SPINA BIFIDA

 SPINA BIFIDA

• "SPINA " means "RELATEd TO SPINE "

• "BIFIDA " means "WITH TO BRANCHES /DIVISIONS"

• DEFINITION = Spina bifida is developmental congenital disorder caused by the incomplete closing of the embryonic neural tube .

• Spina bifida is a birth defect in which there is incomplete closing of the spine and the membranes around the spinal cord during early development in pregnancy.

• Spina bifida is a neural tube defect , results from defective fusion of one or more posterior arches with resultant protrusion of the contents of the spinal canal

• CAUSES = family history

• Folic acid deficiency

• Pregnant women taking valpronic acid have an increased risk to having children with spina bifida.

• Maternal diabetes

• Genetic basis

• Obese women

• Diabetes

• TYPES :- There are two main types:

• A)spina bifida occulta,

(Occulta = HIDDEN)

Spina bifida aperta

( APERTURE = OPENING)

B) Spina bifida Cystica

2. meningocele and

3. myelomeningocele

• Spina bifida cystica can then be broken down into meningocele and myelomeningocele

• LOCATION:- The most common location is the lower back, the lumbar and sacral area , but in rare cases it may be in the middle back or neck.

• CLASSIFICATION:-

• 1. spina bifida occulta :-

• There is merely a radiological defect between the laminae

• Occulta has no or only mild signs,

• About 15% of people have spina bifida occult. 

• which may include a hairy patch, dimple, dark spot or swelling on the back at the site of the gap in the spine.

• Rarely associated with neurological deficit

• Presence of overlying midline skin defect like haemangioma,lipoma and a tuft of hair.

• Caudal regression syndrome

LIPOMYELOMENINGOCELE :-

• One type of spina bifida occulta ..

• Here Lipomatous tissue inserts into the conus, and herniates through the bony defect dorsally to attach to a subcutaneous mass.

• TWO VARIETIES;-

• One that inserts caudally into the conus, and 

• One those attach to dorsal surface of spinal cord

A.Spina bifida aperta

• There is developmental deficiency of laminae ,spinous processes , overlying muscle and skin .

2.meningocele :-

• The spinal cord and nerve roots are intact only the covering membrane projects as a dual sac.

• Meningocele typically causes mild problems, with a sac of fluid present at the gap in the spine. 

• If this defect is closed by early surgery ,paralysis is avoidable and rehabilitation is easy.

• Presents as swelling along the spinal cord

• Lower limbs are normal

• No neurological defects

• SITE :- lumbar region ,

3.myelomeningocele:-

• Myelomeningocele (MMC), also known as meningomyelocele or open spina bifida.

• is the type of spina bifida that often results in the most severe complications and affects the meninges and nerves. 

• Spinal cord is exposed to the surface as a plaque or nervous tissue .

• It is associated with muscle paralysis ,poor ability to walk, sensory loss , impaired bladder or bowel control and deformities . 

• It may be present from 4th thoracic to 1st sacral vertebra.

• It is common in lumbosacral region.

• Clinical features:-

• Motor and sensory deficits vary according to the level and extent of the spinal cord involvement 

• Motor paralysis is usually of the lower motor neuron type

• Sensory deficit is present in the dermatomes that would be innervated by the defective spinal segments and nerve roots. 

1. Sensory impairment

2.Musculoskeletal impairment

• Weakness & paralysis

• Physical problems

• Orthopedic deformities

• Osteoporosis

3. Neurological problems

• Hydrocephalus

• Chairi malformation

• Hydromyelina

4. Bowel and bladder dysfunction

5. Growth & nutrition

1.SENSORY IMPAIRMENT

• Impaired sensation below the level of lesion.

• Sensory loss includes kinesthetic , proprioceptive & sometosensory information.

• Because of the child have to heavily rely on visual & other sensory system to compensate for this loss. 

2.Musculoskeletal impairment

• A) Weakness & paralysis

• Two types of motor dysfunction

• 1.complete loss of function below the level of lesion ,resulting in a flaccid paralysis ,loss of sensation & absent reflexes. 

• 2. Spinal cord remains intact below the level of lesion ,the effect is in the area of flaccid paralysis immediately below the lesion & possible hyperactive spinal reflexes distal to the area. 

• B)physical problems:-

• Leg weakness and paralysis

• Pressure sores and skin irritations

• Abnormal eye movement

• Orthopedic abnormalities (i.e., club foot, hip dislocation, scoliosis)

• Bladder and bowel control problems, including incontinence, urinary tract infections, and poor kidney function. 

• C)Orthopedic deformities :-

• The Orthopedic problems in myelomeningocele may be tye result of Imbalance between muscle group 

• Effect of stress ,posture & gravity

• Associated congenital malformations ,decrease sensation & neurological complications .

• Scoliosis present and vertebral abnormalities.

• D)Osteoporosis

• Paralysed limbs of the child with spina bifida have increased amounts of unmineralised osteoid tissues,

• 3. Neurological problems

• Hydrocephalus

• Develops in almost 80 % of children with meningomyelocele

• Signs of hydrocephalus includes buldging frontanelles & irritability.

• Most obvious effect of the build up of CSF is abnormal increase in head size ,which may be present at the birth or post natally .

• Chairi malformation

The malformation is a congenital anomaly of the hide brain that includes herniation of the medulla & at the times pons, 4th ventricle & inferior aspect of the cerebellum into the upper cervical canal.

• Not all Chairi malformation are Symptomatic

• As a result of Symptomatic Chairi malformation , problems with respiratory & bulbar may be evident in the child with spina bifida. 

• SYMPTOMS may include :-

• Paralysis of vocal cord associated with respiratory stridor

• Problem with aspiration

• Weakness & cry

• Apneic episodes

• Upper extremity weakness

• Difficulty swallowing & abnormal gag reflex

• HYDROMYELIA:-

• Hydromyelina signifies dilatation of the center canal of the spinal cord as hydrocephalus signifies dilatation of the ventricle of the brain. 

• The area of hydromyelina may be focal,multiple or diffuse extending throughout the spinal cord. 

• The increased collection of fluid may cause pressure necrosis of the spinal cord leading to muscle weakness &scoliosis. 

• 4. Bowel and bladder dysfunction :- because of involvement of sacral plexus ,the child with spina bifida commonly deal with some from of bowel and bladder dysfunction 

• Beside various forms of incontinence , incomplete emptying of the bladder remains of constant concern because infection of the urinary tract and kidney damage may be result . 

• 5. Growth & nutrition

• Nutrition intake & weight gain &loss have been found to be problematic in children with meningomyelocele . 

• Early infants may have difficulties with feeding due to abnormal gag reflex & swallowing difficulties & high incidence of aspiration 

• These impairment may lead to nutritional issues & delayed growth & weight gain

• Investigations ;-

• X ray

• MRI

• CT -scan

• Ultrasonography

• Presence of open NTDs can be detected with the measurement of AFP in the amniotic fluid

• PREVENTION

• Pregnant should consume 0.4mg(400 mcg) of folic acid per day to reduce risk of having apregnancy affected with spina bifida or other neural tube defects.

• Foods that contain folate (natural folic acid)include orange juice,leafy green vegetables , beans, and whole -grain products. 

• Multivitamins,fortified breakfast cereals ,and enriched grains products contain a synthetic folic acid that is more easily absorbed by the body than the natural form. 

• IMMEDIATE CARE :-

• Maintain hydration

• Place the child in prone position

• Monitor for associated defects

• Cover the affected area with sterile gauze piece dipped in normal saline .

• COMPLICATIONS :-

• Hydrocephalus

• Meningitis

• Loss of bowel or bladder control

• Frequent urinary tract infections

• Permanent weakness or paralysis of legs

• Difficulty delivery with problems resulting from a traumatic birth, including cerebral palsy and decreased oxygen to the brain. 

• Club foot

• MANAGEMENT:-

1. Surgical

2. PREVENTION

3. Physiotherapy

• TREATMENT:-

• There is no cure for spina bifida

• The nerve tissue that is damaged or lost cannot be repaired or replaced

• Treatment depends on the type and severity of the disorder

• Children with the mild from need no treatment

• Moderate to severe cases ,surgical closure of back lesion within 6 months.

• SURGERY FOR Meningomyelocele :-

• A. position of the patient on the operating table and an elliptical incision at the junction of the membrane and the skin. 

• B. Membrane being eplised to free the neural plaque

• C. Plaque lying in the dural layer

• D.Dura is closed with a continuous suture

• E, skin is closed with interrupted sutures

• PHYSICAL THERAPY MANAGEMENT:-

• PRE- CLOSURE:-

• ROM assessment ,therapeutic positioning for sleeping

• POST-CLOSURE:-

• Sensory assessment ,home programme instruction (ROM exercises ,handling ,and carrying

positions ,and therapeutic positioning for sleeping)

• NEW BORN:- therapeutic pre and post surgery for repair of myelomeningocele .

• Keep an eye out for shunt malfunctions.

• PROGNOSIS

• Spina bifida is a :-

• Static

• Non progressive defects

• With worsening from secondary problems

• The prognosis for a normal life span is generally good for a child with good habits and a supportive family caregiver