Eye Docs Cataract

Cataract (No=175)
A 66-year-old man is referred by his opticians for bilateral, dense nuclear sclerotic cataracts with
VA 6/24 bilaterally. On examination, you note evidence of proliferative diabetic retinopathy
bilaterally, with new vessels at the disc and bilateral macular oedema with central macular
thicknesses over 400 um on OCT.
Regarding his cataracts, what would be the most appropriate advice:

cataract surgery with an intact posterior capsule will increase the chances of neovascular
glaucoma post-operatively
an attempt should be made to perform PRP before cataract surgery
cataract surgery should be performed as soon as possible to aid visualization of the fundus
cataract surgery should be delayed until one cycle of three monthly anti-VEGF injections is
completed
Your answer was CORRECT

Explanation
The proliferative changes take precedence over the cataract or macular oedema in this case and
PRP should be attempted before cataract surgery. Maintenance of an intact posterior capsule is
important to prevent anterior migration of angiogenic factors.
Marfan’s syndrome is typically associated with each of the following EXCEPT:

hypoplasia of dilator pupillae
upward subluxation
glaucoma
keratoconus
hyperopia

Explanation
All of the above are typical ocular associations of Marfan’s with the exception of hyperopia.
Marfan’s is in fact associated with axial myopia and retinal detachment.
When does an anterior sutural cataract develop?

in utero
during adolescence
between birth and 2 years of age
over age 40

Explanation
Sutural cataract is a congenital cataract occurring during development of the fetal lens nucleus.
A 17-year-old boy with mental retardation and short stubby fingers attends clinic. You notice
bilateral inferior lens subluxation.
Given the history, what feature are you MOST likely to encounter?
a small lens
tendency to thrombo-embolic events
increased arm-span
mitochondrial inheritence

Explanation
This patient has Weil Marchesani syndrome. This condition is characterised by:
 AD inheritence
 mental retardation
 short stubby fingers
 downward lens subluxation
 microspherophakia
55-year-old hypermetropic lady develops a sudden, painful right eye with associated headache. On
examination, there is a shallow anterior chamber and mid-dilated pupil with corneal oedema.
What type of lens opacity might develop in association with this episode:
a moderately dense, shield-like anterior subcapsular opacity
small grey-white anterior sub-capsular opacities
fine, crystalline, posterior sub-capsular opacities
cuneiform, spoke-like radial opacities
Your answer was INCORRECT

Explanation
Acute angle closure glaucoma may cause small, grey-white, anterior, capsular or subcapsular
opacities within the papillary area, which are known as glaukomflecken.
A patient is due for right cataract surgery. Biometry of the right shows:
K1: 44.55 @ 45
K2: 45.55 @ 135
Where should the main incision be placed to maximally reduce corneal astigmatism?
45 degrees
90 degrees
135 degrees
0 degrees
Explanation
The main incision should be placed at the steepest meridian, which is 135 degrees
A 15-year-old boy presents with bilateral infero-nasal ectopia lentis. He is tall with malar flush.
Both of his parents appear normal.
Which of the following is LEAST likely to be found in this patient?

increased risk of thromboembolism
decreased serum methionine
mental retardation
red hair
osteoporosis

Explanation
This patient has homocystinuria. Homocystinuria is an autosomal recessive condition caused by
decreased activity of cystathionine beta-synthetase, resulting in systemic accumulation of
homocysteine and methionine. Systemic features include: marfanoid habitus, fair or red hair,
malar flush, osteroporosis, and a tendency to thrombotic episodes. Ectopia lentis is typically
infero-nasal.
Which IOL material has the highest refractive index?
hydrophobic acrylic
hydrophilic acrylic
PMMA
silicone

Explanation
The refractive indices of popular lens materials are:
 PMMA = 1.49
 Silicone = 1.41- 1.46
 Hydrophobic acrylic =1.54
 Hydrophilic acrylic = 1.47
A higher refractive index means the lens can be made thinner for an equivalent power.
All of the following may reduce positive vitreous pressure during ocular surgery EXCEPT:
hypoventilation during general anaesthesia
adjustment of a poorly fitting lid speculum
aspiration of liquid vitreous through the pars plana
IV mannitol

Explanation
Intraoperative manoeuvres to reduce vitreous pressure include:
 reduce vitreous volume by aspiration

 reduce vitreous volume pharmacologically (e.g. IV mannitol)
 relieve pressure on the globe (e.g. speculum)
 prevent squeezing (sedation or general anaesthesia)
 hyperventilation under GA to decrease vitreous pressure
Which is true of galactosaemia type 2?
1. oil droplet cataract

2. deficiency of galactose-1-phosphate uridyl transferase
3. generally healthy child
3 only
1, 2 and 3
2 and 3
1 and 3
1 only

Explanation
Type 1 (classic) galactosaemia is autosomal recessive and is caused by deficiency of galactose-1-
phosphate uridyl transferase. Children are generally unwell, including failure to thrive,
hepatosplenomegaly, CNS disease and renal disease; and the cataract is typically a central oil-
droplet.
Type 2 and Type 3 galactosaemia are also autosomal recessive, but they are less common than
Type 1 and children are less systemically unwell. Cataract is classically lamellar rather than oil
droplet. Type 2 is caused by deficiency of galactokinase while Type 3 is caused by deficiency of
UDP galactose epimerase.
Which is TRUE regarding homocystinuria:

deficiency of methionine
increased risk with general anaesthesia
classic erythema migrans rash
supero-temporal lens subluxation
autosomal dominant
Explanation
Homocystinuria is autosomal recessive. It is caused by decreased activity of cystathionine beta-
synthetase, resulting in systemic accumulation of homocysteine and methionine. Systemic features
include: marfanoid habitus, fair hair, and a tendency to thrombotic episodes. There is an increased
risk of life-threatening complications with general anaesthesia due to thrombosis if the diagnosis
is not known. Ectopia lentis is typically infero-nasal.
A 40-year-old patient with mitral regurgitation presents to clinic. You notice bilateral lens
subluxations and a high-arched palate.
Which is TRUE?
inheritance is AR
accommodation is usually intact
there may be associated mental retardation
lens subluxation is typically downwards

Explanation
This patient has Marfan’s syndrome, which is autosomal dominant. Intellect is normal. Associated
systemic features include arachnodactyly, increased arm span:height ratio, high arched palate and
cardiac valvular anomalies. Lens subluxation is upwards in Marfan’s and zonules are present with
intact accommodation, unlike homocystinuria where subluxation is downwards and zonules are
absent with accommodation lost.
A 6-week-old child is suffering from lethargy, vomiting and diarrhoea. Reducing substances are
found in the urine. The paediatricians request an ophthalmic review.
What feature would you anticipate on ocular examination?

oil droplet cataract
bone-spicule pigmentation
megalocornea
lamellar cataract
sclerocornea

Explanation
The clinical features are consistent with (Type 1) classic galactosaemia, which is associated with
oil-droplet cataract.
All are characteristics of persistent hyperplastic primary vitreous EXCEPT:
autosomal recessive inheritance
progressive cataract
prone to angle closure glaucoma
typically unilateral

Explanation
PHPV is a congenital, non-hereditary ocular malformation. It is typically unilateral. Findings
include:
 elongation of ciliary processes

 prominent radial iris vessels
 persistent hyaloid artery
 microphthalmia
 ectopia lentis
 cataract
Biometry with the Zeiss IOLMaster is most difficult to perform in which scenario?
pseudophakic eye
aphakic eye
silicone filled eyes
dense posterior subcapsular opacities

Explanation
The Zeiss IOLMaster is a non-contact method of biomery that utilizes two coaxial laser beams
that are partially coherent. Because it is light-dependent, dense lenticular opacities are a limitation,
and A-scans may need to be used in these scenarios.
The most common source of organisms leading to post-operative endophthalmitis is:

the operating room air
contaminated instruments and solutions
the surgeon's skin and respiratory tract
the patient's lids

Explanation
The flora of the patients own eyelids and conjunctiva are the most common source of organisms
that lead to post-surgical endophthalmitis.
Which is TRUE regarding posterior polar cataracts?

synonymous with posterior subcapsular cataract
common in myotonic dystrophy
malformation of posterior fibres with attachment to capsule

Explanation
Posterior polar cataract is a rare form of congenital cataract. It is usually inherited in an autosomal
dominant fashion, but can be sporadic. Posterior polar cataracts consist of dysplastic lens fibers,
which, in their migration posteriorly from the lens equator, exhibit progressive lens opacity,
increased degenerative changes, with the formation of a characteristic discoid posterior polar
plaque. There is extreme thinness and fragility of the posterior capsule with adherence of the
opacity to the capsule.
Myotonic dystrophy is associated with posterior cortical and Christmas tree cataracts.
This question came in the 2014 FRCOphth.
Which one of the following is the preferred surgical treatment for severe, bilateral, congenital
cataracts identified in a 1-month-old infant with compliant parents?
lensectomy, anterior vitrectomy, and fitting of contact lens
lensectomy, anterior vitrectomy, and fitting with aphakic glasses
intracapsular cataract extraction with contact lens fitting
aspiration of lens and implantation of posterior chamber IOL

Explanation
There are different views and this area remains controversial.
Intracapsular cataract surgery is best avoided as Weigert's ligament, a connection between the
peripheral posterior capsule and the anterior vitreous, is strong. Excessive vitreous loss and
traction on the retina are likely.
IOL implantation has become a preferred method of treatment by many; however, most feel that
an IOL should not be implanted in children younger than 2 years of age. This is partly a result of
the increase in the size of the anterior segment during the first 2 years of life. Children also have
high rates of capsular opacification.
Contact lenses are preferred over aphakic glasses as the former reduces aniseikonia and
astigmatism.
All are associated with classic (Type 1) galactosaemia EXCEPT:
deficiency of galactokinase
autosomal recessive
renal disease
hepatosplenomegaly
reducing substances in the urine

Explanation
Classic (Type 1) galactosaemia is caused by a deficiency of galactose-1-phosphate uridyl
transferase (GPUT). It is an autosomal recessive condition associated with oil droplet cataract.
Systemic features are common including failure to thrive, hepatosplenomegaly and renal disease.
Type 2 galactosaemia is caused by galactokinase deficiency. This condition is autosomal

recessive, but unlike Type 1, affected children are generally healthy and the cataract is lamellar
(not oil droplet).
The primary abnormality seen in ptosis which develops after cataract surgery is in the:
levator muscle
third nerve nucleus
levator innervation
levator aponeurosis

Explanation
Figure: Aponeurotic ptosis with high skin crease
Aponeurotic dehiscence is the main cause of ptosis after cataract surgery. This may be due to the
effect of anaesthetic injections, lid specula, and bridle sutures.
Which IOL material is most likely to develop late calcification?
hydrophobic acrylic
hydrophilic acrylic
silicone
PMMA

Explanation
Hydrophilic acrylic IOLs are the most susceptible to calcification, though this is a rare event with
modern lenses.
In which of the following congenital scenarios is amblyopia likely to develop MOST quickly:
binocular nuclear cataract
monocular nuclear cataract
monocular lamellar cataract
monocular anterior polar cataract
binocular posterior lenticonus

Explanation
Monocular visual deprivation is more amblyogenic than an equivalent deprivation binocularly.
Nuclear cataract is typically more visually significant than anterior polar or lamellar
cataracts. Every visually significant infantile cataract should be operated on as soon as it is
feasible.
Which of the following is NOT a reported complication after Nd:YAG capsulotomy with properly
focused laser?
subluxation of lens
iritis
retinal detachment
corneal oedema

Explanation
Recognised complications of YAG capsulotomies include:
 lens pitting
 iritis
 raised IOP
 retinal break
 macular oedema
Corneal oedema should not occur if the laser is properly focused on the posterior capsule.
Which cataract is associated with long-term steroid use?

nuclear sclerotic
anterior polar
cuneiform cortical
snowflake
posterior sub-capsular

Explanation
Long-term steroid use is associated with posterior sub-capsular cataract.
Which medication increases the risk of complications during cataract surgery?

oxybutynin
duloxetine
trimethoprim
doxazosin

Explanation
Alpha-1 antagonists such as tamsulosin can cause floppy iris syndrome. Alfuzosin, doxazosin,
terazosin and prazosin may also cause this phenomenon.
Posterior lenticonus is most likely to be found in:

Marfan's syndrome
Alport's syndrome
Lowe's syndrome
Weill-Marchesani
Congenital rubella

Explanation
Posterior lenticonus is associated with Lowe's syndrome. Posterior lenticonus may also occur
sporadically or it may be inherited (as AD, AR or X-linked).
With regard to ophthalmic viscoelastics, which is FALSE:

dispersives offer a better view
dispersives offer better endothelial protection
sodium hyaluronate 1% is a cohesive
cohesives are easier to remove

Explanation
Cohesive viscoelastics offer a better view. Dispersives tend to trap air bubbles which can be a
nuisance for the view during surgery. All other statements are true.
The single most important measure in the prophylaxis against endophthalmitis post-cataract
surgery is:
pre-operative povidone iodine onto the lids and conjunctival sac
pre-operative antibiotics such as chloramphenicol drops
post-operative antibiotic drops such as chloramphenicol
sub-conjunctival antibiotics such as cefuroxime post-procedure
intra-cameral antibiotics such as cefuroxime post-procedure

Explanation
The most important prophylactic measure against endophthalmitis is to thoroughly disinfect the
lids and conjunctiva with an antiseptic such as povidone-iodine to eliminate commensal flora.
This question came in the FRCS (Glasgow) Part 2 October 2014.
Which is TRUE regarding cataracts?

absence of reducing substances in the urine may be related to oil-droplet cataract
myotonic dystrophy causes anterior cortical cataract
steroids cause anterior subcapsular cataract
Wilson's disease causes a characteristic anterior capsular cataract

Explanation
Wilson's disease causes a characteristic sunflower cataract due to copper deposition on the anterior
capsule. Myotonic dystrophy causes a Christmas tree cataract and later a posterior sub-capsular
cataract. Steroids cause posterior sub-capsular cataract. The presence (not absence) of reducing
substances in the urine is indicative of galactosaemia, which is characterised by oil droplet
cataracts.
This question appeared in the 2014 FRCOphth Part 2.
All are associated with acquired ectopia lentis EXCEPT:

pseudoexfoliation
buphthalmos
cataract
hyperopia
ciliary body melanoma

Explanation
High myopia (not hyperopia) is associated with ectopia lentis. All other options are true.
Which is FALSE regarding biometry with the Zeiss IOLMaster?

it is less operator dependent than A-scan biometry
it utilises two fully coherent, coaxial laser beams
it is a non-contact biometry method
it can measure anterior chamber depth

Explanation
The Zeiss IOLMaster is a non-contact method that uses two coaxial laser beams that are partially
coherent. The beams produce an interference pattern that is used to interpret measurements, thus
the technology is referred to as partial coherence interferometry. It is highly reproducible and less
operator-dependent than ultrasound biometry. The machine can measure keratometry, axial length,
anterior chamber depth and corneal white-to-white diameter.
Which of the following statements regarding surgical strategy for paediatric cataract extraction
relative to adults is FALSE?
extracapsular nuclear expression is generally avoided because of the small, relatively soft nucleus
of the juvenile cataract
primary posterior capsulotomy is generally not undertaken because of more prominent
hyaloideocapsular attachments in the young eye
one or more peripheral iridectomies are generally indicated due to the higher risk of exaggerated
inflammation and secondary pupillary block
paediatric cataracts are usually soft and can be aspirated entirely

Explanation
Paediatric patients have a high incidence of aggressive posterior capsular opacification, so many
surgeons advocate primary posterior capsulotomy and even anterior vitrectomy to remove the
hyaloid face at the time of cataract extraction. Note: paediatric eyes have more prominent
hyaloideocapsular attachments, meaning that intracapsular extraction is contraindicated because of
the high chance of inducing retinal tears from traction.
Which statement is FALSE regarding polar cataracts?
anterior and posterior polar cataracts can be sporadic or recessively inherited
anterior polar cataracts may be associated with anterior lenticonus
posterior polar cataracts usually cause more visual disturbance than anterior polar cataracts
posterior polar cataracts may be associated with remnants of the tunica vasculosa lentis

Explanation
Anterior polar cataracts are usually small and non-progressive, do not impair vision and may be
associated with microphthalmos, a persistent papillary membrane and anterior lenticonus.
Posterior polar cataracts cause more visual impairment and tend to be larger than anterior polar
cataracts. They can be associated with posterior lenticonus or a remnant of the tunica vasculosa
lentis.
Both anterior and posterior polar cataracts can be autosomal dominant or sporadic.
In A-scan biometry, a one diopter IOL power error could result from an axial length error of:
1 mm
4 mm
0.4 mm
8mm
0.04 mm

Explanation
0.4mm compression error can result in a 1 diopter error in the calculated IOL power.
Which of the following cases is NOT associated with microspherophakia:
sulfite oxidase deficiency
congenital rubella
small stature, stubby fingers, reduced joint mobility
deafness, nephritis, anterior lenticonus and posterior polymorphous dystrophy

Explanation
Sulfite oxidase deficiency is not associated with microspherophakia.
Sulfite oxidase deficiency is characterised by:

 ectopia lentis (50%)
 enophthalmos
 growth, motor, mental retardation
Causes of microspherophakia:
 Weill-Marchesani syndrome
 Alport's syndrome
 Marfan's syndrome
 Peter's anomaly
 Congenital rubella
 Hyperlysinaemia
A patient who is phakic develops a retinal detachment with proliferative vitreoretinopathy. He

undergoes vitrectomy with silicon oil fill but no lens surgery is performed. What will be the
expected refractive outcome post-op?
hyperopic shift
myopic shift
increased astigmatism
no refractive change

Explanation
In phakic patients, insertion of silicon oil causes a hyperopic shift, usually between 5-9 dioptres,
while in aphakic patients, silicon oil causes a myopic shift (eyes become less hyperopic).
Which statement is TRUE regarding cataracts, cataract surgery and lens implants?
the natural lens of an older patient is less susceptible to the cataractogenic effects of ionizing
radiation than the lens of a younger patient
uncomplicated cataract surgery typically raises IOP by 1 to 2 mmHg long-term
correction of aphakia with spectacles after cataract surgery is less successful in children than in
adults
the preferred design of modern anterior chamber intraocular lens implants involves closed loops
because of fewer complications

Explanation
Routine cataract surgery typically lowers IOP by 1 to 2 mmHg.
The plasticity of the visual system in children allows them to adapt to the various distortions
inherent in aphakic spectacles more readily than adults.
Anterior chamber intraocular lenses with closed support loops are associated with a higher
incidence of complications. They are no longer used for this reason.
A younger patient's lens epithelial cells are more actively growing than an older patient's and are
thus more susceptible to radiation damage.
Which of these antibiotics when given intravitreally is most toxic to the retina?
ceftazime
gentamicin
vancomycin
amikacin
cefuroxime

Explanation
Gentamicin is occasionally used intra-vitreally in endophthalmitis for Gram-negative cover in
combination with cefuroxime. However gentamicin is retino-toxic and alternative regimes
include:
 vancomycin and ceftazidime

 vancomycin and amikacin
Which of the following congenital cataracts is LEAST likely to impair vision significantly?
nuclear
complete
lamellar
posterior polar
anterior capsular

Explanation
Anterior capsular cataracts are opacities of the anterior lens capsule and epithelium that do not
usually affect vision.
A 1-week-old baby is noted by paediatricians to have an abnormal red reflex in both eyes. On
examination, you discover dense, bilateral lamellar cataracts, but no other ocular abnormalities.
Which of the following is the most appropriate therapy for this infant?
patch the eye that best fixes and follows and perform surgery on the other eye
perform surgery on one eye as soon as possible and the other eye after 3 months
perform surgery on one eye with immediate aphakic correction and perform surgery on the
other eye before 3 months of age
observe until 3 months of age and perform cataract surgery if nystagmus develops

Explanation
Bilateral congenital cataracts of significant opacity should be addressed as soon as possible.
Aphakic correction with an aphakic contact lens should be performed immediately post-
operatively. Surgery on the second eye should be done as soon as possible after the first eye.
When indicated, surgery is ideally performed before the child is 3 months of age, as profound
amblyopia and nystagmus develops by this age.
During a complicated cataract operation, the lens and capsular bag are removed and an anterior
chamber lens is placed. On the first post-operative day, the IOP is 50 mmHg and the iris is bowed
forward around the IOL.
Which of the following measures would be most effective?

topical glaucoma medications and close observation
AC paracentesis
laser peripheral iridotomy
urgent vitrectomy

Explanation
This case is characteristic of iris bombe occurring because of pupil block from the ACIOL. The
iris is pushed forward peripherally causing a secondary angle closure. A peripheral iridotomy is
necessary to create an alternative pathway for the aqueous.
Excessive iris prolapse during phacoemulsification may be caused by all EXCEPT:

excessive phaco power
suprachoroidal haemorrhage
wound size too large for phaco tip
infusion bottle height too high

Explanation
Excessive phaco power may cause endothelial damage leading to pseudophakic bullous
keratopathy or corneal wound burn, but does not contribute to iris prolapse.
Which of the following is LEAST likely to cause posterior sub-capsular cataract?

topical steroids
oral steroids
uveitis
diabetes
penetrating surgery

Explanation
This question came in the FRCS (Glasgow) Part 2 exam in October 2014.
We thought this was a difficult question as all the options are recognised causes of posterior
subcapsular cataract. However, topical steroids are much less likely than systemic steroids to
cause cataract (although they are more likely than orals to cause raised intra-ocular pressure) so
we felt this was the answer the examiners were looking for. Recognised causes of posterior
subcapsular cataract:
 oral, topical, inhaled steroids (oral more frequent than others)
 uveitis
 penetrating surgery (e.g. vitrectomy)
 retinal dystrophies (e.g. RP, Usher's, gyrate atrophy)
 degenerative myopia
 chloroquine
 busulfan
 diabetes
This question came in the FRCS (Glasgow) Part 2 exam in October 2014.
Which IOL material is most likely to develop glistenings?

hydrophilic acrylic
silicone
PMMA
hydrophobic acrylic

Explanation
Hydrophobic acrylic IOLs have been associated with glistenings in the optic. These glistenings are
water (from the aqueous) that becomes trapped as droplets in a hydrophobic environment.
Biometry with the Zeiss IOLMaster is most difficult to perform in which scenario?
pseudophakic eye
dense posterior subcapsular opacities
aphakic eye
silicone filled eyes
Explanation
The Zeiss IOLMaster is a non-contact method of biomery that utilizes two coaxial laser beams
that are partially coherent. Because it is light-dependent, dense lenticular opacities are a limitation,
and A-scans may need to be used in these scenarios.
A patient with myotonic dystrophy will classically develop which type(s) of cataract:
1. Christmas tree cataract

2. stellate posterior sub-capsular
3. anterior polar
4. cuneiform anterior cortical
1 and 4
1 only
1 and 2
2 only
2 and 3

Explanation
Myotonic dystrophy is associated with fine cortical iridescent opacities (Christmas tree cataract),
which are visually innocuous and occur in 90% of patients by the third decade. These evolve into
visually disabling stellate posterior sub-capsular opacity by the fifth decade.
A patient with Fuch's dystrophy undergoes cataract surgery. Which proprietary viscoelastic is
most appropriate to form a soft-shell?
Provisc
Viscoat
Healon GV
Healon 5

Explanation
Viscoat is a proprietary dispersive viscoelastic. Dispersives are used to coat and protect the
endothelium in the 'soft-shell' approach to cataract surgery.
The presence of polychromatic cataract in association with chronic progressive external

ophthalmoplegia (CPEO) suggests the diagnosis of:
Kearns-Sayre syndrome
oculopharyngeal muscular dystrophy

myotonic dystrophy
Wilson's disease

Explanation
Causes of chronic progressive external opthalmoplegia (CPEO) include:
 idiopathic
 Kearns-Sayre
 myotonic dystrophy
 oculopharyngeal muscular dystrophy
Myotonic dystrophy is often associated with polychromatic (Christmas tree) cataracts.
A mild myope with accurate, reproducible biometry and a target refraction of emmetropia is found
after routine phacoemulsification and posterior chamber lens implantation to have a refraction of -
1.50.
What is the most likely cause of refractive surprise?

cysoid macular oedema
retained viscoelastic
Anterior IOL dislocation
Posterior IOL dislocation

Explanation
Posterior dislocation of an IOL and cystoid macular oedema would both tend to produce
hyperopic (not myopic) shifts. In routine cataract surgery with in-the-bag IOL placement and
intact zonules, anterior dislocation of the IOL to produce 1.50D of myopic shift is unlikely.
Retained viscoelastic between the lens and the posterior capsule (capsular bag distension
syndrome) is the most probable cause of inducing a myopic shift.
Which cataract is most typical of a patient with Alport's syndrome?
anterior polar
nuclear
cortical
lamellar

Explanation
Alport's syndrome can be inherited in both X-linked recessive (80%) and X-linked dominant (5%)
forms. Where X-linked recessive, heterozygous females may still experience haematuria - a
separate condition termed 'thin basement membrane nephropathy.' Reference: NIH National
Library of Medicine.
Alport's is caused by a defect in Type 4 (basement membrane) collagen. Clinically, it causes:
 glomerular basement membrane nephritis and renal failure

 hearing loss
 anterior lenticonus
 cortical cataract
 juvenile arcus
 posterior polymorphous dystrophy
 dot-fleck retinopathy
A 79 year old lady underwent routine right cataract surgery for gradual reduction in right vision.
Pre-operatively, her right VA was 6/18, left VA 6/6. At her first post-operative visit (at 2 weeks)
her right vision remained 6/18. An OCT was normal, though on examination her right optic disc
appeared slightly pale.
What is the most suitable next test?

MRI brain and orbits
fluorescein angiography
serum electrophoresis
visual fields

Explanation
This patient has evidence of optic neuropathy with reduced VA and disc pallor. While visual fields
may be helpful in this case in confirming and documenting poor nerve function, the most
important tests are to exclude an acute inflammatory cause (ESR, CRP) and to consider
neuroimaging to rule out compression.
The differential diagnosis of childhood leucocoria includes all EXCEPT:

retinoblastoma
persistent hyperplastic primary vitreous
retinopathy of prematurity
Coats' disease
homocystinuria

Explanation
Homocystinuria does not cause leucocoria but may be associated with ectopia lentis.
A patient has sun-flower cataract. Which statement is TRUE?

corneal deposits may be found in Bowman's layer called Kayser-Fleischer ring
the cataract is caused by iron deposits within the anterior capsule
patients may develop bronze diabetes
inheritance is autosomal dominant
the cataract is not usually visually significant

Explanation
Wilson's disease is autosomal recessive. It is caused by abnormalities in copper transport, with
low serum ceruloplasmin, paradoxically low serum copper, high urinary copper, and copper
deposition in tissues including the liver, brain and eyes. It results in liver failure and
neuropsychiatric symptoms. In the eyes, it can cause sunflower cataract from deposition of copper
within the anterior capsule (which is not usually visually significant) and Kayser-Fleischer rings
from deposition of copper in Descemet's membrane.
A 3-week-old child is suffering from lethargy, vomiting, diarrhoea and failure to thrive. Reducing
substances are found in the urine.
What enzyme is most likely deficient?

galactose-1-phophate uridyl transferase
alpha galactosidase
hexosaminidase A
glucocerebrosidase

Explanation
Reducing substances in the urine suggests galactosaemia, which is caused by deficiency of
galactose-1-phosphate uridyl transferase (GPUT).
A young baby with hypotonia and development delay is referred to ophthalmology where
microphakia, posterior lenticonus and cataract are diagnosed. The intra-ocular pressure is
29mmHg bilaterally with Perkins tonometry.
What abnormality would be MOST likely on urinalysis?

aminoaciduria
glucose
reducing substances
red blood cells
leucocytes
Explanation
This patient has features of Lowe's (oculocerebrorenal) syndrome, an inborn error of amino acid
metabolism, which is X-linked recessive. Fanconi's syndrome of the proximal renal tubules occurs
in Lowe's syndrome, with aminoaciduria and low molecular weight proteins found in the urine.
Which organism is most likely to cause post-operative endophthalmitis:
Staphylococcus aureus
Haemophilus influenzae
Staphylococcus epidermidis
Klebsiella spp
Streptococcus pyogenes

Explanation
Staph epidermidis is the most common cause of acute post-operative endophthalmitis. Other
causes (in order of descending frequency):
 Staph aureus
 Strep species
 Pseudomonas spp
 Proteus spp
Which of the following syndromes featuring congenital cataract may be associated with facial
abnormalities?
1. Crouzon syndrome
2. Hallermann-Streiff syndrome
3. Stickler's syndrome
4. Alport's syndrome
1 and 3
4 only
2 and 4
1, 2, 3, and 4
1, 2, and 3

Explanation
In Crouzon syndrome, there is craniosynostosis, small orbits, proptosis, and cranial changes that
affect facial structure. Cataract is a recognised though low-frequency finding. Patients with
Hallermann-Streiff syndrome typically have mandibular hypoplasia with a "bird face." Stickler's
syndrome may be associated with many facial abnormalities, including maxillary and mandibular
hypoplasia, epicanthus, a long philtrum, and the Pierre-Robin's anomaly. Alport's syndrome
features hereditary nephritis, deafness and cataract with normal facies.
According to the Royal College of Ophthalmologists guideline (2010), which formula is best for
an eye with an axial length of 21.6mm?
Holladay 2
SRK/T
Hoffer Q
SRK II

Explanation
The college advises that while most modern formulae work well in the normal axial length range,
for eyes <22mm "the Haigis and Hoffer Q may be slightly better."
A 22-year-old adult with mental handicap, short stature and brachydactyly is referred by his
optician with deteriorating visual acuity. You note bilateral inferiorly subluxed lenses.
What is the most likely diagnosis?
Stickler syndrome
Ehlers-Danlos syndrome
Weill-Marchesani syndrome
Sulphite oxidase deficiency
Hyperlysinaemia

Explanation
The clinical features are in keeping with Weill-Marchesani, which is a rare AD (or AR) inherited
disorder.
Features of Weill-Marchesani include:
 Systemic
o short stature
o brachydactyly
o stiff joints
o mental handicap
 Ocular
o bilateral, inferior ectopia lentis
o spherophakia or microspherophakia
o angle anomaly
o pre-senile vitreous liquefaction
Microspherophakia is most often one component of:
Lowe's syndrome
Peters' anomaly
Alport's syndrome
Marfan's syndrome

Explanation
Microspherophakia, as its name implies, is characterized by a lens with small diameter and a
spherical shape. High myopia is often found in association. It may occur as an isolated hereditary
disorder or in association with other abnormalities - most commonly Weill-Marchesani syndrome.
Causes of microspherophakia include:

 Alport's syndrome
 Marfan's syndrome
 Peter's anomaly
 Congenital rubella
Note: Lowe's syndrome is associated with microphakia
Which one of the following statements about post-operative endophthalmitis is true?

it most commonly presents 7 to 10 days postoperatively, and the most common etiologic agent is
Staphylococcus
it most commonly presents 2 to 5 days postoperatively, and the most common etiologic agent
is Staphylococcus
organisms responsible for these infections are generally part of the surgeon's normal flora
Streptococcus
Streptococcus

Explanation
Post-operative endophthalmitis is usually caused by contamination with bacteria from the patient's
normal lid flora. Staphylococcus epidermidis is the most common agent with presentation 2 to 5
days postoperatively.
Regarding phacodynamics, which is TRUE:
dual linear systems allow control of bottle height and vacuum on different axes of the foot pedal
there is no difference between aspiration flow rate and vacuum in Venturi systems
peristaltic systems do not require occlusion of the phaco tip for vacuum
high aspiration flow rates require a low bottle height to prevent surge

Explanation
High aspiration flow rates require a high bottle height to maintain the anterior chamber.
In peristaltic systems, occlusion of the phaco tip is required to produce vacuum. By contrast, in
Venturi systems the pump generates vacuum by creating negative pressure in a vessel by gas
flowing across its entrance. This means there is no difference between vacuum and aspiration flow
rate in Venturi systems - depressing the pedal increases vacuum, which is not dependent on
occlusion.
In dual linear systems, vacuum and phaco power are controlled on different axes of the foot pedal.
A patient with Marfan's syndrome has bilateral, symmetrical supero-temporal lens subluxations.
The lenses still cover the pupillary plane entirely.
What is the most appropriate initial management?

surgical removal of the lens with aphakic correction
spectacle or contact lens correction of refractive error
surgical removal of the lens with scleral-fixated posterior chamber IOL
pilocarpine to constrict the pupil

Explanation
When the lens still covers the pupillary plane, vision is usually preserved with refractive
correction, and it is therefore most reasonable to treat conservatively. Often the subluxation
remains stable and surgical removal of the lens can be complicated, especially in Marfan's patients
where there is a higher risk of retinal detachment. With conservative treatment, a reading add may
be required, since accommodation may be affected. Pupillary dilatation (not miosis) can help
vision in some patients. If dislocation progresses, removal of the lens with implantation of a
fixated posterior-chamber IOL or anterior chamber IOL is desirable.
Which statement(s) is(are) true regarding unilateral cataract:
1. most are sporadic

2. most are associated with systemic disease
3. most occur in premature infants
3 only
1 only
2 only
1 and 3
2 and 3

Explanation
Unilateral congenital cataracts are usually sporadic without a family history or systemic disease.
Affected infants are usually full-term and healthy.
Which statement is FALSE regarding polar cataracts?

anterior and posterior polar cataracts can be sporadic or recessively inherited
posterior polar cataracts may be associated with remnants of the tunica vasculosa lentis
posterior polar cataracts usually cause more visual disturbance than anterior polar cataracts
anterior polar cataracts may be associated with anterior lenticonus

Explanation
Anterior polar cataracts are usually small and non-progressive, do not impair vision and may be
associated with microphthalmos, a persistent papillary membrane and anterior lenticonus.
Posterior polar cataracts cause more visual impairment and tend to be larger than
anterior polar cataracts. They can be associated with posterior lenticonus or a remnant of
the tunica vasculosa lentis.
Both anterior and posterior polar cataracts can be autosomal dominant or sporadic.
A patient has with-the-rule astigmatism. During routine cataract surgery a toric IOL is placed into
the capsular bag. The day following surgery the lens appears to have rotated 90 degrees.
Which statement is most accurate?

lens adjustments should be delayed for 2 months
the total astigmatism has increased
the lens should have been placed into the ciliary sulcus
a lens exchange is necessary

Explanation
By a rotation of 90 degrees off alignment, the toric IOL is acting to increase (rather than
neutralize) the pre-existing astigmatism.
As a general rule of thumb, a toric IOL loses one third of its power for every 10 degree rotation
off alignment. By 30 degrees, the toric IOL has lost most of its power (equivalent to a spherical
lens).
In the case above, the lens should be re-aligned, ideally within the first few weeks of surgery
before the anterior and posterior capsules fuse together, making further adjustment difficult.
During a cataract operation, the intraocular lens is accidentally placed in the sulcus rather than
within the capsular bag.
This will result in:

with-the-rule astigmatism
irregular astigmatism
myopic shift
hypermetropic shift

Explanation
The lens is placed more anteriorly and therefore results in a myopic shift compared to the
predicted outcome.
Which is TRUE regarding cataracta punctata caerulea:

there is an association with anterior lenticonus
it is associated with deafness and glomerulonephritis
it is associated with excess copper deposition
it is visually insignificant

Explanation
Cataracta punctata caerulea (blue-dot cataract) is a common, innocuous congenital cataract.
A 65-year-old man undergoes an ECCE for a Morgagnian cataract. Postoperatively, he has a

peaked pupil, hypotony, and incarceration of iris into the wound with leakage. Two weeks later, a
grayish white membrane with a scalloped, thickened leading edge was noted on the posterior
corneal surface. Treatment of involved iris tissue with argon laser turns this membrane white.
Which is the MOST appropriate treatment?

beta-irradiation to involved tissues
topical dexamethasone
excision of involved iris tissues and cryotherapy to remaining corneal membranes
photocoagulation of involved iris tissues and cryotherapy to remaining corneal membranes

Explanation
Epithelial downgrowth has been reported most commonly as a complication of cataract surgery,
but it can occur after penetrating keratoplasty, glaucoma surgery and penetrating trauma. It is
more likely when surgery is associated with haemorrhage, inflammation, vitreous loss, or
incarcerated tissue. It usually results in intractable secondary angle closure glaucoma unless
successfully treated. The treatment is difficult and usually unrewarding. All the techniques attempt
to close the fistula and destroy the epithelium inside the eye. The extent of iris involvement can be
outlined using the argon laser. Irradiation and photocoagulation have been abandoned as
ineffective. Excision of involved iris tissue with cryotherapy to remaining corneal membranes is
currently the best technique, although good vision is maintained in only a few cases.
During phacoemulsification cataract surgery you remove the lens uneventfully. However, during
cortical clean-up with automated irrigation-aspiration you notice sudden shallowing of the anterior
chamber and a darkening of the red reflex. The globe is firm to palpation.
What is your next most suitable step?

keep the I/A probe in the eye and request the bottle height to be elevated maximally
remove the I/A probe and suture the corneal wounds
keep your I/A probe in the eye with the irrigation on and with your other hand inject viscoelastic
to reform the chamber
remove the I/A probe and ask for an urgent indirect ophthalmoscope to assess the fundus
remove the I/A probe and inject a high-molecular weight viscoelastic to deepen the iris-capsular
complex

Explanation
The findings are in keeping with a supra-choroidal haemorrhage which is an intra-ocular
emergency. The priority is to prevent expulsive haemorrhage by abandoning the cataract operation
and securing all wounds. If the wounds are large and cannot be sealed before expulsion of intra-
ocular contents is imminent, then digital pressure tamponade of the eye is appropriate.
Ectopia lentis associated with mental retardation may be seen in which of the following disorders:
Down's syndrome
Marfan's
Sturge-Weber syndrome

Explanation
Sturge-Weber can cause infantile glaucoma, with buphthalmos and secondary ectopia lentis.
However, Sturge-Weber patients have normal intelligence; likewise for Marfan's. Down's
syndrome is not associated with ectopia lentis.
The combination of ectopia lentis and mental retardation may be seen in:
 hyperlysinaemia
 homocystinuria
Compared to retrobulbar anaesthesia, peribulbar anaesthesia:

has a higher risk of optic nerve damage
causes greater akinesia
has a greater risk of globe perforation
causes higher intra-orbital pressure

Explanation
Peribulbar anaesthetic requires greater volume than retrobulbar anaesthetic and therefore causes a
higher intra-orbital pressure. It also results in less akinesia compared to retrobulbar and causes
more chemosis. However, the advantage of peribulbar is that there is a lower risk of optic nerve
damage, globe perforation and systemic neurological effects of anaesthesia.
Which is NOT a characteristic feature of Marfan's syndrome:

absence of iris processes on gonioscopy
bilateral, symmetrical, superotemporal lens subluxation
poor iris dilatation following instillation of mydriatics
retinal detachment associated with lattice degeneration

Explanation
Marfan's syndrome is associated with hypoplasia of the dilator pupillae which renders mydriasis
difficult. Angle anomalies are present in 75% of eyes, characterised by dense iris processes and
thickened trabecular sheets, which may be responsible for glaucoma.
What is the most common cause of chronic post-operative endophthalmitis?
Proprionobacterium acnes
Klebsiella sp
Aspergillus fumigatus
Candida albicans

Explanation
The most common causes of chronic post-op endophthalmitis are (in order):
 Propionobacterium acnes
 partially-treated Staphylococcus epidermidis
 fungi spp
All of the following factors will reduce the rate of posterior capsular opacification EXCEPT:
maximal contact between the IOL optic and posterior capsule
a square-edge optic design
a capsulorhexis diameter slightly larger than the size of the optic
anterior capsule polish

Explanation
A capsulorhexis diameter slightly smaller than the size of the optic is recommended to limit the
rate of PCO formation by increasing optic-capsule contact. Other options supplied are true.
Congenital lens dislocations are commonly inherited as:

autosomal dominant
autosomal recessive
mitochondrial
X-linked recessive

Explanation
Simple ectopia lentis is usually transmitted in an autosomal dominant fashion.
What is the most common cause of acute post-operative endophthalmitis?

Haemophilus influenzae
Staphylococcus aureus
Streptococcus pneumoniae
Klebsiella sp

Explanation
The most common cause of acute post-operative endophthalmitis is Staph epidermidis, followed
by Staph aureus and Strep pneumoniae.
Which of the following is LEAST likely to cause delayed-onset post-operative endophthalmitis?

Corynebacterium
Achromobacter
Propionibacterium granulosum
Klebsiella

Explanation
Klebsiella or other Gram-negative bacilli may be seen in endogenous endophthalmitis but would
be an unlikely cause of delayed-onset postoperative endophthalmitis.
The causes of delayed-onset endophthalmitis after surgery include:

 Staphylococcus epidermidis (within 6 weeks)
 Candida (within months)
 Propionibacterium acnes (months to years)
 Others: Propionibacterium granulosum, Achromobacter, Corynebacterium
Which type of congenital cataract does NOT require systemic laboratory evaluation?
anterior lenticonus
bilateral cataract with no family history
monocular cataract with no family history
cataract associated with retinal pigmentary changes

Explanation
Bilaterality makes a systemic condition more likely. Monocular cataracts rarely herald significant
systemic derangements. Anterior lenticonus may be part of Alport's syndrome. Pigmentary
retinopathy raises the possibility of congenital infections.
A white cataract associated with a shrunken and wrinkled anterior capsule due to leakage of water
out of the lens is best described as:
immature
mature
hypermature
Morgagnian

Explanation
Stages of cataract maturity:
 Immature: lens is partially opaque
 Mature: lens is completely opaque
 hypermature: shrunken anterior capsule due to leakage of water from lens
 Morgagnian: hypermature cataract with liquefaction of cortex, allowing the nucleus to
sink inferiorly
Corneal compression can be suspected by comparing the following readings between scans:
corneal thickness
posterior chamber depth
lens thickness
anterior chamber depth

Explanation
The A-scan produces measurements for anterior chamber depth, lens thickness, and total eye
length. The normal ACD reading is between 2.5 and 4 mm. An outlier AC depth measurement
much smaller than others taken for the same eye, suggests compression of the cornea during that
reading.
Part 2 Premium – Tutor Mode

Question 60 of 60
Score: 65 %
What clinical finding might you expect in a patient with Christmas-tree cataract?
wasting of the temporalis

secondary angle closure glaucoma
aminoaciduria
elevated blood sugar

Explanation
Christmas-tree cataract is associated with myotonic dystrophy, occurring in up to 90% of these
patients. While visually insignificant, Christmas-tree cataract may progress in later life to stellate
posterior subcapsular cataract.
All of the following infections can cause congenital cataract EXCEPT:
rubella
herpes zoster
HIV
tuberculosis

Explanation
Intrauterine infections that cause cataract include: (TORCH)
 toxoplasmosis
 other: HIV
 rubella
 CMV
 Herpes: simplex and zoster
Tuberculosis does not cause congenital cataract.
Which cataract is LEAST likely to be found in a 6-week-old infant:

cataracta punctata caerulea
sutural
lamellar
coronary

Explanation
Congenital cataracts tend to be: nuclear, lamellar, coronary, blue-dot (cataracta punctata caerulea),
sutural, anterior polar, posterior polar, oil droplet or membranous.
All of the following are associated with ectopia lentis EXCEPT:
Aniridia
Hyperlysinaemia
Refsum syndrome
Sulphite oxidase deficiency
Stickler's syndrome

Explanation
Refsum syndrome is not associated with ectopia lentis.
Ectopia lentis is associated with:

 Familial ectopia lentis
 Marfan syndrome
 Weill-Marchesani
 Homocystinuria
 Familial microspherophakia
 Hyperlysinaemia
 Sulphite oxidase deficiency
 Stickler syndrome
 Sturge-Weber syndrome
 Crouzon syndrome
 Ehlers-Danlos syndrome
 Aniridia
Posterior lenticonus is associated with:
keratoconus
bilateral lens defect in most cases
posterior polymorphous corneal dystrophy

Explanation
Posterior lenticonus typically occurs sporadically although it can more rarely be a feature of
Lowe's syndrome. It is usually unilateral and causes irregular, lenticular astigmatism.
Anterior lenticonus can occur with posterior polymorphous corneal dystrophy in the context of
Alport syndrome.
Alport's syndrome is characterised by:
 X-linked recessive (80%) and X-linked dominant (5%) inheritance patterns

 defect in Type IV basement membrane collagen
 renal failure secondary to glomerular dysfunction
 megalocornea
 anterior sub-capsular cataract
 corneal arcus
 peripheral retinal flecks
A 68-year-old man presents on the first day following routine cataract surgery complaining of
severe eye pain. Visual acuity is hand movements at 3 meters in the involved eye. Slit-lamp
examination reveals a diffusely shallow anterior chamber and corneal edema without hypopyon.
The next step should be:

dilated fundus examination
pachymetry
tonometry
B-mode ultrasonography
gonioscopy

Explanation
Establishing the IOP is crucial. High IOP secondary to angle closure (e.g. aqueous misdirection)
may be causing severe eye pain and decreased visual acuity. Hypotony (wound leak or
cyclodialysis) can also cause a shallow anterior chamber and cloudy cornea and may predispose to
a painful choroidal hemorrhage.
A neonate fails to thrive and has been vomiting. A reducing substance was identified in the urine.
The paediatrician has noted abnormal red reflexes and is concerned about cataract.
Which cataract are you most likely to encounter in this case?

stellate posterior subcapsular cataract
sun-flower cataract
cerulean cataract

Explanation
Figure: Oil droplet cataract in galactosaemia.
This case is suggestive of galacosaemia, which causes an oil-droplet cataract within a few days of
birth in the majority of patients.
Three months after cataract surgery, a patient returns with low grade uveitis. A white plaque is
noted on the posterior capsule. The most likely cause of this condition is a:
gram- negative diplococci
gram-negative rod
gram-positive anaerobic rod
gram-positive catalase positive cocci

Explanation
This scenario is most suggestive of chronic endopthalmitis due to Proprionibacterium acnes,
which is a gram-positive anaerobic rod.
Which cataract is classically associated with myotonic dystrophy?

polychromatic crystalline
anterior polar
anterior cortical
nuclear
Explanation
Christmas-tree cataract (a polychromatic crystalline needle-like opacity in the deep cortex and
nucleus) is associated with myotonic dystrophy. It occurrs in up to 90% of these patients. While
visually insignificant, Christmas-tree cataract may progress in later life in patients with myotonic
dystrophy to stellate posterior subcapsular cataract.
All are characteristics of persistent hyperplastic primary vitreous EXCEPT:
prone to angle closure glaucoma
progressive cataract
typically unilateral

Explanation
PHPV is a congenital, non-hereditary ocular malformation. It is typically unilateral. Findings
include:
 elongation of ciliary processes

 prominent radial iris vessels
 persistent hyaloid artery
 microphthalmia
 ectopia lentis
 cataract
A patient has posterior polymorphous corneal dystrophy. There is a history of hearing loss.
What is the most likely systemic diagnosis?
Gaucher disease
Lowe syndrome
Cystinosis
Alagille syndrome
Alport syndrome

Explanation
Alport syndrome can be inherited in both X-linked recessive (80%) and X-linked dominant (5%)
forms. Where X-linked recessive, heterozygous females may still experience haematuria - a
separate condition termed 'thin basement membrane nephropathy.' Reference: NIH National
Library of Medicine.
Alport's is caused by a defect in Type 4 (basement membrane) collagen. Clinically it causes:
 glomerular basement membrane nephritis and renal failure

 hearing loss
 cortical cataract
 juvenile arcus
 dot-fleck retinopathy
A 73-year-old woman had an expulsive suprachoroidal haemorrhage in her right eye following
cataract surgery five years ago. She has a dense cataract in her only left eye with vision 6/36.
All of the following may be steps to prevent expulsive haemorrhage in this eye EXCEPT:
use of Honan pressure balloon after retrobulbar anaesthesia
general anaesthetic with deep anaesthesia
single plane corneal incision

closure of wound with nylon sutures
well controlled blood pressure during surgery

Explanation
Risk factors for expulsive choroidal haemorrhage include:
 myopia
 glaucoma
 atherosclerotic disease
 hypertension
 previous expulsive haemorrhage
Steps to prevent supra-choroidal haemorrhage include:
 small incision
 shelved or self-sealing incisions
 decompress globe before surgery with digitial compression or device
 close wound with non-absorbable sutures
 keep blood pressure well controlled during surgery
 deep anaesthesia if GA used
In which of the following individuals is pseudoexfoliation most typically found?

middle-aged East Asian women
young, myopic males
elderly, white women
men with type A personality

Explanation
Psuedoexfoliation is typically found in people of Scandinavian descent and the incidence increases
with age.
Pigment dispersion typically affects young myopic males.
A 28-year-old has progressive dementia, jaundice and akinetic rigidity in his upper limbs.
What is the most likely slit-lamp examination finding in this patient?
cerulean cataract
sun-flower cataract

Explanation
Figure: Sunflower cataract in Wilson's disease.
The clinical description in the question above is in keeping with Wilson's disease.
Wilson's disease is characterised by:

 autosomal recessive inheritance
 abnormalities in copper transport
 copper deposition in tissues including the liver, brain and eyes
 liver dysfunction
 basal ganglia deposition: dementia, neuropsychiatric symptoms
 eyes:
o sunflower cataract (anterior subcapsular)
o Kayser-Fleischer rings from deposition of copper in Descemet's membrane
 chemistry: low serum ceruloplasmin, paradoxically low serum copper, high urinary
copper
Which of the following is NOT typically associated with homocystinuria?

red hair
lens dislocation superiorly
mitral valve prolapse

Explanation
Homocystinuria predisposes to ectopia lentis, which is typically infero-nasal. Other statements are
true.
Marfan’s syndrome is typically associated with each of the following EXCEPT:

hyperopia
glaucoma
keratoconus
hypoplasia of dilator pupillae
upward subluxation

Explanation
All of the above are typical ocular associations of Marfan’s with the exception of hyperopia.
Marfan’s is in fact associated with axial myopia and retinal detachment.
What is the optimum time to operate on a patient with bilateral dense congenital cataracts?
between 2 months and 6 months of age
between 6 months and 1 year of age
as soon as possible, even within the first few weeks of life
between 1 and 2 years of age
Explanation
The critical period of visual development is the first few months of life. Earlier treatment can
result in better visual acuity. Bilateral visually significant cataracts can cause irreversible
amblyopia and sensory nystagmus
Which IOL material is widely held to have the lowest propensity for posterior capsule
opacification?
hydrophilic acrylic
silicone
hydrogel
hydrophobic acrylic
PMMA

Explanation
There are many factors thought to be important in limiting PCO, and foremost among these is the
shape of the optic, which should be square-edged to limit epithelial cell migration. Good cortical
cleanup and capsular polishing at the time of surgery to reduce lens epithelial cells also plays a
role. IOL materials have also been investigated, with hydrophobic acrylic materials believed to
have the lowest propensity for PCO.
A high myope of -14D spherical equivalent right eye has biometry in preparation for cataract
surgery. Keratometry readings are K1: 44.1D and K2: 44.5D. The axial length is measured as
23.8mm.

index myopia
keratoconus
axial myopia
posterior lenticonus

Explanation
This patient has normal K readings and normal axial length. The possibilities are index myopia
due to significant nuclear sclerosis (but a shift as high as 14D is unlikely and the patient was a
high myope to begin with) or an abnormality of the lens such as posterior lenticonus.
A careful look for posterior lenticonus is suggested in cases in which there is a discrepancy
between the biometry and refraction -- and no significant nuclear sclerosis to account for the high
myopia. Surgeons should be aware of dehiscence or thinning of the posterior capsule while doing
cataract extraction in these patients.
Cataract can be induced by all of the drugs below EXCEPT:
hydroxychloroquine
allopurinol
phenothiazide
amiodarone

Explanation
Chloroquine can cause cataract; however, there is no evidence for an association between
hydroxychloroquine and cataract.
Drugs causing cataract include:
 chlorpromazine (a phenothiazide): fine yellow-brown granules on anterior lens capsule

 gold: innocuous, anterior capsular deposits in 50% of patients
 busulphan
 allopurinol
 corticosteroids
 amiodarone
Which topical drop is most helpful in preventing glaucoma in a patient with short stature, stubby
fingers, joint stiffness and associated syndromic ocular features?
atropine
timolol
latanoprost
pilocarpine
dorzolamide

Explanation
This patient has Weill Marchesani syndrome, which is characterized by microspherophakia and
ectopia lentis. Microspherophakia may lead to an angle-closure glaucoma with pupillary block
because the spherical shape increases the degree of iris-lens contact. Miotics further exacerbate
this by shifting the iris-lens diaphragm forward and further increasing iris-lens contact (as the lens
becomes even more spherical). Cycloplegia moves the diaphragm backward and flattens the lens.
It is the medical treatment of choice.
Dilated examination of a 36-year-old woman's eye reveals a dense white spot on the vitreal
surface of the posterior capsule just inferonasal to the center of the posterior capsule.
The patient should be advised that:
she has a benign finding with no visual implications
she should have a glucose tolerance test
pre-senile visually significant cataract formation is likely
she should have urinalysis performed to detect haematuria and proteinuria

Explanation
Mittendorf's dot is a remnant of the posterior tunica vasculosa lentis and results in a white dot
infero-nasally on the posterior capsule of the lens. It is meaningless visually.
Which of the following drugs is LEAST likely to cause cataract formation:

phenothiazine
echothiophate
dexamethasone
pilocarpine
amiodarone

Explanation
The evidence for pilocarpine inducing cataract is tenuous. However, by causing miosis, it may
make cataracts more symptomatic. Among miotic agents, long-acting anticholinesterase drops
(which have now fallen out of common use) such as demecarium and echothiophate were
associated with cataract formation, especially anterior subcapsular vacuoles and posterior cortical
cataracts. However, the shorter-acting cholinergic miotic agents such as pilocarpine have not been
conclusively shown to induce cataract. We are aware that some general text sources still refer to
pilocarpine causing cataract but the evidence is weak. All the other options supplied have been
well documented in causing cataract.
In UGH syndrome which is TRUE?

it is usually bilateral
it is usually asymptomatic
it may be associated with skin depigmentation
it can be caused by a posterior chamber IOL

Explanation
Uveitis-glaucoma-hyphaema (UGH) syndrome is most commonly caused by the placement of an
ill-fitting or mal-positioned anterior chamber IOL. This results in iris and angle trauma, with
chronic inflammation, secondary iris neovasculariation, recurrent hyphaemas and glaucoma. The
syndrome can, however, occur with posterior chamber lenses and suture-fixated lenses.
Which of the following metabolic syndromes can cause pre-senile cataract:
1. hypoparathyroidism
2. pseudo-hypoparathyroidism
3. mannosidosis
4. hypoglycaemia
1 and 3
1, 2 and 3
1, 2, 3 and 4
1 and 4

Explanation
Cataract can be caused by host of metabolic syndromes including:
 hyperglycaemia
 hypoglycaemia,
 hypoparathyroidism
 pseudo-hypoparathyroidism
 mannosidosis
 Fabry's disease
 galactosaemia
 Lowe's syndrome
A 17-year-old boy with mental retardation and short stubby fingers attends clinic. You notice
bilateral inferior lens subluxation.
Given the history, what feature are you MOST likely to encounter?
tendency to thrombo-embolic events
a small lens
mitochondrial inheritence
increased arm-span

Explanation
This patient has Weil Marchesani syndrome. This condition is characterised by:
 AD inheritence
 short stubby fingers
 downward lens subluxation
 microspherophakia
A patient undergoes cataract surgery complicated by a posterior capsular tear with a sulcus IOL
inserted. 3 months later the intra-ocular pressure is 28mmHg.
What is the most likely cause?

pigment dispersion
UGH syndrome
angle recession
aqueous misdirection

Explanation
Sulcus IOL’s often cause pigment chaffing from the iris, which may lead to raised IOP. UGH is
uncommon with modern IOLs, even those in the sulcus.
Which of the following is NOT associated with ectopia lentis?
Homocystinuria
Aniridia
Posterior embryotoxon
Weill-Marchesani

Explanation
Posterior embryotoxon is not associated with ectopia lentis.
Ectopia lentis is associated with:
 Marfan's
 Homocystinuria
 Ehlers-Danlos
 hyperlysinemia
 sulfite oxidase deficiency
 trauma
 syphilis
 aniridia
 Weill-Marchesani
Which is TRUE of cataract surgery in nanophthalmic eyes?
phacoemulsification without IOL insertion is the recommended procedure
anterior sclerotomies may be indicated at the time of surgery
the complication rate is similar to other cataract surgeries
trabeculectomy is contraindicated at the time of cataract extraction

Explanation
Nanophthalmic eyes have a higher complication rate.
Potential complications include:
 retinal detachment
 choroidal effusion
 post-op angle closure glaucoma
 flat anterior chamber
 cystoid macular oedema
 corneal decompensation
 malignant glaucoma
Phaco with IOL is generally the procedure of choice, using the smallest incision possible. In
patients with glaucoma, cataract extraction may be combined with trabeculectomy. When the
anterior chamber is shallow pre-operatively and the choroid is thickened, anterior sclerotomies are
indicated at the time of surgery to reduce the chances of uveal effusion.
Which cataract can be found in a patient with deafness and haemorrhagic nephritis?
lamellar
anterior polar
snowflake
nuclear sclerotic
Christmas-tree

Explanation
The clinical features suggest Alport's syndrome, which is associated with anterior lenticonus and
anterior polar cataracts. Cases of anterior and posterior subcapsular cataract and cortical cataract
have also been described in association with Alport's. Anterior polar cataracts are usually non-
progressive and usually do not affect vision.
Wedl bladder cells are found in:

posterior sub-capsular cataract
anterior polar cataract
nuclear cataract
cortical cataract

Explanation
In cortical cataracts, there is hydropic swelling of lens fibres. Eosinophilic globular material may
be found between lens fibres called morgagnian globules.
In nuclear cataract there is homogenous loss of cellular laminations.
In posterior subcapsular cataract there is posterior migration of lens epithelial cells, which swell to
become Wedl or bladder cells.
Which statement(s) is/are true regarding cohesive and dispersive viscoelastics?
1. Cohesive viscoelastics have a higher molecular weight

2. Dispersive viscoelastics cause higher pressure if left in situ
3. Dispersive viscoelastics are harder to remove
1, 2 and 3
1 and 3
3 only
2 and 3
2 only

Explanation
Cohesive viscoelastics are:
 longer chain molecules and have higher molecular weight

 easier to remove from the eye
 cause a higher rise in IOP if left in the eye
 used to maintain spaces (while dispersives are used to coat or maintain compartments)
Which of the following complications is/are more frequently seen following cataract surgery in
children than in adults?
1. opacification of posterior capsule

2. glaucoma
3. retinal detachment
4. cystoid macular oedema
1, 2, and 3
4 only
1, 2, 3, and 4
1 and 3
2 and 4

Explanation
Posterior capsule opacification, glaucoma (angle closure caused by excessive inflammation), and
retinal detachment (lifetime risk about 3%) are more common postoperative complications in
children undergoing cataract extraction. The incidence of cystoid macular oedema is lower in
children than in adults.
The Early Vitrectomy Study (EVS) found that for patients with post-operative endophthalmitis an
early vitrectomy leads to a better visual outcome compared to standard intravitreal antibiotics if
the presenting vision is:
6/60 or worse
PL or worse
CF or worse
HM or worse

Explanation
The EVS study found that for patients presenting with vision of PL or worse early vitrectomy led
to a three-fold increase in the number of these patients ultimately achieving 6/12 or better.
A cataract in which liquefaction of the cortex has allowed the nucleus to sink inferiorly is best
described as:
Morgagnian
immature
hypermature
mature

Explanation
Stages of cataract maturity:
 Immature: lens is partially opaque

 Mature: lens is completely opaque
 Hypermature: shrunken anterior capsule due to leakage of water from lens
 Morgagnian: hypermature cataract with liquefaction of cortex, allowing the nucleus to
sink inferiorly
A 15-year-old boy presents with bilateral infero-nasal ectopia lentis. He is tall with malar flush.
Which of the following is LEAST likely to be found in this patient?

decreased serum methionine
red hair
increased risk of thromboembolism
mental retardation
osteoporosis

Explanation
This patient has homocystinuria. Homocystinuria is an autosomal recessive condition caused by
decreased activity of cystathionine beta-synthetase, resulting in systemic accumulation of
homocysteine and methionine. Systemic features include: marfanoid habitus, fair or red hair,
malar flush, osteroporosis, and a tendency to thrombotic episodes. Ectopia lentis is typically
infero-nasal.
All are associated with classic (Type 1) galactosaemia EXCEPT:

renal disease
hepatosplenomegaly
autosomal recessive
deficiency of galactokinase

Explanation
Classic (Type 1) galactosaemia is caused by a deficiency of galactose-1-phosphate uridyl
transferase (GPUT). It is an autosomal recessive condition associated with oil droplet cataract.
Systemic features are common including failure to thrive, hepatosplenomegaly and renal disease.
Type 2 galactosaemia is caused by galactokinase deficiency. This condition is autosomal

recessive, but unlike Type 1, affected children are generally healthy and the cataract is lamellar
(not oil droplet).
A patient who had a complicated cataract operation 3 months ago presents with painful blurry
vision. On examination, there is sectoral corneal oedema with an underlying grey membrane on
the corneal endothelial surface. The pupil is distorted and there are cells in the anterior chamber.
The IOP is 34 mmHg.
What is the diagnosis?

epithelial downgrowth
toxic anterior segment syndrome
iridocorneal endothelial syndrome
phacoanaphylactic uveitis

Explanation
Epithelial downgrowth is rare nowadays. It typically presents a few months after surgery or
trauma. Epithelial downgrowth is commonly caused by a persistent wound leak, which allows
conjunctival or corneal epithelium to grow over normal tissues, including corneal endothelium,
iris and angle structures. Glaucoma is caused by synechial angle closure.
Appearances may be similar to iridocorneal endothelial (ICE) syndrome, with glaucoma, corneal
decompensation and iris irregularities but ICE syndrome exists preoperatively.
According to the Royal College of Ophthalmologists guideline for cataract surgery (2010), which
of the following is NOT a recommendation:
a patient at risk of cystoid macular oedema should be considered for pre- and post- operative
topical non-steroidal drops
intravenous access is required for peribulbar anaesthetic
suturing of the corneal section is best avoided if possible in children
if the departmental endophthalmitis rate is higher than that of the published Bolton study
(0.055%), then intracameral cefuroxime should be considered

Explanation
The guideline recommends that the main corneal section is sutured in children to prevent wound
leak and astigmatism.
Intravenous access is recommended for peribulbar and retrobulbar anaesthetic.
Which is TRUE regarding phacoemulsification machine designs?
the speed of rollers in the peristaltic pump allows linear control of aspiration rate
the diaphragm pump allows instantaneous vacuum
the peristaltic pump requires a very slow building of vacuum for aspiration
the Venturi pump has stepwise increases in vacuum

Explanation
Three kinds of aspiration systems exist in phaco machines:
 Peristaltic pump: has rollers along tubing, creating a relatively rapid rise in vacuum
provided the phaco tip is occluded
 Diaphragm pump: has valves over the inlet and outlet of a fluid chamber covered by a
diaphragm, allowing slower build-up of vacuum
 Venturi pump: produces the most rapid increase in vacuum. It works by flow of air over
an opening and is not dependent on phaco tip occlusion
A young child with developmental delay is found to have microphakia, posterior lenticonus and
cataract. There is raised intra-ocular pressure bilaterally.
Given these findings, which kidney impairment is MOST likely?
non-specific glomerulonephritis
Fanconi syndrome
IgA nephropathy
anti-glomerular basement membrane

Explanation
This child has features consistent with Lowe's (oculocerebrorenal) syndrome.
Lowe syndrome is associated with:
 x linked recessive inheritance

 mental handicap
 Fanconi syndrome of proximal renal tubules
 muscular hypotonia
 congential cataract
 microphakia
 posterior lenticonus
 glaucoma
A patient with a strong eyelid squeeze response during cataract surgery has an increased risk of
complications. This is mainly due to:
iridodonesis
excessive mobility of the posterior capsule
positive pressure from the vitreous cavity
zonular instability

Explanation
Squeezing creates positive vitreous pressure, which can shallow the anterior chamber. Chamber
instability increases the chances of posterior capsular tears.
A patient has bone-spicule pigmentation at the retina, with arteriolar attenuation and cataract.
What cataract is most likely?

posterior cortical
Christmas tree
nuclear
anterior polar

Explanation
Retinitis pigmentosa is associated with posterior subcapsular cataract.
A 40-year-old patient with mitral regurgitation presents to clinic. You notice bilateral lens
subluxations and a high-arched palate.
Which is TRUE?
accommodation is usually intact
inheritance is AR
there may be associated mental retardation
lens subluxation is typically downwards

Explanation
This patient has Marfan’s syndrome, which is autosomal dominant. Intellect is normal. Associated
systemic features include arachnodactyly, increased arm span:height ratio, high arched palate and
cardiac valvular anomalies. Lens subluxation is upwards in Marfan’s and zonules are present with
intact accommodation, unlike homocystinuria where subluxation is downwards and zonules are
absent with accommodation lost.
What is the incidence of clinically relevant cystoid macular oedema requiring treatment after
phacoemulsification cataract surgery?
20%
1%
6%
10%

Explanation
The incidence of clinically relevant CMO post-cataract surgery is about 1% across studies,
although angiographic and OCT evidence of CMO may be found in up to 10% of patients.
A 27-year-old man with mental retardation attends clinic. He has bilateral lens subluxation. You
notice pale blond hair and malar flush.
Which is TRUE?
fingers are likely to be short and stubby
there is a risk of osteoporosis
inheritance is autosomal dominant
there is an association with microspherophakia

Explanation
This patient has homocystinuria. This condition is characterized by:
 pale hair (blond or red)

 autosomal recessive inheritance
 osteoporosis
 increased risk of thrombo-embolic events
 downward lens subluxation with absent zonules and accomodation
Note: microspherophakia and short stubby fingers are associated with Weil Marchesani syndrome,
which can also cause downward lens subluxation
A patient with Stickler's syndrome is most likely to develop which pre-senile cataract:
cortical
posterior subcapsular
Christmas-tree
nuclear cataract
anterior subcapsular

Explanation
Hereditary fundus dystrophies such as RP, Leber's congenital amaurosis, gyrate atrophy and
Sickler's syndrome may be associated with posterior subcapsular lens opacities.
Which of the following is associated with lamellar cataract:
galactosaemia
argyrosis
Peter's anomaly
Fabry's disease
Explanation
Lamellar cataract may be inherited or caused by:
 rubella
 diabetes
 galactosaemia
 hypocalcaemia
In which of the following vertically-transmitted infections is congenital cataract LEAST likely?

rubella
CMV
syphilis
HSV
toxoplasmosis

Explanation
Congenital cataract formation secondary to maternal HSV infection is extremely unusual because
the infection is typically acquired at birth, by passage through an infected birth canal. On the rare
occasions where it is acquired earlier in pregnancy, then cataract can occur.
A patient with a history of atopy is most likely to develop which type of pre-senile cataract?
anterior polar
anterior subcapsular with radiating cortical fibres

nuclear
posterior cortical

Explanation
Atopic individuals have an increased incidence of anterior subcapsular cataract with radiating
cortical riders. Another association is keratoconus.
Which is TRUE of Mittendorf's dot:
1. It is a remnant of the anterior tunica vasculosa lentis

2. It is typically located on the infero-temporal posterior capsule
3. It is visually innocuous
1 and 3
3 only
1, 2 and 3
2 and 3
1 only

Explanation
Mittendorf's dot is a remnant of the posterior tunica vasculosa lentis. It is a white dot typically
located on the infero-nasal posterior lens capsule. It is visually innocuous.
Which of the following clinical features can be used to differentiate classic galactosaemia from
galactokinase deficiency?
an abnormal irido-corneal angle
the presence of cataract
hepatosplenomegaly

Explanation
The key feature clinically differentiating true galactosemia (galactose-1-phosphate
uridyltransferase deficiency) from galactokinase deficiency is the presence of
hepatosplenomegaly, mental retardation, and other systemic manifestations.
A 2-week-old infant presents with bilateral nuclear cataracts. What is the most common
identifiable cause?
Hereditary autosomal dominant congenital cataract
Persistent hyperplastic primary vitreous (PHPV)
Intrauterine infection
Galactosaemia

Explanation
Common, identifiable causes of bilateral congenital cataracts:
 autosomal dominant inheritence
 metabolic or systemic disease: e.g. DM, galactosaemia, or Lowe's syndrome
Common, identifiable causes of unilateral congenital cataracts:

 local dysgenesis (sporadic)
 PHPV
 lenticonus
What type of cataract is associated with atopic dermatitis?
anterior capsular sun-flower cataract
anterior subcapsular shield cataract
posterior cortical cataract

Explanation
Figure: anterior sub-capsular shield cataract in atopic dermatitis
Atopic dermatitis is associated with anterior subcapsular shield cataract. Wilson's disease is
associated with sunflower cataract; myotonic dystrophy with Christmas tree cataract and later with
stellate posterior subcapsular cataract; and neurofibromatosis-2 with posterior cortical cataract.
Irrigation of PMMA IOLs just before insertion is intended to:
facilitate insertion through a tight wound
remove static charges which attract debris

hydrate the lens to achieve correct refractive power
wash off residual oils from the manufacturing process

Explanation
PMMA lenses may pick up static charges that attract dust and debris when opened. Therefore the
lens may be rinsed with balanced salt solution before insertion.
A 70-year-old man underwent routine right phacoemulsification with posterior chamber IOL. The
first day, he returns with moderate epithelial and stromal oedema. 1-week later, the oedema is still
present.
Which is NOT a cause for the corneal oedema in this case?
surgical trauma
epithelial downgrowth
elevated IOP
chemical toxicity

Explanation
Epithelial downgrowth takes weeks to develop.
When does an anterior sutural cataract develop?
during adolescence
over age 40
between birth and 2 years of age
in utero

Explanation
Sutural cataract is a congenital cataract occurring during development of the fetal lens nucleus.
A patient undergoes cataract surgery. On review 3 days later, there are prominent Descemet's
folds and deep stromal folds with a central corneal thickness on pachymetry of 630 micrometers.
All of the following are possible causes EXCEPT:
anterior chamber phacoemulsification of a dense nucleus
endothelial toxicity from intracameral antibiotics
Descemet's membrane detachment
retained viscoelastic material within the anterior chamber

Explanation
Retained viscoelastic can cause an elevation in IOP and the development of corneal bullae and
microcystic oedema on the epithelial surface. However, it would not cause the appearance of deep
stromal folds and Descemet's folds, which suggests another aetiology such as endothelial damage
or toxicity.
Which is TRUE regarding posterior polar cataracts?
synonymous with posterior subcapsular cataract
malformation of posterior fibres with attachment to capsule

common in myotonic dystrophy

Explanation
Posterior polar cataract is a rare form of congenital cataract. It is usually inherited in an autosomal
dominant fashion, but can be sporadic. Posterior polar cataracts consist of dysplastic lens fibers,
which, in their migration posteriorly from the lens equator, exhibit progressive lens opacity,
increased degenerative changes, with the formation of a characteristic discoid posterior polar
plaque. There is extreme thinness and fragility of the posterior capsule with adherence of the
opacity to the capsule.
Myotonic dystrophy is associated with posterior cortical and Christmas tree cataracts.

Independent risk factors for retinal detachment following cataract extraction include:
1. retinal detachment in the contralateral eye

2. myopia
3. lattice retinal degeneration
4. Nd:YAG capsulotomy
1, 2, and 3
2 and 4
1, 2, 3, and 4.
4 only
1 and 3

Explanation
All of the above answers are potential risks for retinal detachment following cataract extraction.
The Royal College of Ophthalmologists cataract guidelines (2010) recommend all of the following
to preventing post-cataract endophthalmitis EXCEPT:
sub-conjunctival injection of antibiotic at the end of surgery
treat blepharitis, conjunctivitis and nasolacrimal infections before surgery
povidine iodine 5% instilled into the conjunctival sac pre-surgery
reject lens implants that have inadvertently touched the lid margins

Explanation
The College recommends that if local rates of endophthalmitis are simiar to those reported in the
Bolton study (0.055%), continuation with current measures is reasonable. However, if local rates
are higher, then intracameral cefuoxime may be added as part of a package of measures to lower
the rate of endophthalmitis after a suitable analysis of processes has taken place.
An 82-year-old patient underwent right cataract surgery 4 years previously at another eye hospital
with an anterior chamber IOL inserted. He presents with gradual reduction in vision over 6 to 9
months. His visual acuity in the right eye is 6/12 and slit lamp examination reveals epithelial
microcysts especially superiorly.
What investigation is most likely to reveal the cause of his reduced visual acuity?
corneal topography
specular microscopy
pachymetry
gonioscopy

Explanation
Anterior chamber IOL's can cause corneal decompensation from loss of endothelial cells,
especially if the ACIOL is mobile and ill-fitting. Specular microscopy can clinch the diagnosis by
confirming a low corneal endothelial cell count, which is likely to be less than 500 cells/mm .
2
This question came in the FRCS (Glasgow) Part 2 in October 2014.
A patient underwent complicated cataract surgery, involving a conversion from phaco to extra-
capsular cataract extraction. Pre-operatively the patient had no corneal astigmatism. The corneal
wound, which was located superiorly, was extended to 4mm during surgery and was sutured with
interrupted 10-0 nylon sutures which were tightened more than usual.
Where is the axis of the negative cylinder?
135 degrees
45 degrees
180 degrees
90 degrees

Explanation
The steep meridian is at 90, because of the over-tightened sutures. A steep meridan of 90,
corresponds to a negative cylinder with its axis at 180.
A patient with severe atopic dermatitis is most likely to suffer from which pre-senile cataract:
snowflake
stellate posterior cortical
nuclear sclerotic
christmas-tree
anterior subcapsular

Explanation
Patients with severe atopy are susceptible to cataract, which occurs in 10% of patients by the
second to fourth decades. Shield-like anterior sub-capsular cataract is characteristic. Posterior sub-
capsular cataract may also occur.
A 5-month-old infant presents with unilateral, complete cataract. There is no family history of eye
disease. Which investigation would be MOST helpful in determining the aetiology?
TORCH and syphilis titres
B-scan ultrasound
urinary protein and reducing substances
chromosomal analysis

Explanation
With unilateral cataracts and no family history, screening labs are not warranted, since unilateral
cataracts are usually isolated and sporadic. Metabolic diseases (such as Lowe's and galactosaemia
which are screened by urine), chromosomal abnormalities (such as trisomy 13 and Turners) and
intra uterine infection (TORCH screen: toxoplasma, rubella, cytomegalovirus, herpes simplex) all
cause bilateral congenital cataracts.
In a unilateral cataract, B-scan is very important to rule out secondary causes such as intra-ocular
tumours.
What is the most common cause of posterior capsular opacification following phacoemulsification
and implantation of an IOL?
proliferation of residual lens epithelium

bacterial colonisation
fibroblast ingrowth stimulated by the IOL
inflammatory debris secondary to a foreign body reaction

Explanation
Posterior capsular opacification is caused mainly by proliferation of retained lens epithelium and
capsular fibrosis and contraction.
A 74-year-old man presents 6-months after cataract surgery with a recurrent granulomatous
uveitis. While initially responsive to steroids, the inflammation recurs whenever the steroids are
discontinued. There is a white plaque on the posterior capsule and anterior vitritis.
Which one of the following is the most appropriate therapy for this condition?
intravitreal amphotericin B
oral prednisolone, G. dexamethasone 2 hourly and G. cyclopentolate tds
vitrectomy, posterior capsulectomy, and intravitreal injection of vancomycin
observation
intravitreal cefuroxime and vancomycin

Explanation
The clinical picture suggests delayed-onset postoperative endophthalmitis caused by P. acnes. The
recommended initial therapy includes a pars plana vitrectomy with posterior capsulectomy and
intravitreal injection of vancomycin with or without explant of the IOL.
Cataract surgery in a previously vitrectomised eye has a higher rate of complications, primarily
due to:
zonular instability
excessive mobility of the posterior capsule

positive pressure from the vitreous cavity
iridodonesis
collapse of the globe during surgery

Explanation
The posterior capsule is more mobile in vitrectomised eyes because of the loss of vitreous support.
This causes the capsule to move more posteriorly during irrigation, and more anteriorly during
vacuum/aspiration. As a result capsular tears are more likel
If standard IOL calculations are used to plan an emmetropic outcome with phacoemulsification
cataract surgery in a patient who has previously undergone laser-assisted in situ keratomileusis
(LASIK) surgery to correct myopia what is the MOST likely refractive outcome?
myopia
hyperopia
emmetropia

Explanation
When using standard IOL calculations in patients who have undergone myopic laser-assisted in
situ keratomileusis (LASIK), hyperopia after cataract surgery is common, because the standard
calculations lead to overestimation of central corneal power. The opposite occurs after hyperopic
LASIK.
Ablative laser procedures inherently change the relationship between the anterior and posterior
curvature of the cornea, immediately invalidating assumptions made by keratometers.
Furthermore, the true index of refraction will vary dependent on the amount of laser ablation (and
remaining tissue) further impeding the use of a basic or standardized keratometric index. The
upshot is that for eyes that have undergone myopic ablative refractive procedures, the resulting
keratometry readings obtained by standard keratometry or topography will appear erroneously
high, subsequently leading to an underestimation of the final IOL power and a hyperopic surprise.
Depending on the literature, this value is estimated to represent a difference of between 14% to
25%. The opposite will occur in patients that were previously hyperopic. To overcome this,
special post-LASIK formulas such as the Haigis-L are advised.
A young patient has glaucoma, cataract, mental retardation and renal tubular acidosis.

Gaucher disease
Alport syndrome
Cystinosis
Alagille syndrome
Lowe syndrome

Explanation
Lowe syndrome results in:
 renal tubular acidosis
 angiokeratomata
 corneal keloids
 glaucoma
 cataract
Which statement regarding congenital cataracts is TRUE?
the majority of congenital cataract is unilateral

unilateral cataracts are usually inherited autosomal recessively
bilateral cataract is invariably associated with systemic disease
unilateral cataracts usually affects full-term, healthy infants

Explanation
Unilateral cataracts usually affect full-term, healthy infants and are usually a sporadic occurrence
rather than inherited.
About two-thirds of congenital cataracts are bilateral. Not all bilateral cataracts are associated with
a systemic disorder, but chances are higher than with unilateral cataract
Three months after routine cataract surgery a patient has ongoing uveitis, which flares up
whenever an attempt is made to stop steroids. There is an obvious plaque involving the entire
posterior lens capsule.
What treatment is indicated?
urgent YAG posterior capsulotomy
posterior sub-tenons triamcinolone
vitrectomy, IOL removal with intravitreal vancomycin
oral prednisolone

Explanation
The clinical picture is in keeping with chronic post-operative endophthalmitis. The most common
causes are: Propionobacterium acnes, partially treated Staphylococcus epidermidis and fungi.
The diagnosis should be confirmed by AC tap, vitreous biopsy and consider removal of the
posterior capsule.
Treatment is by vitrectomy, intravitreal vancomycin (or amphotericin B if fungal infection is

suspected) and IOL removal.
A young patient with newly diagnosed diabetes mellitus may present with which pre-senile
cataract?
christmas-tree
snowflake
posterior subcapsular
lamellar
nuclear sclerotic

Explanation
The classic, young diabetic cataract consists of snowflake anterior cortical opacities, which may
resolve spontaneously or mature within a few days. Diabetes mellitus is also associated with
earlier-onset, age-related nuclear cataract.
Hydropic swelling of lens fibres is most consistent with:
anterior polar cataract
nuclear cataract
cortical cataract

Explanation
In cortical cataracts, there is hydropic swelling of lens fibres. Eosinophilic globular material may
be found between lens fibres called morgagnian globules.
In nuclear cataract there is homogenous loss of cellular laminations.
In posterior subcapsular cataract there is posterior migration of lens epithelial cells, which swell to
become Wedl or bladder cells.
Which is the most common type of congenital cataract?
complete
lamellar
capsular
nuclear

Explanation
Lamellar cataracts are the most common type of congenital cataracts and may occur as a result of
toxic influence to the fetus or may be inherited as an autosomal dominant trait.
How many clearly defined peaks should a good ultrasound A-scan have in a phakic eye?
six
four
five
seven

Explanation
There are five A-scan peaks in a phakic eye:

 corneal surface
 lens anterior surface
 lens posterior surface
 ILM of the retina
 sclera
In young children, anterior polar cataracts are typically:
unilateral and visually significant
unilateral and visually insignificant
bilateral and visually significant
bilateral and visually insignificant

Explanation
Anterior polar cataracts are usually bilateral and usually do not impair vision. They are typically
static. Anterior polar cataracts may occur sporadically, in association with other anterior segment
anomalies or may be inherited in an autosomal dominant fashion.
Which is FALSE regarding Nd:YAG laser?
it emits infrared radiation at 946nm
the laser wavelength is invisible to the naked eye
the pulsed laser creates plasma
the laser device is fitted with a helium/neon beam

Explanation
The Nd:YAG emits infrared radiation at 1064 nm. Other statements are true.
A patient taking amiodarone is MOST likely to develop:
snowflake cataract
stellate anterior capsular catarct

polymorphic crystalline cataract
pre-senile nuclear sclerotic cataract

Explanation
Both amiodarone and phenothiazine cause stellate cataracts in the anterior lens capsule.
A hypermetrope with an axial length of 21mm undergoes routine cataract surgery. At 3 weeks
post-op, he sees his optician because of deteriorating vision, which measures 6/36 in the operated
eye. The optician reports "retinal swelling".
uveal effusion syndrome
ciliary body detachment
cystoid macula oedema

rhematogenous retinal detachment

Explanation
Cystoid macula oedema is the most common retinal cause of reduced vision post-op. Uveal
effusion syndrome is predisposed in short eyes, particularly nanophthalmic eyes with an axial
length of 16mm or less. However, it is a rare event.
A young child who was treated for bilateral congenital cataract has recurrent haematuria on
urinalysis. These findings are MOST in keeping with:
Alport's disease
Fabry's disease
galactosaemia
Wilson's disease
Lowe's disease

Explanation
All the options listed above are associated with congenital cataracts and positive findings on urinalysis.
 galactosaemia: reducing substance in urine
 Lowe's: aminoaciduria
 Fabry's: sediments
 Wilson's: copper
 Alport's: blood (glomerulonephritis)
Anterior lenticonus is associated with:

Congenital rubella
Peter's anomaly
Weill-Marchesani
Lowe's syndrome
Alport's syndrome

Explanation
Anterior lenticonus is associated with Alport syndrome. Lowe syndrome is associated with posterior
lenticonus.
A 4-year-old boy has difficulty with vision at pre-school and delay in reaching developmental
milestones. On examination, he is highly myopic, with short stubby fingers and stiff joints.
Given the history, which medication would you avoid to prevent precipitating angle closure?
atropine
topiramate
salbutamol
pilocarpine

Explanation
The history is suggestive of Weill Marchesani syndrome, which is associated with ectopia lentis (usually
inferior) and spherophakia. In Weill Marchesani, the lens may dislocate anteriorly and given the spherical
nature of the lens, it may cause pupil block angle closure glaucoma, which is aggravated by miosis.
Conversely, cycloplegia can decrease pupil block, tightening zonules, decreasing anteroposterior lens
diameter and pulling the lens posteriorly.
Cataract extraction in patients with pseudoexfoliation syndrome can be more hazardous than in
other patients because of:
1. incomplete pupillary dilation

2. abnormally weak lens zonules
3. a brittle anterior capsule
4. abnormally thin sclera
4 only
1, 2, 3, and 4
1 and 3
2 and 4
1, 2, and 3

Explanation
Abnormally thin sclera is not a feature of pseudoexfoliation syndrome. The other three findings are
recognized hazards of cataract surgery in patients with pseudoexfoliation.
Which of the following conditions is/are known to be associated with cataracts in juveniles and
young adults?
1. myotonic dystrophy
2. neurofibromatosis type 1
3. atopic dermatitis
1 only
2 and 3
1, 2, and 3
1 and 3

Explanation
Neurofibromatosis type 2, not type 1, may be associated with posterior subcapsular cataracts (and acoustic
neuromas as well). Other options are true.
A patient with the pre-operative refraction of OS -4.00/+2.00 x 90 has a visually significant

cataract. Keratometry is 40.00 @ 180 and 42.00 @ 90. Which location of incision might help
decrease the amount of astigmatism?
incision at 180
incision at 45
incision at 90
incision at 135

Explanation
This patient has with-the-rule astigmatism (steep axis at 90 degrees) with no obvious lenticular component.
Placing the incision superiorly (at 90 degrees) will reduce the astigmatism post-operatively.
A 65-year-old white man presents with mild discomfort and gradual-onset blurred vision of his
right eye over 1 week. He had phacoemulsification with PCIOL in this eye 4 months previously. A
low-grade granulomatous anterior uveitis is diagnosed, topical steroids are commenced and the
inflammation responds favorably. After discontinuing the steroids, the symptoms return and the
patient again presents with a granulomatous anterior chamber inflammation, including a small
hypopyon and a mild anterior vitritis. There is a white plaque present at the equator of the
posterior lens capsule.
What is the MOST likely cause of this clinical picture?
Propionibacterium acnes
Candida albicans
Mycobacterium tuberculosis

Explanation
This patient has chronic post-operative endophthalmitis.
The causes of delayed-onset endophthalmitis after surgery include:
 Staphylococcus epidermidis (within 6 weeks)

 Candida (within months)
 Propionibacterium acnes (months to years)
 Others: Propionibacterium granulosum, Achromobacter, Corynebacterium
The presence of a white plaque within the capsular bag specifically suggests the diagnosis of P.
acnes. Treatment includes pars plana vitrectomy, posterior capsulotomy, intravitreal vancomycin
plus or minus removal of lens.
A 10 degree rotation of a toric IOL reduces the astigmatic effect of the lens by:
1%
30%
10%
0.1%

Explanation
A 10 degree rotation of a toric IOL reduces the astigmatic effect of the lens by 30%. A 30 degree rotation of
the lens keeps the same magnitude of cylinder but flips the axis to a new meridian.
A 12-year-old boy presents with bilateral infero-nasal ectopia lentis. He is tall with blond hair.
What treatment might be helpful?

vitamin B6
low phenylalanine diet
coenzyme Q
vitamin A

Explanation
This patient has homocystinuria. Homocystinuria is an autosomal recessive condition caused by decreased
activity of cystathionine beta-synthetase. This results in systemic accumulation of homocysteine and
methionine and decreased levels of cysteine. Vitamin B6 and a low methionine-high cysteine diet have
shown promising in treating these patients and in reducing ectopia lentis.
Coenzyme Q has been touted for patients with Leber's herediraty optic atrophy.
Vitamin A may be helpful in some cases of retinitis pigmentosa.
Which is TRUE regarding cataract surgery in patients with uveitis:

uveitis should be quiet for at least 8 months before surgery
patients with pars planitis have a low risk of post-op cystoid macular oedema
in juvenile idiopathic arthritis it is often preferable to leave the child aphakic
Fuch's heterochromic cyclitis has a particularly poor prognosis

Explanation
Cataract surgery in patients with uveitis should ideally be performed after a minimum of 3 months without
significant ocular inflammation, using systemic immunosuppression therapy if necessary. The chances of
complications are higher in patients with uveitis compared to routine cataract surgery, particularly in children
with JIA. There is debate about whether IOLs should be inserted in children with JIA, due to the high
incidence of cyclitic membranes, PCO and lens haze - and it is often advisable to leave such children
aphakic.
Fuch's heterochromic cyclitis has a relatively good surgical prognosis compared to many forms of iritis.
A 64-year-old woman undergoes routine phacoemulsification but the surgeon accidentally leaves
a moderate amount of viscoelastic in the eye.
How long after completion of the case might you expect a pressure spike?
10 hours
4 hours
30 minutes
24 hours

Explanation
Viscoelastics (including sodium hyaluronate, chondroitin sulfate and hydroxymethylcellulose) all produce
pressure spikes at a mean of 4 hours (+/- 1 hour) after surgery

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Cataract (No=175)

Regarding his cataracts, what would be the most appropriate advice:

Your answer was CORRECT

Marfan’s syndrome is typically associated with each of the following EXCEPT:

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When does an anterior sutural cataract develop?

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Which of the following is LEAST likely to be found in this patient?

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Which IOL material has the highest refractive index?

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aspiration of liquid vitreous through the pars plana

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 reduce vitreous volume by aspiration

Which is true of galactosaemia type 2?

1. oil droplet cataract

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Which is TRUE regarding homocystinuria:

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What feature would you anticipate on ocular examination?

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autosomal recessive inheritance

prone to angle closure glaucoma

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 elongation of ciliary processes

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The most common source of organisms leading to post-operative endophthalmitis is:

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Which is TRUE regarding posterior polar cataracts?

Your answer was INCORRECT

This question came in the 2014 FRCOphth.

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Type 2 galactosaemia is caused by galactokinase deficiency. This condition is autosomal

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Figure: Aponeurotic ptosis with high skin crease

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Which cataract is associated with long-term steroid use?

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Which medication increases the risk of complications during cataract surgery?

Your answer was INCORRECT

Posterior lenticonus is most likely to be found in:

Your answer was INCORRECT

With regard to ophthalmic viscoelastics, which is FALSE:

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This question came in the FRCS (Glasgow) Part 2 October 2014.

Which is TRUE regarding cataracts?

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This question appeared in the 2014 FRCOphth Part 2.

All are associated with acquired ectopia lentis EXCEPT:

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Which is FALSE regarding biometry with the Zeiss IOLMaster?

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