1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-4-28 10.15844/pedneurbriefs-4-4-5 Developmental Disorders Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Congenital Contractural Arachnodactyly http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 04 1990 01 08 2016 4 4 28 28 Copyright: © 1990 The Author(s) 1990 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Contractural arachnodactyly with mitral regurgitation and iridodonesis

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.

 Congenital Contractural Arachnodactyly Dolichostenomelia Slit Lamp Biomicroscopy

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.

 Huggon IC Burke JP Talbot JF Contractural arachnodactyly with mitral regurgitation and iridodonesis Arch Dis Childhood 1990 Mar 65 3 317 319 10.1136/adc.65.3.317 2334215