Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content ++ Arthrogryposis multiplex congenita (AMC)AmyoplasiaClassic arthrogryposisGordon syndrome ++ 728.3 Other specific muscle disorders754.89 Other specified nonteratogenic anomalies ++ Q74.3 Arthrogryposis multiplex congenita ++ 4A: Primary prevention/risk reduction for skeletal demineralization5B Impaired neuromotor development +++ Description ++ Children are born with limited range of motionLimited room in the uterus for developmentTerm used to describe two or more congenital, non-progressive joint contractures2 +++ Essentials of Diagnosis ++ Comprises over 300 different disorders +++ General Considerations ++ TypesMyopathic: fixed flexion contractures of joints, chest, and spineAmyoplasia: most common and can have dense fibrous tissueArthrogryposis Ectodermal DysplasiaCote Adamopoulos PantelakisArthrogryposis Multiplex Congenita Neurogenic (AMCN)Arthrogryposis Multiplex Congenita Distal (AMCD)Distal joints more often involved than proximal jointsGordon syndrome: Distal Arthrogryposis, Type 2AAffects joints bilaterally +++ Demographics ++ 1 in 3,000 to 5,100 infants3Genetic: X-linked recessive origin primarily affects males3 +++ Signs and Symptoms ++ Club footHip dysplasiaLimited range of motion of joints including temporomandibular jointAbnormal development of the muscles, joints and bonesScoliosisAbnormal development of the spinal cord and central nervous systemCraniofacial malformationsCardiac, respiratory, or organ anomaliesWebbing skin around joints +++ Functional Implications ++ Limits functional mobilityDevelopmental delays with movement +++ Possible Contributing Causes ++ Prader-Willi Syndrome4Amyoplasia (focal arthrogryposis)4Neonatal neuropathy4Congenital myopathy4Congenital muscular dystrophy4Congenital myasthenia4Myasthenia gravisMultiple sclerosisMyotonic dystrophy4Werdnig-Hoffmann motor neuron disease4OligohydramniosNeuromuscular disorderFetal akinesiaFetal hyperthermiaFetal intrauterine crowdingPrenatal virusFetal vascular compromiseAbnormality of connective tissueMaternal infection or illness +++ Differential Diagnosis ++ Metabolic diseaseMuscular dystrophy +++ Laboratory Tests ++ CPK levels +++ Imaging ++ Computerized tomography (CT)Magnetic resonance imaging (MRI)Radiograph of bony deformitiesElectromyelogramFetal ultrasound +++ Diagnostic Procedures ++ Muscle biopsyElectromyographyNerve conduction velocity ++ Abnormal chromosomes with genetic testingExcessive muscle fibrosis on muscle biopsy +++ Medical Procedures ++ OsteotomyTenotomySerial casting ++ Admission for surgical correctionOrthopedist for muscle and joint development issuesNeurologist for spinal cord and CNS developmentGeneticistOrthotist for bracing and splintingOphthalmologist for retinopathyOccupational therapist for fine motor skillsSpeech therapist for throat musculatureSocial workerCounseling services for family resources ++ Decreased passive and/or active range of motionDecreased strengthInability to transfer independentlyInability to sit independentlyInability to stand independentlyInability to rollInability to walk independentlyDecreased independent with ADL: seating, dressing, toileting... Your Access profile is currently affiliated with [InstitutionA] and is in the process of switching affiliations to [InstitutionB]. Please select how you would like to proceed. Keep the current affiliation with [InstitutionA] and continue with the Access profile sign in process Switch affiliation to [InstitutionB] and continue with the Access profile sign in process Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Error: Incorrect UserName or Password Username Error: Please enter User Name Password Error: Please enter Password Sign in Forgot Password? Forgot Username? Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free profile for additional features.