1994
DOI: 10.1159/000310498
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Echographic Diagnosis of Drusen of the Optic Nerve Head in Patients with Angioid Streaks

Abstract: A total of 58 patients (116 eyes) with angioid streaks, referred to this department over 2 years (1990–1992), underwent a thorough ophthalmic examination, retinal fluorescein angiography, dermatological visit with skin biopsy, and a series of other examinations (blood and biochemistry, skull X-ray, vascular echo Doppler, abdominal ultrasound) and eye ultrasonography. Fifty patients (100 eyes, 86.2%) had pseudoxanthoma elasticum (PXE). In the whole caselist, 21.6% had drusen compared with 21.0% in the group wit… Show more

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Cited by 52 publications
(20 citation statements)
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“…Drusen of the optic disc seem to be more common in PXE than in the general population 27. AFI proved to be efficient in detecting these optic disc drusen which were confirmed on ultrasound.…”
Section: Discussionmentioning
confidence: 69%
“…Drusen of the optic disc seem to be more common in PXE than in the general population 27. AFI proved to be efficient in detecting these optic disc drusen which were confirmed on ultrasound.…”
Section: Discussionmentioning
confidence: 69%
“…By contrast, Mansour [17], in a retrospective study of a large number of patients, suggested that there might be a relationship between optic disc drusen and angioid streaks. Pierro et al [21], reporting on 50/58 patients with angioid streaks and PXE together, found optic disc drusen present in 18 of the 50 patients. Only two of the remaining eight patients, in whom PXE was absent, had both optic disc drusen and angioid streaks.…”
Section: Discussionmentioning
confidence: 97%
“…Optic disc drusen can occur in isolation [15,1], or they can be associated with genetically inherited conditions such as retinitis pigmentosa [8], pseudoxanthoma elasticum (PXE) [21], and Alagille syndrome [20]. When the condition is familial, optic disc drusen can be inherited either as an autosomal dominant trait characterized by incomplete penetrance and a variable expression [14], or as an autosomal recessive trait [15].…”
Section: Introductionmentioning
confidence: 99%
“…Another asymptomatic ocular sign present in 50% of PXE patients is the presence of paired hyperpigmented smudges, like the wings of a predator bird, aligning an angioid streak [12]. A non-specific ocular finding, associated with angioid streaks, is the presence of optic disc drusen that can be present in up to 20% of patients [21].…”
Section: Ocular Signs and Symptoms Of Pxementioning
confidence: 99%