1/16. Anomalies of the human hyaloid system.We describe two asymptomatic cases of persistent anomalies of the human hyaloid system. fluorescein angiography of a cyst of the hyaloid system shows leakage from the hyaloid vessels.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/16. Spondylocarpotarsal synostosis with ocular findings.We report on three sibs presenting with spondylocarpotarsal synostosis, short-trunk dwarfism of postnatal onset, scoliosis, unsegmented thoracic vertebrae with unilateral bar, and carpal bone fusion. Tarsal bone fusion and dental abnormalities were noted in some of them, indicating pleiotropy and intrafamilial variability. Lens opacities, rarefaction of retinal pigmentation, and narrowing of retinal vessels, detected in two patients, are findings that have not been described to date in this condition.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/16. optic disk pallor and retinal atrophy in sotos syndrome (cerebral gigantism).PURPOSE: To report a case of sotos syndrome (cerebral gigantism) with megalophthalmos, optic disk pallor, and retinal atrophy. methods: Case report. RESULTS: A 22-year-old man was diagnosed with sotos syndrome with optic atrophy. His corneal diameters were 13 mm horizontally, and his optic disks were pale. His retinal vessels were attenuate in diameter, and small white spots were found on the retina. The results of a Goldmann visual field test were normal. His visually evoked potential to flash stimuli showed extended latent times and normal amplitudes. Keratometry was 40.5 diopters (8.33 mm) in both eyes. The axial length was 25.9 mm in the right eye and 25.4 mm in the left eye. CONCLUSION: sotos syndrome may be associated with optic disk pallor and retinal atrophy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/16. Type a syndrome of insulin resistance: anterior chamber anomalies of the eye and effects of insulin-like growth factor-I on the retina.BACKGROUND: The purpose of this work was to describe the anterior chamber and iris anomalies as well as to evaluate the effects of recombinant human insulin-like growth factor-I (rhIGF-I) on the retinal vessels in 2 diabetic patients with type A syndrome of insulin resistance, a rare condition associated with acanthosis nigricans. methods: Ophthalmologic examinations, including photographs and fluorescein angiograms, were performed before, and 2 and 4 weeks after starting subcutaneous rhIGF-I treatment, and 3 months after withdrawal of rhIGF-I treatment. RESULTS: Both patients had goniodysgenesis with mild elevation of the intraocular pressure. Before and after 2 weeks of treatment with rhIGF-I, the fundus and the fluorescein angiograms were mainly normal. After 4 weeks of rhIGF-I treatment both patients' retinas revealed leakage of fluorescein. Three (case 1) and 4 months (case 2) after withdrawal of rhIGF-I, the fundus of all four eyes were again without leakage. CONCLUSIONS: The anterior chamber anomalies found in these patients may be part of the type A syndrome of insulin resistance and could alert clinicians that these patients might not have the usual type of diabetes. Moreover, the data show that exogenous rhIGF-I administration in patients with type A syndrome of insulin resistance alters the permeability of the superficial layer of retinal capillaries which is comparable to the earliest angiographic changes in childhood diabetic retinopathy. Whether this is a direct effect of rhIGF-I, as suggested by experiments in an animal model, or an indirect effect due to the near-normalization of the glucose levels by rhIGF-I warrants further investigations. Finally, this work points to an important caveat regarding the therapeutic use of rhIGF-I in this patient population.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/16. Vascular anastomoses between the iris and persistent hyperplastic primary vitreous.Prominent radial iris blood vessels created small but definite pupillary notches in five children with persistent hyperplastic primary vitreous and persistence of the tunica vasculosa lentis. Detection of these abnormal iris vessels suggested the presence of persistent hyperplastic primary vitreous and related syndromes even when the diagnosis was obscured by the presence of an opaque lens. These vessels represented early developmental arrest and may have been associated with abnormal development of the macula and optic nerve. Surgical and amblyopia therapy, therefore, may not provide useful vision, and should be undertaken with caution.- - - - - - - - - - ranking = 3keywords = vessel (Clic here for more details about this article) |
6/16. Prepapillary vascular loops.A case is reported of a 10-year-old girl with prepapillary vascular loops. These are normal blood vessels of excessive length, usually arterial, that project from the optic disc into the vitreous cavity. Stereoscopic photographs and fluorescein angiographs of the condition are presented.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/16. Juxtapapillary subretinal pigment epithelial polypoid pseudocysts associated with unilateral tilted optic disc: case report with clinicopathologic correlation.OBJECTIVE: To describe the unique preoperative appearance, successful postoperative clinical course, and histopathologic features of a cluster of progressively enlarging pseudocysts that arose at the temporal margin of a unilateral tilted optic disc. STUDY DESIGN: Case report. methods: Clinical observation, color fundus photography, fluorescein angiography, and optical coherence tomography, as well as routine histologic and immunohistochemical studies of tissue removed by subretinal surgery. RESULTS: Subretinal surgical excision of the lesions resulted in retinal reattachment with improved postoperative visual acuity. Histologic examination disclosed a cluster of fluid-filled polypoid pseudocysts lined by small vessels of choroidal origin lying beneath the basement membrane of the overlying retinal pigment epithelium (RPE). CONCLUSIONS: We postulate that buds of small vessels of choroidal origin grew through or around the edge of Bruch's membrane at the temporal margin of the tilted optic disc and then passed under the juxtapapillary RPE. Ensuing leakage of proteinaceous fluid from these vessels eventuated in formation of a cluster of polypoid pseudocysts and subsequent localized papillomacular retinal separation with visual loss. The lesions were amenable to subretinal surgical removal with restoration of visual acuity.- - - - - - - - - - ranking = 3keywords = vessel (Clic here for more details about this article) |
8/16. Congenital retinal macrovessel: a case report.BACKGROUND: A 24-year-old woman with symptoms of chronic, recurrent headache, presumably secondary to migrainous etiology, came to our clinic and was found to have a congenital retinal macrovessel encircling an area superior to (but not passing through) the macula. CONCLUSIONS: Congenital retinal macrovessels are rare, aberrantly large branches of retinal arteries or veins that typically cross the horizontal raphe to either supply or drain the macular area. These unusual vascular entities must be distinguished from prepapillary vascular loops, racemose hemangiomas, Wyburn-Mason syndrome, or other arteriovenous malformations with potential neuro-ocular sequelae.- - - - - - - - - - ranking = 6keywords = vessel (Clic here for more details about this article) |
9/16. The ateliotic macula: a newly recognized developmental anomaly.PURPOSE: We present a macular phenotype resulting from 1 or more abnormalities in the developmental pathway of the central retina. methods: We describe the clinical and genetic characteristics of 7 patients observed since shortly after birth with regard to visual acuity, refractive error, anterior segment status, retinal findings including foveal structure, and natural history. RESULTS: The patients varied in age from 18 months to 18 years. All patients were examined for the first time during their first year of life and by us at the age of 5 years or younger. The longest follow-up period was 16 years. The abnormal appearance of the macula consisted of thinning of the retina, rarefication of the pigment epithelium with excess visibility of the large choroidal vessels, and absence of the foveal reflex. The visual acuities varied from 20/20 in the better eye to light perception. A retinal detachment was noted in 1 patient at age 2 1/2 years. The refractive errors varied from -2.50 to -16.50 diopters of spherical equivalent. The disease was limited to the retina in 4 patients. In 2 patients, however, developmental abnormalities of the anterior segment were also present; they consisted of malformation of the iris in 1 patient and Peters' anomaly in the other. The electroretinogram (ERG) showed reduced but not absent photopic responses and some reduction in scotopic responses. CONCLUSION: The phenotype of ateliotic macula is being defined as characterized by an unfinished or primordial appearance. In the 7 patients studied, visual loss was noted shortly after birth. The visual outcome was variable with regard to visual acuity, but many patients showed improvement. There was no evidence of significant worsening of the disease with age except in 1 patient who had a retinal detachment. The ERG responses showed primarily photopic but also scotopic changes. The better-preserved ERG differentiates this disorder from Leber's congenital amaurosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/16. telangiectasis as a cause of intra-schitic haemorrhage in optic disc pit maculopathy.PURPOSE: To present a patient with the novel finding of vascular telangiectasis as a cause of intra-schitic haemorrhage, occurring in optic disc pit-associated maculopathy. methods: A clinical history was detailed. Clinical examination included visual acuity assessment and slit-lamp microscopy. fluorescein angiography was performed. RESULTS: A temporal optic disc pit, macular retinoschisis and a circumscribed detachment of the outer retinal layer and inner leaf holes were noted. A retinal haemorrhage extending into the schitic cavity was present, along with an associated vitreous haemorrhage. fluorescein angiography showed telangiectatic vessels in association with the haemorrhage. CONCLUSION: This is the first reported case of vascular telangiectasis as a cause of intra-schitic haemorrhage occurring in optic disc pit-associated maculopathy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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