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Lecture 2

NURS 3012 Lecture 2: Topic 2 – Neural Tube Defects

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NURS 3012
Lori Robbins

Topic 2 Neural Tube Defects & Hydrocephalus Neural Tube Defects Etiology o Neural tube defects occur when there is a failure of the neural tube to close during the embryo’s first 3-5 weeks o The etiology of NTDs is multifactorial. The cause is thought to be a combination of the following: • Medications – for example, valproic acid (an epileptic drug) • Radiation – for example, x-rays during pregnancy • Maternal malnutrition – low maternal Vitamin B12 and maternal obesity, maternal diabetes • Chemicals • Genetic mutations in the folate pathways – recommended that women of childbearing age take 0.4mg of folic acid daily to prevent risk o Prenatal diagnosis is possible through maternal serum alpha feto-protein (MSAFP) and through ultrasound Types o Anencephaly is the first type of neural tube defects • Anencephaly occurs when minimal brain tissue is developed • Babies are usually born live with this condition, but die within the first few hours of life o Spina bifida is the second type of neural tube defect, which can be broken down into two further types: • Spina bifida occulta – occurs when babies do not have complete closure of the spinal cord by vertebrae. The baby may have a back dimple or tuft of hair on back. There usually are no deficits/risks associated with spina bifida occulta. • Spina bifida cystica – refers to a visible defect with an external saclike protrusion. Spina bifida cystica refers to two types of defects; meningocele and myelomeningocele ▪ Meningocele – sac-like protrusion that encases meninges and spinal fluid. This defect usually does not cause neurological deficits. ▪ Myelomeningocele – sac-like protrusion that encases meninges, spinal fluid and nerves. This defect is the most common, accounting for 90% of spinal cord lesions. This defect usually causes neurological deficits of varying severities. o These babies are born via C-section, if diagnosed prenatally. o If the sac ruptures, then the babies are at extreme risk for infection. Clinical Manifestations o The degree of neurological dysfunction is dependent on the anatomical level of the defect and the amount of nerve tissue involved o Sensory disturbances are parallel to motor dysfunction (motor/sensory function is impaired below the defect) o Almost all babies will have bladder and bowel dysfunction, and most babies will have joint deformities • For example, kyphosis, scoliosis, hip dislocation or subluxation o Is associated with hydrocephalus and Chiari II malformation in about 80% of spina bifida babies • Hydrocephalus is a condition in which there is an abnormal build up of CSF in the ventricles of the brain • Chiari malformations are structural defects in the cerebellum (part of the brain that controls balance). Normally, the cerebellum and parts of the brain stem sit in an indented space at the lower real of the skull, above the foramen magnum. When part of the cerebellum is located below the foramen magnum, it is called a Chiari malformation. • Type II involves the extension of both cerebellar and brain stem tissue into the foramen magnum. Management  Fetal surgery would be ideal for this condition, however it is still in early stages of development and is high risk to the mother and for premature birth. This is not commonly practiced today. o Support is multidisciplinary for care of babies with this condition, and thus requires intensive nursing skills o As for family support and newborn care… • Proper care of the sac should be maintained and taught to parents. Dressing should be applied and changed q2-4 hrs (dependent on the doctor’s orders), and always moistened with normal saline. During dressing changes, the sac should be inspected for leaks, abrasion, irritations or any signs of infection. • Babies should lay in prone positioning. The baby should never lay supine as this puts the child at risk for sac rupture. • Proper skin care should also be performed to reduce risks of infection and skin breakdown o Neurosurgery usually takes place within 24 hours – the surgeon will return the spinal cord and nerves to normal positioning and close the hole. • If the baby suffers from hydrocephalus, the surgeon may perform a shunt procedure. VP shunts are most commonly used in this procedure. • Post-surgery vital signs, I&O and pain management should be monitored. Neuro signs should also be monitored, including whether the baby cries, pupil response, and lethargy. • Babies should also be monitored for signs of increased intracranial pressure • Post-surgery, the babies should remain in prone positioning until directed by the surgeon. Surgeons might allow side-lying for feeds, unless there are other contraindications such as hip issues. Babies may be held as long as pressure is not placed on site of the defect. o Orthopaedic considerations involve prevention of joint contractures, correction of existing deformities, prevention/minimization of effects of motor and sensory deficits, and prevention of skin breakdown. • To prevent joint contractures, parents should be taught to perform range of motions on their children • Common deformities that exist in spina bifida are club foot, or deformities of the hip (subluxation), knee, and spine. • Because children with spina bifida usually have sensory deficits in the lower extremities, skin care is extremely important. It is important to take proper precautions to reduce risk for skin breakdown and to perform thorough assessments. o Myelomeningocele commonly causes neurogenic bladder dysfunction and varying degrees of fecal incontinence. o Bladder considerations include the following: • The characteristics of bladder dysfunction in children vary according to the level of the neurological lesion and the influence of bony growth and development on the spine. Thus, ongoing urological monitoring is essential. • Clean, intermittent catheterization is taught to the children and parents to regularly empty the child’s bladders. This may
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