Though CME occurs in upto 26% to 38%percent of patients with RP(9,18), MH has been reported variedly tooccur in 0.5% to 10.5% eyes.(9,12) Majority of MH develops after the 4thdecade of life. MH has also been reported in an adult patient with Ushersyndrome, which is an autosomal recessive form of RP with congenital sensoryneural hearing loss.(19) Loss of central field with MHformation in these eyes can be potentially devastating as the visual field isalready constricted. Intra-retinal cysts and cuff of subretinal fluid arefrequently present in these MH.
(10,13,17) Visual acuity at presentation hasbeen variedly reported from 20/50 to 20/1000, depending upon the status ofneurosensory retina and RPE at the macula. Pars plana vitrectomy with ILMpeeling has proved successful in MH closure in most of the eyes with final BCVAbetter than or equal to 20/50.(9,11,13,17) However spontaneous closure withBCVA improvement has also been reported in a single case with atrophic macularhole.(14) Peripapillarypigmentary retinal degenerationPericentral pigmentary retinopathy orperipapillary pigmentary retinal degeneration is a disorder of retina andchoroid with annular atrophic patches with bone spicule pigmentationcharacteristically involving the peripapillary area. Durlu et al. reported MHin 2 such eyes and ascribed foveal ischaemia and VMT as the probable cause.(20) Best vitelliformmacular dystrophyBest vitelliform macular dystrophy isan 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 dysfunctionwhich leads to accumulation of lipofuschin and abnormal fibrillar material withthe RPE and subretinal space at the macula.
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(22) The disease follows a coursestarting from vitelliform type (macular egg yolk lesions) to vitelliruptivetype and then finally scarring. MH develops in these eyes following cystrupture in the vitelliform stage and retinal atrophy in late stages with minorrole of VMT in these eyes.(23–29) Majority of these patients develop MHby 4th decade of life. Characteristically these MH have intraretinalcystic changes, NSD, subretinal deposits and degenerated outer retina-RPElayers on OCT. The lesions exhibit hyper auto fluorescence with fundus autofluorescence imaging. RD occurs in more than 50% of these eyes with MH.(23,24,26) This occurs secondary to widespreadRPE dysfunction which leads to accumulation of fluid in the potentialsubretinal space.
Unlike myopic eyes which frequently have RD associated withMH, these eyes are emmetropic and have characteristic macular lesions or theirsequalae. Although vitrectomy and ILM peeling have been tried for these MH andleads to MH closure, visual outcomes have been dismal probably due tounderlying long standing outer retinal and RPE atrophy.(23,25,26)