Angioid streaks. I. Ophthalmoscopic variations and diagnostic problems. (1/26)

Fifty-six patients with angioid streaks were evaluated ophthalmologically. Most had repeated fundus photography and fluorescein angiography during a follow-up period of 6 months to 7 years. The ophthalmoscopic variations and diagnostic difficulties which occurred were noted. In most instances, the angioid streaks were not initially recognized and the patient was referred with another diagnosis. In several cases, the peripapillary, macular, and peripheral changes seen with angioid streaks were found to simulate other better known fundus conditions, resulting in the erroneous diagnosis and improper treatment. In some cases, the angioid streaks were so subtle that they were overlooked and in others they were observed, but initially interpreted as something else. Because of the medical significance of angioid streaks, ophthalmologists should be aware of their variable features. These are discussed, with emphasis upon those subtleties which differentiate angioid streaks from other conditions which they may simulate. On the basis of these observations, an ophthalmoscopic differential diagnosis of angioid streaks is proposed.  (+info)

Angioid streaks and traumatic ruptures of Bruch's membrane. (2/26)

Minor blunt trauma may cause typical haemorrhages and probably enlargement of breaks or new breaks in patients affected with the Groenblad-Strandberg syndrome.  (+info)

Elastic tissue abnormalities resembling pseudoxanthoma elasticum in beta thalassemia and the sickling syndromes. (3/26)

The development of clinical and histopathologic manifestations of a diffuse elastic tissue defect, resembling inherited pseudoxanthoma elasticum (PXE), has been encountered with a notable frequency in patients with beta thalassemia, sickle cell disease, and sickle thalassemia. The PXE-like clinical syndrome, consisting of skin, ocular, and vascular manifestations, has a variable severity in these hemoglobinopathies and it is age-dependent, with a generally late onset, after the second decade of life. The defect is believed to be acquired rather than inherited and related to the consequences of the primary disease. The high prevalence of the findings implicates the elastic tissue injury as one of the main comorbid abnormalities encountered in beta thalassemia and the sickling syndromes. In these patients a number of complications, sometimes serious, has been recognized to be related to ocular and vascular elastic tissue defects. Because several organ systems are involved, each medical specialty should be aware of the phenomenon. This coexistence, on the other hand, introduces a novel pathogenetic aspect of PXE and an important research challenge.  (+info)

Angioid streaks and sickle haemoglobinopathies. (4/26)

Five patients had angioid streaks associated with sickle cell haemoglobinopathy. Other diseases associated with angioid streaks were ruled out, as was elastic tissue degenegation in sickle cell patients. After studying over 350 patients, we believe the incidence of angioid streaks in sickle cell disease to be between 1 and 2 per cent.  (+info)

Intravascular ultrasound findings of coronary wall morphology in a patient with pseudoxanthoma elasticum. (5/26)

Pseudoxanthoma elasticum (PXE) is an inherited disorder characterised by progressive calcification of the elastic fibres in the skin, eye, and cardiovascular system. Recently, mutations in the ATP binding cassette transporter gene (ABCC6) were identified as cause of this disease. Although patients with PXE often have coronary artery disease, little is known about the process and the mechanism of coronary artery disease in PXE. In this report, intravascular ultrasound (IVUS) imaging was performed in a female patient with PXE seven years after the onset of skin lesion to assess the coronary wall morphology in detail. IVUS showed a unique five layer appearance without acoustic shadowing along the vessel wall observed in the angiographically normal portion. These findings may reflect the earlier stage of coronary artery disease caused by PXE before calcification of the internal elastic laminae.  (+info)

Photodynamic therapy with verteporfin for choroidal neovascularization in patients with angioid streaks. (6/26)

PURPOSE: To evaluate the functional and anatomic outcomes of photodynamic therapy (PDT) for choroidal neovascularization (CNV) in patients with angioid streaks. METHODS: The authors retrospectively evaluated 6 consecutive patients (6 eyes) with CNV secondary to angioid streaks. All patients were treated with standard PDT with verteporfin protocol. Standardized protocol refraction, visual acuity testing, ophthalmologic examination, color photographs, fluorescein angiograms and indocyanin angiograms were used to evaluate the results of PDT with verteporfin. Main outcome measures were visual acuity and CNV size. RESULTS: Their mean age was 61.3+/-5.50 years (range, 53-68 years). Follow-up time ranged from 12 to 38 months with mean of 20.5+/-10.91 months. The mean visual acuity at baseline was 20/100 (range 20/25-20/500), and the mean visual acuity at the last examination was 20/320(range 20/125-counting finger). The mean greatest linear dimension (GLD) at baseline was 2400+/-766.81 micrometer, and the mean GLD at the last examination was 3483+/-444.59 micrometer. CONCLUSIONS: PDT for CNV associated with angioid streaks seemed to slow down but not prevent the progression of the disease and associated visual loss.  (+info)

Intravitreal bevacizumab (Avastin) in choroidal neovascular membrane in angioid streaks. (7/26)

Angioid streaks are crack-like dehiscences in the Bruch's membrane, which predispose to the development of a choroidal neovascular membrane (CNVM) that carries a poor visual outcome. We report successful treatment in a 25-year-old woman with bilateral angioid streaks and subfoveal CNVM in the left eye who received two doses of intravitreal bevacizumab (1.25 mg) injections six weeks apart, resulting in rapid regression of the CNVM.  (+info)

Non-vascular vision loss in pseudoxanthoma elasticum. (8/26)

Pseudoxanthoma elasticum patients with angioid streaks are well-known to have acute vision loss due to choroidal bleeding. However, chronic vision loss due to macular atrophy is less well characterized. We describe a patient with sub-acute vision loss in one eye due to loss of macular retinal pigment epithelium function. Autofluorescence and pattern electroretinogram were useful adjuncts to help diagnose the source of her vision loss.  (+info)

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