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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On general examination patient may have intra cerebral calcifications, arachnodactyly, increased susceptibility to infections and mental retardation.3 The prevalence of this disease is 1 to 4 per million people.
Arachnodactyly, as defined in one or more of the followings: (i) positive wrist sign (Steinberg sign) on both sides; (ii) positive thumb sign (Walker sign) on both sides 10.
He was tall and slender (body weight: 58 kg; height: 183 cm; BMI: 17.3 kg/cm2) with arachnodactyly which is considered as a marfanoid habitus.
Haim-Munk syndrome It is a rare autosomal recessive genodermatosis characterized by palmoplantar hyperkeratosis, onychogryphosis, arachnodactyly and acroosteolysis.
Craniosynostosis, dolichostenomelia, proptosis, low-set ears, arachnodactyly, camptodactyly, pes planus, pectus excavatum or carinatum, scoliosis, joint hyperextensibility or contractures, hydrocephalus, dilatation of lateral ventricles, Chiari 1 malformation may be seen in this syndrome.
The proband (individual II: 2) was a 29-year-old woman who presented typical marfanoid cardiovascular features, facial features, and arachnodactyly, but without ectopia lentis.
Physical examination showed clubfoot, low-set ears with dysplastic lobes and dysmorphic antihelix, anteverted nostrils, arachnodactyly, low-set thumbs, bilateral clinodactyly of 5th finger of the hands, and varus foot.
On physical examination, the patient with the long-facial appearance had high-arched palate, long limbs and legs, purple-guinea-colored transverse striae of back, arachnodactyly, genu recurvatum, joint laxity, hypermobility, pes planus, scoliosis, and pectus excavatum.
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.
Her fingers and toes appeared elongated (arachnodactyly), although lengths were not objectively measured, and her thumb held abducted (see Figure 1).
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).