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Congenital Contractural Arachnodactyly Cover

Congenital Contractural Arachnodactyly

Open Access
|Apr 1990

Full Article

An infant girl with arachnodactyly and spontaneously resolving contractures who died in cardiac failure is reported from the Paediatric Unit, Northern General Hospital and Department of Ophthalmology, Royal Hallamshire Hospital, Sheffield, England. In addition to the arachnodactyly the infant had dolichostenomelia, iridodonesis, and mitral and tricuspid incompetence. There was no evidence of lens subluxation on slit lamp biomicroscopy. Chromosome studies and urinary homocystine were normal. [1]

COMMENT. Congenital contractural arachnodactyly has been described as an autosomal dominant syndrome distinct from classical Marfan’s syndrome and usually unassociated with serious ocular and cardiovascular complications. This case report questions this distinction and emphasizes the importance of cardiovascular and ophthalmic assessment of patients with contractural arachnodactyly. As an editorial comment from Springfield, Illinois, I cannot omit the frequent reference to President Abraham Lincoln and Marfan’s syndrome.

Language: English
Page range: 28 - 28
Published on: Apr 1, 1990
Published by: Pediatric Neurology Briefs Publishers
In partnership with: Paradigm Publishing Services

© 1990 J Gordon Millichap, published by Pediatric Neurology Briefs Publishers
This work is licensed under the Creative Commons Attribution 4.0 License.