Aio Vol 1 Issue 1 Sep 2013
Aio Vol 1 Issue 1 Sep 2013
Aio Vol 1 Issue 1 Sep 2013
Fig 6
Right eye ptosis causing the pseudo lid retraction of left eye
16 AIO Journal of Ophthalmology
REVIEW
EvALuATIOn Of PTOSIS
History
To suggest the provisional diagnosis of the ptosis:
l Duration: Congenital / Acquired.
l Pre-disposing factors: h/o pregnancy and delivery,
trauma, surgery, medical conditions.
l Progression.
l Variability: time of day, jaw winking.
l Functional limitation: restricted superior feld / full
feld of vision. difculty while reading
l Associated symptoms: diplopia, dysphonia, dys-
phagia, dyspnoea (Myasthenia gravis)
l Family history: Blepharophimosis, some forms of
congenital ptosis.
Examination
To confrm provisional diagnosis and decide on treatment.
Full ocular and adnexal examination with emphasis on:
l Facial examination: Chin up, frontalis overaction,
brow ptosis, dermatochalasia, telecanthus, epican-
thus inversus.
Presence of ptosis
l Vertical palpaebral fssure height: Patient fxates on
a distant object in primary gaze. Measure the widest
point between upper and lower eyelid (Fig 7 ). Value
of < 9 mm indicates ptosis.
l Margin refex distance (MRD):
MRD
1
= With patient looking at the torch in the primary
gaze, the distance from the upper eyelid margin to the cor-
neal light refex is measured.
Normal MRD
1
value is 4 to 4.5
mm.
Light may be obstructed
by the eyelid in severe cases
and therefore have a zero or
negative value.
MRD
1
helps to exclude
cases of lower lid malposi-
tions which lead to decreased
palpaebral fssure height.
Grading of ptosis
In unilateral cases, one can compare the droop with the
contralateral eyelid position. However in bilateral ptosis
(can be used also for unilateral ptosis), we have to measure
the diference between a point 9 mm above the inferior
limbus and the upper eyelid margin in primary position.
Using MRD
1
, ptosis can be graded as the amount in
mm, the MRD
1
value is less than the normal value of 4 mm.
2 mm is Mild ptosis
3 mm is Moderate ptosis
4 mm is Severe ptosis.
Levator function
It is measured as the amount of upper eyelid excursion
from extreme downgaze to extreme upgaze, with the
frontalis muscle immobilised by the examiner manually
fxing the eyebrow in its normal position (Fig 8 ).
Levator action in mm Functional classifcation
10 15 Excellent
8 9 Good
5 7 Fair
0 4 Poor
l In infants and uncooperative young children, the ILI-
FF sign is elicited to determine the levator function. When
the upper lid is everted in infants with fair to good levator
function, the agitated infant is able to fip back the lid to
its normal position. If levator function is poor to nil, the lid
will not return to its normal position and will have to be
manually reinverted.
l Lid position in downgaze:
Lid lag compared to the contralateral upper eyelid on
downgaze in the absence of previous surgery or trauma
suggests dystrophy of the levator muscle which will nei-
ther relax nor contract properly (Fig 9). This is the impor-
tant fnding in congenital ptosis. In involutional ptosis,
drooping of the lid may worsen on downgaze because of
frontalis muscle relaxation.
Cong. Myogenic ptosis Acquired Aponeurotic ptosis
Lid crease Absent / weak Higher position
Levator function Reduced Near normal
On downgaze Eyelid lag Eyelid drop
Comparison between:
Fig 7: Measurement of
Palpebral fissure height
17
Blepharoptosis
Fig 8 : Measurement of levator function Fig 9 : Lid lag on down gaze Fig 10 : Bells phenomenon
l Upper eyelid skin crease:
Skin crease is often shallow or absent in congenital ptosis.
Margin crease distance (MCD) is the distance between
the upper eyelid crease and the eyelid margin. The high
crease (large MCD) suggests an aponeurotic defect.
l Aberrant eye movements:
On lateral jaw movement or eye movements:
Marcus Gunn jaw winking Synkinesis.
Aberrant regeneration of the Vth cranial nerve.
Duane retraction syndrome.
l Refractive error and vision:
To diagnose amblyopia due to occlusion of pupil, high
astigmatism or strabismus.
l Pupillary examination:
Horner syndrome will present with miosis while IIIrd cra-
nial nerve palsy will present with mydriasis.
l Ocular motility:
To diagnose
Ptosis with superior rectus weakness.
IIIrd cranial nerve palsy
Chronic progressive external ophthalmoplegia
Myasthenia gravis
l Ancillary tests:
Tests for Horner syndrome:
Cocaine and amphetamine test.
Test to rule out Pancoast tumor, carotid artery aneurysm
or dissection.
Tests for Myasthenia gravis:
Edrophonium chloride (Tensilon) test
Ice pack test
Acetylcholine receptor antibody test.
l Test to predict post-op complication:
Lagophthalmos
Bells phenomenon (Fig 10 )
Evaluation for dry eye Corneal sensation
TREATMEnT (Table 3)
Choice of surgery depends upon:
l Type of ptosis.
l Amount of ptosis.
l Degree of levator function.
l Surgeons experience with various type.
Ptosis
Moderate Mild Severe
Good
Fasanella Servat
Fair Good Poor
Large Resection
Moderate Resection Supramaximal Maximal Resection/
Sling
Sling
Fair Poor
Table 3. Decision making in ptosis surgery
AIO Journal of Ophthalmology
18 AIO Journal of Ophthalmology
REVIEW
Surgical Procedures
Fasanella Servat procedure:
Principle: The upper border of tarsus is excised with the
lower part of Mullers muscle and the conjunctiva (Fig 11).
Indications:
l Mild congenital ptosis with a levator function better
than 10 mm
l Horners syndrome
l Mild involutional ptosis if not associated with frank
aponeurotic weakness.
Levator resection:
The extent of the resection is modifed by the degree of
the ptosis.
Levator function Resection
8 10 mm 14 18 mm
6 7 mm 18 22 mm
4 5 mm 22 26 mm
If the superior rectus muscle is weak, the resection should
be increased by about 4 mm.
Anterior approach levator resection:
Principle
The aponeurosis is ap-
proached through a skin
incision.
The muscle is shortened
and sutured to the tarsus.
The skin crease reformed
with sutures which pick
up the underlying levator
muscle (Fig 12).
Fig 11A, Fig 11B and Fig 11C Fasanella Servat procedure
Indications
A ptosis with 4 mm or more of levator function;
Lid exploration;
Maximum levator resection;
Preservtion of tarsus and conjunctiva;
Skin crease defect.
Aponeurosis surgery:
It is indicated for patients with an aponeurotic defect and
good levator function i.e. usually better than 10 mm. Intra
operatively, the lid is set at the same level or a little higher
than the other side.
Anterior approach aponeurosis repair:
Principle
The aponeurosis is approached through a skin incision. It
is advanced and sutured to the tarsus or the defect is re-
paied directly. Excess skin is excised and the skin crease
reformed with sutures which pick up the underlying
aponeurosis.
Indications
Repair or exploration of an aponeurotic defect, especially
if there is an excess skin to be excised.
Brow suspension
Principle
The frontalis muscle normally lifts the eyebrow and con-
tributes to eyelid elevation. This action of lifting the eyelid
is enhanced by connecting the frontalis muscle and eye-
brow to the eyelid with a subcutaneous sling for which
various materials can be used. A unilateral sling will always
produce asymmetrical result on downgaze. For a defnitive
cosmetic procedure it is better to do a bilateral suspension
with weakening of other levator muscle if this is normal.
Fig 12: Ligation of LPS during
LPS resection surgery
AIO Journal of Ophthalmology 19
Blepharoptosis
Indications
Ptosis with less than 4 mm of levator function;
The prevention of amblyopia in an infant with severe pto-
sis in whom an assessment of levtor function is not pos-
sible;
Following a levator excision.
Levator division or excision:
Principle
The action of the levator muscle is abolished by dividing
or excising it.
Indications
The abolition of aberrant eye movement e.g. jaw winking.
To promote the use of frontalis muscle and create sym-
metry in patients undergoing a bilateral brow suspension
procedure for a unilateral ptosis.
Post Operative Complications:
Exposure keratitis.
Undercorrection
Overcorrection
Contour defects, notching of lid margin.
Lash ptosis and entropion
Lash eversion and ectropion
Defective skin crease
Conjunctival prolapse
Symblepharon
Extra ocular muscle involvement (Strabismus)
Haemorrhage
Infection.
Congenital ptosis with superior rectus weakness:
If the eye is straight in the primary position of gaze but the
superior rectus muscle is weak, more extensive ptosis sur-
gery is required to lift the lid by a given amount since the
superior rectus muscle normally contributes to upper lid
movement via its common sheath with the levator mus-
cle. When a levator resection is performed, an extra 4 mm
of levator muscle is arbitrarily resected. If the eye is hypo-
tropic in the primary position, it must be elevated before
the ptosis correction is undertaken.
Blepharophimosis:
l The treatment of the ptosis depends on the levator
function. This is usually poor and bilateral brow sus-
pension procedure is required. The epicanthus and
telecanthus should be corrected 6 months prior to
ptosis surgery. For telecanthus trans nasal wiring or
medial canthoplasty can be done.
Marcus Gunn jaw winking Synkinesis:
l If the ptosis is the main problem, it can be corrected
with the ptosis surgery based on levator function.
If the aberrant movement of the eyelid is the
main problem, it can be corrected by leva-
tor excision and brow suspension procedure.
This can be done bilaterally to obtain the most sym-
metrical result.
References
l Orbits, eyelids and lacrimal system. Section 7. Basic
and clinical science course. Edition: 2000-2001. The
Foundation of the American Academy of Ophthal-
mology. Blepharoptosis. Pages: 189 204.
l J.R.O. Collin. Manual of systematic eyelid surgeries.
2nd Edition. Butterworth Heinemann. Ptosis. Pages:
41 72.
l Homblass A (Ed). Oculoplastic, Orbital, and recon-
structive surgery, 1988, Williams & Wilkins, Balti-
more, MD
l Ptosis, Chapter 24, Repair and Reconstruction of or-
bital region, C.Beard and J.C. Mustarde, pages 411-
443. l
Dr. Kunal Kumar, DO, FMRF - (Orbit, Oculoplasty &Reconstructive Surgery)
Sankara Netralaya., Chennai is currently working as Orbit & Oculoplastic
surgeon in the Al Salama group of Eye Hospitals Calicut & Perinthalmanna
20 AIO Journal of Ophthalmology
REVIEW
Recurrent Corneal Erosion Syndrome
Etiology
R
ecurrent corneal erosion is a disorder of the eyes
characterized by the failure of the cornea's outer-
most layer of epithelial cells to attach to the under-
lying basement membrane (Bowman's layer). Defective
adhesion of the epithelium to the Bowman's membrane
causes recurring cycles of epithelial breakdown. Multiple
recurrences are common, because the epithelial cells re-
quire at least 8-12 weeks regenerating or repairing the
epithelial basement membrane .The condition is excruci-
atingly painful because the loss of these cells results in the
exposure of sensitive corneal nerves.
Typically, recurrent corneal erosion syndrome (RCES)
develops 3-10 days after the insult but in some cases, it
may not develop until several months later. There is often
a history of previous corneal injury (corneal abrasion or
ulcer), but also may be due to corneal dystrophy or cor-
neal diseases. In other words, one may sufer from corneal
erosions as a result of another disorder, such as map dot
fngerprint disease. [1]
The common causes that trigger the process
of are:
a) superfcial trauma to the cornea including corneal
abrasions due to contact lens wearing
b) A clean cut abrasion (such as a paper cut) is more
likely to cause the problem than a ragged abrasion.
c) Other types of trauma may include alkali burns, for-
eign bodies and exposure keratopathy,entropion,ectropio
n,blepharitis,lagophthalmos.
There may be more unusual trigger factors (all involv-
ing disruption of the epithelial basement membrane) such
as following cataract surgery or refractive surgery. Other
iatrogenic causes include vitrectomy (surgical removal of
the vitreous) and photocoagulation.[2]
Systemic diseases which may predispose to this
condition include
l Diabetes[1]
l Junctional epidermolysis bullosa
l Alport's syndrome
l Mnchausen's syndrome
l Malnutrition
Drugs associated with the development of RCES
include
l Thiomersal (found in contact lens solutions).
l High-dose topical neomycin.
l Topical paromomycin.
l Topical diamidines and propamidine.
To a certain extent, all topical anaesthetic medication
causes some degree of epithelial cell damage but this is
most marked with cocaine, which is associated with RCES
more than others. A variety of RCES associated with au-
tosomal dominant inheritance has been described but is
rare.
Epidemiology [2]
l There are no accurate statistics relating to the exact
incidence and prevalence of this condition as it often
goes undiagnosed or misdiagnosed.
l It tends to occur slightly more commonly in females.
l It is a problem seen in adulthood, usually from the
fourth decade of life onwards, unless it is associated
with one of the corneal dystrophies (eg Reis-Bcklers
dystrophy) or Alport's syndrome, in which case it can
arise in children.
Symptoms
Symptoms include recurring attacks of severe acute ocular
pain, foreign-body sensation, photophobia (i.e. sensitivity
to bright lights, this occurs if corneal oedema develops)),
and tearing often at the time of awakening or during sleep
when the eyelids are rubbed or opened, decreased visual
acuity. In its early stages, the condition may be asympto-
matic. Later, there may be recurrent attacks of acute pain
(typically towards the end of sleep or on waking) associ-
ated with lacrimation, photophobia and a foreign body
sensation. There may be an associated blepharospasm
(inability to open the lids). The problem may be unilateral
Dr. Divya Menon, D.O.M.S., FICO
AIO Journal of Ophthalmology 21
RCES
or bilateral. Symptoms may gradually subside over the
course of the day and then start all over again the next
morning. The unpredictability of these episodes may lead
to an associated anxiety.
Signs
Signs of the condition include corneal abrasion or lo-
calized roughening of the corneal epithelium, some-
times with map-like lines, epithelial dots or micro-
cysts, or fngerprint patterns. An epithelial defect may
be present, usually in the inferior interpalpebral zone.
Loose edges, intraepithelial cysts,stromal
edema,blepharospasm. (Fig 1)
Diagnosis
Diagnosis is made on history and examination. Severe cas-
es may warrant corneal topography to outline the degree
of corneal damage or help in the diagnosis of a corneal
dystrophy (most of which are diagnosed on examination).
The erosion may be seen by a doctor using the magnifca-
tion of a slit lamp biomicroscope , although usually fuo-
rescein stain must be applied frst and a blue-light used.
(Fig 2). In vivo confocal microscopy and videokeratogaphy
are other methods of diagnosis.
Management of episodes
The type of tears being produced has little adhesive prop-
erty. Water or saline eye drops tend to be inefective. Rath-
er a 'better quality' of tear is required with higher 'wetting
ability' (i.e. greater amount of glycoproteins) and so artif-
cial tears are applied frequently. Individual episodes may
settle within a few hours or days but additional episodes
(as the name suggests) will recur at intervals.
The easy remedy for recurrent attacks is to apply a
pressure patch.
Prevention
Since episodes tend to occur on awakening and managed
by use of good 'wetting agents', approaches has to be tak-
en to help prevent episodes .Prophylaxis revolves around
long-term lubrication in the frst instance, eg night-time
lubricating ointment for three months.[7] The success of
this approach depends on the patient understanding the
importance of continuing this treatment in the absence
of symptoms. More severe cases may require a protective
bandage contact lens.
There is evidence to suggest that a 12-week course of
systemic tetracyclines (eg oxytetracycline 250 mg bd) may
be benefcial (they inhibit matrix metalloproteinase activ-
ity and promote epithelial stability)
Fig 1 Fig 2
Prevention includes
a) Environmental
Ensuring that the air is humidifed rather than dry, not
overheated and without excessive airfow over the face.
Also avoiding irritants such as cigarette smoke.[2]
l Use of protective glasses especially when gardening
or playing with children.[2]
b) General personal measures
l Maintaining general hydration levels with adequate
fuid intake.[2]
l Not sleeping-in late as the cornea tends to dry out
the longer the eyelids are closed.[2]
c) Pre-bed routine
l Routine use of long-lasting eye ointments applied
before going to bed.[2]
l Occasional use of the anti-infammatory eyedrops
before going to bed if the afected eye feels infamed,
dry or gritty
l Use of a hyperosmotic ointment before bed reduces
the amount of water in the epithelium, strengthen-
ing its structure
l Use the pressure patch as mentioned above.
d) Waking options
l Learn to wake with eyes closed and still and keeping
artifcial tear drops within reach so that they may be
instilled under the inner corner of the eyelids if the
eyes feel uncomfortable upon waking.[2]
l It has also been suggested that the eyelids should be
rubbed gently, or pulled slowly open with your fn-
gers, before trying to open them, or keeping the af-
fected eye closed while "looking" left and right to help
spread lubricating tears. If the patient's eyelids feel
stuck to the cornea on waking and no intense pain is
present, use a fngertip to press frmly on the eyelid to
push the eye's natural lubricants onto the afected
22 AIO Journal of Ophthalmology
REVIEW
area. This procedure frees the eyelid from the cornea
and prevents tearing of the cornea.[2]
Surgical
A punctal plug may be inserted into the tear duct by an
ophthalmologist, decreasing the removal of natural tears
from the afected eye. [5]
The use of thin contact lenses may help prevent the
abrasion during blinking. However they need to come for
frequent follow-up visits because there is an increased risk
of infection.[2]
Alternatively, under local anaesthetic, the loose cor-
neal layer may be gently removed with a fne needle and
cautery (heat or laser) or 'spot welding can be attempted
with laser. The procedures are not guaranteed to work,
and in a minority may exacerbate the problem.
Anterior Stromal Puncture is an efective and simple
treatment. This is a minor procedure carried out under
topical anaesthesia in the eye clinic. Numerous tiny punc-
tures are made away from the central visual axis, which
induces cicatrisation and promotes more permanent epi-
thelial adherence. It may be performed using Nd-YAG la-
ser.[5] or 24-26G needle.
An option for minimally invasive and long-term efec-
tive therapy [6] is laser phototherapeutic keratectomy.
Laser PTK involves the surgical laser treatment of the cor-
nea to selectively ablate cells on the surface layer of the
cornea. It is thought that the natural regrowth of cells in
the following days are better able to attach to the base-
ment membrane to prevent recurrence of the condition.
Laser PTK has been found to be most efective after epi-
thelial debridement for the partial ablation of Bowman's
lamella[7], which is performed prior to PTK in the surgical
procedure. This is meant to smoothen out the corneal area
that the laser PTK will then treat. In some cases, small-spot
PTK[8], which only treats certain areas of the cornea may
also be an acceptable alternative
.Keratectomy is the removal of corneal tissue and is
reserved for severe and difcult cases (usually in patients
with associated corneal dystrophies).[3] It involves either
laser treatment or surgical removal (knife, diamond burr):
the entire epithelium is removed and allowed to regrow
from new over the following 5-7 days. It can be carried out
more than once if the problem recurs. It can also be com-
bined with a refractive correction.
Presence of blepharitis,meibomitis,and flaments over
the corneal surface should be treated accordingly.
30-60 days autologous serum drops can be given. We
have to educate the patient regarding the refrigeration
and sterile preservation of the drops .
Alcohol delamination is a newer procedure that has
shown good efcacy and safety.[6]
Medical
Patients with recalcitrant recurrent corneal erosions often
show increased levels of matrix metalloproteinase (MMP)
enzymes.[9] These enzymes dissolve the basement mem-
brane and fbrils of the hemidesmosomes, which can lead
to the separation of the epithelial layer.
Treatment with oral tetracycline antibiotics (such as
doxycycline or oxytetracycline) together with a topical
corticosteroid (such as prednisolone), reduces MMP activ-
ity and may rapidly resolve and prevent further episodes
in cases of unresponsive to conventional therapies.[10]
[11] Some have now proposed this as the frst line therapy
after lubricants have failed.[3]
Assessment
l Check the patient's visual acuity (a drop of local an-
aesthetic will temporarily relieve the pain and the as-
sociated blepharospasm).
l Examine as much of the anterior segment of both
eyes (even if symptoms are unilateral). This includes
fuorescein staining of the cornea. The idea is to rule
out other possible diferentials .
Differential diagnosis
l Corneal abrasion.
l Corneal foreign body.
l Contact lens-related problem.
l Dry eye syndrome.
l One of a variety of corneal dystrophies.
l Floppy eyelid syndrome.
l Herpes simplex keratitis.
l Other form of keratitis.
Recurrent corneal erosion syndrome (RCES) can also occur
following prolonged eye patching.
Staging
There is no formal staging of recurrent corneal erosion
syndrome (RCES) but a note is made of the depth of cor-
neal involvement, as this dictates treatment options and
prognosis.
Management
First if there is an abrasion, cover with an antibiotic (such
as a topical fuoroquinolone given as directed in the pre-
scribing information) and a bandage contact lens until
healed. Once the cornea is reepithelialized, the following
AIO Journal of Ophthalmology 23
RCES
regimen can be followed for the treatment of RCE.
a) 5%hypertonic saline (5% NaCl) ointment at bedtime
for 2 months. This helps reduce overnight edema and
improves epithelial adherence just prior to the morn-
ing (when most RCEs occur).
b) Preservative free lubricating drops 4 times a day.
c) (loteprednol etabonate 0.5%) or fuoromethalone four
times a day for 2 weeks, then twice a day for 6 weeks
(check intraocular pressure within 1 month).
d) Oral doxycycline (either 20mg or 50 mg) twice a day for
2 months.
Long-term, management, especially in cases of epithelial
basement membrane dystrophy, should include:
a) Cyclosporine 0.05% twice a day
b) Artifcial tear substitution
c) Omega-3 free fatty acid supplementation
d) Lubricating eye ointment for 2 months during night
time. The negative pressure in the sac is neutralised
with this.
e) Soft BCL for 2-3 weeks.
Note: Avoid topical steroid till the epithelium is healed .
Complications
l Infectious keratitis.
l Corneal scarring.
l Decreased visual acuity (due to either of the above).
Prognosis
With the right treatment, generally the prognosis is good
with most patients responding well to topical treatment.[2]
This relies on any underlying conditions being diagnosed
and treated and good patient education on the long-term
management. Few patients will have their vision perma-
nently afected.
further reading & references
1. Kanski J. Clinical Ophthalmology; A Systematic Ap-
proach (5th Ed) Butterworth Heinemann (2003)
2. Recurrent corneal abrasions/erosion, Kinshuck D, Good
Hope Eye Department, Heart of England NHS Founda-
tion Trust; (excellent patient resource with good dia-
grams)
3. Ramamurthi S, Rahman MQ, Dutton GN, et al; Patho-
genesis, clinical features and management of recurrent
corneal erosions. Eye (Lond). 2006 Jun;20(6):635-44.
Epub 2005 Jul 15.
4. Verma A et al, Corneal Erosion, Recurrent, Medscape,
Jun 2009
5. Ewald M, Hammersmith KM; Review of diagnosis and
management of recurrent erosion syndrome. Curr
Opin Ophthalmol. 2009 Jul;20(4):287-91.
6. Watson SL, Barker NH; Interventions for recurrent
corneal erosions. Cochrane Database of System-
atic Reviews 2007, Issue 4. Art. No.: CD001861. DOI:
10.1002/14651858.CD001861.pub2.
7. Tsai TY, Tsai TH, Hu FR, et al; Recurrent corneal ero-
sions treated with anterior stromal puncture by neo-
dymium: Ophthalmology. 2009 Jul;116(7):1296-300.
Epub 2009 May 8.
8. Singh RP, Raj D, Pherwani A, et al; Alcohol delamina-
tion of the corneal epithelium for recalcitrant recur-
rent corneal Br J Ophthalmol. 2007 Jul;91(7):908-11.
Epub 2007 Feb 14.
9. Das S, Seitz B; Recurrent corneal erosion syndrome.
Surv Ophthalmol. 2008 Jan-Feb;53(1):3-15
10. Review of Ophthalmology, Friedman NJ, Kaiser PK,
Trattler WB, Elsevier Saunders, 2005, p. 221
11. Verma A, Ehrenhaus M (August 25, 2005) Corneal Ero-
sion, Recurrent at eMedicine
12. Wang L, Tsang H, Coroneo M (2008). "Treatment
of recurrent corneal erosion syndrome using the
combination of oral doxycycline and topical cor-
ticosteroid". Clin. Experiment. Ophthalmol. 36 (1):
812. doi:10.1111/j.1442-9071.2007.01648.x. PMID
18290949.
13. Liu C, Buckley R (January 1996). "The role of the ther-
apeutic contact lens in the management of recurrent
corneal erosions: a review of treatment strategies".
CLAO J 22 (1): 7982. PMID 8835075.
14. Tai MC, Cosar CB, Cohen EJ, Rapuano CJ, Laib-
son PR (March 2002). "The clinical efcacy of sili-
cone punctal plug therapy". Cornea 21 (2): 1359.
doi:10.1097/00003226-200203000-00001. PMID
11862081.
15. Baryla J, Pan YI, Hodge WG (2006). "Long-term ef-
cacy of phototherapeutic keratectomy on recurrent
corneal erosion syndrome.". Cornea 25 (10): 1150
1152. doi:10.1097/01.ico.0000240093.65637.90.
PMID 17172888.
16. Kampik D, Neumaier K, Mutsch A, Waller W, Geerling
G (2008). "Intraepithelial phototherapeutic keratec-
tomy and alcohol delamination for recurrent corneal
erosions--two minimally invasive surgical alterna-
tives.". Klinische Monatsbltter fr Augenheilkunde
AIO Journal of Ophthalmology 35
Posterior Embryotoxon
with Alagille syndrome.
4, 5
Most children are evaluated when younger than 6
months for either neonatal jaundice (70%), or cardiac mur-
murs and symptoms (17%). Patients who are less afected,
such as family members, are often diagnosed after an index
case. Ocular abnormalities are common
9
. The most frequent
ophthalmologic fnding is a posterior embryotoxon, which
was observed in more than 75% of patients in one large se-
ries conducted by Emerick et al
10
.
Lab work up demonstrates fat-soluble vitamin defcien-
cies, Prolongation of prothrombin time (PT) or activated
partial thromboplastin time (aPTT), Hypercholesterolemia
(>200 mg/dL) and hypertriglyceridemia (500-2000 mg/dL),
raised Gamma-glutamyl transpeptidase (GGT) and alkaline
phosphatase levels, elevated Serum bile acids .Diagnostic
tests include-abdominal USG for abnormal hepatobiliary
tree pattern and hepatic and renal parenchyma. Chromo-
somal analysis for mutations within the JAG1 gene (20p12)
confrms diagnosis of Alagille syndrome
Isolated PE
Shields
9
showed no increased risk for glaucoma in the pres-
ence of isolated PE, but the association of other anterior
segment abnormalities with glaucoma is well recognised.
The extent of PE viewed externally does not predict the
presence of any underlying condition, and no link between
refractive error, astigmatism, and the position of PE has
been demonstrated . The presence of PE may be helpful
in putting together the features of Alagille syndrome syn-
drome, but it is not diagnostic and would not necessarily
prevent the need for more invasive investigations.
In one study the prevalence of PE in the general oph-
thalmic clinic was found to be 6.8%. No particular features of
PE suggest that it is pathological rather than physiological,
but the presence of other anterior segments abnormalities
is signifcant. The presence of PE should prompt careful an-
terior segment examination and gonioscopy. It is important
to identify any anterior segment syndromes, such as Axen-
feldReiger, that will require long-term follow-up because
of the risk of glaucoma. Children seen in the ophthalmic
clinic with PE should be carefully examined for evidence of
anterior segment dysgenesis, and any associated systemic
or genetic abnormalities. A family history should be sought
and because of the variability of expression an examination
of parents and siblings considered.
References
1. Waring GO, Rodrigues MM & Laibson PR. Anterior cham-
ber cleavage syndrome. Surv Ophthalmol 1975; 20: 327.
2. Azuma N & Yamada M. Missense mutation at the C termi-
nus of the PAX6 gene in ocular anterior segment anoma-
lies. Invest Ophthalmol Vis Sci 1998; 39: 828830.
3. Forsius H, Eriksson A & Fellman J. Embryotoxon corneae
posterius in an isolated population. Acta Ophthamol
1964; 42: 424
4. Alkemade P. Dysgenesis Mesodermalis of the Iris and the
Cornea Charles C Thomas: Assen, Netherlands 1969;.
5. Ozeki H, Shirai S, Majima A, Sano M & Ikeda K. Clinical
evaluation of posterior embryotoxon in one institution.
Jpn J Ophthalmol 1997; 41: 422425
6. Hingorani M, Nischal KK, Davies A, Bentley C, Vivian A &
Baker AJ et al.. Ocular abnormalities in Alagille syndrome.
Ophthalmology 1999; 106: 330337. |
7. Burian HM, Braley AE & Allen L. Visibility of the ring of
Schwalbe and the trabecular zone. Arch Ophthalmol
1955; 53 (6): 767782. Johnston MC, Noden DM & Hazel-
ton RD. Origins of avian ocular and periocular tissue. Exp
Eye Res 1979; 29: 2743.
8. Shields MB. AxenfeldRieger syndrome: theory of mech-
anism & distinctions from the iridocorneal endothelial
syndrome. Trans Am Ophthalmol Soc 1983; 81: 73678
9. Alward LWM. AxenfeldRieger syndrome in the age of
mol. genetics. Am J Ophthalmol 2000; 130: 107115.
10. Hanson IM, Fletcher JM, Jordan T, Brown A, Taylor D & Ad-
ams RJ et al.. Mutations at the PAX6 locus are found in
heterogeneous anterior segment malformations includ-
ing Peter's anomaly. Nat Genet 1994; 6: 168173.
11. Jones EA, Clement-Jones M & Wilson DI. JAGGED1 expres-
sion in human embryos: correlation with the Alagille syn-
drome phenotype. J Med Genet 2000; 37: 658662. l
Dr. Maneesh Shyamkul
M.S. (Ophthalmology), Fellow LVPEI is currently working as Consultant in
Pediatric Ophthalmology in the Al Salama group of Eye Hospitals Calicut &
Perinthalmanna
36 AIO Journal of Ophthalmology
CASE KORNER
Ankyloblepharon Filiforme Adnatum
Dr. Maneesh Shyamkul, M.S. (Ophthalmology), Fellow LVPEI
A
nkyloblepharon Filiforme Adnatum is a rare con-
genital anomaly wherein the lid margins are con-
nected by fne bands of extensile tissue which
reduce the palpebral fssure by interfering with the move-
ments of the lids. ( Figure 1)
Ankyloblepharon fliforme adnatum can present as an
isolated fnding,, or as part of a well-defned syndrome . We
report a case of ankyloblepharon fliforme adnatum in a
newborn, with congenital anomalies.
Introduction
The developing eyelid margins remain fused until the ffth
month of gestation, but may not be completely separated
until the seventh month [1].anomalies occurring between
7 -15 weeks may give rise to eyelid anomalies. Anky-
loblepharon fliforme adnatum (AFA) is a rare benign con-
genital anomaly which is characterised by partial or com-
plete fusion of the lid margins. However, it can occur in an
autosomal dominant pattern associated with cleft lip and
palate.
Case presentation
Baby R., female, aged 25 days presented at the outpatient
section of Alsalama Eye Hospital ,Calicut Kerala on 16th
January 2012. This baby was unable to open her eyes as
both the lids were adherent on both sides since birth.
Baby 'R' is the frst child of the parents and is a full term
baby with normal delivery. The pregnancy was normal and
the mother denied having taken any drugs or being ex-
posed to X rays .
family history
Father aged 30yrs Years and mother 26 yrs are healthy and
without any congenital abnormality. There was no family
history of congenital anomalies or consanguinity.
Ophthalmic examination
Revealed multiple fne bands of elastic tissue connecting
the upper and lower lid margins (Figure 1,2,3,4). The elastic
adhesions were divided with a Wescott conjunctival scissors
without any bleeding. The posterior surfaces of the eye-
lids appeared normal, as were ocular movement, anterior
segment,retinoscopy and fundus examination. Neither se-
dation nor local anaesthetic
was necessary. The vestigial
remnants on the eyelid mar-
gins disappeared complete-
ly within 48 hours.
Histological
examination
The band was composed of
central vascular connective tissue surrounded by squamous
epithelium. The connective tissue was highly cellular. No
muscle fbers or signs of infammation could be found.
Discussion:
AFA was frst described by Josef von Hasner in 1881, and
is characterized by partial or complete adhesion of the cili-
ary edges of the upper and lower eyelids [2]. Usually, AFA
constitutes a solitary malformation of sporadic occurrence.
However, it can occur in an autosomal dominant pattern
associated with cleft lip and palate. In addition, it has been
reported in the context of trisomy 18 (Edwards syndrome),
Hay-Wells syndrome (a variant of the ectodactylyecto-
dermal dysplasia-cleft lip palate syndrome), the popliteal
pterygium syndrome (characterized by intercrural webbing
of the lower limbs), and CHANDS (curly hair-ankyloblepha-
ron-nail dysplasia). Other associations may include hydro-
cephalus, meningocoele, an imperforate anus, bilateral
syndactyly, infantile glaucoma and cardiac problems such
as patent ductus arteriosus and ventricular
septal defects [3].
Neuman and Shulman (1961) described a 3-day-old boy
with AFA who had in addition adhesions between the alve-
olar processes of both upper and lower jaws, a papilla and a
sinus on the left side of the lower lip, and a cleft palate(6). Bi-
lateral syndactyly of the fourth and ffth toes, partial adher-
ence of the left ear to the skin of the head, and a ventricular
septal defect of the membranous type with a patent ductus
arteriosus were associated abnormalities reported by Rog-
ers (1961) in a premature baby(7). A 'familial' tendency was
invoked by Howe and Harcourt (1974), who described the
condition in identical twins, and by Khanna (1957), who
reported the condition in sisters, one of whom had cleft
lip and palate(8). Hecht and Jarvinen (1967) described 2
Figure 1
AIO Journal of Ophthalmology 37
Ankyloblepharon Filiforme Adnatum
families in whom a mother and
2 of her children were afected.
They noted wide variability of
expression of the syndrome and
postulated autosomal domi-
nant inheritance(9). Gorlin et al.
(1968) also suggested that the
syndrome was inherited as an
autosomal dominant trait with
incomplete penetrance and variable expressivity(10).
The aetiology of AFA is unknown, but failure of ap-
optosis at a critical stage in eyelid development has been
suggested [4]. Timely separation of the eyelids is crucial to
avoid the development of occlusion amblyopia.
Conclusion
A case of Ankyloblepharon Filiforme Adnatum associated
with cleft-lip and cleft palate which had no hereditary back-
ground is reported. The relevant literature is reviewed.
This case report also demonstrates the simplicity in treating
AFA; and highlights that its presence should alert the clini-
cian to the possibility of an underlying congenital disorder.
References
1. Sharkey D, Marlow N, Stokes J: Ankyloblepharon fli-
forme adnatum. J Pediatr 2008, 152:594.
2. Jain S, Atkinson AJ, Hopkisson B: Ankyloblepharon fli-
forme adnatum. Br J Ophthalmol 1997, 81:708.
3. Williams MA, White ST, McGinnity G: Ankyloblepharon
fliforme adnatum. Arch Dis Child 2007, 92:73-74.
4. Mohamed YH, Gong H, Amemiya T: Role of apoptosis
in eyelid development. Exp Eye Res 2003, 76:115-123.
5. Duke-Elder, S. (1964). System of Ophthalmology, vol. 3,
part 2, p. 869.
6. Neuman, Z., and Shulman, J. (1961). Congenital sinus-
es of the lower lip. Oral Surgery, 14, 1415-1420
7. Rogers, J. W. (1961). Ankyloblepharon fliforme adna-
tum.Archives of Ophthalmology, 65, 114-117. Sood, N.
N., Agarwal, T. P., and Ratnaraj, A. (1968).Ankyloblepha-
ron fliforme adnatum with cleft lip and palate. Journal
of Pediatric Ophthalmology, 5, 30-32
8. Khanna, V. N. (1957). Ankyloblepharon fliforme adna-
tum.American Journal of Ophthalmology, 43, 774-777.
9. Hecht, F., and Jarvinen, J. M. (1967). Heritable dysmor-
phic syndrome with normal intelligence. Journal of
Pediatrics, 70, 927-935.
10. Gorlin , R. J., Cervenka, J., and Pruzansky, S. (1971). Fa-
cial clefting and its syndromes. Birth Defects: Original
Article Series, 7, 15.
11. Howe, J., and Harcourt, B. (1974). Ankyloblepharon fli-
forme adnatum afecting identical twins. British Jour-
nal of Ophthalmology, 58, 630-632.
12. Judge, H. V., Mott, W. C., and Gabriels, J. A. C. (1929).
Ankyloblepharon fliforme adnatum. Archives of Oph-
thalmology,2, 702-708.
13. Kazarian, E. L., and Goldstein, P. (1977). Anky-
loblepharon fliforme adnatum with hydrocephalus,
meningomyelocele,and imperforate anus. American
Journal of Ophthalmology,84, 355-357.
14. Gorlin, R. J., Sedano, H. O., and Cervenka, J. (1968).Pop-
liteal pterygium syndrome. Pediatrics, 41, 503-509
15. Long, J. C., and Blandford, S. E. (1962). Ankyloblepha-
ron fliforme adnatum with cleft lip and palate. Ameri-
can Journal of Ophthalmology, 53, 126-129.
16. Ankyloblepharon fliforme congenitum associated
with harelip and cleft palate. Acta Ophthalmologica,
48, 465-467.
17. Escobar, V., and Weaver, D. (1978). Popliteal pterygium
syndrome: a phenotypic and genetic analysis. Journal
of Medical Genetics, 15, 35-42 l
Figure 2 Figure 3
Dr. Maneesh Shyamkul
M.S. (Ophthalmology), Fellow LVPEI is currently working as Consultant in Pediatric
Ophthalmology in the Al Salama group of Eye Hospitals Calicut & Perinthalmanna
Figure 4
KNOW THY INSTRUMENT
38 AIO Journal of Ophthalmology
Optical Coherence Biometry: How Coherent is the Master?
Dr. K. Preetha, M.S. (Ophthalmology)
C
ataract surgery, in recent times, has undergone a
paradigmal shift entering into the realm of refrac-
tive surgeries with the emphasis now being on
achieving the desired refractive outcome in a consistent,
reproducible & accurate fashion. Sutureless incisions, state
of the art phacoemulsifcation systems, improved fuidics,
premium IOLs (with increasingly sophisticated IOL de-
signs, IOLs in 0.25-diopter steps, aspheric designs for pow-
ers as high as +40.0 diopters, and the limited correction of
higher-order aberrations) have ushered in a new era in cat-
aract extraction. However without an accurate IOL power
calculation, an appreciation of these advancements is lost.
visual outcome after surgery
The refractive outcome after cataract surgery is depend-
ent on several factors, including axial length, keratometry,
anterior chamber depth, IOL power calculation formula,
optimized lens constant, and confguration of the capsu-
lorhexis. The preoperative measurement of axial length
(AL) is considered to be the key determinant in calculating
the IOL power to be implanted with 54% of postoperative
refractive surprises being attributed to axial length errors
1
If keratometry is inaccurate by 0.50 diopters, then the
fnal refractive outcome will be inaccurate by that same
amount. If the capsulorhexis is made larger than the optic
of the IOL, an anterior shift in position may occur during
capsular bag contraction, resulting in more postoperative
myopia than anticipated. Optimization of IOL constants
and the IOL power calculation formulae are the other pa-
rameters that also infuence the refractive outcome. Errors
in axial length measurement by 0.1mm will cause 0.3 di-
opters of IOL power variation.
2
Biometry over the ages
The history of IOL power calculation began in 1949 when
Harold Ridley implanted the rst IOL in a blind eye. The
surgery was reported to be successful (the patient still
could not see) but the refractive error was found to be -18
DS/-6DC X 120.
3
Since those pioneering days, biometry
has evolved over time, from putting in a standard IOL of
fxed power to the precise calculation of IOL power in each
eye. A-mode contact ultrasound ocular biometry has been
considered the gold standard for decades for AL and ACD
measurement.
4, 5
Optical biometers are new entrants in the feld with
the laser partial coherence interferometry (PCI) based
IOLMaster (Carl Zeiss Meditec AG, Jena, Germany) being
introduced in 1999. More recently, a new biometry device,
the Lenstar LS 900 (Haag Streit AG, Bern, Switzerland) us-
ing low-coherence optical refectometry (LCOR) technol-
ogy was introduced in 2008. This article is a brief overview
of the IOL master recently acquired at Al Salama Calicut
ultrasound vs optical biometry
4
A-scan ultrasonography, with a longitudinal resolution of
approximately 200 m and an accuracy of approximately
100150 m, is routinely employed in the measurement of
the ocular Axial Length (AL). A special crystal embedded
in a probe oscillates to generate a high-frequency sound
wave that penetrates the eye. This results in a one-dimen-
sional time-amplitude representation of echoes received
along the beam path. The distance between the echo
spikes provides an indirect measurement of tissue such as
globe length or lens thickness (LT). The height of the spike
is indicative of the strength of the tissue sending back the
echo. There are two types of A-mode ultrasound biometry
available, including contact applanation biometry and im-
mersion biometry. Contact type biometry requires a probe
placed on the cornea and is prone to errors due to corneal
Figure 1 Figure 3
AIO Journal of Ophthalmology 39
Optical Coherence Biometry
indentation and of-axis measurements besides carrying
the risk of transmitting infections. Immersion type biome-
try requires placing a saline flled scleral shell between the
probe and the eye. As no pressure is applied on the eye,
corneal indentation is prevented. Although diferences in
the AL between immersion and applanation ultrasonogra-
phy up to 0.36 mm have been reported, owing to pressure
exerted by the transducer during applanation ultrasonog-
raphy, the latter continues to be very commonly used.
6
Perhaps the greatest single step forward in biometry
measurement has been the switch from measuring dis-
tance using ultrasound to measuring using light. Optical
biometry uses a 780-m light wave that has eight to nine
times the resolution of a 10-MHz sound wave & measure-
ment accuracy is increased from 0.10 mm to between 0.02
mm and 0.01 mm, an improvement of approximately fve
times
Its precision of about 20 pm is unrivalled by the
US technique. The total accuracy is better (< 50 nm com-
pared to 120 to 200 nm) which is operator independent; it
is easy to use, less time consuming and very comfortable
for the patient. It is a noncontact technique that requires
no anesthesia and avoids the risk of corneal infection.
5
However, signifcant media opacities limit the use of
IOL Master. PCI has a signifcant failure rate, particularly
in the presence of dense cataracts, besides in certain ran-
domized controlled trials the calculation of IOL power
based on ocular AL measurement using PCI technology
provided no clinical advantage over conventional AUS, as
measured by postoperative refractive outcome
8
.
IOL Master the frst commercial version of Optical co-
herence biometry has been in clinical practice for over a
decade now
9, 10
. The IOL master 500 is the latest version of
the biometer launched in 2011 with better technical speci-
fcations .( Figure 1)
Principle
7, 11, 13
IOL Master employs the principle of Optical coherence
Biometry (OCB). It uses partially coherent infrared light
beams of 780 nm diode laser with coherence length of
130 micron
11
. Special feature incorporated is use of dual
beams. In interferometer eye needs to be absolutely sta-
ble so as not to disturb interference patterns. Use of dual
beams makes IOL Master insensitive to longitudinal move-
ments and measurements can be made with ease. Laser
light emitted is split up into two beams in a modifed
Michelson Interferometer. One mirror of interferometer is
fxed and other is moved at constant speed making one
beam out of phase with other. Both beams are projected
in the eye and get refected at cornea and retina. The light
Figure 2
refected from the cornea interferes with that refected by
the retina if the optical paths of both beams are equal. This
interference produces light and dark band patterns which
is detected by a photo detector. The signals are amplifed,
fltered and recorded as a function of the position of the
interferometer mirror. An optical encoder is used to con-
vert the measurements into axial length measurements .
(fgure 2) OCB measures the axial length from corneal apex
to retinal pigmentary epithelium while A scan measurers
up to vitro retinal interface only. IOL Master thus gives the
true refractive length rather than anatomical axial length.
Furthermore, it measures the corneal curvature, the
anterior chamber depth, and the corneal diameter and it
calculates the optimum IOL power by the acquired biom-
etry data, employing several IOL power calculation formu-
las built into its computer software.
Procedure
12,14,15,2
After entering patient data, overview mode (fgure 3)
is activated for coarse image alignment prior to taking
measurements. The patient is asked to look straight at
the small yellow fxation light and then the instrument-
to-patient distance is adjusted until the six light spots
(refex points on the patients pupil) appear to be in focus.
The small circle of lights and the cross hairs should be
approximately centered in the patients pupil.
In the IOL master 500 version a trafc light display changes
from red to yellow to green as the optimum measurement
position is reached. This optimum position is the starting
point for fne adjustments in the subsequent measurement
modes.
Axial Length Measurement Mode
Switching to ALM mode will automatically change the
LASER LIGHT
REFERENCE MIRROR
PHOTO
DETECTOR
AMPLIFIER
PCI
DISPLAY
AXL
ROTATING
MIRROR
R2
R
C
C2
C1
R1
BEAM
SPLIT
ER 2
BEAM
SPLIT
ER 1
40 AIO Journal of Ophthalmology
KNOW THY INSTRUMENT
magnifcation ratio: a
smaller section of the eye
becomes visible with the
refection of the align-
ment light and a vertical
line. Patient is asked to
look directly at the small
red fxation light. On the
display a cross hair with
a circle in the middle
appears. The operator
adjusts the focus of the
video image to get the
refection of the light in the centre of the cross hair (Figure
4). This alignment causes axial length measurements to
be made to the centre of the macula, giving the refractive
axial length, rather than the anatomic axial length. Meas-
urements with and without glasses are exactly the same.
Measurements through contact lenses lead to measure-
ment errors. If patient is using contact lens its use should
be discontinued at least 24 hrs prior to measurements. In
high refractive error (more than 6.00 D), measurement is
to be taken with the patient's glasses in place to ensure
adequate fxation. For eyes with high to extreme myopia,
with a type 1 peripapillary posterior staphyloma, being
able to measure to the fovea is an enormous advantage
over conventional Ascan ultrasonography.
To measure aphakic eyes, pseudophakic eyes flled
with silicone oil, corresponding mode is selected from the
AL settings menu. The instrument will automatically be re-
set to the phakic mode by changing the side (moving to
other eye), or by measuring a new patient. In AL setting 9
diferent modes are available. (Figure 5)
Interpretation of Signal Curves
Axial lengths measurements are displayed in the form of
signal curves graphs. The Signal to Noise Ratio value is a
gauge of the quality of measurement. Ideal display of axial
length graphs termed as valid signal curves characteristi-
cally shows very good signal-to-noise ratio (SNR > 10). It
has narrow primary maxima with central terminal peak.
Secondary maxima are present symmetrically on either
side, separated by approximately 800 m from primary
peak. Tertiary and quaternary peaks are visible with low
and even base line (Figure 6,7). Clear signal has SNR >2.0.
Borderline SNR as displayed in graph (Figure 8) has SNR
value between 1.6 -2.0. Graph display is uneven with pri-
mary maxima having short peak and indistinct secondary
maxima. Such display is seen with moderate grade cata-
ract and should be interpreted with caution.
Invalid signal displays are seen with SNR value less
than 1.6 with indistinguishable primary maxima. Display
graph is irregular (Figure 9) with high variations in axial
length displayed. Also machine gives "error" message and
these results need to be discarded. Unsteady (non fxat-
ing) patient, strong ametropia and dense medial opacity
along the visual axis gives invalid signal display.
The IOLMaster 500 displays a single signal of the axial
length measurement in red and marked with an S on the
ordinate. The SNR (signal-noise ratio) is shown on the x-
axis. In contrast, the compos-
ite signal is shown in blue
and marked with a C on the
ordinate. The increased SNR
of the composite signal is
likewise shown on the x-axis.
The SNR ranges are displayed as a trafc light.
l Red = MeasuringError
l Yellow = Uncertain or Borderline Value
l Green = Value with Good SNR
Take at least FIVE individual measurements:The frst
Figure 6
Figure 4
Figure 5
AIO Journal of Ophthalmology 41
Optical Coherence Biometry
four measurements are displayed as usual . From the ffth
measurement on, a composite signal is calculated in the
background. After which, for each individual measure-
ment, the axial length signal is displayed for about 1 sec-
ond (red), followed by the display of the composite sig-
nal (blue). If no axial length reading appears after the frst
fve individual measurements, additional measurements
should be taken. With stronger lens opacities; it may be
advisable to defocus the device.. Also try measurements
by height variation (turning the joystick) of the refocused
refection at the lower and/or upper edge of the circle on
the display. Defocusing and shifting the refection within
the circle will have no efect on the result, because inter-
ferometric axial length measurement is completely inde-
pendent of distance. Avoid measurements of eyes with
retinal detachment. In such cases, measuring errors can-
not be precluded
SnR Categories
While the system is internally calculating the axial length
from the interference signal, it automatically analyzes the
SNR.
Reasons for Low Snr:
l Dense medial opacity along the visual axis
l Restless patient
l Alignment of device to patient eye is not optimal
l Very high ametropia (> 6 D)
l Corneal scars
l Pathological changes in the retina.
RED SnR display --> read-
ing should not be used
This means that the true
measuring signal does not
stand out sufciently from
the noise. As a rule, the results
of such readings are not us-
able and should be rejected.
Keratometry
After activating Keratometry
mode, patient is asked to fo-
cus straight and blink several
times to get an even tear flm.
On video screen examiner
sees 6 peripheral points lo-
cated in the feld between the
two auxiliary circles (Figure
10). The instrument is aligned
so that the six peripheral
measuring points are sym-
metrical, within the circular
cross hairs and appear op-
timally focused.The central
point is usually not focused
and is not evaluated.W hen
the optimum measurement
position has been reached,
the trafc light will change
from red to yellow to green
(Figure 11). Five internal in-
dividual measurements are
taken for a single keratometer measurement within 0.5
seconds. If a measurement point is not correctly identi-
fed, a blue fashing dot will appear. In the printout this
will be marked by an x. These readings should not be used
and a new measurement should be taken as a precaution.
Keratometer measurements may be repeated as often as
desired; however, only the last three measurements will be
displayed.
The IOL Master requires THREE keratometer measure-
ments to be taken! Only then will a mean value be passed
on to the IOL calculation.The corneal curvature results
are displayed in mm or diopters along with respective
axial orientation and the astigmatic diference. If cornea
is completely spherical then only one radius or power is
displayed. Knowing the steeper axis helps in planning the
incision during surgery.
Figure 10
Green SnR display --> reading is valid (Fig. 7)
Yellow SnR display --> reading is uncertain
("Borderline SNR") (Fig. 8)
Figure 11
Fig. 9
42 AIO Journal of Ophthalmology
KNOW THY INSTRUMENT
It needs to be remembered that IOL Master measures
central 2.5 mm diameter compared to manual keratom-
eter which measures central 3 mm zone. Since IOL Mas-
ter measures more centrally, keratometry readings show
steeper K value than manual keratometry. In pseudopha-
kic eyes multiple images appear due to refection from an-
terior surface of IOL leading to measurement errors. Tip in
such cases is to move the IOL Master approximately 1 mm
away (defocusing). Corneal image becomes slightly larger,
while the artifacts of the IOL become fainter. Measure-
ment is then possible.Presence of corneal scars, severe dry
eyes, epithelial defects and partially concealed refections
due to half open lids or ptosis leads to measurement er-
rors. The following plausibility tests will be made with the
keratometer reading:
l R > 8.4 mm 2 Possible fat corneal curvature,
keratoglobus
l R < 7.2 mm 2 Very steep corneal curvature
l R < 7.0 mm 2 Possible keratoconus
l |R1 - R2| > 0.5 mm 2 High astigmatism
Dual Measurement Mode
IOL Master 500 has a dual
mode where both axial length
& keratometry can be meas-
ured in a single measurement
step using twin technology.
The basic principles are same
as for individual measure-
ment. (fgure 12)
Anterior Chamber Depth Mode
Anterior chamber depth on
the IOL Master is interpreted
as the distance between the
anterior vertex of the cor-
nea and the anterior vertex
of the lens. Hence, the dis-
played distance includes
the thickness of the cornea.
On activating ACD measurement lateral slit illumination
will automatically be turned on. The fxation point is dis-
played in optimum focus in the rectangle on the screen
(Figure13). Throughout the examination the patient is di-
rected straight ahead on a small yellow fxation light. Five
internal individual measurements are taken for a single
anterior chamber depth measurement within 0.5 seconds,
Calculation of the anterior chamber depth requires the
input of the corneal radius. If a valid keratometer meas-
urement was performed prior to ACD measurement, the
system will automatically use the measured radius for the
calculation. ACDmeasurement values are required in IOL
power calculation formulae like Holladay 2, and Heigis.
The anterior chamber depth may only be measured on
phakic eyes! ACD measurements of pseudophakic eyes
result in measuring errors.
White-to-White Measurement (WTW)
IOL Master can give easy measurements of WTW distance.
As operator activates the function, a cross bar appears.
Trick is to focus on the iris rather than cornea. Align the IOL
Master so that the six peripheral light spots are symmetri-
cal to the cross hairs and the iris structures or the edge of
the pupil appears optimally focused (Figure 14). The fxa-
tion point in the centre of the six light dots is usually not
in the centre of the pupil or iris, because only in the rarest
cases does the visual axis correspond to the optical axis of
the eye. Measuring WTW distance is useful in calculation
of phakic IOL. The WTW value is the horizontal diameter
of the iris. In addition to the WTW value, the deviation of
the visual axis from the centre of the iris (x, y) will also be
displayed . The values are stated in millimetres with refer-
ence to a Cartesian coordinate system, the zero point of
which is assumed to be in the established centre of the iris
or pupil. If the visual axis is above the iris or pupil centre,
the Y value will be positive; if it is below, the value will be
negative. X values to the left of the centre are negative;
those to the right are positive.
IOL Power Calculation
Before the system calculates IOL options, you must have
entered the desired lens types into database. The IOL for-
mulas HoferQ, Holladay, Haggis, SRK/t and SRK 11 are
listed across the top.
After taking axial length and keratometry readings, IOL
Figure 14
Figure 13
Figure 12
AIO Journal of Ophthalmology 43
Optical Coherence Biometry
power mode is activated. The values of mean axial length
and keratometry are passed on automatically. Operator
needs to select the surgeon to enable the activation of
IOL power calculation. Surgeon must have entered the
parameters of his preferred IOLs in database beforehand.
Soft ware then calculates IOL power for the diferent types
of lenses. Results for four diferent types of lenses are
displayed at a time (Figure 15). Up to 20 surgeons name
and correspondingly data of their preferred lenses can be
entered. Only constants optimised for optical biometry
should be used for calculating the suggested strength of
the intraocular lens to be implanted with the IOL Master,
not the manufacturers IOL constants
IOL powers are displayed in the steps of 0.5 diopters
or 0.25 diopters. Included in the standard IOL master soft
ware package are fve popular IOL power calculation for-
mulae, Haigis, Holladay, SRK II, SRK/T and Hofer Q. While
calculating IOL power surgeon can choose his preferred
formula from the task bar.IOL Master 500 has mode for
phakic IOL and post refractive surgery patients. After re-
fractive corneal surgery the Haigis-L or Prior refractive sur-
gery tabs may be selected.
Optimisation
Included in the IOL Master is the most important part that
is ability to optimise or fne tune the IOL power as per
the surgeons choice of IOL. It must be remembered that
A constants which are provided by the manufacturer are
based on contact A scan and manual keratometry. This val-
ue represents where we anticipate the IOL to sit in relation
to cornea. Specifcally how near or far from cornea. The
"constant" will decrease with ACIOL as compared to PCIOL.
As ACIOL sits closure to cornea, less power is needed. The
manufacturer estimates the A constant by approximating
from similar lens models. The fnal lens position in capsular
bag will be infuenced by IOL style (optic - haptic design,
fexibility, angulations, reaction to fbrosis) and surgical
technique (rhexis, incision, phaco technique). Therefore
A constant provided by manufacturer needs to be opti-
mised as per surgeon's technique.
Initially, surgeon can use optical lens constants avail-
able on the ULIB web page which gives lens constants for
diferent types of lenses as per data pooled in from world
wide IOL Master users. To derive his own optimized A con-
stant surgeon needs to select minimum of 20-25 cases.
All these cases should be operated by same surgeon with
same surgical technique using same type of lenses. Post
operative data and fnal post operative refraction at end
of 6 weeks are noted. This data needs to be entered in IOL
Master which then calculates the new optimised lens con-
stant for that type of lens for that particular surgeon
IOL MASTER in practice
IOL Master has become the most important tool now a
days available to refractive surgeons who aim for perfec-
tion in their every aspect of surgery. IOL Master gives the
most accurate and consistent IOL power for most choices
of IOL.
16
In individual practice studies, the new IOLMaster 500
has been shown to complete all measurements in as few
as 80 seconds
17
The average time needed to take a reading
on the IOLMaster 500 is up to 4 times faster compared to
other optical devices
18
OptiIical biometry along the visual
axis is highly accurate. High ametropia, pupil size as well
as state of accommodation do not afect the accuracy of
measurement.
Whereas the principal application of the device will be
in cataract surgery , the relatively high order of dioptric
resolution for axial length (~plus or minus 0.03 D) is espe-
cially valuable in studies of myopia the IOLMaster is likely
to fgure signifcantly in future myopia research
Limitations
4,13
IOL Master is not suitable in cases of:
l In dense cataract
l Restless patient with inability to fxate light
l Patient with nystagmus
l Retinal detachment
l Very high ametropia
l Thick posterior capsular plaque
l In severe dry eye or scarred cornea keratometry is not
possible
l Anterior chamber depth cant be measured in apha-
kic eyes.
l media opacities like vitreous hemorrhage
Figure 15