Condition Definition Symptoms Spina Bifida Occulta a failure of fusion of the vertebral arches posteriorly Lipomyelomeningocele and lipomeningocele: like a tethered spinal cord, except attached to benign fatty tumor; Thickened filum terminale: end of the spinal cord too thick; Fatty filum terminale: fatty lump at the inside end of the spinal cord; Diastematomyelia (split spinal cord) and diplomyelia: spinal cord split in two, usually by piece of bone or cartilage; Dermal sinus tract (SC involved): spinal canal + skin of back connected by band of tissue. Spina Bifida Aperta Variations in the failure of total closure of the embryonal neural tube or of mesodermal tissue to invest it. Commonest in T/L region Rachischisis: • most severe, neural tube open and exposed on the surface • CSF leaks from the exposed upper end of the Central Spinal Canal Myelomeningocele: • NT closed by membrane but skin covering is deficient • SC + nerve roots displaced post into sac + outside line of the vertebral canal Meningocele: • Bulging sac only meninges ad fluid, nerve elements normally situated. Often no neurological involvement, possible impairment of nerve function caused by tethering of the dura, then spinal cord, to the skin surface by a fibrous membrane. Txx of the cord gradually worse as disproportion between spinal column and spinal cord >> slow progression of neurological deficit • “My skin feels weird in the bottom of my spine” • “My Feet changed in the last years” • “My surroundings noticed I was limping” • “I suffer from some incontinence and it never happened to me before” • “My back hurts” • “My legs hurt/feel weak” • “I feel some changes in sensation the my legs/ back" Varying degress of motor, sensory, and visceral paralysis, Consequent orthopaedic disability in LEx complex. Primary paralysis at birth due to failure of dvp of part of SC Secondary Paralysis after birth from damaged to exposed nerve tissues Motor paralysis: • mainly lower limbs, trunk to some extent. • Degree of paralysis correspond to degree of dysplasia or of sencondary damage in SC (from mild to very severe) • Uneven paralysis>>mm imbalance>>secondary contractures with fixed deformity of hips, knees, feet. Hip dislocation very common (glut+abductor paralysis with strong flexors+adductors) Sensory paralysis: same distribution as the motor paralysis Visceral paralysis: bladder and bowel incontinence present in high proportion Hydrocephalus: usually due to Arnold-Chiari malformation of the hind-brain Signs Tests Medical Tests Medical Treatments No correlation between severity of bony defect and degree of neurological impairment often none might feel bifide SP on palpation if neurological involvement: • Dimple, a thuft of hair, a lipomatous mass, dermal sinus, dark spot or a birth mark (red), a hypopigmented spot on the overlying sk • muscle imbalance in LEx, • selective m wasting in foot, • equino-varus [inverted feet] or pes cavus If neuro symptoms Neuro Test in LEx • DTR’s, muscle power, light touch, sharp/dull • SLR? Plain radiograph MRI and radiculography required if neuro involvement + incontinence If structural lesions found: neurosurgical intervention may be required Important objective: determine the level at which normal function of SC ceases. Obs mvt of the child+correlate them with root levels. Sensibility tested from limbs to trunk Admition to special centre with team of experienced specialists Program depending on the pt: - Correct deformity - Maintain correction - Promote the best possible function in the affected Orthopaedic ttt deferred until age 1 to 3 Contraindications If neuro test significantly concerning ? avoid any HVT or strong technique in the concerned area in the spine avoid any HVT or strong technique in the concerned area in the spine