Key points 9 Flashcards
What are some systemic associations of CHRPE?
Familial adenomatous polyposis (FAP): widespread bowel polyps with increased risk of bowel cancer
Gardner syndrome: FAP with osteomas of the skull, mandible and long bones and soft tissue tumours such as epidermoid cysts, lipomas and fibromas
Turcot syndrome: FAP with tumours of the CNS such as medulloblastoma and glioma.
If Optokinetic nystagmus abnormalities are present, where is the brain lesion?
Parieto-occipital or frontal lobe
Causes of bilateral congenital cataracts?
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.
What are Herbert’s pits
punched-out limbal lesions representing necrotic limbal follicles associated with trachoma.
What deficiency is present in Hunter syndrome?
deficiency of iduronate sulphate sulphatase.
The ocular effects are:
corneal clouding
pigmentary retinopathy
optic atrophy
What is a dacryocystocele
presents at birth as an enlargement of the lacrimal sac due to a blockage at the valve of Hasner.
Which blood tests for syphilis would be expected to normalise following treatment?
The Venereal Disease Research Laboratory (VDRL) and rapid plasma reagin (RPR) tests reflect treponemal infection and both would revert to normal when treated.
What is the MOST important prognostic factor for 5-year survival in a child diagnosed with retinoblastoma:
tumour location. If retinoblastoma is strictly intraocular, there is a 95% 5-year survival, while if the tumour has extraocular extension (e.g. optic nerve involvement) the 5-year survival is as low as 5%.
What features can VHL present with?
retinal capillary haemangiomas (which can cause exudative RD)
cerebellar haemangioblastomas
pancreatic, hepatic, and renal cysts
renal cell carcinoma
pheochromocytomas
What are iris pearls pathognmonic for?
pathognomic sign of uveitis secondary to lepromatous leprosy. Bacteria invade the iris, and dead bacteria form slowly enlarging and coalescing iris pearls, which pedunculate and shed into the anterior chamber.
When are the A and V patterns significant?
A pattern: significant if 10 prism diopters
V pattern: significant if 15 prism diopters
Where are the firm points of vitreoretinal attachment?
major retinal blood vessels
the vitreous base
the edge of lattice retinal degeneration
the edge of retinal scars
optic nerve
Dot hemorrhages are due to rupture of capillaries in which layer:
rupture of capillaries in the outer plexiform layers. They are smaller and more circumscribed than flame hemorrhages.
In untreated eyes with pre-proliferative disease, the probability of progression to proliferative retinopathy over 2 years is approximately:
The risk is approximately 50% over 2 years.
Which feature is most likely to be found in association with snail-track degeneration?
Snail-track degeneration is most often associated with round retinal holes. Rarely, vitreous traction at the posterior edge of the lesion may cause a retinal tear following a PVD. Snail-track degeneration is characterised by tightly packed snow-flakes which give the retina a white frost-like appearance.
A 66-year-old lady presents with gradual onset metamorphopsia in the left eye over 3 to 6 months. Visual acuities are 6/6 right eye and 6/12 left eye. 6 years previously she was diagnosed with a left branch retinal vein occlusion, which was treated with sectoral pan-retinal photocoaguation.
What is the most likely diagnosis?
ERM the most likely cause in this case.
Where do the 3rd nerve fibres cross in the brain?
cross the red nucleus and medial cerebral peduncle
A 28-year-old healthy West African man presents with recurrent vitreous haemorrhage. Slit lamp biomicroscopy reveals peripheral retinal haemorrhages, vascular sheathing and fine new vessels in the right superotemporal quadrant. The left eye is normal.What is the MOST likely diagnosis?
Sickle-cell and diabetic retinopathy should be bilateral and fairly symmetrical. Ocular ischaemic syndrome is unlikely in a healthy 28-year-old.
Eales disease is:
a primary vasculitis
more common in the Indian subcontinent
probable association with TB
peripheral venous inflammation especially superotemporal
neovascularisation
bilateral in 50-90% of patients
What is the Argyll-Robertson pupil
due to a lesion in the dorsal midbrain. Specifically, the pretectal interneurons to the Edinger Westphal nucleus are involved while sparing ventrally located accommodative reflex neurons; hence the light-near dissociation seen.
What are the features of Argyll Robertson pupil?
small irregular pupils
typically bilateral
asymptomatic
caused by lesion in dorsal midbrain
light-near dissociation (responds better to near stimulus than light)
respond poorly to pharmacological dilation
In the landmark Ocular Hypertension Treatment Study, a reduction in IOP of 20% from baseline reduced the relative risk of progression of visual fields and/or optic discs over 5 years by:
50%
Which of the following conditions is autosomal dominant and is characterised by facial abnormalities, deafness, myopia, ectopia lentis and radial lattice-like degeneration?
The features described above, together with an optically empty vitreous, joint hypermobility and arthritis, are typical of Sticker’s syndrome. Stickler’s is the most common inherited cause of retinal detachment in childhood.
What are the features of Interstital keratitis?
Interstitial keratitis (IK) usually produces intense pain and photophobia. The immune response in IK is felt to be an immune response to treponemal antigens (and not live organisms). Standard regimens for neurosyphilis are sufficient to treat luetic IK. Although results of the rapid plasma reagin (RPR) and Venereal Disease Research Laboratory (VDRL) tests may be negative in congenital syphilis, those of the fluorescent treponemal antibody-absorption (FTA-ABS) are usually positive.
