Brain and spinal cord abnormalities related to SB can lead to lifelong impairments. Closed spinal dysraphism conditions with similar care needs include lipomyelomeningocele, congenital tethered cord, and sacral agenesis/caudal regression. Patients with closed (or skin-covered) forms of SB do not have associated brain anomalies, such as hydrocephalus or Chiari II malformation, unlike patients with myelomeningocele (MMC). With advancements in pediatric care, 85% of individuals with SB live well into adulthood. SB is often classified by function according to the Hoffer scale: thoracic (flaccid lower extremities), high lumbar (hip flexion present), mid lumbar (knee flexion present), low lumbar (ankle dorsiflexion present), and sacral (ankle plantar flexion present). Common reasons for hospitalization in adults with SB are ventricular shunt malfunction, urinary tract infections, pressure injuries, bowel problems, and osteomyelitis. Common causes of death in adults with SB are sepsis, pneumonia, respiratory failure, and renal failure. People with SB commonly have a latex allergy or require latex precautions. For more information, visit the Spina Bifida Association's website.

Concerns

Neurocognitive Abilities

Information

  • Most people with SB do not have intellectual disability, but there is variability. Those with MMC and shunts are more likely to have cognitive deficits.
  • Common challenges include: reading comprehension (making inferences), functional math skills (price comparison, time, estimation), executive function (planning, initiating tasks, attention switching, and problem solving).
  • Relative strengths include: language fluency, following routine/practiced rule-based tasks. 

Follow-Up

  • SBA Neuropsychology Guideline 
  • Additional Resources: Burke, R., Liptak, G. S., the Council on Children with Disabilities; Providing a primary care medical home for children and youth with spina bifida. Pediatrics. 2011;128(6): e1645–e1657. doi:10.1542/peds.2011-2219
  • Queally JT, Barnes MA, Castillo H, Castillo J, Fletcher JM. Neuropsychological care guidelines for people with spina bifida. J Pediatr Rehabil Med. 2020;13(4):663–673. doi:10.3233/PRM-200761

Mental Health

Information

  • Common concerns may include: depression, anxiety, attention deficit disorder, and interpersonal and intrafamily stressors
  • Community involvement and participation in peer groups can support mental health.

Follow-Up

  • SBA Mental Health Guideline
  • SBA Family Functioning Guideline
  • Additional Resources: Burke, R., Liptak, G. S., the Council on Children with Disabilities; Providing a primary care medical home for children and youth with spina bifida. Pediatrics. 2011;128(6): e1645–e1657. doi:10.1542/peds.2011-2219
  • Kritikos TK, Smith K, Holmbeck GN. Mental health guidelines for the care of people with spina bifida. J Pediatr Rehabil Med. 2020;13(4):525–534. doi:10.3233/PRM-200719

Nervous System

Information:

  • Hydrocephalus occurs in about 40% to 80% of those with MMC, frequently in higher SB levels.
  • Treatment consists of ventricular shunt or endoscopic ventriculostomy (ETV). Ventriculoperitoneal (VP) shunts are often placed in the neonatal period. Shunt malfunctions occur more frequently during childhood but can occur in adulthood.
  • Hydrocephalus symptoms include: severe headache, seizures, memory/attention changes, swallowing changes, vision deficits, vomiting, lethargy, respiratory suppression.
  • Tethered cord/syringomyelia can occur in open and closed SB. Symptoms include: worsening back pain, spinal curvature, bowel/bladder incontinence, change in strength/tone/deformity or sensation of the lower extremities.
  • Chiari II malformation is associated with MMC; decompression is more common in childhood. Symptoms (apnea, dysphagia, cranial nerve abnormalities) are most commonly due to shunt malfunction.

Follow-Up

Pulmonary

Information

  • Central apnea (due to Chiari II) and obstructive sleep apnea (due to body habitus) are common.
  • Restrictive lung disease is common due to scoliosis, especially in higher SB lesions, and may be asymptomatic except in cases of surgery or pulmonary illness. 

Follow-Up

Cardiovascular

Information

  • Hypertension is common, often starting in adolescent/young adult years and may be due to renal scarring, medications, family history, or obesity.
  • Patients with VA shunt are at risk for right heart failure. 

Follow-Up

Renal/Urinary: Neurogenic Bladder

Information

  • All levels of SB are associated with neurogenic bladder; function can change with age.
  • Common surgeries to address neurogenic bladder include: ileocystoplasty augmentation, vesicostomy/urostomy, catheterizable abdominal channel (see handout), bladder neck surgery.
  • Comorbidities include: urinary tract infections (UTIs), bladder/kidney stones, hydronephrosis, kidney disease, and bladder cancer.
  • Chronic bacteriuria of the urinary tract is common. Antibiotic suppression is usually discouraged as it causes increased antibiotic resistance. Increased water intake, routine cathing, bladder irrigation with water, and improving bowel continence may help with malodorous and/or cloudy urine.
  • Signs of concern include: increased creatinine, worsening acidosis, or cystatin C (follow trend), worsening incontinence or new nocturnal enuresis despite adherence to cathing, increased UTIs, hematuria or persistent microhematuria, new upper tract dilation on imaging.

Follow-Up

Bowel

Information

  • Bowel movements every 1-2 days can help prevent incontinence and constipation.
  • Regimen to goal: predictable bowel movements every 1-2 days, minimal accidents, and constipation.
  • Adults with SB may have:
    • Malone Antegrade-Colonic Enema stoma or CHAIT tube cecostomy for antegrade enema. These regimens use 300-1000 ml water (or saline) in feeding bag or syringe through a straight 16” catheter inserted into the channel placed in the cecum. Sometimes baby soap, glycerin, or laxatives are added to the water to improve output timing. Stool is evacuated from the rectum into the toilet in usually 30-60 minutes.
    • Transanal irrigation system or cone enema. This device allows the individual to flush out their bowels through the rectum using 300-1000 ml water (or saline).
    • Ileostomy or colostomy, which occurs less commonly.

Follow-Up

Reproduction and Sexual Health

Information

  • For people of all genders, clinicians should review intimate partner safety and sex education, sexual health/family planning, interest, and function (positioning/sensation concerns), and bowel/bladder management during sex, latex-free barrier options.
  • Females:
    • Review menses management and concerns.
    • Review sexual health/family planning.
    • Pregnancy is possible. For females with SB or who have had a baby with SB, there is an increased risk of having a child with SB (4%). Pregnancy risks include: bowel/bladder regimen changes, UTI. Less common are: complications from restrictive lung disease, tethered cord, shunt.
  • Males:
    • Erectile dysfunction (ED), retrograde ejaculation, oligospermia are common especially with lesions L2 and above.

Follow-Up

Obesity

Information

  • Obesity is common due to decreased activity and low lean muscle mass.
    • BMI—for non-ambulatory patients, height can be estimated using the arm span length (cm) x 0 x 0.95 (mid lumbar) and 0.90 (high lumbar/thoracic).

Follow-Up

Skin

Information

  • Pressure injuries are common, especially in cases of difficulty transferring, prolonged hospitalization, new equipment (orthotics/wheelchair), crawling or skeletal deformity, worsening urinary or fecal incontinence.

Follow-Up

Bone Health

Information

  • Risk factors include: wheelchair dependency, antiepileptics, antipsychotics, decreased testosterone/estrogen, poor nutrition, low calcium/vitamin D, chronic steroids, chronic acidosis/renal failure, medroxyprogesterone. 
  • Common fractures if wheelchair dependent include: long bones (femur/tibia) and spine.

Follow-Up

Musculoskeletal

Information

  • Assess assistive devices, orthotics, wheelchair seating and positioning, mobility.
  • Assess for musculoskeletal injury and pain (e.g., neck, shoulders, back).
  • Assess for progressive joint contractures.

Follow-Up

Preventive Care

Information

  • Use adaptive exam tables for routine exams.
  • Follow general age/risk-based guidelines.

Follow-Up

Last Updated

03/17/2025

Source

American Academy of Pediatrics