Unit 7 - Benign lesions Flashcards
What is a congenital anomaly which has a loss of RPE, temporal to fovea pointing towards the fovea?
Torpedo maculopathy
Which variant of CHRPE is of concern?
Multiple irregular CHRPE in all 4 qudrants that are pisciform in shape and bilateral. Linked to familial adenomatous polyposis
Are CHRPE elevated?
May be minimally elevated
Which associations are there with Angioid streaks?
P – PXE (ABCC6)
E - Ehlers Danlos syndrome (probably not_
P - Paget’s disease of bone
S- Sickle cell disease (and thalassaemia)
I – Idiopathic
What are the four common types of haemangiomas
Capillary
Cavernous
Vasoproliferative
Racemouse
Which haemangioma is more likely in VHL?
Capillary haemangioma
Which haemangiomas either don’t respond to treatment or don’t need it?
Racemouse
Cavernous
Which haemangioma is more likely if there is a history of ocular disease?
Vasoproliferative tumour of the ocular fundus
How often does VHL present with a capillary haemangioma?
68%
What types of choroidal haemangioma are there?
Diffuse
Circumscribed
Which type of choroidal haemangioma has systemic associations?
Diffuse, may have other haemangiomas on fac and or Sturge-Weber
What will a diffuse choroidal haemangioma look like on ultrasound?
HIgh reflectivity
What signs would you see in a diffuse choroidal haemangioma?
Tortuous vessels, srf, subretinal fibrosis
What types of hamartoma are there?
Simple
Astrocytic
Combined hamartoma of retina and rpe
Which lesion is termed a mulberry lesion?
Astrocytic hamartoma
Which hamartoma is due to glial proliferation?
Combined hamartoma of retinal and RPE
What are the signs of a simple hamartoma?
retinal exudation and pigmented vitreous cells (20%)
Minimally dilated feeder vessels
solitary pigmented nodule
In which layer of the retina is an astrocytic harmartoma located?
nerve fibre
Which hamartomas may be asscoiated with NF?
Type 1 - astrocytic
Type 2 - Combined
What does a choroidal osteoma look like?
Yellow well defined lesion
Looks like aero on FAF
Highly reflective on US
How does vision loss occur with choroidal osteomas?
Bone de-calcifies and kills photoreceptors
Which peripheral retinal degeneration has an association with RD and by how much?
Lattice - 1% risk of RD
What types of Ret tels are there?
Coats,
Mac tel 1,2,3
Sickle cell
Describe Coat’s disease?
Unilateral severe form of tel
Light bulb aneurysm, exudation, capillary dropout and fibrosis
Which mac tel is congenital?
Type 1 - probably a form of Coats
Which Mac Tel is associated with cerebrovasculopathy?
Type 3
With which mac tel can you get CNVM?
Type 2
Which mac tel patients retain good vision?
Type 1 - 6/12 ish
If you see drusen in young people what do you want to check for?
Membranoproliferative glomerulonephritis type 2
Which patients are more likely to get choroidal melanoma?
White, fair, blue eyed men with NF1, BAP gene with a naevus of ota and are welders!
What is the mortality of choroidal melanoma?
50% at 5 years
What treatment is more successful plaque brachytherapy or enucleation?
Equally
Is choroidal melanoma similar to cutaneous melanoma?
No different disease
what types of retinoblastoma are there?
Exophytic, subretinal growth
Endophytic, growth into virtreous cavity
How can you differentiate Coats from exophytic retinoblastoma?
Don’t get lightbulb telangiectasia in retinoblastoma
Which type of retinoblastoma simulates Coats?
Exophytic
What is the survival in retinoblastoma?
95%, worse if extraocular
Which mac tel is bilateral?
Type 2, and type 3
What features does Alport syndrome have?
Kidney failure
Deafness
Corneal RE
Dot and fleck retinopathy
Mid peripheral flecks
Temporal macular thining
Anterior lenticonus
What is the genetics in Alport syndrome?
X-linked 85%
15% AR
AD is very rare
Who gets adult onset vitelliform macular dystrophy and why?
women in their 30-40s
May be heriditary trait
Yellow material is thought to be lipofuscin and photoreceptor debris
What will the FFA show with adult onset vitelliform macular dystrophy
Hyper fluorescent staining with a corona sign present
What is the prognosis in adult onset vitelliform macular dystrophy?
VA quite good until GA develops
What is the diff dx in AFMVD
Best’s disease but would be present earlier
AMD but AFMVD lesions appear earlier and have more discrete sub-foveal deposits
What symptoms do you get with primary intraocular lymphoma?
Vitreous floaters
Systemic history of lymphoma
What are the sign in primary intraocular lymphoma?
Subretinal yellow infiltrates
VA better than signs
Mean age 50-60
60-80% develop intracranial lymphoma within 29 months
What is often the problem in primary intraocular lymphoma?
Treated as a uveitis with steroids. Need to be off steroids for 2 weeks before biopsy
What percentage of uveal metastases are choroidal?
88%
