Wk.6 L2 - Spina Bifida Flashcards
(11 cards)
LO
- Describe the pathophysiology and major subtypes of spina bifida
- Explain common treatments and management approaches for spina bifida
- Explain the public health management of an identified risk factor for spina bifida
Spina bifida
- Neurodevelopmental disorder
- One of the most common of the neural tube defects (NTDs)
- Errors in the development of the caudal spinal cord in utero
- Global prevalence of 1 in 1000 births
- Vertebral arches fail to develop, leading to maldevelopment of vertebral column, spinal cord and nerves and associated muscles
- A sac-like cyst may protrude from the spine
- Defects can be “open” or “closed”
- Estimated 214,000–322,000 affected pregnancies worldwide annually
Spina bifida Occulta
- Mildest form of NTDs
- Affects lower back (L5-S1 regions fail to form)
- Cutaneous stigmata at lesion level
- May be associated with syrinomyelia (cyst in spinal cord) or tethered cord syndrome
- Often associated with anorectal and/or urogenital malformations, bowel, bladder and sexual dysfunction is common.
Meningocele
- Sac-like cyst of meninges filled with spinal fluid
- Usually with no involvement of spinal cord, but possible spinal abnormalities
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Myelomeningocele
- Most serious form and most common ~90% cases
- Protrusion of meninges and spinal cord through vertebral defect
- 80% in lumbar and sacral regions
- Involvement of neural tissue with paralysis of limbs and abdominal muscle, loss of sensation and incontinence
- Surgery usually required, prior to or soon after birth
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Possible complications of myelomeningocele:
Chiari II malformation
* Condition where brainstem and cerebellum protrude down through hole of base of spine into vertebral column
* Compresses brain tissue, damaging
* Results in sensory and motor symptoms through to paralysis, brainstem damage and hydrocephalus
* May require prenatal surgical decompression of brain as an emergency
hydrocephalus
“Water head” - CSF builds up in ventricles of the brain
CSF puts pressure on cerebral hemispheres, damaging tissue and also swells the babies soft skull
Severity is associated with the severity if the psi and neurological outcomes
Treatments and management
- Diagnosed in utero through ultrasound imaging of brain and spinal cord
- Depending on lesion on spinal cord determines functional effects
- No cure, aim is to manage symptoms and maintain QOL.
- Mean survival of Spina bifida patients ~50yo
Good management:
* Early diagnosis
* Surgical treatment in pregnancy
* Proactive and life-long special care
Specialists to help maintain QOL
Urology
Neurosurgery
Orthopaedics
Physical medicine and rehab
Physchology
Assistive tech services
Cause of spina bifida is likely multifactorial
Mostly sporadic condition but genetic predisposition likely; effect of sex and ethnicity
Env risk factors:
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Folate (B9 vitamine) is crucial in closure of the nerual tube
- Brussel sprout, avo, leafy greens, asparagus
Reducing risk in the Australian population
- mandatory supplementation of wheat flour with 2-3mg folic acid/kg flour.
- A low cost intervention
- 32 fewer NTDs/year, $1,500,000 in health system savings
- Net benefit on society
