SPINA BIFIDA

DEFINITION

It is the congenital defects of neural tube closure. Failure of neural tube closure produces defects that may involve the entire length or maybe restricted to a small area.

SPINA DYSRAPHIA or SPINA BIFIDA

defect in closure of the vertebral column with varying degrees of tissue protrusion through the bony cleft.

MYELODISPLASIA

all inclusive term that refers to defective development of any part of the spinal cord; usually used to describe abnormalities without gross superficial defects.

CLASSIFICATIONS

SPINA BIFIDA OCCULTA

fusion failure of posterior vertebral arches without accompanying herniation of the spinal cord or meninges; usually not visible externally.

SPINA BIFIDA CYSTICA

defect in closure with external saccular protrusion through the bony spine with varying degrees of nerve involvement.

TYPES OF SPINA BIFIDA CYSTICA

MENINGOCELE

consists of a sac-like cysts of meninges filled with spinal fluid.

MYELOMENINGOCELE

consists of hernial protrusion of a saclike cysts containing meninges, spinal fluid and a portion of of the spinal cord with its nerves.

MORE SEVERE CASES OF NEURAL TUBE DEFECTS

RACHISCHISIS

fissure in the spinal column that leaves the meninges and spinal cord exposed.

ENCEPHALOCELE

herniation of brain and meninges through a defect in the skull that produces a fluid-filled sac in the occipital region.

ANENCEPHALY

absence of brain; exposed vascular mass and no bony covering.

CLINICAL MANIFESTATION

SPINA BIFIDA OCCULTA

NO OBSERVABLE MANIFESTATION Maybe associated with one or more cutaneous manifestation: - Skin depression - Port-wine angiomatous nevi - Dark tufts of hair - Soft, subcutaneous lipomas Maybe neurological disturbances : - Progressive disturbance of gait with foot weakness - Bowel and bladder disturbances

SPINA BIFIDA CYSTICA

Sensory disturbances usually motor dysfunction - Flaccid, areflexic partial paralysis of lower extremeties - Sensory deficit - Overflow incontinence with constant dribbling of urine - Lack of bowel control - Rectal Prolapse - Maybe saddle anesthesia with bladder and anal sphincter paralysis Joint deformities (sometimes produced in utero) - Talipes vagus or varus contracture - Kyphosis - Lumbosacral scoliosis - Hip Dislocations

DIAGNOSTIC PROCEDURES

Basis of clinical manifestation Examination of the meningeal sac If it is transilluminated - meningococele If it is not transilluminated - myelomeningococele Plain radiography -to disclose the bony defect in the symptomatic lesion. Spinal tomograms and Myelography -used to differentiate spina bifida and other spinal disorders. Skull Tomography -establish the presence or absence of hydrocephalus PRENATAL DETECTION Ultrasound Alpha-fetoprotein test -it can indicate the presence of anencephaly or myelomeningococele. (16 - 18 weeks of gestation)

Medical Management
Meningocele & Myelomeningocele Immediate surgery MRI (Magnetic Resonance Imagining) Ultrasound of the back. MRI or ultrasound of the brain may be done to look for hydrocephalus (extra fluid in the brain). Appropriate antibiotics

Nursing Management
Responsibility of nurse in Meningocele and Myelomeningolocele Until the operation is performed the newborn should be : 1. Kept flat on his abdomen with a single layer of sterile gauze. 2. The genitalia and buttocks must be kept clean. 3. The ankles should be supported with foam rubber pads so that the toes do not rest upon the bed. 4. Antibiotics must be given if infection is suspected. 5. Emptying the infants bladder every 2 hours during the day and once at night, pressure should be applied firmly but gently beginning in the umbilical area and slowly progressing under the symphysis pubis and toward the anus. 6. If evidence of urinary infection occur, culture should be done to determine the antibiotics. 7. The infant should be held for his feeding. 8. The nurse records the activity of the legs and the degree of continence, whether there is constant or intermittent dribbling, noting whether there is retention of urine or fecal impaction. All the vital signs should be taken and recorded with extreme care.

Responsibility of the nurse Postoperatively

1. The nurse is responsible for observing Temperature, Pulse Rate, Respiratory Rate, symptoms of shock, and abdominal distention. 2. Head circumference of the infant must be measured frequently. 3. Surgical dressing should be kept clean. 4. Cast applied to the child legs should be positioned properly and handled carefully. 5. Nutrition is important.

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