Unique Considerations for Care and Treatment of Individuals with SA/CRS


There are many similarities for treatment of these conditions for those with spina bifida, spinal cord injury and SA/CRS.  For general medical care, concerns address fever (for which UTI/kidney infection should be ruled out first), constipation (for which risk of bowel impaction should be addressed) skin health (for which pressure sores inside casting or braces should be addressed) in the same way as for other patients with spinal involvement.

There are some differences for individuals with SA/CRS.

  • Nerve pathways and sensation tend to be more unique.  Rather than overall decreased sensitivity below the level of spinal agenesis, often the individual with SA/CRS has some areas of reduced sensitivity and some areas of heightened sensitivity below the level of spinal cord termination.
  • The child with SA/CRS has more competition for space in the torso area, depending on the amount of spine missing, and sometimes experiences more esophageal reflux as a result but is usually not compromised in terms of organ function.
  • Children with SA/CRS are more likely to have fused/fixed knee/ankle joints and hips that splay outward.
  • The child with SA/CRS may have a reduced appetite due to a  smaller stomach size and need more frequent smaller meals.  The child is likely to be smaller in size ( which is a benefit for mobility).
  • A child with SA/CRS may not have some organs in the expected place. For example, if doing a mitrofanoff procedure using the appendix, it might be a good idea to find out first if the appendix is in the typical place and not for example, retrocecal.
  • Children with tapered end spinal cords and SA/CRS appear to have issues more frequently with tethered cord.
  • Children with SA/CRS may experience more complex bowel , bladder and kidney issues and may be unable to achieve catherterization due to heightened sensitivity in the genital area. They may be delayed in self-catheritization due to difficulty inserting the catheter independently due to seating position or heightened sensitivity in the genital area or unique physical structures of the urethra.
  • Keep in mind that children with SA/CRS  (without spina bifida-as some children may have both) do not typically have any issues with hydrocephalus or Chiari malformation. They typically have average intellectual functioning.
  • Individuals with SA/CRS may have other conditions that are unrelated to SA/CRS, or they may have syndromes or conditions that include SA/CRS along with other conditions.