What is the recommended management for occult spina bifida?

Management of Occult Spina Bifida

The recommended management for occult spina bifida requires early neurological and urological assessment with regular surveillance for tethered cord syndrome, even in asymptomatic patients, to prevent progressive neurological deterioration and urinary tract damage. 1, 2

Diagnostic Evaluation

• MRI examination of the spine is essential for accurate diagnosis and characterization of intraspinal and perispinal abnormalities in patients with suspected occult spina bifida 2
• Comprehensive uroneurological assessment is crucial as patients with occult spina bifida may present with urinary symptoms as the sole initial complaint without obvious neurological abnormalities 1
• Neurological examination should focus on identifying both upper and lower motor neuron signs, as occult spina bifida typically presents with dominant upper motor neuron signs 1

Management Approach

Urological Management

• Regular urodynamic testing is recommended to detect bladder dysfunction before upper tract changes develop 3
• Clean intermittent catheterization (CIC) with or without anticholinergic medications should be initiated based on urodynamic findings 3
• Two main approaches exist for urological management 3:
  • Proactive approach: Early and regular urodynamic testing with CIC and/or pharmacotherapy initiated before development of upper tract changes
  • Expectant approach: Clinical monitoring with periodic ultrasound, with urodynamics and CIC initiated only when clinical deterioration or hydronephrosis develops

Neurosurgical Management

• Continued surveillance for tethered cord syndrome is indicated in all patients with occult spina bifida 3
• Surgical decompression should be considered for symptomatic patients with progressive neurological deficits, chronic pain, or urological dysfunction 2
• Intraoperative neurophysiological monitoring can assist in identifying the appropriate level for decompression 2

Special Considerations

Pain Management

• MRI is essential before performing interventional pain procedures in patients with suspected occult spinal dysraphism 4
• Lumbar epidural steroid injections should be avoided or approached with extreme caution due to anatomical abnormalities including potential arteriovenous malformations and fatty substitution of paravertebral tissues 4

Long-term Surveillance

• Regular follow-up is necessary even in asymptomatic patients with occult spina bifida, as it can be associated with various pathologies that may develop over time 5
• Monitoring should include assessment for:
  • Urological dysfunction (detrusor hyperreflexia, low compliance detrusor, detrusor-sphincter dyssynergia) 1
  • Neurological deterioration (progressive weakness, sensory changes) 2
  • Chronic pain syndromes 2, 4

Transitional Care

• Adequate transitional care from pediatric to adult services is extremely important for patients with spina bifida 3
• Transition of urological care to adulthood remains a major avenue for improvement in disease management 3
• With improved attention to factors that promote independence early in childhood, emerging adults with spina bifida may be less dependent on others for care 6

Potential Complications

• Urological issues can be a significant source of morbidity and mortality in patients with spina bifida 3
• Chronic kidney disease due to poor bladder dynamics is a serious long-term complication 3
• Pressure sores, mobility limitations, and incontinence are common complications that impact quality of life 7

Surgical Interventions

• Surgical intervention is indicated for those at risk for renal deterioration or who fail to achieve satisfactory continence with medical management 3
• Major reconstructive urological surgeries play an important role in protecting the upper urinary tract and achieving continence 3
• For patients with fecal incontinence, the Antegrade Continence Enema (MACE) procedure can achieve complete or near-complete fecal continence in 77% of patients 6