Angioid Streaks in Ehlers-Danlos Syndrome

An update on the ocular phenotype in patients with pseudoxanthoma elasticum

“…Paget’s disease of the bone has been reported to be associated with angioid streaks in 1.4–14% (Scholz, 1941) and peau d’orange in 0–22% of cases (Clarkson and Altman, 1982). It has been reported that angioid streaks may also occur in the absence of any of the PXE-like systemic or ocular alterations (Clarkson and Altman, 1982), including two members of a family with Ehlers–Danlos syndrome (Green et al, 1966). However, up to date phenotyping in such patients would be needed to support such associations.…”

Variants of the Ehlers-Danlos syndrome. Clinical, biochemical, haematological, and chromosomal features of 100 patients.

“…The chromosomes of the peripheral blood of three related patients with the EDS were found to be normal by Pommerening and Antonius (1966), and in another family chromosome analysis in two related patients affected by the EDS and in a third unaffected relative did not reveal any significant abnormality (Green, Friedman-Kien, and Banfield, 1966).…”

Tenuity of Cornea With Ehlers‐danlos Syndrome

“…Report of a case 3 10/ 12-year-old girl. Anamnestically it was.informed that the girl often fell and easily caught hematomae.…”

“…Cot-tinil2 has reported angioid streaks in a patient suffering from Ehlers-Danlos syndrome as well as from Groenblad-Strandbergs syndrome. Green et al 3 have observed two perfect cases of Ehlers-Danlos syndrome with angioid streaks. Pemberton et al 6 have scrutinized three generations of a family, six out of seven members of which suffered from myopia and amotio retinae.…”

“…Visus 0. dxt. 3 After section of the prolapsed iris tissue and irrigation of the lens masses suturing of the rupture of cornea was attempted; this, however, proved impossible as the sutures cut through the thin, fragile cornea tissue. Thereafter, grafting of Tenon's capsule was tried, but this likewise was so thin and delicate that grafting was not possible.…”