Spina bifida

• Myelomeningocele
• Spina bifida cystica
• Myelodysplasia
• Spinal dysraphism
• Meningocele
• Myelocele
• Meningomyelocele
• Spina bifida occulta
• Spina bifida aperta

• 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

• 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

• Pattern 5C: Impaired Motor Function and Sensory Integrity Associated With Nonprogressive Disorders of the Central Nervous System - Congenital Origin or Acquired in Infancy or Childhood

Description

• 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

Essentials of Diagnosis

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

General Considerations

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

Demographics

• 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)

Signs and Symptoms

• Open lesion on spinal column
• Skin-covered cephalocele
• Hydrocephalus
• Muscle weakness and/or paralysis in lower extremities
• Sensory deficits in lower extremities
• Talipes equinovarus (clubfoot)
• Latex allergy
• Visual perceptual deficits
• Cognitive deficits
• Speech delay
• Signs and symptoms of tethered cord ...