Bilateral hypermetropia, myelinated retinal nerve fibers, and amblyopia

A 14-year-old hyperopic female with poor vision in both eyes was evaluated for ophthalmic and systemic diseases. The patient had bilateral retinal fiber myelination and greater vision loss in the more hyperopic eye. This was a rare case of reverse Straatsma syndrome, the clinical presentation which may be accompanied with significant vision loss

Quantitative and qualitative corneal endothelial morphology of Omani patients with pseudoexfoliation syndrome

Pseudoexfoliation [PEX] syndrome is one of the leading causes of secondary open angle glaucoma and blindness. This study explored whether in PEX eyes, preoperative changes in corneal endothelial cell morphology might be a risk factor for postoperative corneal decompensation. One hundred twenty six eyes of 69 preoperative cataract patients [43 males, 26 females] were enrolled in this cross-sectional study from the Ophthalmology Department at Sultan Qaboos University Hospital between 2003-2005. All patients were subjected to confocal biomicroscopy. The mean age of patients with PEX eyes was 63.2 years. One hundred and eight [85.7%] eyes with PEX had endothelial cell counts within the normal range [1650-3500/mm[2]]. The qualitative morphology of the endothelium of PEX corneas was highly abnormal in term of polymegathism and pleomorphism. Twelve eyes had endothelial cell counts higher than normal for that age group. Only 6 eyes had endothelial cell counts lower than normal. The mean value for the pleomorphism was found to be significantly lower than normal and for polymegathism significantly more than normal. The relationship between pleomorphism and polymegathism was stronger for males than for females and stronger for patients under 60 years than patients over 60 years. The same relationship between pleomorphism and polymegathism showed a stronger relationship for the glaucoma group as compared to the non-glaucoma group. This study revealed that corneal decompensation in PEX eyes can occur in presence of abnormalities in polymegathism and pleomorphism, even when the endothelial cell counts may be normal

Optical coherence tomographic view of persistent primary fetal vasculature

To report on the posterior segment changes in a patient with bilateral persistent primary fetal vasculature as detected by optical coherence tomography. An 18-year-old lady with poor vision, left esotropia and bilateral posterior polar cataract was found to have dysplasia of the macula in both the eyes. Fundus fluorescein angiography, optical coherence tomography, [A] scan biometry and genetic work up was performed as a part of investigation. There was increase in thickness of the macular area in both the eyes [450-500mm]. The left eye showed a [sail like] fold extending over macula, from nasal to temporal side. The tissue had the same sensitivity and thickness as inner the retinal layers [180-200mm]. There was no detectable nerve fibre layer in the macula of either eye. Fundus fluorescein angiography was normal in the right eye, and showed hyperfluorescence at the inferior pole of the disc in the left eye corresponding to the Bergmeister papilla. There was no staining of the membrane with the dye. Evaluation of the posterior segment is important in predicting the visual outcome in patients with any form of PFV. Optical coherence tomography is an adjuvant to direct visualization and aids in further delineating posterior segment changes seen in this condition