White dot syndromes

White dot syndromes

Source: Kanski, Yanoff, Sankara Netralaya atlas of FFA

	Acute multifocal posterior placoid pigment epitheliopathy(AMPPPE)	Birdshot Retinochoroidopathy Syn.Name: vitiliginous chorioretinitis	Multifocal Evanescent White Dot Syndrome (MEWDS)
Laterality	Bilateral	Bilateral	Unilateral
Sex incidence	Men = women	Women > Men	Women > Men
Sp. Feature	The absence of substantial anterior chamber or vitreous inflammation in a young, healthy patient who has a viral prodrome with typical fundus lesions is highly suggestive of APMPP	90% of patients who have birdshot retinochoroidopathy have positive HLA-A29 levels. This is the highest association of any HLA antigen with a human disease.
Presentation	3rd-5th decades Viral prodromal symptoms Sudden, painless loss of vision in one or, more typically, both eyes	3rd   and 6th   decades blurred vision, floaters, central & peripheral photopsias and, later, nyctalopia and color blindness.	3rd to 5th decades Sudden decreased vision with photopsia Typically Temporal field of vision is affected
White Dots	Multiple large cream or greyish white coloured sub retinal plaque like lesion begin at post pole then spread to post equatorial fundus		Numerous very small deep white dots involving the posterior pole but sparing the fovea OD oedema & enlargement of blindspot Retinal venoussheathing
AC inflammation	x	Non granulomatous uveitis.	x
Vitritis	Absent, mild if present	Diffuse vitritis with pars plana exudates	Mild
FFA	early hypofluorescence of these white placoid lesions with late staining of these same lesions due to leakage of dye from damaged RPE cells	Early lesion may remain silent. Established cases shows  OD staining, Vascular leakage. ICG> FFA. The patches are more  prominent ophthalmoscopically than angiographically	normal early phase & late phase shows hyperfluorescence which may have a “wreathlike” appearance
ERG		Subnormal	Decrease a wave amplitude
Course	self-limited course	Long-term visual prognosis is guarded. Self limited in 20%	After a few week visual acuity recovers the white dots fade & the disc oedema resolves
Treatment	_	Steroid Cyclosporin	Self limiting disease

	SERPIGINOUS CHOROIDITIS Syn. Name : helicoid and geographical choroidopathy	PUNCTATE INNER CHOROIDOPATHY	MULTIFOCAL CHOROIDITIS AND PANUVEITIS
Laterality	Bilateral	Bilateral	Bilateral
Sex incidence	Men = women	Women > Men	Female>Male
Sp. Feature	An association with herpes and tuberculosis has been suggested		An association with Epstein-Barr virus has been suggested
Presentation	2nd to 7th Decades typically experience paracentral or central scotomata with blurred vision, metamorphosia	4th to 5th decades Blurred vision Photopsia Paracentral scotoma	4th Decade Blurring of vision Floaters Photopsia
White Dots	Grey white to yellow white subretinal infiltrates with hazy borders Typically starts around OD & then gradually spread outwards in a snake like manner towards the macula	Multiple small deep yellow indistinct spots After a few weeks the acute lesions resolve  leaving behind sharply demarcated atrophic scars which may enlarge & become pigmented	Multiple, discrete, small, round or ovoid, deep, yellowish grey located at the posterior pole & periphery. Enlargement of blind spot, mild disc oedema.
AC inflammation	Mild	x	Present in 50% cases
Vitritis	present	Absent	universal
FFA	early hypoflourescence due to blockage & hyperfluorescence due to leaking	Hyperfluorescence due to window defect, with leakage in late phase	Early hypofluorescence due to blockage and late staining.
ERG	subnormal	Normal	Normal or mildly subnormal
Course	Relentlessly progressive and foveal destruction eventually occurs. Central visual acuity is lost in 20% or more of eyes with serpiginous choroiditis	Some patient develop foveal scarring or CNV with in a yr. Prognosis is relatively good with without macular involvement.	Prognosis is guarded as central vision may be impaired by direct foveal involvement, CNV, scarring, Macular oedema etc.
Treatment	Triple therapy with steroids, azathioprine & cyclosporin	Only in case of CNV -Laser photocoagulation -Systemic steroid -Surgical excision of subfoveal CNVM	Systemic steroid, immunosuppressant, In case of CNV = Laser

        

	Acute retinal pigment Epitheliopathy
Laterality	unilateral
Sex incidence
Sp. Feature	Inflammatory condition of macular RPE
Presentation	3rd-5th decade Sudden impairment of central vision Metamorphopsia
White Dots	Discrete clusters of few, subtle, small, brown or grey spots at the level of  RPE which may be surrounded by hypopigmented yellow haloes Fovea shows a blunted reflex.
AC inflammation
Vitritis
FFA	Small hyperfluorescent dots with hypofluorescent centres (Honeycomb appearance) with out leakage.
EOG	subnormal
Course	Within 6-12 weeks the acute fundus lesion resolves and visual acuity returns to Normal
Treatment	Self limiting disease