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Condition/Disorder Synonyms

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  • Myelomeningocele

  • Spina bifida cystica

  • Myelodysplasia

  • Spinal dysraphism

  • Meningocele

  • Myelocele

  • Meningomyelocele

  • Spina bifida occulta

  • Spina bifida aperta

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ICD-9-CM Codes

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  • 741 Spina bifida

  • 741.0 Spina bifida with hydrocephalus

  • 741.00 Spina bifida unspecified region with hydrocephalus

  • 741.01 Spina bifida cervical region with hydrocephalus

  • 741.02 Spina bifida dorsal (thoracic) region with hydrocephalus

  • 741.03 Spina bifida lumbar region with hydrocephalus

  • 741.9 Spina bifida without mention of hydrocephalus

  • 741.90 Spina bifida unspecified region without hydrocephalus

  • 741.91 Spina bifida cervical region without hydrocephalus

  • 741.92 Spina bifida dorsal (thoracic) region without hydrocephalus

  • 741.93 Spina bifida lumbar region without hydrocephalus

  • 742.0 Encephalocele

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ICD-10 Diagnosis Codes

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  • Q01 Encephalocele

  • Q01.0 Frontal encephalocele

  • Q01.1 Nasofrontal encephalocele

  • Q01.2 Occipital encephalocele

  • Q01.8 Encephalocele of other sites

  • Q01.9 Encephalocele, unspecified

  • Q05 Spina bifida

  • Q05.0 Cervical spina bifida with hydrocephalus

  • Q05.1 Thoracic spina bifida with hydrocephalus

  • Q05.2 Lumbar spina bifida with hydrocephalus

  • Q05.3 Sacral spina bifida with hydrocephalus

  • Q05.4 Unspecified spina bifida with hydrocephalus

  • Q05.5 Cervical spina bifida without hydrocephalus

  • Q05.6 Thoracic spina bifida without hydrocephalus

  • Q05.7 Lumbar spina bifida without hydrocephalus

  • Q05.8 Sacral spina bifida without hydrocephalus

  • Q05.9 Spina bifida, unspecified

  • Q07.01 Arnold Chiari malformation

  • Q07.3 Arnold Chiari syndrome with spina bifida and hydrocephalus

  • Q76.0 Spina bifida occulta

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Preferred Practice Pattern1

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Key Features

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Description

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  • Group of disorders involving failure of closure in the caudal end of the spinal column in utero

  • Lipomenigocele: fatty tissue mass without typical neurologic involvement or hydrocephalus

  • Anencephaly: failure of closure of caudal end of neural tube, incompatible with life

  • Occipital meningocele: meningeal sac at the occipital level

  • Encephalocele: meningeal sac containing cerebral tissue

  • Spina bifida occulta: mildest defect characterized by dimple in skin and/or tuft of hair, asymptomatic

  • Meningocele: meningeal sac filled with cerebrospinal fluid2

  • Meningomyelocele: severe defect characterized by meningeal sac that contains a portion of the spinal cord

  • Spina bifida aperta: rare, most severe type of defect that contains neural plate and rectum in addition to spinal cord

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Essentials of Diagnosis

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  • Often involves the meninges and/or spinal nerves resulting in hydrocephalus and/or Arnold-Chiari malformation

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General Considerations

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  • Extent of neural involvement ranges from anencephaly, sacral agenesis, meningocele, or myelomeningocele

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Demographics

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  • Probability of occurrence in siblings 1 to 2%3

  • Probability of occurrence in offspring 5%3

  • Meningomyelocele accounts for 94% of all spina bifida occurrences.4

  • Incidence 1:1000 live births

  • Irish and Celtic families at increased risk (4.5:1000 live births)

  • Japanese families at lowest risk (0.3:1000 live births)

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Clinical Findings

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Signs and Symptoms

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  • Open lesion on spinal column

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