Le syndrome de Grônblad et Strandberg (stries an-gioides de la rétine et pseudoxanthome élastique) et ses rapports avec ďautres affections du mésenchyme

Pseudoxanthoma elasticum (PXE), also known as Gröenblad-Strandberg syndrome, is an heritable multi-system disorder, characterized by aberrant mineralization of soft connective tissue resulting in fragmentation of elastic fibers, involving primarily the …

Fundus examination revealed angioid streaks and subretinal hemorrhages on OU (Oculus Uterque), macular fibrosis on OD and “peau d’orange” pigmentary mottling on OS. Leakage areas on fundus fluorescein angiography (FFA) revealed active CNV on OU, which was confirmed by Optical Coherence Tomography (OCT).

In the course of the last fifty years various cases of angioid streaks in the ocular fundus have been reported in the literature. In all the cases the fundus showed about the same characteristic picture, consisting of a network of streaks varying in color from reddish to brownish black.

2008年4月1日 · Grönblad-Strandberg syndrome is a rare congenital hereditary dysplasic disorder of the connective tissue, characterized by a progressive abnormal mineralization and dystrophic calcification...

They typically have their onset after the skin lesions appear. The fundus may also have areas of yellow mottling temporal to the fovea suggestive of an orange peel surface. These are sometimes labeled 'peau d'orange' and their appearance frequently precedes the …

Results: Best-corrected visual acuity (BCVA) on presentation was 20/ 200 OD (Oculus Dexter) and 20/ 60 OS (Oculus Sinister). Fundus examination revealed angioid streaks and subretinal hemorrhages on OU (Oculus Uterque), macular fibrosis …

2022年12月29日 · fundus features such as Peau De orange appearance, choroidal neovascularization and disciform macular degeneration. Visual impairment occurs eventually in over 70% of patients. The systemic disease most commonly associated with angioid streaks is pseudoxanthoma elasticum or Gronblad-Strandberg syndrome, a predominantly autosomal recessive disorder.

The Gr6nblad-Strandberg syndrome is considered to be a hereditary systemic disease of elastic tissue. In 1935, Franceschetti and Roulet named the syndrome after Gr6nblad and Strandberg to commemorate their important research in this field [5, 10, 14]. Abnormal collagen is …

The Grönblad-Strandberg syndrome, also known as pseudoxanthoma elasticum, is a rare congenital dysplasic disorder of the connective tissue, characterized by a progressive abnormal mineralization and dystrophic calcification of elastic tissues and collagen (1).