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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Haim-Munk syndrome also exhibits arachnodactyly, acroosteolysis, atrophy of nails, and deformity of the phalanges in the hands.
Subsequently, several anomalies were evidenced: narrow palate, pectus excavatum, recurrent hernias, arachnodactyly, general marfanoid physical aspect, and joint laxity with a high degree of elbow extension, besides cardiac and ocular anomalies.
The limb abnormalities after VPA exposure may include pre- and postaxial polydactyly, overlapping digits, talipes (clubfoot), clinodactyly, arachnodactyly, hip dislocation, limb deficiencies, preaxial and postaxial polydactyly, reduction malformations of the arms and hands and radial ray defects19,20 .
Primary affection of hands and feet; part of this is the contractural arachnodactyly (So called distal arthrogryposis; autosomal dominant).
5,6] Molecular analysis of Marfan-like conditions has unveiled that patients with congenital contractural arachnodactyly (CCA or Beals syndrome) carry germline mutations of the fibrillin 2 (FBN2) gene on chromosome 5q23-q31.
Retrospectively, medical historians thought that Gabrielle had been born with congenital contractural arachnodactyly.
The Steinberg test for arachnodactyly, in which the thumb is adducted across the palm, was considered a possible indication of marfanoid habitus, if the thumb projected beyond the ulnar boarder of the hand.
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.
Shprintzen-Goldberg syndrome (SGS) is characterized by craniosynostosis (involving the coronal, sagittal, or lambdoid sutures), distinctive craniofacial features, skeletal changes (dolichostenomelia, arachnodactyly, camptodactyly, pes planus, pectus excavatum or carinatum, scoliosis, joint hypermobility, or contractures), neurologic abnormalities, mild-to-moderate intellectual disability, and brain anomalies.
It is characterized clinically by palmo-plantar keratoderma (PPK), aggressive early onset of periodontitis, onychogryphosis, arachnodactyly, acroosteolysis and pes planus.
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.
In addition to typical bilateral lens subluxation, arachnodactyly and long and thin extremities were found in the patient.