What is acrodysostosis?

Anonymous

∙ 11y ago

Updated: 8/21/2019

Acrodysostosis is a variety of dysostosis, a bone development disorder, which affects the joints of the hands or feet.

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∙ 11y ago

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Acrodysostosis?

DefinitionAcrodysostosis is an extremely rare disorder that is present at birth (congenital). People with this condition have problems in the bones of the hands, feet, and nose, and mental retardation.Alternative NamesArkless-Graham; Acrodysplasia; Maroteaux-MalamutCauses, incidence, and risk factorsMost patients with acrodysostosis have no family history of the disease. However, sometimes the condition is passed down from parent to child. Parents with the condition have a 1 in 2 chance of passing the disorder to their children.There is a slightly greater risk with fathers who are older.SymptomsFrequent middle ear infectionsGrowth problems, short arms and legsHearing problemsMental deficiencyUnusual looking faceSigns and testsA physical exam confirms this disorder.Findings may include:Advanced bone ageBone deformities in hands and feetDelays in growthProblems with the skin, genitals, teeth, and skeletonShort arms and legs with small hands and feetShort head, measured front to back (brachycephaly)Short heightSmall, upturned broad nose with flat bridgeUnusual features of the face (short nose, open mouth, jaw that sticks out)Unusual headWide-spaced eyes (hypertelorism), sometimes with extra skin fold at corner of eyeIn the first months of life, x-rays may show spotty calcium deposits, called stippling, in bones (especially the nose). Infants may also have:Abnormally short fingers and toes (brachydactyly)Early growth of bones in the hands and feetShort bonesShortening of the forearm bones near the wristTreatmentTreatment depends on the physical and mental problems that occur.Orthopedic care, early intervention, and special education are recommended.Expectations (prognosis)Problems depend on the degree of skeletal involvement and mental retardation. In general, patients do relatively well.ComplicationsArthritisCarpal tunnel syndromeWorsening range of movement in the spine, elbows, and handsCalling your health care providerCall your health care provider if your child seems to be growing or developing slowly or abnormally, or if you notice physical abnormalities in your infant.PreventionConsider genetic counseling to help with diagnosis, testing, and identifying risk.

What is acrodysostosis?

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Acrodysostosis?

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