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 12-year-old girl presented with decreased vision in the right eye since 6 months following penetrating injury, for which primary repair was done elsewhere. Physical examination by Internist revealed OI. Best corrected visual acuity (BCVA) was 20/1000 in the right eye and 20/80 in the left eye. Refractive error was previously documented as –11 DS and –18 DS in the right and left eye, respectively. Examination revealed blue sclera in both eyes. Adherent leucoma with irregular anterior chamber and peripheral anterior synechiae were noted in right eye; fundus evaluation was not possible. Ultrasound was done and keratoplasty advised. Left eye fundus was unremarkable, except myopic appearance. However, she was lost to follow-up and presented again 3.5 years later with sudden diminution of vision in the left eye since 3 days.\nBCVA was 20/1000 in the right eye and 20/630 in the left. There were no additional findings on biomicroscopic examination in the right eye as compared to the initial visit. Fundus evaluation of the left eye showed the presence of a subfoveal choroidal neovascular membrane (CNVM) []. The clinical findings were confirmed on fundus fluorescein angiography (FFA) and optical coherence tomography (OCT). FFA of the left eye revealed an active subfoveal classic CNVM with profuse leakage. Blocked choroidal fluorescence due to the overlying hemorrhage was also noted [Fig. and ]. OCT revealed subfoveal CNVM and sub-retinal fluid []. The treatment options were explained and informed consent was obtained. The patient was treated with intravitreal injections of Bevacizumab (1.25 mg/0.05 ml). At 1-month follow-up, the patient had a BCVA of 20/200 in the treated eye. Fundus evaluation showed a yellow-white linear streak deep to the retinal layers, suggestive of lacquer crack []. The patient received two re-treatments with intravitreal Bevacizumab at 1-month intervals, following which the CNV regressed. At the last follow-up, 6 months following the last injection, the fundus remained stable [] and visual acuity stabilized at 20/200.