Management of Spina Bifida Occulta
Most patients with spina bifida occulta require regular urodynamic surveillance and proactive bladder management, with surgical intervention reserved for those developing upper tract deterioration or failing conservative management.
Initial Diagnostic Evaluation
Urodynamic testing is the cornerstone of management to detect bladder dysfunction before upper tract changes develop, even in asymptomatic patients 1. This proactive approach is critical because:
- Urological issues represent a significant source of morbidity and mortality in spina bifida patients 1
- Chronic kidney disease from poor bladder dynamics is a serious long-term complication 1
- Patients may present with urinary symptoms as the sole initial complaint without obvious neurological abnormalities 2
- Spinal MRI is essential for diagnosis, particularly in adults with chronic symptoms that fail standard management 3
Common urodynamic abnormalities include:
- Detrusor hyperreflexia (42% in occult form) 2
- Low compliance detrusor (67% in occult form) 2
- Detrusor-sphincter dyssynergia (27% in occult form) 2
- Upper motoneuron signs are typically dominant in occult spina bifida 2
Conservative Management Algorithm
First-line treatment consists of clean intermittent catheterization (CIC) with or without pharmacotherapy, initiated before upper tract changes develop 1. This approach is critical because:
- Early intervention prevents renal deterioration 1
- Up to 50% of patients followed conservatively with CIC ± pharmacotherapy through puberty spontaneously achieve continence 4
- There is no correlation between neurological findings and urodynamic abnormalities, making objective testing essential 2
Surveillance schedule should include:
- Regular urodynamic testing at intervals determined by initial findings 1
- Upper tract imaging (typically renal ultrasound) and serum creatinine annually in stable patients 5
- Continued monitoring for tethered cord syndrome in all patients 1
Surgical Intervention Criteria
Surgery is indicated for patients at risk for renal deterioration or those failing to achieve satisfactory continence with medical management 1. Major reconstructive urological surgeries play an important role in protecting the upper urinary tract and achieving continence 1.
Specific surgical considerations:
- Bladder augmentation (enterocystoplasty) creates a large, low-pressure reservoir to protect upper tracts, though it commits patients to lifetime catheterization 4
- Bladder neck procedures address outlet incontinence issues 4
- Tethered cord release in symptomatic adults generally results in improvement or stabilization of symptoms with low complication rates 6
- Early surgical treatment is reasonable even in asymptomatic adults, with special consideration for older patients with poor medical condition 6
Critical Pitfalls to Avoid
Do not delay diagnosis in adults with chronic low back pain or non-specific symptoms. Spina bifida occulta can present with chronic pain as the predominant symptom, and delayed diagnosis decreases quality of life 3. MRI examination is required for characterization of intraspinal abnormalities when chronic symptoms fail expected management 3.
Do not assume neurological examination predicts urodynamic dysfunction. There is no correlation between neurological findings and urodynamic abnormalities, making objective urodynamic testing mandatory 2.
Transition to Adult Care
Adequate transitional care from pediatric to adult services is extremely important and remains a major avenue for improvement 1. Structured transition programs with meetings involving both adult and pediatric medical and nursing staff together improve satisfaction 7. Most providers believe transition should occur at age 18-21 years 5.
Adult care requires multidisciplinary collaboration with neurosurgery/neurology (87% of providers identify as needed), social work (84%), and orthopedics (73%) 5.