Arnold-Chiari malformation
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Synopsis

Type II CM is usually diagnosed in infants and young children. It is almost always associated with myelomeningocele (spina bifida). Manifestations in infants include dysphagia and difficulty feeding, stridor, episodes of apnea, and extremity weakness due to brainstem dysfunction. Manifestations in childhood include progressive hydrocephalus and syringomyelia; scoliosis may occur.
Common complaints in children include severe head and neck pain, occipital headache, blurred vision, dizziness, balance issues, and spasticity. Type II CM often results in the development of a syrinx (a fluid-filled cavity in the spinal cord). More severe symptoms include limb weakness, changes in breathing patterns (apnea), or abnormal eye movements (nystagmus).
Codes
ICD10CM: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
SNOMEDCT:
373587001 – Chiari malformation type II
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Vertigo
- Cerebellar ataxia
- Cervical spondylosis
- Cervicogenic headache
- Occipital neuralgia
- Sleep apnea
- Laryngeal nerve paralysis
- Pseudotumor cerebri
- Syrinx
- Intracranial hypotension (idiopathic versus traumatic versus iatrogenic from lumbar puncture or shunt)
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Last Reviewed:03/12/2018
Last Updated:04/11/2022
Last Updated:04/11/2022