arachnodactyly


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arachnodactyly

[ə¦rak·nə′dak·tə·lē]
(medicine)
A rare congenital defect of the skeletal system marked by abnormally long hand and foot bones.
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Joint hyperlaxity, arachnodactyly, pecus excavatum or carinatum, scoliosis and contractures of the feet (talipes equinovarus) are seen in LDS patients, and are also common in Marfan syndrome.
In regard to the aforementioned syndrome patients we have to note that they presented with a variety of the expected typical characteristics, more specifically MVP, arachnodactyly, muscular atrophy and ectopia lentis for the Marfan patients, and MVP skin and joint hyperextensibility (8), ectopia lentis and highgraded myopia for the Ehlers-Danlos patients.
Beals Hecht syndrome, also known as congenital contractural arachnodactyly, is a member of arthrogryposis multiplex congenita family.
Marfan syndrome and classic homocystinuria represent the top 2 in the list of differential diagnoses for a patient presenting with external features of tall stature, disproportionately long limbs, arachnodactyly, scoliosis, and, after ophthalmologic investigation, lens dislocation.
The diagnosis of MS was established on the characteristics of tall stature, intermaxillary narrowness, myopia, retinitis pigmentosa, blue sclera, scoliosis, pectus excavatum, arachnodactyly and diminished ratio of Us/Ls.
The latter condition has a phenotypic expression similar to PLS, plus in addition arachnodactyly, atrophic changes of the nails, and deformity of the phalanges of the hand [Hart and Shapira, 1994].
Key Words: congenital contractural arachnodactyly, Beals syndrome, "crumpled ear," fibrillin-2 protein
She is unusually tall, with an expansive reach and long, reed-thin fingers and toes-traits marking a condition so distinctive that ancient physicians named it arachnodactyly, after the Greek word for spider.
Clinically, she had severe manifestations of MFS--she was tall, with limb length disproportion and marked arachnodactyly.
The researchers, led by Francesco Ramirez of the Mount Sinai School of Medicine in New York City, also report finding a gene on chromosome 5 that codes for a different version of fibrillin, which may cause a related disorder of the fingers called congenital contractural arachnodactyly.
Physical findings in Marfan syndrome Skeletal Arm span > height Arachnodactyly Ligament laxity Kyphosis Pectus excavatum High arched palate Cardiovascular Aortic insufficiency murmur Mitral valve prolapse Ophthalmologic Lens dislocation Myopia
Arachnodactyly is one of the major skeletal manifestations of Marfan syndrome.