A previously healthy, 12-year-old male presented for a routine visit with complaints of a 2-month history of decreased visual acuity in his left eye (LE). Six months before the visit, he suffered blunt trauma to the LE during a struggle, with no medical observation. At the initial visit, BCVA in the LE was counting fingers and in the right eye, it was 20/20. He had neither restricted eye movements nor afferent pupillary defects. Biomicroscopic examination of the anterior segment was normal. Fundus examination of the LE showed a central macular lesion of 1 disc diameter with fibrosis, increased retinal thickness and intraretinal hemorrhage (fig. , left). Peripheral retinal examination was normal. Spectralis OCT (Heidelberg Engineering Inc., Heidelberg, Germany) showed disruption of the IS/OS junction with corresponding increased reflectivity, loss of the outer nuclear layer, cell infiltration of the retinal wall and subretinal fibrosis. Retinal thickness was 289 µm at the site of the lesion (fig. , right). A fluorescein angiography (FA) revealed early impregnation with no diffusion, increasing with time (fig. ). With support from Pediatrics, off-label, high-steroid pulse therapy (intravenous methylprednisolone 500 mg for 3 days and oral prednisolone 30 mg, tapering for 10 days) was done, resulting in an increase of LE BCVA to 20/200 and a decrease of retinal thickness by 71 µm to 218 µm 1 week after treatment. In an attempt to achieve additional improvement without undesired systemic effects, off-label intravitreal triamcinolone acetonide (IVTA; 0.05 ml/2 mg) was administered 2 weeks after oral treatment. LE BCVA improved to 20/150, and retinal thickness decreased by 10 µm 3 weeks after IVTA. Three months after initial presentation, LE BCVA was 20/125 and retinal thickness was 208 µm. However, the alterations in fundoscopy and OCT remained (fig. ).
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.