Tilted Disk Syndrome with Superior Staphyloma

European Journal of Ophthalmology, 2009

Purpose. Many complications associated with tilted disk syndrome may occur in the area bordering on the inferior staphyloma, including macular pigmentary changes, choroidal neovascularization, macular serous retinal detachment, polypoidal choroidal vascular anomalies, and radially orientated chorioretinal folds. The purpose of the present article is to describe an additional complication, Tshaped pigmentary changes, orientated radially from the border of the inferior staphyloma, in eyes with tilted disk syndrome.. Methods. Retrospective series of 6 eyes of 6 patients (3 men and 3 women) aged from 35 to 67 years. All eyes were studied by fluorescein angiography. Results. All 6 eyes exhibited pigmentary changes located along the border of the staphyloma, and also radially to it, giving a particular T-shaped pattern of pigmentary changes. These changes were visible as either a linear strip or a triangular or oval-shaped area. Three eyes had a documented history of subretinal leakage that mimicked central serous chorioretinopathy, a well-known complication of tilted disk syndrome. Conclusions. T-shaped pigmentary changes may develop in the course of tilted disk syndrome, and should be added to the list of this syndrome's possible retinal complications. These changes may indicate the presence of chronic leakage in the area around the superior border of the inferior staphy

IOSR Journals , 2019

Tilted disc syndrome is a congenital anomaly of the eye characterized by the inferior or inferonasal tilting of the optic disc, congenital conus, ectasia, situs inversus of the retinal vessels at the level of the optic disc, myopic astigmatism and visual field defects. There is also thinning of the retinal pigment epithelium and choroid in the inferior nasal fundus that may be ophthalmologically visible as fundus depigmentation.This syndrome occurs equally in men and women in 1-2% of the population. It is generally accepted that the syndrome shows no hereditary patterns. We present a case of 45 year female with tilted disc syndrome with bilateral situs inversus and inferonasal staphyloma. Typical fundus picture, optical coherence tomography, visual fields,B scan ultrasonography and MRI of the patient would be instructive to a clinician.

American Journal of Ophthalmology, 2002

Scripta Medica

We present a 51-year-old female with a unilateral congenital excavated optic disc anomaly. After clinical examination and appropriate diagnostic procedures we were unable to determine with certainty whether it is a morning glory anomaly or a peripapillary staphyloma. The atypical fi nding was an optic disc with characteristics of both states. The affected eye had almost normal visual acuity (0.9 Snellen chart), which is a rare fi nding in congenital anomaly of the optic disc. Confocal scanning laser ophthalmoscopy (Heidelberg Retina Tomograph, HRT 3.0) was not of diagnostic value in comparison with optical coherence tomography (OCT).

Ophthalmology, 1999

Medical Journal Armed Forces India, 2015

Optometry - Journal of the American Optometric Association, 2009

BACKGROUND: Tilted disc syndrome (TDS) is a congenital optic nerve coloboma occurring from embryonic dysgenesis. Several features characterize TDS, including an inferiorly located conus, situs inversus of the major retinal vessels, and an anomalous disc shape. Commensurate with axonal dysgenesis, visual field defects may often occur from TDS, the most common of which involve the temporal and superior temporal visual field. These visual field defects can mimic those seen in chiasmal compression from a mass lesion. CASES: Five patients from New South Wales, Australia, with distinct TDS and bitemporal visual field defects on frequency doubling threshold perimetry seemingly respecting the vertical hemianopic line are presented. Neuroimaging and medical evaluation of each failed to show intracranial chiasmal pathology in any patient. CONCLUSIONS: TDS can present with visual field loss resembling that seen in chiasmal disease. Although most cases of temporal visual field loss from TDS do not respect the vertical hemianopic line and are not true quadrantanopsias, there are instances in which this does occur, likely caused by the testing modality used. It is essential that patients with suspected intracranial pathology undergo immediate neuroimaging, even in the face of TDS.

Retina (Philadelphia, Pa.), 2016

Pigmentary alterations along the presumed edge of staphyloma in wide-field fundus images have been reported to be highly correlated with the eye shape in three-dimensional magnetic resonance images. The purpose of this study was to analyze Optos images in a large series of highly myopic patients to determine the prevalence, types, and features of staphylomas. One thousand and sixty eyes of 541 patients with high myopia (axial length ≥ 26.5 mm) in at least one eye were retrospectively analyzed in Japan and Spain. To determine the presence and types of staphyloma, the authors focused on pigmentary abnormalities along the presumed edge of staphylomas with at least one positive finding in fundus images, autofluorescent images, and infrared images by Optos. Posterior staphyloma was detected in 552 of 1,060 eyes (55%) in Optos images. Wide macular type was the most common (79%), followed by narrow macular (15%), then peripapillary (3%), inferior, and finally nasal. In the 60 non-highly my...

Cornea, 2002

Purpose. To evaluate the corneal topography in patients with tilted-disc syndrome to determine the relationship between optic disc dysversion and corneal astigmatism and the pattern of astigmatism in these patients. Methods. The study included 23 eyes of the 13 tilted-disc syndrome patients with spheric refractive errors ranging between +1.00 D and −9.00 D (mean −4.00 ± 3.4 D) and astigmatic errors ranging between −0.50 and −4.50 D (mean −1.95 ± 0.93 D). Corneal topography was performed by computerassisted videokeratoscope topographic modelling system 2 (TMS-2) and incidence of corneal astigmatism, corneal topographic patterns, and mean values of the topographic indices were determined. Results. Corneal topographic analysis showed corneal astigmatism in 22 out of 23 patients with tilted discs. Corneal astigmatism was symmetric bow tie pattern in 10 eyes (45.45%), asymmetric bow tie pattern in 11 eyes (50%) and irregular in 1 eye (4.5%). Among the patients with bow tie pattern group (21 eyes), 14 eyes had with-the-rule astigmatism, 1 eye had against-the-rule astigmatism, and 6 eyes had oblique astigmatism. In 18 eyes, astigmatism was corneal, whereas combined corneal and lenticular in 4 eyes and lenticular in 1 eye. Conclusion. In the majority of tilted-disc cases, ocular astigmatism is mainly corneal. Morphogenetic factors in the development of the tilted disc might possibly influence the corneal development in such a way to result in corneal astigmatism.

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