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scientific articles

Serious Ophthalmological Complications in the Ehlers-Danlos Syndrome

key information

source: The British Journal of Ophthalmology

year: 1970

authors: Beighton P

summary/abstract:

The main features of the Ehlers-Danlos syndrome (EDS) are cutaneous hyperextensibility, articular laxity, and fragility of the tissues. Systemic ramifications may lead to orthopaedic, cardiovascular, and gastrointestinal problems. Minor ophthalmological changes, including epicanthic folds, strabismus, and myopia are not infrequent, but serious ocular complications are rare. However, there have been a few reports of patients with impaired vision from retinal detachment or displacement of the lens.

The EDS is a generalized disorder of connective tissue, which is usually inherited as a dominant trait. There is some scanty evidence to indicate the existence of an uncommon recessive form, and it is of great interest that the majority of the reports of sight-threatening lesions have pertained to individuals who could well have had this genetic background. The purpose of this paper is to describe an affected brother and sister who both became blind from bilateral ocular catastrophes. The transmission of the EDS in their kindred was consistent with recessive inheritance of the trait.

organization: Johns Hopkins Hospital

DOI: 10.1136/bjo.54.4.263

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