Bilateral Microphthalmos Without Microcornea Associated With Unusual Papillomacular Retinal Folds and High Hyperopia

High Hyperopia with Papillomacular Fold

“…Ultrasonography and CT scan were necessary to confirm the diagnosis of post erior microphthalmos in our case, also show ing an increase in choroidal thickness (2.5 mm) and probably of the optic nerve sheaths. Similar cases have already been published [2,10,11], There is always a relative amblyo pia: visual acuity ranges from 0.5 to 0.6. In our case, there is a progressive increase in am blyopia, probably organic amblyopia, be cause of the characteristic macular fold.…”

“…The vit reous is clear and undetached; there are no remains of the hyaloid artery, the retinal pe riphery is normal; no choroidal folds can be seen. Therefore, it seems, as suggested by Boynton and Purnell [2] inspired by the expe riences of Coulombre and Coulombre [3] on chickens, that the retina has developed inde pendently from underneath the tissue lavers, creating a fold. This type of development could also concern the choroid and pigmen tary epithelium, creating an abnormal choroi dal thickness.…”

“…This syndrome was described precisely by Boynton and Purnell in 1975 [2] and Spitznas et al in 1983 [10]. It includes a progressive high hyperopia with a relative organic amblyopia.…”

High-hyperopia database, part I: clinical characterisation including morphometric (biometric) differentiation of posterior microphthalmos from nanophthalmos

“…Various studies published at different time points have used different and sometimes overlapping biometric criteria to define these two clinical entities. [6][7][8][9][10][11][12][13][14][15][16] We used a single measurement of horizontal corneal diameter of o11 mm for differentiating NO from PM in simple microphthalmic eyes with axial length of o20.5 mm in our high-hyperopia cohort. Detailed biometry evaluation of the two groups revealed many interesting findings.…”

“…7 Various other ocular findings reported include microcornea, a high lens/eye volume ratio, angle-closure glaucoma (ACG), 8 yellow macular pigmentation, chorioretinal folds, macular hypoplasia, retinal cysts, 9 crowded optic discs, 9 retinal striae and folds, 10 wrinkling of ILM, 11 thickened sclera with abnormal collagen fibrils, 12 recurrent or persistent choroidal effusions, 2,6 and non-rhegmatogenous retinal detachments. 11 Eyes with short axial length but normal corneal diameter were first reported in 1975 13 and the term 'PM' was introduced to differentiate these patients from NO that have small corneal diameter. Clinical features reported in PM include reduced axial length with a normal-sized cornea and normal anterior segment and a disproportionately small size of the posterior segment.…”