A 43-year-old married Syrian woman presented at the age of 40 with distortion of central vision in the right eye. Choroidal dystrophy was suspected in Damascus (Syria) ( and ). Rheumatology and infectious workup was negative (syphilis, tuberculosis, anti-double-stranded DNA, anti-single-stranded DNA, rheumatoid factor). CNV was also detected in the macular area in Dubai (United Arab Emirates) and intravitreal injections were advised. She subsequently received three monthly intravitreal ranibizumab injections in Barcelona (Spain) (). Past ocular history revealed myopia of 2.5 D as per patient recollection. She had undergone bilateral refractive surgery (excimer laser 5 years ago followed by laser in situ keratomileusis [LASIK] 3 years ago). Medical history was negative. There was a strong family history of myopia and no parental consanguinity. Fifteen months after initial right eye involvement, the patient expressed the same symptoms in the left eye, such as seeing broken lines and asymmetric faces. She was advised to undergo treatment for CNV in the macular region. She received one injection in Beirut (Lebanon), and subsequently, three monthly injections in Dubai to the left eye. She reported further worsening of vision after each injection. Consultation in London (UK) revealed a best-corrected visual acuity of 20/80 in the right eye and 20/30 in the left eye. Dilated fundoscopy revealed bilateral areas of vertically linear atrophy, more extensive in the right eye, without subretinal fluid or hemorrhage by optical coherence tomography. Fundus autofluorescence imaging revealed areas of reduced autofluorescence corresponding to these areas of atrophy. The reported clinical impression then was myopic chorioretinal degeneration versus lacquer crack-associated myopic degeneration. She was advised to have regular checkup without intravitreal injections as long as the macula stays dry. The patient was frustrated because of both inability to pinpoint a definite diagnosis and gradual decline in vision bilaterally. Nine months later, she initially presented to our clinic with 20/200 visual acuity. Fundoscopy revealed definite widening of the vertical macular choroidal rupture bilaterally (). Stippled changes in the retinal pigment epithelium were observed. Peau d’orange appearance was not present. The patient was emmetropic without keratoconus on corneal topography. Axial length measurement with the IOL Master (Carl Zeiss Meditec AG, Jena, Germany) was 27.35 mm in the right eye and 27.31 mm in the left eye. Keratometry readings were 38.6 D in the right eye and 38.2 D in the left eye. The skin of the neck had tiny pinkish papules suspicious for early goose flesh (). Two separate dermatology consultations and skin biopsies did not favor the diagnosis of pseudoxanthoma elasticum. Even further widening of the choroidal rupture occurred 9 months after presentation. Our working diagnosis was lacquer crack from high myopia (without pseudoxanthoma elasticum) () possibly initiated by LASIK and exacerbated by ocular rubbing and intravitreal injections (). The patient was instructed to avoid ocular rubbing, head-down position, and intravitreal injection (unless an exudative component was present) and to have prior paracentesis before any future intravitreal injections.
A 7-year old boy presented 1 day after injury to the right eye. The left was normal. The right eye vision was 6/18. There were abrasions on the cornea. Anterior chamber had cells of 1+ and flare of 2+. Fundus examination showed a double humped sub-foveal bleed. The retina over the macula looked edematous clinically. OCT done at presentation showed blood between the RPE and ellipsoid layer. The ellipsoid and ELM layer over the blood lost its clear distinction and looked thickened due to edema. OCT line scan showed two areas of breaks in the RPE/BM complex measuring 103 and 185 μm [ pre]. One week later since the sub-macular blood persisted, he underwent pars plana vitrectomy, sub retinal tissue plasminogen activator injection, and SF6 gas replacement of the vitreous cavity using the technique described previously.[] A month later the vision improved to 6/9. The fundus picture and OCT line scan after surgery is shown in . Clinically macular area showed multiple stellate lines under the retina that did not correspond to areas of choroidal rupture. The ELM layer and ellipsoid layer were continuous under the foveola. There was scar tissue between the photoreceptor layer and RPE corresponding to the yellowish gray area under the fovea seen clinically. Full thickness neurosensory retinal defect was seen at the site of retinotomy. The RPE/BM complex break near the retinotomy was plugged with scar tissue. There were some cystic changes in the margin of the retinotomy site. At the site of the rupture corresponding to the area in pre, there was scarring and closure of the BM break like in the previous two cases [ post].\ngives an overview of the cases presented. As has been noted above besides a break in the BM, the SSOCT showed blood between RPE and ellipsoid layer, and variable amounts of edema of the inner retinal layers in all the three acute cases. The gap in the BM ranged from 103 to 465 μm. The inner neuro-sensory retina and the Sattler's and Haller's layers of the choroid did not show any disruption in the acute stage.\nOCT after a month of the injury showed scar tissue involving Sattler's layer, choriocapillary layer, BM, RPE, ellipsoid layer, ELM, and outer nuclear layer in all the cases.