silk sutures attached to the ends of the muscle
when recessing the medial rectus muscle more than 5 mm. In adjustable suturing technique, a procedure that was introduced
this procedure, the detached muscle was sutured to the sclera by Jampolsky5 in 1970’s. Adjustable suture modification
at two points separated by a distance equal to the width of the to traditional surgery was introduced to improve surgical
original insertion and located 5 mm behind it. The loose ends of
the suture ends were tied over a 2-mm diameter probe and the outcome and reduce frequency of reoperations by eliminating
muscle was allowed to recess another 5 mm for a total recession undesirable early post-operative under or over-corrections6-10.
of 10 mm. This technique of loop recession underwent many
modifications and in the early 1970’s with the advent of high The hemi hang-back muscle recession was described
tensile strength synthetic absorbable sutures, the suspension by Potter et al in 199211. The technique is derived from three
recession or hangback techniques were introduced (Figure 1).
Mills and coworkers3 conducted the first study of nonadjustable procedures mentioned in the literature: conventional scleral
hang-back recession on patients with either horizontal or
vertical strabismus. Although not controlled, the study found suturing, hang-back muscle recession and loop suspension
that 55 of 62 (89%) patients had a successful outcome, which
was defined as any alignment that did not require reoperation. recession technique. In the hemi-hang-back muscle recession

This procedure was advocated as a safe, easy and effective technique, the muscle is reattached and suspended from
alternative to conventional muscle recession technique4.
Success here depends upon attachment of the cut muscle to the the sclera posterior to the original insertion. This procedure
sclera before complete hydrolysis of the suture material occurs.
is illustrated in Figure 2. After disinserting the muscle, it is

82 DOS Times - July-August 2017 Kamlesh et al. Hangback Recession

Techniques

suspended, with the help of sutures, from Figure 2: Hemi Hang-Back Recession Technique.
a point midway between the original
insertion and desired muscle insertion Figure 3: Anchored Hang back Recession Technique.
site. This technique is recommended
for management of difficult strabismus at the desired site of muscle recession ‘Modified Anchored Hang-back muscle
cases as in paralytic strabismus; large and the muscle is suspended from the recession’ technique. In this technique,
deviations or following maximal original muscle insertion using the same after disinserting the muscle, a superficial
recessions with residual large deviations sutures (Figure 3K-O). The conjunctival scleral anchoring bite is placed midway
and in cases with good vision in one flap is then sutured back (Figure 3P). between the intended recession site and
eye when the deviating eye has already the original insertion (Figure 4: A-I). This
undergone maximal surgery. Although Although anchored hang-back procedure on one hand maintains the
there are many advantages of hangback muscle recession appears to overcome width of the muscle preventing central
recession, there are some possible most of the disadvantages of the hangback bowing and on the other hand allows for
disadvantages also: muscle recession, it lost the major intra and postoperative customization.
a) Central posterior muscle bowing advantage of customization in terms of
b) Late over-corrections may occur if post or intraoperative adjustment offered New Developments
by the hang-back recession.
muscle reattachment lags suture Over the last decade hang- back
hydrolysis and the muscle slips In order to integrate the advantage muscle recession has undoubtedly
posteriorly12-14. of both techniques; Kamlesh et al16 proven to be a safe, easy and effective
c) Possibility of a sideslip of the sutures in 2002 described a new procedure
up or down over the surface of the
globe with vertical movement of the
eye, producing temporary upshoots
or downshoots
d) Extraocular muscle might attach
to the globe above or below the
horizontal meridian, thereby
producing an A or V pattern
strabismus or possibly permanent
upshoots or downshoots
To tackle these disadvantages,
Macleod et al15 described a modified
suspension recession technique - an
anchored hang-back muscle recession
technique. As shown in the Figure 1, the
central posterior bowing noted with
hangback recession led to enhanced
recession making the results less
predictable. In addition, the muscle
cannot be displaced accurately upwards
or downwards as may be required for
correction of A and V patterns or vertical
deviations. The tendency of the muscle to
bunch centrally leads to lack of control
over the final vertical position of the
muscle thus hindering the correction
of coexisting vertical deviation of A
and V pattern. Thus, anchored hang-
back muscle recession is designed to
overcome the problems of hang-back
muscle recession while maintaining the
ease and safety of the latter procedure.
This technique is illustrated step by step
in Figure 3. The muscle is exposed using
a paralimbal fornix based incision and
carefully hooked (Figure 3A-C). Locking
bites are taken to secure the muscle
with 6-0 vicryl suture (Figure 3D-G). The
muscle is then severed from the original
insertion and markings taken for the site
of desired insertion site (Figure 3H-J).
Then a superficial anchoring bite is taken

Kamlesh et al. Hangback Recession www. dos-times.org 83

Techniques

Figure 4: Modified Hand back Recession Technique 8. Pratt-Johnson JA. Adjustable suture
strabismus surgery: a review of 255
surgery in both routine as well as complications were encountered. This consecutive cases. Can J Ophthalmol.
complicated strabismus procedures is the only study, which has evaluated 1985;20:105-109.
for both horizontal and vertical recti the outcome of suspension recession
muscles. Modified anchored hang-back technique on the inferior oblique muscle. 9. Franklin SR, Hiatt RL. Adjustable suture
surgery has added a new armamentarium in strabismus surgery. Ann Ophthalmol.
to the existing surgical techniques as a Conclusion 1989;21:285-89.
procedure overcoming the disadvantages
of hang-back surgery and at the same Hang-back muscle recession offers 10. Kushner BJ. Adjustable sutures
time offering an opportunity for intra as an exciting and challenging alternative to in strabismus surgery. J of Ocular
well as postoperative customization. conventional recession for the correction Therapeutic and Surgery. 1983;2:11-
of strabismus. 15.
Recently Awadein et al compared
non-absorbable versus absorbable REFERENCES 11. Potter WS, Nelson LB. Hangback and
sutures in hangback medial rectus muscle Hemi-hangback recession techniques
resection17. They concluded that using 1. Jameson PC. Correction of squint by in strabismus surgery. Ophthalmology
nonabsorbable suture for hang-back muscle recession with scleral suturing. Clinics of North America 1922; 5:47-
medial rectus recessions; greatly reduces Arch Ophthalmol 1922; 51:421-432. 56.
the incidence of over-corrections that can
occur when absorbable suture dissolves. 2. Gobin MH. Recession of medial rectus 12. Weakley DR, Stager DR, Everett ME.
muscle with a loop. Ophthalmologica Seven-millimeter bilateral medial
Hang back recession was carried out 1968; 156: 25-27. rectus recessions in infantile esotropia.
on the inferior oblique muscle for the first J Pediatr Ophthalmol Strabismus.
time in the history of strabismus surgery 3. Mills PV, Hyper TJ, Duff GR. Loop 1991;28:113-15.
by Kamlesh et al18. They evaluated recession of the recti muscles. Eye.
15 patients of V pattern strabismus 1987; 1:593-96. 13. Rosenbaum AL. The use of adjustable
with inferior oblique overaction and suture procedures in strabismus
performed 10 mm Hangback recession 4. Repka MX, Guyton DL. Comparison of surgery. Am Orthopt J. 1978;28:88-94.
of the inferior oblique muscle. In this hang-back medial rectus recession with
study, a mean V pattern correction of conventional recession. Ophthalmology 14. Wise J, Flanders M, Williams F.
20.2 prism diopters was obtained. In 1988;95:782-87. Adjustable sutures in strabismus
85.37% patients, the V pattern was found surgery. Can J Ophthalmol.
to be fully corrected. The mean inferior 5. Jamplosky A. Current techniques of 1982;17:157-60.
oblique overaction correction was found adjustable strabismus surgery. Am J
to be 19.04 prism dioptres. No significant Ophthalmol 1979;88406-18. 15. MacLeod JD, Rhatigan MC, Luff AJ,
Morris RJ. Bimedial rectus recession
6. Hiatt RL, Ivens MY. Adjustable sutures using the anchored hangback
in strabismus. J of Ocular Therapeutics technique. Ophthalmic Surg. 1997; 28:
and Surgery 1985;4:147-51. 343-46.

7. Fells P. Techniques and applications 16. Kamlesh, Dadeya S Bhola R. A modified
of adjustable sutures. Aust N Z J hang-back recession technique for
Ophthalmol. 1987;15:35-41. horizontal strabismus. J of Pediatr
Ophthalmol Strabismus. 2002; 39:195.

17. Awadein A, Marsh JD, Guyton DL.
Nonabsorbable versus absorbable
sutures in large, hang-back medial
rectus muscle recessions. JAAPOS.
2016;20:206.

18. Kamlesh, Prasad HN, Monga S, Bhola R.
Hang-back recession of inferior oblique
muscle in V-pattern strabismus with
inferior oblique overaction. JAAPOS.
2008;12:401-4.

Correspondence to:
Dr. Kamlesh,
Director and Head of Ophthalmology,
Guru Nanak Eye Center,
Maulana Azad Medical College,
New Delhi, India.

84 DOS Times - July-August 2017 Kamlesh et al. Hangback Recession

SNAPSHOT

One and a Half Syndrome

Deepali Garg Mathur MS

Squint and paediatric ophthalmology, Max Eye Care, Delhi, India.

Summary: The one and a half syndrome is a rare clinical disorder of extraocular movements characterized by a conjugate
horizontal gaze palsy of one side with an internuclear ophthalmoplegia on the other side. It occurs due to a single unilateral
lesion of the dorsal pontine tegmentum involving the ipsilateral paramedian pontine reticular formation or the abducens
nucleus with interruption of ipsilateral median longitudinal fasciculus causing failure of adduction of ipsilateral eye. The
main causes are demyelination and stroke. Uncommon causes include tumors, av malformations, basilar artery aneurysms,
vasculitis, brainstem tuberculoma and neurocysticercosis.

Case Figure 1: Ocular movements showing left exotropia with loss of other
horizontal movements and preserved vertical eye movements.
A 25-year-old female was referred from the inpatient
neurology department for an ophthalmic evaluation. She Figure 2: MRI brain with T2 weighted imaging showing hypo intense
complained of an inability to move the eyes for the past 2 weeks lesion in dorsal pons indicative of a haemorrhage.
and vertigo on looking to the left. On examination, the patient
was conscious, cooperative, responding to verbal commands. of the paramedian pontine reticular formation or the abducens
She had a naso gastric tube in situ due to associated dysphagia. nucleus on one side (causing the conjugate gaze palsy), with
Bedside visual acuity was brisk finger counting at 6 meters in interruption of adjacent median longitudinal fasciculus1.
both eyes. Pupils were normal in size and reacting normally Reported causes of this syndrome include demyelination as in
to light. Ocular motility examination revealed a left exotropia multiple sclerosis2, stroke, tumors, av malformations, basilar
in primary position. There was a total loss of horizontal artery aneurysm and rarely vasculitis, and neurocysticercosis.
movements in the right eye with loss of adduction in left eye Brainstem tuberculoma as a cause of one and a half syndrome
(Figure 1). On attempted right gaze, there was widening of has been reported only twice with complete resolution of
palpebral fissure in both eyes and she was able to get the left eye symptoms following antitubercular treatment3.
just short of midline. The only preserved horizontal movement
was abduction in left eye accompanied by abducting nystagmus. Pontine hemorrhages represent only 5% of intracranial
The loss of movements was not overcome by Vestibulo ocular haemorrhage4. The prognosis and clinical picture and recovery
reflex. Convergence was normal. Vertical eye movements were largely depend on the volume of insult. While the massive
within normal limits. Anterior segment and fundus were within variety produces the classic picture of coma, quadriplegia,
normal limits. decerebrate posturing and eventual demise, the hemipontine
syndromes have preserved consciousness with gaze paresis
INVESTIGATIONS

MRI brain revealed a hypo intense lesion in the dorsal pons
on the right side in T2 weighted images indicative of a right
sided pontine haemorrhage (Figure 2).

INTERVENTION

The patient was under follow up for 4 weeks under
Neurology Department. Dysphagia improved completely
though there was little recovery of ocular movements. The
patient was diplopia free in primary position.

Comment

Supranuclear horizontal eye movements are mainly
controlled in the pons. The final common pathway is the
abducens nucleus and paramedian pontine reticular formation.
The combination of ipsilateral horizontal gaze palsy and
internuclear ophthalmoplegia (INO) is known as one and a
half syndrome. This peculiar syndrome was first described by
Freeman et al and later dubbed as one and a half syndrome by
Fisher.

The syndrome is usually due to a single unilateral lesion

Mathur DG. One and a Half Syndrome www. dos-times.org 85

SNAPSHOT

and motor sparing. Nuclear magnetic References 4. Kushner J. Michael, Bressman Susan.
resonance imaging allows a definite Clinical manifestations of pontine
diagnosis during the acute phase without 1. Wall M, Wray SH. The one and a half haemorrhage. Neurology. May 1985 Vol
recourse to invasive investigations. syndrome: a unilateral disorder of 35 No.5, 637.
pontine tegmentum: a study of 20 cases
In primary position, the eyes and review of literature. Neurology Correspondence to:
may be orthotropic or there may be an 1983; 33:971-980. Dr. Deepali Garg Mathur,
exotropia of the contralateral eye also Senior Consultant, Squint and Paediatric
called paralytic pontine exotropia. There 2. Martyn CN, Kean D. The one and a half Ophthalmology,
may be associated dissociated abduction syndrome. Clinical correlation with Max Multispeciality Hospital, New Delhi, India.
nystagmus and skew deviation. Recovery a pontine lesion demonstrated by
of eye movement abnormalities occurs nuclear magnetic resonance imaging
with multiple sclerosis and infarction in a case of multiple sclerosis. Br J
though it may be incomplete and variable. Ophthalmol 1988: 72: 515-517.

3. Menon V, Gogi M, Saxena R, Singh
S, Kumar A. Isolated one and a half
syndrome with brainstem tuberculoma.
Indian j paedtr. 2004; 71: 469-471.

86 DOS Times - July-August 2017 Mathur DG. One and a Half Syndrome

SNAPSHOT

Myopic Strabismus Fixus

Shailja Tibrewal MS, Regina Lalramhluni MS, Rolli Khurana MS, Suma Ganesh MS
Dr. Shroff Charity Eye Hospital, New Delhi, India.

Abstract: Myopic strabismus convergens is a rare restrictive strabismus characterized by large esotropia and limitation of
abduction and elevation. It is described as both congenital and acquired forms. This report presents a case of acquired myopic
strabismus convergens occurring in a 35-year-old high myope. Various theories have been proposed regarding pathogenesis
of this entity in high myopia. Recent MRI studies have helped in understanding the disease process and led to the development
of newer surgical modalities. Careful preoperative evaluation and MRI of the orbit ensures correct diagnosis and choice of
appropriate surgical procedure.

A35-year-old male presented to the strabismus Figure 2: Nine gaze clinical photograph of the patient showing
clinic of Dr. Shroff Charity eye hospital in May extremely large angle esotropia and left hypotropia with limitation of
2017 with complaints of inward deviation of movement (left>right).
left eye since childhood (Figure 1). He had
been using high myopic glasses for the past 30 Magnetic resonance imaging of the orbits revealed
years. The deviation was progressing with age bilateral posterior staphyloma with elongation of the globes in
and had increased after he underwent cataract surgery with supero-temporal direction. As a result, the lateral rectus was
intraocular lens implantation in his right eye two years back. He seen plastered to the lateral orbital wall (Figure 3). Coronal
also had a history of cataract surgery without intraocular lens
implantation of left eye one year later after right eye cataract
surgery. He is a known asthmatic since the past 5-6 years.

His uncorrected visual acuity in right eye was 20/40 and in
left eye was finger counting at 1 meter with eccentric viewing
due to inability to move the left eye outwards up to midline. His
best corrected vision in right eye was 20/30 with a refraction of
+1.00DC @ 180 degree. Refraction in left eye was not possible
due to extreme position of the globe. Slit lamp evaluation of the
anterior segment revealed pseudophakia and aphakia in the
right and left eye respectively. Fundus examination of both eyes
showed posterior staphyloma, chorio-retinal atrophic patches
in the posterior pole and peripapillary area and mild pallor of
the optic disc. The axial length in right eye was 35.72 mm and
left eye was 36.06 mm.

Orthoptic examination revealed bilateral convergent
strabismus fixus. The patient maintained a small right face
turn while reading the distance chart. The left eye was almost
immobile with negligible movement in abduction, elevation
and depression. The right eye showed limitation of elevation
(-3) and abduction (-2). The strabismus was estimated by
Hirschberg reflex to be around 15 degrees esotropia in his right
eye and >70 degree esotropia and 10 degree hypotropia in his
left eye (Figure 2).

Figure 1: Primary gaze photograph showing inward deviation of both Figure 3: Axial scan through mid-orbit showing posterior staphyloma
eyes, the left eye being in an extreme position. and superotemporal elongation of the globes (asterisk). The lateral
Tibrewal et al. Myopic Strabismus Fixus Convergens rectus is plastered to the lateral orbital wall (bold arrow).

www. dos-times.org 87

SNAPSHOT

sections of the MRI showed a polyester suture. Suture loop

that the superior rectus (SR) myopexy poses the potential

muscle path was deviated nasally complications of muscle cheese

and lateral rectus (LR) muscle wiring and/or strangulation

path was deviated inferiorly in of the anterior ciliary vessels.

both eyes (Figure 4). Therefore, other materials

Based on history, clinical have been used to prevent
findings, axial length and MRI the above. Wong et al did loop

reports, a diagnosis of myopic myopexy of lateral rectus and
superior rectus using a silicone
strabismus fixus convergens 240 band and tightened it with
was made. Bilateral modified

Loop myopexy of LR and SR a sleeve. Shih et al used Gore-

with medial rectus recession is Figure 4: Coronal scan through the orbit showing inferior displacement Tex sling to unite the LR-SR
planned for the correction of of lateral rectus (bold arrow) muscle path and nasal displacement of bellies. Shenoy et al modified
deviation. A discussion of the superior rectus (asterisk) and inferior rectus muscle paths.
loop myopexy by Wong by

various pathological mechanism passing the 240-silicone band

and treatment modalities for myopic lateral rectus and superior rectus muscle through a scleral tunnel. This additional

strabismus fixus follows below. as a result of differential elongation of step secures the silicone band to the

COMMENT the globe in the supero-temporal region. sclera, thereby preventing its anterior
Herzau and Loannakis were the first to migration.

Myopic strabismus fixus (MSF) observe an abnormal path of the LR in Loop myopexy procedure alone
convergens is a rare type of acquired MSF intra-operatively in 199611. This can correct up to 40 PD of esotropia. A
was later demonstrated in magnetic combination of loop myopexy procedure
strabismus seen in high myopes wherein resonance scans of the orbit of patients and recession of the MR muscle is effective
one or both eyes are fixed in extreme with myopic strabismus fixus by Krzizok in correcting larger deviations. A medial
adducting position1. In the more common et al also12. rectus recession is advisable especially if
it is found to be tight on forced duction
form, however the patient presents Based on the above theory, Krzizok testing. Careful handling of the medial
et al. treated MSF by performing a large rectus muscle is suggested as it may be
with esotropia and hypotropia of the recession of medial rectus and fixing extremely tight leading to pulled in two
the lateral rectus with posterior fixation syndrome while recessing it. Also the
affected eye with limitation of elevation suture in physiological meridian with passage of sutures through the extremely
and abduction2,3. Congenital type of non-absorbable suture after resection13. thin sclera and construction of the scleral
strabismus fixus is thought to be caused However, certain patients with MSF also tunnel in high myopes can be extremely
due to congenital fibrosis of extraocular displayed restriction of elevation and challenging and should be dealt with
muscles and subsequent loss of their consequent hypotropia. It was Yokoyama utmost care to avoid perforation.
elasticity. Villasecca4 and Martinez5 were et al who in 2001 demonstrated the
the first to describe strabismus fixus as an nasal-ward shift in path of SR along For the present case, the plan of
acquired condition due to fibrosis of the with inferior shift in path of LR in high bilateral modified loop myopexy by
medial rectus as a consequence of lateral myopia. Several other studies thereafter scleral fixation along with bimedial rectus
found similar pathology and supported recession has been made since the angle
rectus paralysis. Yokoyama’s theory14. of deviation is extremely large and the
LR and SR muscle paths are deviated
Thereafter several theories Surgical modalities for treatment significantly on both sides. To conclude,
of MSF can be grossly divided into two myopic strabismus fixus is a fairly
have been proposed regarding the groups. Firstly, those procedures that complex strabismus entity. MRI of the
alter the muscle forces which include the orbit forms an important part of diagnosis
pathogenesis of this type of motility classical recess-resect procedure15-17. The and further plan of management. Surgical
second group consists of procedures that procedure should be decided based on the
derangement (a) Bagolini et al suggested aim to correct the deviated muscle paths amount of deviations, grade of limitation
by either muscle belly union,18-23 myopexy of movement and anatomical factors.
myopathic paralysis of lateral rectus of LR at equator or transposition surgery24. Recent advances in the understanding
The choice of the procedure is guided by of the pathophysiology of the condition
due to compression against orbital wall the amount of limitation of abduction have led to development of successful
and elevation, angle of esodeviation and treatment modalities.
and enlarged globe (b) Hugonnier and the amount of deviation of LR and SR
Magnard6 proposed a theory of myositis path deviation based on MRI evaluations. REFERENCES
In general, belly union or transposition
of the lateral rectus muscle (c) Aydin procedures are preferred when the 1. Bagolini B, Tamburrelli C, Dickmann
et al7 proposed the long globe theory limitation of movement is so severe that A, et al. Convergent strabismus fixus in
the eye fails to cross midline. Yokoyama high myopic patients. Doc Ophthalmol
wherein there is mechanical limitation et al were the first to perform a full loop 1990;74:309–20.
myopexy of lateral rectus and superior
especially to abduction due to the rectus 15 mm behind the insertions using 2. Krzizok TH, Kaufmann H, Traupe H.
large globe filling the entire orbit and New approach in strabismus surgery
subsequently long standing esotropia in high myopia. Br J Ophthalmol

causing foreshortening of the medial

rectus muscle. Additionally, certain case
reports of strabismus convergence fixus
occurring in cases with amyloidosis of
lateral rectus muscle8 and mitochondrial
myopathy9 and non-myopic subjects10

have led to proposition of alternate

mechanisms.

Recently, the more widely accepted
theory for strabismus fixus in high myopia
is the displacement of the paths of the

88 DOS Times - July-August 2017 Tibrewal et al. Myopic Strabismus Fixus Convergens

SNAPSHOT

1997;81:625–30. high myopia by magnetic resonance strabismus fixus. J AAPOS 9:589–591.
imaging. Arch Ophthalmol 1997; 115: 20. Yamaguchi M, Yokoyama T, Shiraki K.
3. Yokoyama T, Tabuchi H, Ataka S, et al. 1019-27.
13. Krzizok TH, Kaufmann H, Traupe H. Surgical procedure for correcting globe
The mechanism of development in New approach in strabismus surgery in dislocation in highly myopic strabismus.
high myopia. Br J Ophthalmol 1997; 81: Am J Ophthalmol 2010;149:341–6.
progressive esotropia with high myopia. 625-30. 21. Leo SW, Del Monte MA. Surgical
14. Aoki Y, Nishida Y, Hayashi O, et correction of myopic strabismus fixus
In: de Faber J-T, ed. Transactions: 26th al. Magnetic resonance imaging by modified loop transposition with
measurements of extraocular muscle scleral myopexy. J AAPOS 2007;11:95.
Meeting, European Strabismological path shift and posterior eyeball 22. Shih MH, Li ML, Huang FC. A
prolapse from the muscle cone in preequatorial Gore-Tex sling to restore
Association, Barcelona, Spain, 2000. acquired esotropia with high myopia. rectus muscle pathways in myopic
Am J Ophthalmol 2003;136:482–9. strabismus fixus. J AAPOS 2012;16:80–
Lisse (Netherlands): Swets & Zeitlinger, 15. Mohan K, Sharma A, Gupta R, Gupta 2.
A. Treatment of strabismus fixus 23. Shenoy BH, Sachdeva V, Kekunnaya
2001:218–22. convergens. J Pediatr Ophthalmol R. Silicone band loop myopexy in
4. Villasecca A: Strabismus fixus. Am J Strabismus 1999; 36:94–97. the treatment of myopic strabismus
16. Bagheri A, Adhami F, Repka MX. fixus: surgical outcome of a novel
Ophthalmol 1959;48:51. Bilateral recessionresection surgery for modification. Br J Ophthalmol.
5. Martinez L: A case of fixed strabismus. convergent strabismus fixus associated 2015;99:36-40.
with high myopia. Strabismus 24. Larsen PC, Gole GA. Partial Jensen’s
Am J Ophthalmol 1948;31:80. 2001;9(4):225–230. procedure for the treatment of myopic
17. Hayashi T, Iwashige H, Maruo T. Clinical strabismus fixus. J AAPOS 2004;8:393–
6. Hugonnier R, Magnard P. Les features and surgery for acquired 395.
progressive esotropia associated with
déséquilibres oculomoteurs observé en severe myopia. Acta Ophthalmol Scand Correspondence to:
1999;77:66–71. Dr. Shailja Tibrewal,
cas de myopie forte. Ann Oculist (Paris) 18. Yokoyama T, Ataka S, Tabuchi H, Shiraki Consultant, Pediatric Ophthalmology and
K, Mihi T. Treatment of progressive Strabismus Services,
1969;202:713–24. esotropia caused by high myopia—a Dr Shroff Charity Eye Hospital,
new surgical procedure based on its Daryaganj, New Delhi, India.
7. Aydin P, Kansu T, Sanac AS. High myopia pathogenesis. In: de Faber J-T (ed)
causing bilateral abduction deficiency. J Transactions: 27th Meeting, European
Clin Neuro Ophthalmol 1992;12: 163– Strabismological Association, Florence,
Italy, 2001. Swets & Zeitlinger; Lisse
165. (Netherlands),2002. pp 145–148.
19. Wong I, Seo-Wei L, Boo-Kian K (2005)
8. Sharma P, Gupta NK, Arora R, Prakash P. Loop myopexy for treatment of myopic
Strabismus fixus convergens secondary
to amyloidosis. J Pediatr Ophthalmol

Strabismus. 1991;28:236-7.

9. Venkatesh CP, Gayathri N, Murthy
KR. Myopic strabismus fixus: a
mitochondrial myopathy? Am J

Ophthalmol. 2003;135:720-2.

10. Mendonca TM, Tibrewal S, Sachdeva V,

Kekunnaya R. Convergent strabismus
fixus without high myopia. J AAPOS.
2016;20:83-5.

11. Herzau V, Loannakis K. Zur Pathogenese

der Konvergenz und Hypotropie

bei hoher Myopie. Klin Monatsbl

Augenheilkd 1996;208:33–6.

12. Krzizok TH, Kaufmann H, Traupe H.

Elucidation of restrictive motility in

Tibrewal et al. Myopic Strabismus Fixus Convergens www. dos-times.org 89

Photo Essay

An Interesting case of Head Tilt

Elizabeth Joseph K, Joseph John
Little Flower Hospital, Angamaly, Kerala, India.

Summary: We report the case of a two year old child who had 3 episodes of acquired Brown’s syndrome. He had abnormal
head posture in the form of face turn to left and chin elevation. He had left hypertropia, restriction of levoelevation of right
eye. Imaging studies revealed bulky right superior oblique tendon and sinusitis. ANA, RA factor were negative. We treated with
systemic and periorbital steroids as well as systemic antibiotics to which the child responded remarkably well.

Acquired Brown’s syndrome is not so uncommon.
Abrams MS has reported progressive Brown’s
syndrome in a two-year-old girl1. Depras A et al
diagnosed a case of idiopathic acquired Brown’s
syndrome in a ten-year-old boy2. Rao VB et al
has reported acquired Brown’s syndrome in 6
patients who had cysticercosis3. Kraft SP et al. has described
acquired Brown’s syndrome in six patients aged between 2-8
years4. Acquired Brown’s syndrome is also described in a nine-
year-old boy with post-streptococcal reactive arthritis5 and in a
child with juvenile idiopathic arthritis6.

Case report Figure 3

A 2-year-old child presented to us with abnormal head
posture (Figure 1) of three days duration. He had fever and
upper respiratory tract infection 1 week prior to this episode.

On examination, visual acuity was 6/7.5 both eyes with
Cardiff chart. He had a face turn to left with chin elevation.
Corneal reflex test showed left hypertropia of 10 degrees
(Figure 2). We noticed -4 limitation of laevoelevation of right
eye (Figure 3). Lang test showed stereoacuity of 550 seconds of
arc. Krimsky test showed 20 prism diopters of left hypertropia.

Figure 1

Figure 2 Figure 4
90 DOS Times - July-August 2017
Joseph KE et al. Unusual case of Head Tilt

Photo Essay

Figure 6

Figure 5 Figure 7

The left hypertropia was more on hydrocortisone for 3 days and crystalline recovered completely.
laevoversion and on head tilt to left. penicillin for 5 days. He did not show 1 year later, he again came back
any improvement after 3 days. We gave
Pupil was 3mm round, direct and triamcinolone injection 1ml in the right with the same head posture (Figure
consensual reflexes were brisk in both paratrochlear area. 2 weeks later the child 8), left hypertropia, -4 limitation of
eyes. There was fundus incyclotorsion showed remarkable improvement with laevoelevation of right eye (Figure 9).
in right eye, but was otherwise normal. no limitation of laevoelevation of right Stereoscopic acuity was 550 seconds of
Forced duction test was positive eye (Figure 6). On follow up at 4 weeks, arc with Lang’s test.
indicating restrictive strabismus. Family the results were maintained (Figure 7).
album scan showed normal head posture, ESR was 15mm/ 1st hour, ANA and
orthophoria and full ocular movements in A year later, he came back with RA factor negative.
the past (Figure 4). face turn to left and chin elevation. He
had left hypertropia of 10 degrees and MRI at this stage showed bulky right
Hematology shows relative -4 limitation of laevoelevation of right superior oblique tendon (Figure 10).
lymphocytosis, normal peripheral eye. Prism cover test showed 16 prism
smear. Erythrocyte sedimentation rate dioptres left hypertropia. Investigations We treated with paratrochlear
(ESR) is 25mm/ 1st hour. Antinuclear revealed normal peripheral smear and triamcinolone injection and systemic
antibody (ANA), Rheumatoid factor (RA) ESR 16mm/ 1st hour with negative RA antibiotics. 1 week later since there was
are negative. CT Brain and Orbits show factor negative. CT scan showed normal no improvement, we gave intravenous
bulky right superior oblique tendon, mild paranasal sinus and no evidence of hydrocortisone for 3 days. He responded
surrounding fat stranding in the region thickening of right superior oblique very well (Figures 11,12) and we tapered
of trochlea and right maxillary sinusitis tendon. We treated with intravenous the steroid. Two weeks later he had full
(Figure 5). hydrocortisone, crystalline penicillin recovery (Figures 13,14).
as well as triamcinolone injection in
We consulted with Pediatrician, the right paratrochlear area. The child Discussion
Pediatric rheumatologist, Neurologist, started to improve and 2 weeks later he
Otorhinolaryngologist and started Patients with acquired Brown’s
treatment with intravenous syndrome should be evaluated medically
for coexisting systemic disease. If a
disorder, such as rheumatoid arthritis

Joseph KE et al. Unusual case of Head Tilt www. dos-times.org 91

Photo Essay

Figure 8 Figure 9 Figure 10

Figure 12 Figure 13

Figure 11 Figure 14 JD, Budning AS, De Faber JT, Ellis GS
Jr, Pritchard C. Bilateral idiopathic
or sinusitis, is identified, that has to References Brown’s syndrome with delayed onset
be treated accordingly7. Once systemic in the second eye. J AAPOS. 2000
1. Abrams MS. A new mechanism Jun;4(3):158-63.
disease is excluded, patients who have for Brown’s syndrome. J Pediatr 5. Faust AO, Gillenwater JM, Saulsbury
Ophthalmol Strabismus. 2009 Mar- FT. Acquired Brown’s syndrome
acquired Brown syndrome with signs of Apr;46(2):115-7. in a child with poststreptococcal
inflammation can be treated with anti- reactive arthritis. J Rheumatol. 2001
inflammatory medication. Oral ibuprofen 2. Depras A, Chabrier S, Allard D, Gérard Dec;28(12):2748-9.
is a good first-line choice. Local steroid C, Lauras B. Radiological case of the 6. Akar S, Söylev M, Onen F, Ada E,
injections in the area of the trochlea month. Acquired idiopathic Brown’s Birlik M, Akkoc N. Acquired Brown’s
syndrome in a 10-year-old boy. Arch syndrome with juvenile idiopathic
and oral corticosteroids can be used for Pediatr. 2002 Jul;9(7):737-8. arthritis: resolution with early steroid
inflammation. Once the inflammatory treatment. Clin Exp Rheumatol. 2001
disease process is controlled, patients 3. Rao VB, Sahare P, Varada V. Acquired May-Jun;19(3):354.
with inflammatory Brown syndrome may brown syndrome secondary to superior 7. Kenneth WW and Edsel I. “Brown
show spontaneous resolution. oblique muscle cysticercosis. J AAPOS. Syndrome Treatment & Management:
2003 Feb;7(1):23-7. Medical Care, Surgical Care,
Consultations”. Emedicine.medscape.
4. Kraft SP, Nabi NU, Wilson ME, Roarty com. N.p., 2017. Web. 1 June 2017.

Correspondence to:
Dr. Elizabeth Joseph,
Chief, Pediatric Ophthalmology and Strabismus,
Little Flower Hospital,
Angamaly, Kerala, India.

92 DOS Times - July-August 2017 Joseph KE et al. Unusual case of Head Tilt

Photo Essay

Glioma Masquerading as Acquired Non-
Accommodative Comitant Esotropia in a Child

Neena R., A. Giridhar
Giridhar Eye Institute, Kochi, Kerala, India.

Summary: We report a case of CNS Glioma masquerading as Acquired Comitant Nonaccommodative Esotropia (ANAET) in a
9-year-old boy who was referred to us for squint surgery. He had visual acuity of 6/7.5, N6 OD and 6/6, N6 OS respectively.
Worth Four Dot Test showed alternate suppression. Stereopsis was nonrecordable. Pupils were normal in size and reaction.
Ocular movements appeared full OU. Alternate Prism Bar Cover Test showed 40 PD esotropia for distance and 20 PD esotropia
for near with either eye fixing separately. Cycloplegic retinoscopy was +1.00DS OU. Dilated fundus examination showed large
discs with pallor. Systemic examination did not reveal any focal neurological deficit. Optical Coherence Tomography (OCT)
showed thinning of temporal retinal nerve fibre layer (RNFL) both eyes. MRI Brain showed hyperintense lesions extending
from bilateral optic tracts to tectum of midbrain up to pons. MR Spectroscopy showed increased Choline with reduction in NAA
(N-Acetyl Aspartate) suggestive of glioma.

Acomitant esotropia, with no limitation of parents, esotropia was first noticed at the age of 4 years and since
ocular movements, is generally thought to be then he has been on occlusion therapy. He seemed to be a very
active, playful child with no systemic diseases. Visual acuity was
benign and does not warrant any neurological 6/7.5, N6 OD and 6/6, N6 OS respectively. Worth Four Dot Test
showed alternate suppression. Stereopsis was nonrecordable.
workup. However, there are reports of Pupils were normal in size and reaction. Ocular movements
appeared full OU (Figure 3). Hirschberg test showed 20-degree
intracranial lesions presenting with comitant right esotropia. Alternate Prism Bar Cover Test showed 40 PD
esotropia for distance and 20 PD esotropia for near measured
nonaccommodative esotropia without over either eye. There was no incomitance in any gaze. Ocular
neurological deficit in early stage. Sometimes acute-onset movements were normal. Cycloplegic Retinoscopy was +1.00DS
nonaccommodative esotropia may be the only presenting sign

of an intracranial neoplasm. A careful history and a meticulous
clinical evaluation will pick up the red flags in an atypical case
and prompt the need for neuroimaging to confirm the cause.

Case Report

A 9-year-old boy with Right esotropia was referred from
Middle East for squint surgery (Figure 1,2). According to the

Figure 1,2 Figure 3: Extraocular movements of the child

Neena R et al. Glioma Masquerading as Strabismus www. dos-times.org 93

Photo Essay

Undilated fundus showed large discs with Figure 4
temporal pallor both eyes.

Investigations: Family album
showed that child had straight eyes in
early childhood and the sudden onset
of squint at 4 yrs. of age (Figure 4). OCT
RNFL showed thinning in temporal
quadrants OU and inferior quadrant OD
(Figure 5).

MRI Brain and Orbits were done
which showed predominantly white
matter lesions involving bilateral optic
tracts, extending to tectum of midbrain
up to pons on left side, mild enhancement
of both thalami with subtle contrast
enhancement and no diffusion restriction
(Figure 6). There was mild hyperintensity
noted extending to right temporal white
matter lateral to temporal horn (Figure
7). MR Spectroscopy showed increased
choline with reduction in NAA (N-acetyl
aspartate) (Figure 8).

Diagnosis: CNS Glioma; possibly
Low grade.

Management: Patient was referred
to Paediatric Neurosurgeon and
Paediatric Oncologist; found to have
Neurofibromatosis-Type I. He was advised
to undergo biopsy and chemotherapy but
parents refused. He is currently on follow
up.

Discussion

Acquired nonaccommodative

esotropia (ANAET) is a less common

disorder of ocular alignment

characterized by a nonaccommodative

esodeviation which can occur in older

children, adults, and even the elderly1-3.

Onset can be acute and associated with

diplopia when it is called Acute acquired

comitant esotropia (AACE) or it can result

from deterioration of existing, previously

controlled, esotropia4-6. AACE is classically
is divided into five different subtypes.

The Swan type (type I) occurs after a

period of interrupted binocularity7. Type

II AACE, known as Burian-Franceschetti

has minimal hypermetropia and

diplopia that is often associated with

physical or psychological stress8. The

Bielschowsky type (type III) is associated Figure 5

with patients with myopia, convergence prompted us to image:
1) Sudden Onset of Esotropia at the age
spasm and divergence paralysis9-10. Type Many brain tumors such as cerebellar
of 4.
IV / Refractive-accommodative type is 2) Normal Alignment Prior to 4 Years astrocytomas, medulloblastomas,

characterized by high hypermetropia Noted in Family Pictures. pontine gliomas, astrocytoma of the
3) Large Distance Near Disparity
that can be adequately controlled with 4) Temporal Pallor OU (possibly due to corpus callosum (with hydrocephalus)

the refractive correction alone11. Type Va, involvement of optic tracts) and Arnold Chiari Malformation are
5) OCT Showing RNFL thinning
lesser common entity, is associated with associated with acute acquired comitant

intracranial pathology, most commonly a esotropia in childhood without any
neurological deficit15-20. Sometimes acute-
posterior fossa lesion12-16.

Red Flags in Our Case which onset nonaccommodative esotropia is

94 DOS Times - July-August 2017 Neena R et al. Glioma Masquerading as Strabismus

Photo Essay

Figure 6 Figure 7: Mild hyper-intensity noted extending to right temporal white matter
lateral to temporal horn
the only presenting sign of intracranial
neoplasm. Tumors involving the corpus Figure 8
callosum and posterior fossa, especially
gliomas give rise to no distinctive signs a child, it may prove costly. So, we feel prospective study. Eye. 1999; 13:617-
and are typically known to present with that neuroimaging should be done in
only acute-onset comitant esotropia any patient presenting with acquired 620.
with no other systemic signs such as comitant nonaccommodative esotropia,
papilledema. It is important in these especially when there is a high index of 5. Sturm V, Menke MN, Töteberg M,
cases of acquired esotropia to rule out the suspicion. Jaggi GP, Shoeffler C. Early onset of
possibility of intracranial tumor, Arnold
Chiari malformation or other neurological References acquired comitant non-accommodative
abnormalities.
1. Kothari M. Clinical characteristics esotropia in childhood. Klin Monbl
N-acetyl aspartate (NAA) is an of spontaneous late-onset comitant
indicator of neuronal density. Gliomas acute nonaccommodative esotropia Augenheilkd. 2012; 229:357-361.
have disruption of neuronal integrity with in children. Indian J Ophthalmol
axonal loss and thus reduced NAA. Choline 2007;55:117-20. 6. Burian HM, Miller JE, Comitant
is a compound involved in membrane
synthesis and degradation. Choline peak 2. Legmann Simon A, Borchert M. convergent strabismus with acute
correlates with cell density and increased Etiology and prognosis of acute, late-
choline is seen with increased membrane onset esotropia. Ophthalmology 1997; onset. Am J Ophthalmol. 1958; 25:55-
turnover. As grade of glioma increases, 104:1348–1352.
choline levels increase. 63.
3. Clark AC, Nelson LB, Simon JW, et al.
The exact mechanism responsible Acute acquired comitant esotropia. Br 7. Swan KC. Esotropia following occlusion.
for acute comitant esotropia in patients J Ophthalmol 1989; 73:636–38.
with brain tumors is not clear. Comitant Arch Ophthalmol. 1947;36:444-51.
strabismus might result from involvement 4. Lyons CJ, Tiffin PA, Oystreck D. Acute
of supranuclear mesencephalic acquired comitant esotropia: a 8. Franceschetti A. Acute concomitant
structures, which control vergence eye
movements. Some have ascribed acquired strabismus. Ophthalmologica
comitant esotropia to infranuclear insults,
such as varying degrees of bilateral sixth 1952;123:219-2.
nerve paresis. Spread of comitance is
another suggested mechanism. 9. Bielschowski A. Das Einwärtsschielen

Although most children with this der Myopen. Ber Dtsch Ophthalmol
form of esotropia are otherwise healthy,
central nervous system lesions must be Ges. 1922; 43:245-49.
considered and ruled out before planning
surgery. If you miss a brain tumor in 10. Campos EC. Why do the eyes cross? A

review and discussion of the nature and

origin of essential infantile esotropia,

microstrabismus, accommodative

esotropia, and acute comitant

Neena R et al. Glioma Masquerading as Strabismus www. dos-times.org 95

Photo Essay

esotropia. J AAPOS. 2008; 12:326-31 15. Williams AS, Hoyt CS. Acute comitant 20. Lee JM, Kim SH, Lee JI, et al. Acute
esotropia in children with brain tumors. comitant esotropia in a child with a
11. Gunter K von Noorden. Esodeviations. Arch Ophthalmol. 1989;107:376–78. cerebellar tumor. Korean J Ophthalmol
2009; 23:228–31.
In : Gunter K von Noorden, Emilio 16.. Zweifach PH. Childhood esotropia with
delayed appearance of cerebellar tumor. Correspondence to:
Campos Editors. Theory and Neuroophthalmology 1981;1:291-3. Dr. Neena R.
Consultant,
Management of strabismus in binocular 17. Liu GT, Hertle RW, Quinn GE, et al. Giridhar Eye Institute,
Comitant esodeviation resulting from Kadavanthara, Kochi, Kerela, India.
vision and ocular motility. 5th ed. The neurologic insult in children. J AAPOS
1997; 1:143–46.
C.V. Mosby Company: USA; 1990. p.
18. Dikici K, Cicik E, Akman C, et al.
309. Cerebellar astrocytoma presenting
with acute esotropia in a 5-year-old
12. Astle WF, Miller SJ. Acute comitant girl. Int Ophthalmol 2001;23:167-70.

esotropia: a sign of intracranial disease. 19. Musazadeh M, Hartmann K, Simon F.
Late onset esotropia as first symptom
Can J Ophthalmol. 1994;29:151–54. of a cerebellar tumor. Strabismus
2004;12:119-23.
13. Hoyt CS, Good WV. Acute onset

concomitant esotropia: when is it a

sign of serious neurological disease? Br

J Ophthalmol. 1995;79:498–501.

14. Macpherson H, De Becker I, MacNeill

JR. Beware: armed and dangerous-

-acquired non-accommodative

esotropia. Am Orthopt J. 1996;46:44–

56.

96 DOS Times - July-August 2017 Neena R et al. Glioma Masquerading as Strabismus

Case Reports

Is there an Age Limit While Treating
Amblyopia in Adults?

Sameera Irfan FRCS
Lahore, Pakistan.

Summary: A 56-year lady presented with poor vision in right eye since child-hood. She had been refused therapy by various
ophthalmologists for her “Lazy Eye’ since she was considered past the “treatable age”. A detailed ophthalmological assessment
of her visual status, Best Corrected Visual Acuity for distance and near was done. She was found to have anisomyopic amblyopia.
Full-time occlusion therapy was started. Within 6 weeks, her near vision improved from worse than N18 to N6 while her
distance vision improved from 0.1 to 0.8 (decimal fraction) in further 3 months. Her progress was monitored for 2 years, and
no regression in visual acuity was noted.

Amblyopia or lazy eye, is a disorder of the and near, pupil, colour vision, stereopsis, status of muscle balance
visual system in which visual loss is out of by cover-uncover test, slit-lamp and fundoscopy, assessment of
proportion to any structural abnormality foveal fixation by visuoscope was done. She was found to have a
in the eye1. It results from disuse of an eye, BCVA of 0.9 (Decimal fraction on TSCP-700 Chart Projector) OD
either due to an inadequate foveal or greater and Counting finger (CF) OS with a correction of OD = -0.5DS
peripheral retinal stimulation (where there and OS= -5.50DS / -2.00Dcyl@90. She could only read a font
is a lesser concentration of cones), or due to an abnormal much larger than N18 with OS (newspaper headlines only with
binocular interaction resulting from variable visual inputs difficulty), with no improvement for near after an add of +2.5DS
from both fovea2. Human brain is designed to allow both eyes into her distance correction. She was prescribed glasses for full-
to function together to explore space. If signals from one eye time use and asked to return for follow-up after 1 month.
are blurred or absent, brain blocks visual input from that eye.
In the neuronal visual pathway, the synapses are broken due to On first follow-up, her BCVA improved from CF to 0.1 so she
disuse of the amblyopic eye3,4. This may occur due to constant was asked to continue with glasses only for a further period of
strabismus, disproportionately high refractive error in one eye, one month. On second follow up, her BCVA had not shown any
a combination of both factors, or blocked vision in an eye due improvement. She was prescribed full-time occlusion therapy
to a droopy upper lid, media opacity like cornea/vitreous or of her good eye along with active usage of the amblyopic eye
congenital cataract5. by forced reading, writing, for 4-5 hours per day. She was
explained to start reading a large font that she was comfortable
Amblyopia has been estimated to affect 1–5% of the with and shift to smaller fonts every day. The need for regular
population6,7. It is generally believed to be fully treatable only follow-up was stressed. A written consent form was obtained
till the age of 6-8 years; beyond that age only some visual and commercially available eye patches to be worn over good
improvement is considered to be possible. Many clinicians eye were prescribed for all waking hours daily, to be removed
refuse therapy after the age of 8-12 years. at night while going to sleep. After 2 weeks of starting full-time
occlusion therapy, her near vision improved to N18 but there
An adult person with unilateral amblyopia is at three times was no improvement in distance vision. On further follow-up,
greater risk and a child, 17 times that of a normal person for the near vision showed a steady, gradual improvement to N6
losing vision in the better eye8. There are anecdotal evidences with 6 weeks of occlusion therapy and the distance vision also
of spontaneous improvement of vision in an amblyopic eye after improved to 0.2. With regular follow-up, the distance vision
loss of vision in the good eye. This spontaneous improvement of also showed a gradual improvement and after 4 months of
visual acuity to a usable level (6/24 or better) is relatively low full-time occlusion therapy, her BCVA for distance vision was
(<17%) unless a complete visual loss occurs in the better eye9. 0.8 while the good eye VA remained at 0.9. After that, a gradual
weaning protocol of occlusion therapy was started with 1 day
Full-time occlusion therapy for treating amblyopia has off-patching in first week with 6 days full-time patching, 2 days
been practiced since decades. The case discussed here shows off-patch in the second week with 5 days full-time patching and
that it can result in improvement of visual acuity at any age. follow-up after every 2 weeks. Since the BCVA was maintained
at 0.8, weaning schedule was continued till patching was totally
CASE REPORT off after 7 weeks. She was kept under regular follow-up for 2
years and no regression in BCVA was noted. She was strictly
A 56 years old lady presented at our tertiary care centre counselled to wear spectacle correction and have a regular
accompanying her 6 years old grandson for the treatment of his follow-up.
lazy eye. While the grandson was being explained the rational
of therapy, she queried if it was possible to treat her ‘Lazy Eye’ Review
as well at her age, though she had been refused therapy by
various ophthalmologists since her childhood. In general, the amblyopic adult patients are refused

A detailed ophthalmological assessment including her
visual status, Best Corrected Visual Acuity (BCVA) for distance

Irfan S. Age Limit for Amblyopia. www. dos-times.org 97

Case Reports

therapy beyond a certain age because to long-term bimanual typing, suggesting 3. Barrett BT, Bradley A, McGraw PV.
of misinterpretation of “Critical Period that learning can affect not only function Understanding the neural basis of amblyopia.
for Visual Development.” According to but brain structure as well in adults19. Neuroscientist 2004;10:106-117.
Wiesel and Hubel10, this period exists Dopamine is another neurotransmitter
from 1-5 years of age. It means that that stimulates receptors and turns them 4. Greg Mischio. Why New Brain Rewiring
during this period, an individual’s retina “On”. It is present in retina and cerebral Study Should Excite Amblyopia Pa-tients.
and brain are most sensitive to outside cortex but does not cross the blood- Neurobiology June 1996:Vol. 93: 6830-34.
environment and stimuli than at other brain barrier. Its precursor, Levodopa,
periods of life. This does not mean that crosses that barrier and is converted to 5. American Academy of Ophthalmology.
the visual cortex becomes unresponsive Dopamine in the brain. These studies Basic and clinical science course. Pediatric
to retinal stimulation once that period is prove that neural stem cells (progenitor Ophthalmology and Strabismus. Section
over and the “gates’ leading from retina cells) can be made to generate neurons in 6. San Francisco: American Academy of
to visual cortex close tightly; rather these various brain areas of mammals20. Adults Ophthalmology, 2006.
gates remain very slightly open and get continue to learn throughout life and this
rustic by disuse. is because of continued neurogenesis in 6. Ciuffreda KJ, Levi DM, Selenow A. Amblyopia.
the memory area. Boston: Butterworth-Heinemann, 1991:1-
Recent findings of neuroplasticity 64.
have shown that brain is not a Hence, in case of amblyopia the
physiologically static organ and it closed, rustic gates in the visual pathway 7. Flom MC, Neumaier RW. Prevalence of
can modify throughout life11,12. Its can be fully opened and turned active amblyopia. Public Health Rep 1966; 81:329-
development does not end after a certain again but this needs a strong, persistent 41
age; it can be stimulated to form new stimulation, without any inhibitory
connections between existing brain cells influence. The brain favors neural 8. Tommila V, Tarkkanen A. Treatment of
and strengthen older ones in any part of transmission from the good eye; it is amblyopia after loss of vision in the healthy
the brain, any time in an individual’s life a known fact that the good eye has an eye. Ophthalmic Pediatr Genet 1982; 1:177-
by strong, persistent and appropriate inhibitory influence over the amblyopic 82.
stimulation. This ability is strong in early eye. As shown in this case-report,
childhood when maximum brain growth full-time occlusion of the good eye 9. Vereecken EP, Brabant P. Prognosis for vision
occurs, slows down with age, but it never removed this inhibitory effect over the in amblyopia after the loss of the good eye.
stops13. The molecule responsible for development of neural connections of Arch Ophthalmol 1984; 102:220-4.
neuroplasticity is a protein receptor14 the amblyopic eye for the whole duration
which is in an “OFF” mode in adults of therapy. This was combined with the 10. Wiesel TN, Hubel DH. Single-cell responses in
but can be turned “ON” by continued, active use of amblyopic eye till neural the striate of kittens deprived of vision in one
active brain stimulation. GABA (Gamma connections became fully functional. eye. J Neurophysiol 1963;26:1003-1017.
Amino Butyric Acid)15,16, acts as an Once that was achieved, the connections
excitatory neurotransmitter in immature, were given adequate time to stabilize to 11. Chakraborty R, Chatterjee A, Choudhart
developing brains and regulates avoid regression of amblyopia; this was S, Chakraborty PK. Neuroplasticity—a
proliferation of neural progenitor cells, provided by following a very slow and paradigm shift in neurosciences. J Indian
proliferation and elongation of neurons gradual weaning protocol for occlusion Med Assoc 2007;105:513-4,516-8,520-1.
and formation of synapses by releasing therapy in this case.
Brain-Derived-Neurotrophic Factor. This 12. Levin HS. Neuroplasticity and brain imaging
results in important brain functions like This case report proves the concept research: implications for rehabilitation.
memory, learning, speech, motor control. of neuro-plasticity and shows that Arch Phys Med Rehabil 2006;87(12 Suppl
It not only gradually decreases with age amblyopia in adults can be fully treatable. 2):S1.
but in mature brains, it has an inhibitory But this needs highly motivated and
affect by activating GABA-receptors inspired patients who are ready to 13. Hook BM, Chen C. Critical periods in the
and causing cell arrest in the S-phase cooperate and comply with therapy. They visual system: Changing views for a model
(static phase). GABA given exogenously must manage their lives for a period of of experience-dependent plasticity. Neuron
cannot cross the blood-brain barrier. 2-3 months by keeping their good eye 2007;56:312-26
Researchers obtained GABA secreting closed and actively using an eye that had
neurons from young mice while they were been neglected for decades. This is not 14. Ge S, Yang CH, Hsu KS, et al. A critical period
in their “critical period” and transplanted an easy job either for the patient or the for enhanced synaptic plasticity in newly
into the brains of adult, amblyopic mice. treating ophthalmologist, but this is the generated neurons of the adult brain. Neuron
After some time, they found new neural only way to ensure full visual recovery 2007;54:559-66.
connections forming in the visual pathway by very simple means with no economic
and restoration of normal eyesight in burden either on the patient or the health 15. Haydar TF, Wang F, Schwartz ML, Rakic P.
those adult, amblyopic recipient mice17,18. services. Once the visual recovery is “Differential modulation of proliferation
Similarly, in other studies, plasticity of achieved, it may be permanent. in the neocortical ventricular and
brain was shown to improve in specific subventricular zones”. J. Neurosci. 2000;20:
regions by a specified stimulus. The REFERENCES 5764–74.
brain receptors which were turned “Off”
with age, could be turned “On” by GABA 1. Hess RF, Field DJ, Watt RJ: The Puzzle of 16. Behar TN, Schaffner AE, Scott CA, O’Connell
released in response to a stimulus. An Amblyopia. Vision: Coding and Efficiency. C, Barker JL. “Differential response of cortical
increase in the gray matter volume has Cambridge University Press 1990; 267-80. plate and ventricular zone cells to GABA as
been observed in professional typists’ due a migration stimulus”. J. Neurosci. 1998;18:
2. Flynn JT. 17th annual Frank Costenbader 6378–87.
Lecture Amblyopia revisited. Pediatr
Ophthalmol Strabismus 1991;28(4):183- 17. Barbin G, Pollard H, Gaïarsa JL, Ben-Ari
201. Y. “Involvement of GABAA receptors in
the outgrowth of cultured hippocampal
neurons”. Neurosci. Lett. 1993;152: 150–54.

18. Maric D, Liu QY, Maric I, Chaudry S, Chang
YH, Smith SV, Sieghart W, Fritschy JM, Barker
JL. J. Neurosci. 2001;21: 2343–60. PMID
11264309.

19. Ben-Ari Y. “Excitatory actions of gaba during
development: the nature of the nurture”. Nat.
Rev. Neurosci. 2002;3: 728–39.

20. Ben-Ari Y, Gaiarsa JL, Tyzio R, Khazipov R.
“GABA: a pioneer transmitter that excites
immature neurons and generates primitive
oscillations”. Physiol. Rev. 2007;87: 1215–84.

Correspondence to:
Dr. Sameera Irfan, FRCS
Pediatric Ophthalmologist & Oculoplastic Surgeon,
Lahore, Pakistan.

98 DOS Times - July-August 2017 Irfan S. Age Limit for Amblyopia.

Case Reports

Utility of Ice Pack Test in Diagnosis of
Myasthenia Gravis

Mandeep Tomar MS, Gaurav Sharma MS, Nikhil Verma MBBS, Richa Dhiman MBBS, Vinod Dhiman
Dr. Rajender Prasad Government Medical College, Tanda, Himachal Pradesh, India.

Summary: Ice pack test is a simple clinical test performed for Myasthenia gravis (MG) in the office and is of great value in
diagnosing and minimizing costly neuroimaging referrals. A 16 year old male child presented with hoarseness, dysphagia and
bilateral ptosis after having local honey and meals in neighbourhood. Marginal Reflex Distance was 1mm in right eye and zero
mm in the left. Possibility of botulism was kept. Ice test for 3 minutes was positive. MG was suspected and confirmed by positive
edrophonium test. MG suspects should undergo complete ophthalmological examination with ice pack test to differentiate from
other neurological mimickers.

Myasthenia gravis is an autoimmune disease with palpebral aperture 8mm and 7mm in right and left eye
in which antibodies bind to acetylcholine respectively, marginal reflex distance of 1mm in the right eye
receptors or to functionally related and zero in the left. There was a good LPS (levator palpabrae
molecules in the postsynaptic membrane at superioris) action. Ice pack test for 3 minutes was done as a
the neuromuscular junction. The antibodies part of routine ptosis workup and came out to be positive
induce weakness of skeletal muscles, (Figure 1). After positive ice pack test, a strong possibility of
which is the sole disease manifestation1-3. The weakness can myasthenia gravis was suspected and further investigations
be generalized or localized, is more proximal than distal, and were carried out.
nearly always includes eye muscles, with diplopia and ptosis.
With an annual incidence of 8 to 10 cases per 1 million persons Cogan lid twitch sign and lid fatigability test were also
and a prevalence of 150 to 250 cases per 1 million4, myasthenia positive bilaterally. Neostigmine test was done in neurology
gravis and its various subgroups are the major diseases department under anaesthetist supervision with monitoring of
that affect the neuromuscular junction. The Lambert–Eaton cardiovascular function, and was found positive (Figure 2).
myasthenic syndrome and neuromyotonia are additional, rare,
presynaptic autoantibody disorders characterized by skeletal- Anti-acetylcholine esterase antibody levels (binding type)
muscle dysfunction5. Congenital myasthenic syndromes and were negative i.e. value<0.15 nmol/l (normal range 0.0-0.4
toxin-induced conditions (e.g., botulism) can also affect the nmol/L) and anti-muscle specific kinase levels (Musk) were
neuromuscular junction and lead to muscle weakness. normal. CT thorax to rule out thymoma revealed a normal
study. Thyroid profile revealed decreased levels of T3 [77
Case report ng/dl (normal 80-180 ng/dl)], T4 [7nmol/l (normal 58 - 161
nmol/l)] and elevated TSH [6.8mIU/L (normal-0.4-4 mIU/l)].
A 16 years old male child presented in paediatrics routine Lactate dehydrogenase levels were 404 u/l (normal-140-280
OPD with insidious onset of inability to speak properly, u/l), rest of the investigations were normal. Ultrasonography
hoarseness and dysphagia since last 7 days following meals
and local honey in the neighbourhood. It was soon followed Figure 1: On day of presentation before ice pack test and after.
by asymmetrical drooping of bilateral eyelids since last 3 days.
There was no history of associated diplopia and restriction Figure 2: Before and after neostigmine test.
of ocular movements. Child also complained of weakness of
both upper limbs which was followed by bilateral lower limbs
weakness since the last 1 day. There was history of difficulty
in lifting heavy objects and climbing upstairs. There were no
diurnal variations, no aggravating and relieving factors, no
history of vomiting, diarrhoea, fever or any drug intake.

A provisional diagnosis of botulism with descending
paralysis with multiple cranial nerve palsy in the form of
3rd, 5th ,7th and 10th cranial nerve, was kept in paediatrics
department, keeping in mind the history of food ingestion
associated with it and child was referred to department of
ophthalmology for further evaluation.

On ocular examination, visual acuity was 6/9 (p) both
eyes improving to 6/6 with pin hole. Pupils were reactive and
ocular movements were full. There was severe bilateral ptosis

Tomar M et al. Ice Pack Test For Myasthenia www. dos-times.org 99

Case Reports

of the neck was in view of a midline the sensitivity and specificity of this test Electrodiagnostic test (RNS) was
swelling in the neck which revealed was 76.9% and 98.3%, respectively12. not preferred in this case as acute severe
heterogeneous echotexture with diffuse generalized or bulbar MG (≤ 4 weeks of
hypoechogenicity in the thyroid gland Among electrodiagnostic tests, disease) shows only 11% to 40% positive
suggestive of thyroiditis. Repetitive Nerve Stimulation (RNS) (2 rates17. Moreover due to strongly positive
to 3 Hz) is the most frequently used edrophonium test, diagnosis was certain
A final diagnosis of generalized electrodiagnostic test for MG. The nerve and RNS test was not considered.
myasthenia gravis with ocular to be studied is electrically stimulated
involvement was made in consultation 6-10 times at 2 or 3 Hz (slow rate) with a About 85% of patients with
with neurology department and child supramaximal stimulus and the compound generalized MG have antibodies to AChRs,
was started on oral pyridostigmine 60 muscle action potential (CMAP) is but only approximately 50% of patients
mg 3 times/day and 180 mg (sustained recorded with surface electrodes. In MG, with purely ocular manifestations
release) bedtime and tablet azathioprine as the number of individual muscle fiber (ocular MG) have detectable antibodies18.
50 mg once a day. Child was kept under action potentials reduce, the CMAP also Antibodies to another protein at the
follow up with marked recovery on reduces in both amplitude and area with neuromuscular junction (muscle-
subsequent visits. a resulting decremental response13. In specific kinase, or MuSK) are present in
MG, a characteristic decrement (>10%) approximately 40% of AChR antibody-
Review in muscle action potential amplitude negative patients with generalized MG19.
is typically seen by the fourth or fifth Elevated AChR-Ab titers confirm the
Myasthenia gravis (MG) is an response in a series of low-frequency RNS, diagnosis of MG. However, a normal titer
autoimmune disorder characterized by whereas the amplitude remains the same does not exclude the disease.
reduction in the acetylcholine receptors in normal individuals14. This decremental
(AChRs) at neuromuscular junctions response is seen in only 33% of patients Coexisting conditions are common
due to the effects of autoantibodies. It with pure OMG15. A decremental response in patients with MG and should always
is characterized by a variable weakness to RNS is not specific and may also be seen be considered. Approximately 15% of
of skeletal muscles, which improves in Lambert-Eaton myasthenic syndrome, patients have a second autoimmune
on resting. Weakness is exacerbated motor neuron diseases, and myopathies. disease20,21, which occurs most frequently
by repetitive contraction6. Generalized in patients with early-onset myasthenia
myasthenia involves the bulbar, limb, and Conventional electromyography gravis and thymic hyperplasia. Thyroiditis
respiratory muscles; ocular myasthenia (EMG) is not useful for the diagnosis of is the most common coexisting
gravis (OMG) is a subtype of MG where the myasthenia, but may be indicated in these condition, followed by systemic lupus
weakness is clinically isolated to the EOMs, patients when concurrent nerve or muscle erythematosus and rheumatoid arthritis.
levator, and orbicularis oculi. The pattern disease is in question16. Single fibre EMG Additional testing for thyroid dysfunction
of involvement is usually symmetric, (SFEMG) needs more patient cooperation may also be considered in patients
apart from the eye involvement, which is than do these tests. The diagnostic yield with myasthenia, since about 4-5% of
often markedly asymmetric and involves is significantly higher. Some patients patients with MG may have concurrent
several eye muscles. In 15% of all patients considered to have myasthenia gravis autoimmune thyroid disease22.
with myasthenia gravis, symptoms and do not show any abnormalities with this
signs are confined to ocular muscles7. technique, particularly those with the Although diagnosed as a case of
Ptosis and diplopia are common initial pure ocular form. generalized myasthenia gravis, it was
symptoms, but the disease remains a case of diagnostic dilemma for the
restricted to ocular muscles in only a
minority of patients. Differences between myasthenia gravis and botulism

The Ice Pack Test is a simple, but MG Botulism
effective clinical test that can be used to
confirm the diagnosis of MG. An icepack is Pathology Autoantibody formation Endotoxin prevent release of
placed over the patient’s closed eyelids for against AChR Ach
a period of 2 min (for ptosis) to 5 min (for
ophthalmoparesis). The ocular motility Hall Mark Features Fatigable weakness, ptosis Descending paralysis cranial
deficits and ptosis must be measured neuropathy bilateral
before and after the test. Although there
are no strict guidelines regarding the Weakness Yes Yes
interpretation of this test8 it is, usually, Reflexes Present Absent
considered positive when the upper
eyelid elevates by at least 2 mm following Distribution Eyes + cranial nerves Descending +Symmetrical
ice application. Cooling may reduce
cholinesterase (ChE) activity, which Progression Relapsing Acute
increases the amount of available ACh at
the neuro-muscular junction9. There is Fatigue Yes (characteristic) No
thus an increase in the efficiency of ACh
in eliciting depolarization at the motor Delayed nerve No No
end plate10. Resolution of ptosis has been conduction
reported in over 90% of OMG patients
after the ice test11. According to one study, Fade + post tetanic Yes Small motor response On
count facilitation repetitive stimulation

Response to Yes Yes
Anticholinesterase

Response to plasma- Yes No
pheresis

100 DOS Times - July-August 2017 Tomar M et al. Ice Pack Test For Myasthenia

Case Reports

presenting physician, due to abrupt 7. Kerty E, Elsais A, Argov Z, Evoli A, 17. Liik M, Punga AR etal. Repetitive
onset of the disease, history of intake Gilhus NE. EFNS/ENS guidelines for the nerve stimulation often fails to detect
of contaminated meals associated with treatment of ocular myasthenia. Eur J abnormal decrement in acute severe
complaints, facial picture mimicking Neurol 2014; 21: 687-93. generalized Myasthenia Gravis.
multiple cranial nerve palsy and ClinNeurophysiol. 2016;127:3480-84.
motor neuropathy, absence of diurnal 8. Conti-Fine BM, Milani M, Kaminski HJ.
variations and simultaneous onset of Myasthenia gravis: Past, present, and 18. Drachman DB. Myasthenia gravis. N
bilateral ptosis. Thorough and meticulous future. J Clin Invest. 2006;116:2843– Engl J Med 1994;330:1797-1810.
ophthalmological examination with ice 54.
pack test helped in this case to make the 19. Zhou L, McConville J, Chaudhry V , et al.
final diagnosis. 9. Sethi KD, Rivner MH, Swift TR. Ice pack Clinical comparison of muscle-specific
test for myasthenia gravis. Neurology. tyrosine kinase (MuSK) antibody-
References 1987;37:1383–5. positive and -negative myasthenic
patients. Muscle Nerve 2004;30:55-60.
1. Gilhus NE, Skeie GO, Romi F, Lazaridis K, 10. Hubbard JI, Jones SF, Landau EM. The
Zisimopoulou P, Tzartos S. Myasthenia effect of temperature change upon 20. Nacu A, Andersen JB, Lisnic V, Owe JF,
gravis — autoantibody characteristics transmitter release, facilitation and Gilhus NE. Complicating autoimmune
and their implications for therapy. Nat post-tetanic potentiation. J Physiol. diseases in myasthenia gravis: a review.
Rev Neural 2016; 12: 259-68. 1971;216:591–609 Autoimmunity 2015;48:362-8.

2. Gilhus NE, Verschuuren JJ. Myasthenia 11. Kubis KC, Danesh-Meyer HV, Savino 21. Gilhus NE, Nacu A, Andersen JB, Owe
gravis: subgroup classification and PJ, Sergott RC. The ice test versus JF. Myasthenia gravis and risks for
therapeutic strategies. Lancet Neurol the rest test in myasthenia gravis. comorbidity. Eur J Neurol 2015;22:17-
2015; 14: 1023-36. Ophthalmology. 2000;107:1995–8. 23.

3. Querol L, Illa I. Myasthenia gravis and 12. Chatzistefanou KI, Kouris T, Iliakis E, 22. Chen CS, Lee AW, Miller NR, Lee AG.
the neuromuscular junction. Curr Opin Piaditis G, Tagaris G, Katsikeris N, et Double vision in a patient with thyroid
Neurol 2013; 26: 459-65. al. The ice pack test in the differential disease: What’s the big deal? Surv
diagnosis of myasthenic diplopia. Ophthalmol. 2007;52:434–9.
4. Carr AS, Cardwell CR, McCarron PO, Ophthalmology. 2009;116:2236–43.
McConville J. A systematic review of Correspondence to:
population based epidemiological 13. Juel VC, Massey JM. Myasthenia gravis. Dr. Mandeep Tomar
studies in myasthenia gravis. BMC Orphanet J Rare Dis. 2007;2:44. Senior Resident, Department of Ophthalmology
Neurol 2010; 10:46. Dr. RPGMC Tanda, HP, India.
14. Ozdemir C, Young RR. The results to be
5. Verschuuren J, Strijbos E, Vincent A. expected from electrical testing in the
Neuromuscular junction disorders. diagnosis of myasthenia gravis. Ann N
Handb Clin Neurol 2016; 133: 447-66. Y Acad Sci. 1976;274:203–22.

6. Grigg J. Extraocular muscles: 15. Costa J, Evangelista T, Conceição
Relationship of structure and function I, de Carvalho M. Repetitive nerve
to disease. Aust N Z J Ophthalmol. 1999; stimulation in myasthenia gravis –
27:369–70. relative sensitivity of different muscles.
Clin Neurophysiol. 2004;115:2776–82.

16. Stalberg E. Clinical electrophysiology
in myasthenia gravis. Journal of
Neurology, Neurosurgery, and
Psychiatry, 1980;43:622-33

Tomar M et al. Ice Pack Test For Myasthenia www. dos-times.org 101

Case Reports

A Case of Congenital Third Cranial
Nerve Palsy

Komal Batra, Sandra Ganesh
Aravind Eye Hospital, Coimbatore, India.

Summary: Congenital third nerve palsy can present with a myriad of features such as ptosis, pupillary involvement, amblyopia,
partial or complete loss of function of the extraocular muscles, leading to ocular motility defects. A 23-year-old female presented
to us with limitation of movements of her left eye. Based on clinical findings of severe limitation of adduction of the left eye and
mild ptosis, a diagnosis of congenital third nerve palsy with residual ptosis and exotropia was made. Surgical correction by
fixating the globe to medial orbital wall was attempted, aiming to fix the eye in primary position of gaze.

Third cranial nerve supplies four of the six Through a medial limbal conjunctival incision, medial
extraocular muscles and is associated with rectus (MR) muscle was isolated (Figure 2). A transcutaneous
presence of associated factors such as ptosis, incision was made over the anterior lacrimal crest and
amblyopia, pupillary involvement, aberrant insertion of medial palpebral ligament (MPL) was exposed by
regeneration, lateral rectus muscle contracture blunt dissection (Figure 3). Two arms of double armed 5-0 non-
leading to ocular motility defects. Third nerve absorbable (ethibond) suture were passed through the superior
palsy can be isolated or can occur in association with other and inferior part of MPL insertion. The suture ends were tied
ocular nerve palsies. Isolated third nerve palsy may be unilateral to each other (Figure 4). An empty semicircular needle was
or bilateral, complete or partial, pupil involving or pupil used as a guide to bring out the two needles of the suture onto
sparing, and congenital or acquired1. Surgery is the mainstay the subconjunctival space (Figure 5). The sutures were then
of treatment for management of unrecovered third nerve tied tightly at the insertion of the MR directly onto the sclera,
palsy. Surgical management is challenging and is different for at the same time as the assistant rotated the globe medially
partial and complete third nerve palsy. For partial involvement, thus adducting the eye with the aim of slight overcorrection
surgical intervention is according to the nature and extent of (Figure 6). Post operatively the eye was aligned in adduction
extraocular muscle involvement. For complete third nerve with mild hypertropia.
palsy, supramaximal recession-resection of the recti, superior
oblique tendon transposition, lateral rectus transposition or Figure 1: Clinical Pre-operative 9 Gaze Photograph Showing Left Eye in
globe anchoring procedures are attempted. Abduction in Primary Position.

Case Report Figure 2: Isolation of the Medial Rectus (MR) muscle.
Batra et al. Case of Third Nerve Palsy
A 23-year-old lady presented with limitation of movements
of her left eye. She had undergone 9mm lateral rectus (LR)
recession in her left eye in February 1996 and two Frontalis
sling operation were performed elsewhere subsequently for
left upper lid ptosis in September 1997 and December 2000 to
prevent amblyopia. On examination, her visual acuity was 6/6
in right eye and 6/9 in her left eye improving to 6/6 with glasses.
Left eye showed mild upper lid ptosis, 15 degrees of exotropia on
Hirschberg’s corneal light reflex and absent adduction on cover
test. Movements were restricted in all direction of gazes in her
left eye with severe limitation of adduction (-4) and inability
to move the left eye to midline. There was >50 prism diopters
of exotropia of her left eye on modified Krimsky test. Right eye
motility was normal (Figure 1). When fixing with involved eye,
there was marked secondary deviation in the right eye. There
was no evidence of aberrant regeneration. She was diagnosed
with congenital oculomotor nerve palsy with residual ptosis
and residual exotropia. As she wanted cosmetic correction, she
was operated for residual exotropia with her left eye medial
rectus (MR) fixated to medial palpebral ligament under GA to
fix the globe in primary position of gaze.

102 DOS Times - July-August 2017

Case Reports

Figure 3: Transcutaneous incision over the anterior lacrimal crest. Figure 4: A 5-0 non absorbable(ethibond) suture passed through
superior and inferior part of medial palpebral ligament (MPL) insertion.

Figure 5: Sutures being passed through an empty semicircular needle. Figure 6: Sutures passed through the sclera at the superior and inferior
aspect of medial rectus (MR) muscle while the globe is adducted
medially.

Figure 7: Clinical Pre-Operative Photograph Showing Left Exotropia in Figure 8: One week post-operative photograph showing Left Eye in
Primary Position. Primary Position.

Review (14-16 mm) and MR resection (8-14 mm) significant medial rectus function and in
for correcting the horizontal deviation in the presence of contracture of the lateral
Management of strabismus due to primary position as initially described rectus muscle. Disinsertion of the lateral
complete third nerve palsy is a surgical by Helveston3. But the conventional rectus muscle and reattachment to the
challenge. Due to the unopposed action of recession-resection procedure usually lateral orbital wall or globe fixation of
lateral rectus and superior oblique muscle, drifts the eye back to exotropia. Superior medial rectus muscle to the medial wall
the eye is in a divergent and depressed oblique tendon transposition procedures4 of the orbit at anterior lacrimal crest
position. In complete third nerve palsy the to medial aspect of globe were advocated can be performed. This can be achieved
goal of surgery is realignment of the eye by some workers but the procedure is by transcutaneous approach as done in
in primary position, compromising the technically little difficult and the results this case or precaruncular approach. It
ocular motility of the involved eye. While were not found to be satisfactory. Y can be achieved by using either 5-0 non-
the globe is aligned in primary gaze, with split and lateral rectus transposition to absorbable polyester suture as described
very little or no horizontal movement of superior and inferior border of medial by Sharma P et al6, fascia lata, superior
the eye, no meaningful area of binocular rectus muscle was described by Taylor et oblique tendon or silicon band.
single vision can be achieved2. al5.
Though the technique of medial
Surgical options for strabismus Globe anchoring procedures are the rectus fixation to medial palpebral
resulting from third nerve palsy include mainstay of treatment in the absence of ligament limits the horizontal movements
a very large supramaximal LR recession

Batra et al. Case of Third Nerve Palsy www. dos-times.org 103

Case Reports

Figure 9: Three-week post-operative References Correspondence to:
photograph showing Left Eye in Primary Dr. Sandra Ganesh,
Position. 1. Rosenbaum AL, Santiago AP. Clinical Consultant,
strabismus management: Principles Department of Paediatric Ophthalmology and
of the eye and reduces the field of and surgical techniques. David Hunter Strabismus
binocular single vision, it provides a 1999, p. 251-8. Aravind Eye Hospital Coimbatore, India.
better cosmetic appearance by fixing
the eye in primary position of gaze. 2. Singh A, Bahuguna C, Nagpal R, Kumar
This procedure is mainly attempted in B. Surgical management of third nerve
cases of congenital third nerve palsy (no palsy. Oman Ophthalmol 2016;9:80-6.
binocular vision) for cosmetic alignment
as there could be intractable diplopia in 3. Helveston EM. Muscle transposition
patients with acquired palsies. procedures. Surv Ophthalmol
1971;16:92.

4. Scott AB. Transposition of the superior
oblique. Am Orthopt J 1977; 27:11-4.

5. Taylor JN. Surgical management of
oculomotor nerve palsy with lateral
rectus transplantation to the medial
side of the globe. Aust N Z J Ophthalmol
1989; 17: 27–31.

6. Pradeep Sharma MD, Madhurjya Gogoi
MD, Sachin Kedar MD, Rahul Bhola MD.
Periosteal fixation in third-nerve palsy.
J AAPOS 2006; 10: 324–27.

104 DOS Times - July-August 2017 Batra et al. Case of Third Nerve Palsy

news watch

DOS Times Quiz 2017-18

Episode-1

Last date: Completed responses to reach the DOS Office by e-mail or mail before 5 pm on 15st September, 2017

Q1. Identify the test? Q5. Identify the disorder -

(a)

(b) Q6. 9-year-old female with inability to move either
eye sideways along with drooling of saliva, since
(c) childhood. Figure attached. Identify the possible
syndrome.
Q2. The following features are seen in Leber’s congenital
amaurosis except:

A. Nystagmus
B. Normal fundus at the early stage
C. Myopic refraction
D. Arteriolar narrowing in later stage

Q3. All are features of Blepharophimosis syndrome

except:
A. Autosomal dominant inheritance
B. Epicanthus inversus
C. Nasal bridge hypoplasia
D. Mental retardation

Q4. Identify the disorder -

Compiled by:
Guru Nanak Eye Centre, New Delhi, India.

Dr.Yashpal Goel MS

www. dos-times.org 105

news watch

Q7. A 2-week-old infant is suspected of having persistent fetal vasculature DOS Times Quiz Rules
(PFV). On anterior segment examination, what findings would support this
diagnosis? 1. DOS Times Quiz will now feature as 5
Episodes (Episode 1: July-August, Episode 2:
A. Congenital bilateral cataracts September – October, Episode 3: November
B. Normal axial lengths – December, Episode 4: January – February,
C. Iris hypoplasia Episode 5: March – April). Entries will have to
D. Anteriorly displaced ciliary processes be emailed before the last date mentioned in
the contest questions form. Late entries will
Q8. Individuals at greater risk for developing anomalous retinal correspondence not be entertained.
include which one of the following?
2. Please email (as scanned PDF Only)
A. 7-year-old boy with a history of congenital esotropia completed responses for the quiz along
B. 12-year-old girl with a 3-week history of decreased vision in her right eye with details of the contestant filled in and
C. 15-year-old boy with a I-week history of esotropia signed to [email protected] (with cc to
D. 7-year-old girl with a history of exophoria [email protected]) or mail to DOS Times
Quiz, Dr. Subhash Dadeya, Room No. 205, 2nd
Q9. Identify the condition depicted Floor, OPD Block, Guru Nanak Eye Centre,
by the photographs Maharaja Ranjit Singh Marg, New Delhi.

Q10. Which of the following statements does not accurately describe the use of 3. Nonmembers may also send in their entries
indirect ophthalmoscopy to screen for retinopathy of prematurity (ROP)? but will be required to send along with
their completed entries, the completed
A. Screening should be performed on all premature neonates of less than 30 membership application (with the required
weeks gestation. documents) to enroll as member. Failing
this their entries into the contest will not be
B. Screening should be repeated biweekly on neonates who demonstrate any considered.
stage of ROP on initial examination.
4. Contestants are requested to attempt all the
C. Screening should be performed before hospital discharge, or by 4- 6 weeks of 5 episodes of the Quiz contest and send in
age. their applications within the date specified.
No entries will be entertained after the last
D. Screening should be performed on all premature neonates with a birth weight date. The scores of each contestant for all
<1500 g. 5 episodes together will be compiled at
the end of episode 5 and the winner will be
announced in the DOS Annual Conference
in April 2018. In the event of more than one
winning contestants, a draw of lots will decide
the winner. Winner of each episode will also
be published in the next episode along with
the previous episode answers.

5. Please write to [email protected] or
[email protected] for further

clarifications if any.

## # # #

Q. No. Completed Responses for DOS Times Quiz: Episode 1

1. __________________________________________________________________ 6. __________________________________________________________________
2. __________________________________________________________________ 7. __________________________________________________________________
3. __________________________________________________________________ 8. __________________________________________________________________
4. __________________________________________________________________ 9. __________________________________________________________________
5. __________________________________________________________________ 10. __________________________________________________________________

Contestant Details
Name: ________________________________________________________________________________________________ Degree: _______________________________
Designation:_________________________________________________________________________ Address:_______________________________________________
_______________________________________________________________________ State _______________________________ Pin _______________________________
Mobile No: ________________________________________________________________________________________ DOS Membership no: ___________________
Email ID: _______________________________________________________________________________________Signature: ___________________________________

106 DOS Times - July-August 2017

DOS Crossword news watch
7
Episode-1

Dr. Richa Agarwal MS
Guru Nanak Eye Centre,
New Delhi, India

1
23

4

56

8

9 10
11
12
13

Across Down

2. Treatment for non refractive accommodative esotropia(7) 1. 3 step test for vertical deviation(5)
4. Press-on prism(7) 2. Superior oblique tendon sheath syndrome(5)
5. Syndrome most commonly seen with Posterior 3. Common cause of proptosis in children(10)
5. Lazy eye(9)
embryotoxon(8) 6. Syndrome that violates Sherrington law(6)
8. Chemotherapeutic agent used in retinoblastoma(11) 7. Head tilt test for SOP(12)
9. Neurotoxin for treatment of squint(5) 10. Surgery for MED(5)
11. Law violated by DVD(6) 12. Pharmacological treatment for amblyopia(8)
13. Benign vascular tumor of infancy(10)

www. dos-times.org 107

news watch

Delhi Ophthalmological Society

(Life Membership Form) Paste
Photo

The Photo Identity Card will be issued after
your Membership is ratified by the General
Body during the next Annual DOS Conference

Name (In Block Letters)________________________________________________________________________________________________

S/D/W/o_____________________________________________________________________________ Date of Birth____________________

Qualifications_________________________________________________________________________ Registration No._________________

Sub Speciality (if any)_________________________________________________________________________________________________
Address
_____________________________________________________________________________________________________________
_____________________________________________________________________________________________________________
______________________________________________________________________________ Phone________________________

Email _____________________________________________________________ Mobile No.______________________________

Proposed by

Dr.________________________________________________ Membership No.___________ Signature__________________________

Seconded by

Dr.________________________________________________ Membership No.___________ Signature__________________________
[Must submit a photocopy of the Address proof, Pan Card, MBBS/MD/DO, State Medical Council / MCI Certificate for our records.]

Declaration: I hereby declare that the above details are correct. I wish to be Life member. I have carefully read the instructions overleaf. I shall abide
by the Rules, Regulation & Bye-Laws of the Society as in force and any subsequent amendment(s) made from time to time
(Life membership fee Rs. 5600/- payable by DD for outstation members. Local Cheques acceptable, payable to Delhi Ophthalmological
Society)
Please find enclosed Rs.___________in words ____________________________________________________ by Cash _________________
Cheque/DD No.________________________________ Dated_______________ Drawn on__________________________________________

Signature of Applicant Three specimen signatures for I.D. Card.
with Date

For Official Use Only

Dr._______________________________________________________________has been admitted as Life Member of

the Delhi Ophthalmological Society by the General Body in their meeting held on________________________________

His/her membership No. is _______________. Fee received by Cash/Cheque/DD No._______________ dated_________

drawn on __________________________________________________________________. (Secretary DOS)

www. dos-times.org 109