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Defect in the vertebral arch of the spinal cord – incompletely formed or absent.
Deformity occurs during development in utero
Epidemiology
Incidence 1-2 per 1000, Highest rate in British isles (Ireland , Whales). Africans
and African Americans have lowest rates
Familial tendency
Female predominance (60% children affected are female)
Etiology
Multifactorial inheritance
Environmental factors – nutrition and folic acid;
Teratogens–Drugs especially Carbamezapine and Valproic acid (anticonvulsants)
Pathogenesis
>Embryology
Trilaminar germ disk – 3rd week
Ectoderm cells overlying the notocord thicken to form the neural plate.
Lateral edges of neural plate elevate – neural folds with neural groove at the center
Neural crest cells migrate into mesenchyme and will form dorsal roots
Neural folds approach and fusei from the cervical area caudally and cranially forming
neural tube
Anterior and posterior neuropores close at day 25 and 27 respectively
Spinal cord develops from neural tube
4th week, mesoderm proliferates at midline forming paraaxial mesoderm. This is
arranged longitudinally forming somites. Each somite forms sclerotome around spinal
cord which will differentiate to form the vertebrae
Malformations
Failure of fusion of dorsal vertebrae or failure of neural groove to close
Classification
1. Spina bifida cystica
Menigocele – herniation of meninges
Meningomyelocele – spinal cord and nerve roots herniated. 94%
Lipomeningocele – lipomatous mass herniates and attaches to spinal cord
tethering it
Myelocelemyelocele, or myeloschisis- open neural plate covered by epithelium
and the neural plate has spread out onto the surface.
2. Spina bifida occulta - Lesion covered by skin, no herniation.
Skin stigmata i.e. hairy patch, dimple, dermal sinus tract, lipoma, haemangioma etc
seen. Lesion usually above the gluteal fold along the spinal cord
1
Associated anomalies
Meningomyeloce show increased incidence of Chiari II malformation, Hydrocephalus
Syringomyelia, Brainstem malformations, Cerebral ventricle abnormalities (cranial nerve)
Midline abnormalities – VACTREL
Pathophysiology
Problem caused when spinal cord contents herniated or the neural tube is
completely open and the ependymal layer is exposed (myelocele or myeloschisis)
Lack of protection of nervous tissue causes damage mechanically or from lack of
vascular support, desiccation, or from other insults. This results in features like
neurogenic bowel and bladder, a contracted bladder leads to hydronephrosis along
with infections and renal failure,
Asymmetric innervation causes muscular imbalance, which leads to joint
contractures and developmental deformities such as hip dislocation and spinal
deformities.
Seizure activity secondary to the neural tube defect may be noted.
Chiari II malformations can cause deterioration of neurologic system with time and
may present with signs of brainstem compression (stridor, central apnea, dysphagia,
quadriparesis, nystagmus, myelopathy
EVALUATION
Initial examination
>Local
>General
Type 1 – segmental level with flacid paralysis, loss of sensation and loss of reflexes.
Type 2 – Interruption of long tracts only either partially or complete
3. Associated hydrocephalus
4. Associated orthopaedic deformity (hip dislocation, CTEV, vagus and varus
deformities, scoliosis etc)
5. Other associated deformities –vertebral, cardiac, anorectal, esophageal, renal.
Give attention also to the anus to assess function of the sacral root
2
Investigation
Imaging Studies:
• CT scan
• MRI
o Superior anatomical detail and assists the surgeon in choosing the best
treatment. Necessary in endoscopic treatment,
o The lateral ventricles have a characteristic appearance in almost all patients
with spina bifida the occipital horns are more dilated than the frontal horns,
and the long axis of the lateral ventricles tend to be parallel.
Supportive investigations
Full haemogram, urea and electrolytes, urine cultures and urinalysis, skin sensitivity tests,
CSF studies
TREATMENT
>Immediate treatment
Cover open NTD with saline moist sponge to avoid rupture or drying
Give systemic antibiotics if leaking
Constitute a management team
>Definitive
Step 1: Circumnavigate the neural placode without injuring any of the neural elements.
Once completed, neural placode is placed into the spinal canal. Many methods available
Step 2: Identification and dissection of the dura then covering the neural placode covered
using the dura. If the dura is absent, the muscle fascia is used.
3
Shunt placement during myelomeningocele closure
Prevention
4
i
Surgery Grand round
Spina bifida
Presentor: Gregory Ganda