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TABLE 1

Spina Bifida: Types, Description, and Implications

Spina Bifida TypeDescriptionClinical Implications
Myelomeningocele Open NTD posterior vertebral defect and extrusion of spinal cord elements into a meningeal sac; location: cervical, thoracic, lumbar, and/or sacral spine; leads to paraplegia and insensitivity below the lesion and neurogenic bowel and bladder; associated defects include structural brain anomalies (Chiari II malformation, hydrocephalus, brainstem dysfunction abnormalities of the cerebrum and corpus callosum, learning disabilities, dislocated hips and clubbed feet) Complex multisystem disorder that requires ongoing monitoring by spina bifida team, enhanced primary care in the medical home with bidirectional communication and comanagement with the multispecialty spina bifida care team, early-intervention and special education services, physical therapy and adaptive equipment, and developmental and learning monitoring 
Meningocele Closed NTD posterior vertebral defect without extrusion of spinal cord elements into a meningeal sac; location: cervical, thoracic, lumbar, and/or sacral spine; motor deficits are less likely than with myelomeningocele; structural brain anomalies and Chiari II malformation are less likely Early closure of spina bifida sac; follow-up by spina bifida team; additional monitoring in the medical home; ongoing monitoring for neurologic function; early-intervention monitoring; periodic monitoring for possible late onset of neurologic signs 
Occult spinal dysraphism/lipomeningocele Closed NTD posterior vertebral defect and with fatty tumor that might contain neuronal elements; location: typically lumbar and/or sacral spine; leads to motor deficits if neuronal elements are involved; associated defects include tethered cord; structural brain anomalies and Chiari II malformation are unlikely Early neurosurgical intervention; follow-up by spina bifida team; additional monitoring in the medical home; enhanced primary care in the medical home and with follow-up by the multispecialty spina bifida care team; ongoing monitoring for neurologic deficits; early-intervention monitoring 
Spina bifida occulta Benign closed NTD posterior vertebral defect only without a meningeal sac; location: lumbar-sacral spine; usually asymptomatic but can be associated with occult spina dysraphism; usually no associated defects Monitoring and reassurance within the medical home 
Tethered cord Traction injury to the distal spinal cord caused by anomalous attachment of the spinal cord, which causes subtle and progressive loss in neural function; can occur with any NTD at any time, but often occurs with growth spurts; might be precipitated by ventricular shunt failure Monitoring of all children and youth with open or closed NTD for signs of tethering; changes in lower extremity strength, function, or sensation; urinary incontinence or enuresis; changes in bowel function; or worsening scoliosis 
Spina Bifida TypeDescriptionClinical Implications
Myelomeningocele Open NTD posterior vertebral defect and extrusion of spinal cord elements into a meningeal sac; location: cervical, thoracic, lumbar, and/or sacral spine; leads to paraplegia and insensitivity below the lesion and neurogenic bowel and bladder; associated defects include structural brain anomalies (Chiari II malformation, hydrocephalus, brainstem dysfunction abnormalities of the cerebrum and corpus callosum, learning disabilities, dislocated hips and clubbed feet) Complex multisystem disorder that requires ongoing monitoring by spina bifida team, enhanced primary care in the medical home with bidirectional communication and comanagement with the multispecialty spina bifida care team, early-intervention and special education services, physical therapy and adaptive equipment, and developmental and learning monitoring 
Meningocele Closed NTD posterior vertebral defect without extrusion of spinal cord elements into a meningeal sac; location: cervical, thoracic, lumbar, and/or sacral spine; motor deficits are less likely than with myelomeningocele; structural brain anomalies and Chiari II malformation are less likely Early closure of spina bifida sac; follow-up by spina bifida team; additional monitoring in the medical home; ongoing monitoring for neurologic function; early-intervention monitoring; periodic monitoring for possible late onset of neurologic signs 
Occult spinal dysraphism/lipomeningocele Closed NTD posterior vertebral defect and with fatty tumor that might contain neuronal elements; location: typically lumbar and/or sacral spine; leads to motor deficits if neuronal elements are involved; associated defects include tethered cord; structural brain anomalies and Chiari II malformation are unlikely Early neurosurgical intervention; follow-up by spina bifida team; additional monitoring in the medical home; enhanced primary care in the medical home and with follow-up by the multispecialty spina bifida care team; ongoing monitoring for neurologic deficits; early-intervention monitoring 
Spina bifida occulta Benign closed NTD posterior vertebral defect only without a meningeal sac; location: lumbar-sacral spine; usually asymptomatic but can be associated with occult spina dysraphism; usually no associated defects Monitoring and reassurance within the medical home 
Tethered cord Traction injury to the distal spinal cord caused by anomalous attachment of the spinal cord, which causes subtle and progressive loss in neural function; can occur with any NTD at any time, but often occurs with growth spurts; might be precipitated by ventricular shunt failure Monitoring of all children and youth with open or closed NTD for signs of tethering; changes in lower extremity strength, function, or sensation; urinary incontinence or enuresis; changes in bowel function; or worsening scoliosis 

Spina bifida is a general term for several malformations of the spine and its neural elements, which may be associated with neurologic, neuromotor, developmental, and orthopedic anomalies and are related to structural abnormalities of the brain and cranium.

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