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JW Kim, JL Kinyoun, D Boes; Optical Coherence Tomography Imaging in Bilateral Posterior Microphthalmos with Papillomacular Fold and Novel Features of Retinoschisis, Dialysis, and Submacular Fluid . Invest. Ophthalmol. Vis. Sci. 2002;43(13):1189.
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© ARVO (1962-2015); The Authors (2016-present)
Purpose: To increase awareness of an uncommon hereditary eye abnormality while reporting a novel case of retinoschisis and dialysis associated with bilateral posterior microphthalmos and papillomacular fold. In addition, original optical coherence tomography (OCT) data is presented to elucidate the abnormal anatomy of the posterior segment in this condition. Methods: Ten-year follow-up of a family in which three of five siblings have posterior microphthalmos with papillomacular fold. Ophthalmoscopy, biomicroscopy, fundus photography, fluorescein angiography, ultrasonography, and OCT were used to study these three affected subjects. Results: Three siblings presented with poor vision (20/200 OU); examination revealed high hyperopia (+18.00 sphere OU), bilateral horizontal papillomacular fold, and reduced axial lengths (15 mm OU) with normal corneal diameters (11.5 mm OU). One of the siblings initially presented with retinoschisis that later developed into a retinal dialysis without subretinal fluid in the superotemporal left eye. The right eye developed retinoschisis in the superotemporal area during follow-up. Biomicroscopy, ophthalmoscopy, and OCT of the macular folds revealed cystoid macular edema with shallow subretinal fluid in the macula for all three patients; fluorescein dye did not leak into the cysts during angiography. Conclusion: Posterior microphthalmos with papillomacular fold has characteristic findings as revealed by ophthalmoscopy, biomicroscopy, fundus photography, fluorescein angiography, and OCT. The novel findings of retinoschisis, dialysis, and submacular fluid as well as the OCT data may help us to better understand the limited development of the posterior ocular coats as originally described. Furthermore, this case shows the importance of a thorough peripheral retinal examination these patients.
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