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CONGENITAL CONDITIONS-ANENCEPHALY (Signs and Symptoms (No bones on the…
CONGENITAL CONDITIONS-ANENCEPHALY
Definition
Anencephaly is the absence of a major portion of the brain, skull, and scalp that occurs during embryonic development. It is a cephalic disorder that results from a neural tube defect that occurs when the rostral (head) end of the neural tube fails to close, usually between the 23rd and 26th day following conception
Health Education
Parents of babies with anencephaly should be educated about preventive measures for future pregnancies. Consultation with a genetic counselor may be helpful.
Signs and Symptoms
No bones on the back of the head
Missing bones on the front and sides of the head
Folding of the ears
Blindness
Deafness
Causes
During pregnancy, the brain and spine begin as a flat plate of cells. This plate rolls into a tube called the neural tube. The tube is completely formed 28 to 32 days after conception. If all or part of the neural tube fails to close, this leaves an opening. The opening is called an open neural tube defect. The opening in the tube may be left exposed. Or it may be covered with bone or skin. Anencephaly is when the neural tube fails to close at the base of the skull.
Diagnostic Studies
Blood test.
Prenatal Ultrasound
Amniocentesis
Calssification
Meroanencephaly
Meronanencephaly is a rare form of anencephaly characterized by malformed cranial bones, a median cranial defect, and a cranial protrusion called area cerebrovasculosa.
Holoanencephaly
The most common type of anencephaly, where the brain has entirely failed to form, except for the brain stem. Infants rarely survive more than one day after birth with holoanencephaly
Craniorachischisis
The most severe type of anencephaly where area cerebrovasculosa and area medullovasculosa fill both cranial defects and the spinal column. Craniorachischisis is characterized by anencephaly accompanied by bony defects in the spine and the exposure of neural tissue as the vault of the skull fails to form
Pathophysiology
In the normal human embryo, the neural plate arises approximately 18 days after fertilization. During the fourth week of development, the neural plate invaginates along the embryonic midline to form the neural groove. The neural tube is formed as closure of the neural groove progresses from the middle toward the ends in both directions, with completion between day 24 for the cranial end and day 26 for the caudal end. Disruptions of the normal closure process give rise to NTDs. Anencephaly results from failure of neural tube closure at the cranial end of the developing embryo. Absence of the brain and calvaria may be partial or complete.
Risk Factors
A shortage (deficiency) of folate appears to play a significant role
Diabetes Mellitus
Obesity
Anti Seizure Medication
Exposure to high heat
Surgical Management
Surgical management is not applicable in anencephaly
Medical Mangement
Medical Mangement is not applicable
Nursing Mangement
There is no treatment for anencephaly; instead, the child is provided with supportive care. The infant should be kept warm and any areas of the brain that are exposed must be protected. Sometimes a special bottle is used to help feed babies who may have difficulty swallowing fluid
As these infants die within days or weeks of being born grief management should be offered to the parents and family
Nursing Care Plan
Grieving
Desired Outcomes
Patient participates in self-care activities of daily living (ADLs), as able.
Patient recognizes impact/effect of the grieving process (e.g., physical problems of eating, sleeping) and inquires proper help.
Patient identifies and expresses feelings (e.g., sadness, guilt, fear) freely.
Patient looks toward/plan for future, one day at a time.
Nursing Interventions
Allocate a private room if patient wants it, with regular contact by care providers. Encourage of feelings unlimited visiting by family and friends.
Support free flow of emotional expression. Only restrict behavior that is dangerous to well-being of patient/couple (e.g., pulling out IV, using fists to pound on abdomen).
Include partner in planning care. Grant opportunity for partner to be seen individually. Reinforce discussion of concerns.
Discover the magnitude of the loss for both members of the couple. Regard how strongly couple desired this pregnancy.
