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Condition/Disorder Synonyms

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  • Arnold-Chiari malformation

  • Chiari II malformation

  • Arnold-Chiari II

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ICD-9-CM Code

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  • 348.4 Compression of brain

  • 742.2 Congenital reduction for deformities of brain

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ICD-10-CM Code

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  • G93.5 Compression of brain

  • Q04.8 Other specified congenital malformations of brain

  • Q07.0 Arnold-Chiari syndrome

  • Q07.00 Arnold-Chiari syndrome without spina bifida or hydrocephalus

  • Q07.01 Arnold Chiari syndrome with spina bifida

  • Q07.02 Arnold-Chiari syndrome with hydrocephalus

  • Q07.03 Arnold-Chiari syndrome with spina bifida and hydrocephalus

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Preferred Practice Pattern1

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Key Features

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Description

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  • Defect in cephalad portion of spinal column

  • Herniation of portions of the brainstem and spinal cord through the foramen magnum, including the cerebellum, medulla, and cervical spinal cord

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Essentials of Diagnosis

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  • Characteristic signs and symptoms with diagnosis confirmed by MRI

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General Considerations

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  • Most often occurs in presence of spina bifida

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Demographics

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  • 90% of patients with Chiari malformation also have hydrocephalus

  • Three times more common in females than males2

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Clinical Findings

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Signs and Symptoms

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  • Respiratory difficulties

  • Vision difficulties

    • Nystagmus

    • Diplopia

    • Blurred vision

    • Esotropia

  • Bradycardia

  • Cranial nerve palsies

  • Ataxia

  • Vertigo

  • Hydrocephalus

  • Headache

  • Neck pain

  • Decreased balance

  • Spasticity, numbness or tingling in hands

  • Poor fine-motor skills

  • Slurred speech

  • Developmental delay

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Functional Implications

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  • Delayed motor development

  • Difficulty swallowing

  • Poor balance

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Possible Contributing Causes

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  • Abnormality during fetal development

  • Teratogens

  • Possible genetic link

  • Infection

  • Insufficient maternal vitamins or minerals during gestation

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Differential Diagnosis

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  • Brain tumor

  • Syringomyelia

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Means of Confirmation or Diagnosis

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Imaging

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  • MRI

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Findings and Interpretation

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Treatment

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Medical Procedures

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Referrals/Admittance

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  • Geneticist

  • Neurologist

  • Orthopedic surgeon

  • Occupational therapist

  • Speech therapist

  • Admittance for surgical procedures, such as

    • Tracheostomy

    • Gastrostomy

    • Posterior fossa decompression

    • Cervical laminectomy

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Impairments

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  • Delayed motor milestones

    • Rolling

    • Sitting

  • Decreased balance in sitting

  • Additional impairments typically associated with spina bifida

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Tests and Measures

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  • Pediatric Evaluation of Disability Inventory (PEDI)

  • Manual muscle test

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Intervention

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  • Therapy

  • Child should avoid activities associated with risk for cervical injury

  • Developmental activities to facilitate gross motor development

    • Prone on elbows

    • Rolling

    • Sitting

  • Balance training

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