Though dystrophy Best vitelliform macular dystrophy is an autosomal

Though CME occurs in upto 26% to 38%
percent of patients with RP(9,18), MH has been reported variedly to
occur in 0.5% to 10.5% eyes.(9,12) Majority of MH develops after the 4th
decade of life. MH has also been reported in an adult patient with Usher
syndrome, which is an autosomal recessive form of RP with congenital sensory
neural hearing loss.(19) Loss of central field with MH
formation in these eyes can be potentially devastating as the visual field is
already constricted. Intra-retinal cysts and cuff of subretinal fluid are
frequently present in these MH.(10,13,17)                                      

Visual acuity at presentation has
been variedly reported from 20/50 to 20/1000, depending upon the status of
neurosensory retina and RPE at the macula. Pars plana vitrectomy with ILM
peeling has proved successful in MH closure in most of the eyes with final BCVA
better than or equal to 20/50.(9,11,13,17) However spontaneous closure with
BCVA improvement has also been reported in a single case with atrophic macular
hole.(14)

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Peripapillary
pigmentary retinal degeneration

Pericentral pigmentary retinopathy or
peripapillary pigmentary retinal degeneration is a disorder of retina and
choroid with annular atrophic patches with bone spicule pigmentation
characteristically involving the peripapillary area. Durlu et al. reported MH
in 2 such eyes and ascribed foveal ischaemia and VMT as the probable cause.(20)

Best vitelliform
macular dystrophy

Best vitelliform macular dystrophy is
an autosomal dominant dystrophy with characteristic ‘egg-yolk or scrambled egg’
like subretinal deposits and generalised reduction in Arden ration on electro-oculogram
(EOG).(21) There is generalised RPE dysfunction
which leads to accumulation of lipofuschin and abnormal fibrillar material with
the RPE and subretinal space at the macula.(22) The disease follows a course
starting from vitelliform type (macular egg yolk lesions) to vitelliruptive
type and then finally scarring. MH develops in these eyes following cyst
rupture in the vitelliform stage and retinal atrophy in late stages with minor
role of VMT in these eyes.(23–29)  

Majority of these patients develop MH
by 4th decade of life. Characteristically these MH have intraretinal
cystic changes, NSD, subretinal deposits and degenerated outer retina-RPE
layers on OCT. The lesions exhibit hyper auto fluorescence with fundus auto
fluorescence imaging. RD occurs in more than 50% of these eyes with MH.(23,24,26) This occurs secondary to widespread
RPE dysfunction which leads to accumulation of fluid in the potential
subretinal space. Unlike myopic eyes which frequently have RD associated with
MH, these eyes are emmetropic and have characteristic macular lesions or their
sequalae. Although vitrectomy and ILM peeling have been tried for these MH and
leads to MH closure, visual outcomes have been dismal probably due to
underlying long standing outer retinal and RPE atrophy.(23,25,26)

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