Spina bifida

SPINA BIFIDA
• Congenital disorder in which the 2 posterior vertebral arches
(or several arches) fail to fuse.
• Occur in 1st 6 wk of gestation.
• Associated with mal development of neural tube.
• Subsequent mal development of the overlying skin.
• Dysraphism refers to be the sum of; Bony, Neural, skin
defects.
• Site: Lumbar or Lumbosacral.
• If neural element:Paralysis, Hyposthesia, Incontinence.
 Pathology
• Spina Bifida Occulta (Mildest form).↓
• Midline defect between 2 laminae.
• If multi segment → skin (tuft of hair ± Naevus).
• Sometimes: -Conus tethering.
• Caudal lipoma.
• Caudal cysts.
• Diastomatomyelia (Cord splitting).
 Spina Bifida Cystica (severe)
• Missed lamina.
• Vertebral prolapse.
• 2 Sub forms:Meningocele, Meningomyelocele.
 Meningocele
• Incidence around 5%.
• Cord & root in the canal.
• Dura is open posteriorly.
• CSF filled sac protrude.
• No CNS manifestations.
 Meningomyelocele
• Commonest.
• Cord & roots prolapse into the meningeal sac.
• Neurological deficit below the level.
• If neural tissue is exposed to air→ulceration →infection
→Meningitis.
• Hydrocephalus as sequelae→cord tethering→cerebellar and
brain stem herniation through foramen
magnum→obstruction of
CSF→hydrocephalus→↑ICT→cerebral atrophy + MR.
 Meningomyelocele

Closed Open
- Neural tube is fully formed - Primitive cord
- Covered by membrane and - Unfolded neural tube → form
skin the sac roof
 Epidemiology
• Meningocele 5% 2:100
• Spina bifida cystica. Second child
• Folic acid administration in 1st trimester
→↓2:1000incidence.
• Neural tube defect →↑α Fetoprotein in amniotic
fluid after 1st trimester.
Clinically:
1- SB Occulta:
• discovered incidentally in X ray.
• Tuft of hair.
• Pigmented nevus.
• Sometimes neurological manifestation:
• Paresis.
• Hyposthesia.
• Enuresis.
• Incontinence.
 Clinically::

2- SB Cystica:
Hydrocephalus:
• Starts even at birth.
• No ICT.(after closure) ICT↑
Paralysis:
• Level vary according to the lesion level.
• Increase if the case was neglected (↑ root traction).
• Saccular lesion over Lumbosacral region. Deformities:
(ð ms imbalance, IU position, independent)
• • Hip: Dislocation (5%)
• • Knee: Genu recurvatum
• • Ankle: TEV + claw toes
 Treatment
• Defect closure→Ms chart at the time of closure is mandatory for assesment.
• WITH IN 48 HRS.
• Neural tissue is preserved,dura is closed,Skin is undermined&closed .

Assess
Hydroceph
alus

• ventriculoatrial drainage via first valve shunt.

Till 6
yrs(tendency to
form)↓

• removal of shunt.
• Tethereed cord is invetible↑Release,if↑pain,Nrological deficit are
present.
• DEFORMITIES:
• optimum treatment calls for team work(ortho,neuro,physiotherapist.).
• Reapet MS chart after defect closure and healing.Start with strech and
strap,asses after 4 yrs of initil emergent surgery→ORTHOTICS.
• Child never becomes independent.
• UL&mental skills are more are more than walking.
• children with below L4 lesions have QF→can walk.
• Avoid prolonged immobilization→fracture.
• Avoid urinary problems.
 Kypho scoliosis: common in MMC,MS.
weakness,imbalance,vertebral anamoly,teethered cord.