Spina bifida ( Latin : "split spine") is a developmental congenital disorder caused by the incomplete closing of the embryonic neural tube . vertebrae overlying the spinal cord are not fully formed and remain unfused and open
three categories: spina bifida occulta , spina bifida cystica with meningocele , and spina bifida cystica with myelomeningocele .
Spina bifida occulta – “hidden” - The bony vertebra is open, but the spine is within the spinal canal The skin may have a lipoma (small benign fatty tumor), some discoloration (birthmark), or a small tuft of hair overlying the spinal defect Most patients with spina bifida occulta do not know they have it There may be tethering of the spinal cord
Spina bifida cystica – meningocele The bony vertebra is open, part of the meninges is protruding out of the spinal canal Since the spinal cord is not protruding, there is often normal function Some cases of tethering have been reported
Spina bifida cystica – myelomeningocele The bony vertebra is open, part of the meninges and part or all of the spinal cord is protruding out of the spinal canal Since the spinal cord is protruding, it is often not fully developed Involved nerve roots are often not developed resulting in weakness, pain, and/or paralysis Higher the lesion More deficits are present
Etiology Genetics Exposure of alcohol + Fetal alcohol syndrome Irish and Americans of Irish descents Lack of folic acid h/ o SPINA BIFIDA
Diagnosis and Detection Amniocentesis Alpha Feto Protien - indication of abnormal leakage Blood test Maternal blood samples of AFP Ultrasonography For locating back lesion vs. cranial signs
Clinical features Motor and sensory impairments including loss of bowel and bladder control No clear direct relationship with level
musculoskeletal deformities (scoliosis) joint and extremity deformities (joint contractures, club foot, hip subluxations , diminished growth of non-weight bearing limbs) osteoporosis abnormal or damaged nerve tissue
Other Complications Hydrocephalus Hydromyelia Seizures
hydrocephalus Anatomical changes associated with a cervical lesion: An enlarged head caused by hydrocephalus (“water on the brain” Hydrocephalus
Spina bifida cystica – myelomeningocele Arnold Chiara malformation II is often associated with myelomeningocele and occurs when the cerebellum is forced downward This can result in life-threatening situations because the build-up of cerebrospinal fluid can cause pressure on the brain Patients with Arnold Chiari malformations often require placement of a shunt to drain the excess fluid
Health Problems associated with the Arnold Chiari syndrome Cranial Nerve Palsies Visual Deficits Pressure from the enlarged ventricles affecting adjacent brain structures
Motor dysfunction: Upper limb in coordination: halting and deliberate movement instead of smooth continuous movement Spasticity: related to upper motor neuron lesions
Spina bifida occulta – tethered spinal cord Often occurs later in life Caused by limitations of movement of the spinal cord within the spinal column Patients often have low back pain, weakness in the legs, and/or incontinence depending on the site of tethering
Related Problems Skin Breakdown Decubitus ulcers and other types of skin breakdown Obesity Latex Allergy
Physiotherapy intervention 1 st stage Pre-closure: MMT, ROM assessment, therapeutic positioning for sleeping. Post-closure: MMT, sensory assessment, home program instruction
Goals Prevent secondary complications Promote age appropriate sensory motor development Prepare child for ambulation Educate family bout appropriate strategy
Postoperative positioning Prone positioning and side lying Avoid frog leg position W sitting Heel sitting Cross leg sitting
Sensory precaution Prevention of contracture – ROM
Newborn Therapeutic positioning pre- and post-surgery for repair of myelomeningocele . Keep an eye out for shunt malfunction.
The Young Toddler Typically seen in a transdisciplinary clinic for management of multiple and varied medical, surgical needs, and therapeutic needs. Transdisciplinary teamwork enhances communication, prevents delays in care, coordinates management. Transdisciplinary team consists of: neurosurgeon, orthopedist, urologist, PT, OT, nurse, social worker, and may include others.
Concerns for the Young Toddler Developmental delay: delayed and abnormal head and trunk control, righting, and equilibrium responses Handling/Positioning: The child needs to develop upright head control in many positions
Therapeutic handling
Orthoses and Equipment typical for children with SB Total contact orthosis A-frame (Toronto standing frame) parapodium () Star Cart Reciprocating gait orthosis HKAFO rollator walker floor reaction AFO (a.k.a. anti-crouch orthosis) articulating ankle joints in S1-level lesions
Example of a Parapodium Commonly used for children with high lesions (T12-L3) Offers support to the hips, knees, and ankles.
Activities for the Young Toddler Stimulate automatic balance responses against gravity in all positions to activate responses in the lower extremities. Encourage brief periods of well-aligned weight-bearing throughout the day to stimulate acetabular development (reducing likelihood for hip dysplasia) and prevent osteoporosis. Avoid infant walkers, jumper seats, swings, bouncer chairs, excessive use of infant car seats.
The Adolescent Psychosocial issues: dependency on parents or caretakers poor personal hygiene from lack of independence and motivation, need for vocational training
The Adult Need to focus on health promotion and fitness. Watch for overuse syndrome, especially in upper extremities. Also, low back pain. Monitor for safe and properly fitting equipment (wheelchair, bathroom devices, supportive & protective shoes Model advocacy to improve access to community-based resources . Thank you