Chorioretinal Vascular Abnormalities Associated With Angioid Streaks and Pseudoxanthoma Elasticum

Abstract

Objective: To analyze the retinal and choroidal vascular abnormalities in eyes with angioid streaks (AS) associated with pseudoxanthoma elasticum (PXE). Methods: Color photographs and fluorescein angiograms of 54 eyes of 27 consecutive patients with AS and PXE were examined retrospectively. Results: Four (7%) of the 54 eyes had a major vascular abnormality at the level of the disc; this took the form of a large vascular loop corresponding to an arteriovenous communication between retina and choroid in 3 eyes (6%) and an anastomosis between 2 retinal arteries in 1 eye (2%). Conclusion: Analysis of the vascular network in these eyes showed several vascular abnormalities, among which chorioretinal arteriovenous communications appear to be the most dramatic.

Targeted Ablation of the Abcc6 Gene Results in Ectopic Mineralization of Connective Tissues

“…It is unclear at this point whether the mice develop loss of visual acuity and possible development of angioid streaks, features of ophthalmologic findings in PXE (48,65).…”

Pseudoxanthoma elasticum

“…7 15 Major vascular abnormalities at the optic disc such as arteriovenous anastomosis have been found in PXE patients with angioid streaks. 16 Angioid streaks are not pathognomonic and have been described in a variety of other systemic disorders including Ehlers-Danlos syndrome, Paget's disease of the bone, Marfan's syndrome, sickle cell anaemia, thalassaemia, and lead poisoning.…”

A rare and unusual retinal arterioarterial communication in a prepapillary vascular loop

“…Retinal arterioarterial communications are rare; the coexistence of retinal arterioarterial communication and prepapillary vascular loops is extremely rare. 4,5,7,8 Nishimura et al described a 27-year-old man with arteroarterial communication at the peripapillary region. 4 Tamura et al 5 described a 25-year-old man with arterioarterial communication; however, the patient had a history of branch retinal artery occlusion.…”

“…4 Tamura et al 5 described a 25-year-old man with arterioarterial communication; however, the patient had a history of branch retinal artery occlusion. Two cases of arterioarterial communications with angioid streaks have been reported, 7,8 both showing vascular loop formation. This is the first reported case of a patient with arterioarterial communication, located at the posterior pole of the eye, and a prepapillary vascular loop.…”