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Congenital conditions (Encephalocele (Signs and symptoms (Neurologic…
Congenital conditions
Encephalocele
Pathophysiology
Two distinct and critical processes are involved in the formation of the neural tube: primary neurulation and secondary neurulation (i.e., canalization).
Primary neurulation (weeks three and four during embryogenesis, forming the early brain and spinal cord)
The neural folds elevate, approximate each other, and start fusing along the dorsal midline, thus forming the neural tube. This begins during the third week after conception.
The cranial neuropore closes during the fourth gestational week. The last area to close is the commissural plate.
The point of initial closure occurs at the caudal rhombencephalon, or cranial neuropore, on day 25 of embryogenesis.The cutaneous ectoderm fuses first, followed by the neuroectoderm.
Secondary neurulation (canalization: weeks five and six, forming the early sacral and coccygeal cord). This characterizes further neural development caudal to the caudal neuropore after the termination of primary neurulation, and the caudal neuropore closes
This process includes formation of the filum terminale and conus medullaris from a poorly differentiated cell mass of the medial eminence.
Because of differential growth between the vertebral column and the spinal cord, the conus becomes more rostral during later development.
By 8 weeks after conception, spinal cord tissue runs the entire length of the spinal cord.
Causes
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It is more common in families with a history of spina bifida and anencephaly, both of which are severe neurological disorders.
Exposure to certain environmental substances before or during pregnancy may inhibit the proper development of the neural tube during the embryonic stage.
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Spina bifida
Pathophysiology
Neural tube defects are the result of a teratogenic process that causes failed closure and abnormal differentiation of the embryonic neural tube.
Neural tube defects occur between the 17th and 30th day of gestation, at a time when the mother may not be aware that she is pregnant and the fetus is estimated to be about the size of a grain of rice.
The most common neural tube defects are anencephaly and myelomeningocele. Anencephaly results from failed closure of the rostral end of the neural tube, resulting in incomplete formation of the brain and skull.
The malformation can occur in any region of the the spinal cord but most in lumber or sacral region.
Signs and symptoms
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A build up of cerebrospinal fluid (CSF), leading to hydrocephalus,
and possibly brain damage
Causes and risks factors
Plant proteins, iron, magnesium, and niacin: A low intake of these nutrients before conception may be associated with an increased risk of neural tube defects by two to five times.
Family history: If one infant is born with spina bifida, there is a 4 percent chance that a future sibling will have the same condition.
Medications: Drugs such as valproate, used to treat epilepsy or bipolar disorder, have been associated with a higher risk of giving birth to babies with congenital defects, such as spina bifida.
Diabetes: A woman who has diabetes is more likely to have a baby with spina bifida, than one who does not.
Folic acid: Spina bifida is more likely if a mother does not have enough folic acid during the pregnancy, although the reason is unclear.
Nursing management
Prevent infection by monitoring the newborn’s vital signs, neurologic signs, and behavior frequently; administer prophylactic antibiotic as ordered
Prevent contractures of lower extremities.massage the knees and other bony prominences with lotion regularly, then pad them, and protect them from irritation.
Promote skin integrity. Placing a protective barrier between the anus and the sac may prevent contamination with fecal material, and diapering is not advisable with a low defect.
Maintain the newborn in a prone position so that no pressure is placed on the sac; after surgery, continue this positioning until the surgical site is well healed.