Anencephaly

Neural tube defect (NTD); occurs in approximately 1 out of 1,000 births. 

The neural tube, which acts as the foundation of the spinal cord and brain, develops during the embryonic period (fertilization until the eight gestational week).  Defects in neural tube development manifest during this time. Gaps in the formation of the tube leave the spinal cord and brain unprotected and susceptible to damage. The type of NTD that develops, its severity, its affect on fetal development, and its potential for future complications will depend upon where the opening is and what tissues are involved [1].  Genetic and environmental causes have been implicated as the causative agents in this birth defect, with folic acid deficiency in the mother being the most notable.  Research suggests that maternal supplementation of folic acid can decrease the incidence of neural tube defects by 50% [4]. Unfortunately, the neurological system is developing at such an early time in gestation that the mother may not even realize she pregnant, resulting in supplementation deficits.

There are two types of NTDs, closed and open. Open NTDs are most common and occur when the brain and/or spinal cord is exposed at birth due.  During development, the neural tube fails to close properly at its cephalic (head) end, preventing the complete formation of brain and skull. Anencephaly is a type of open NTD. The majority of infants born with anencephaly are stillborn; an infant born alive is typically blind, deaf, unconscious, and unable to feel pain, dying within several hours to days of being born.

Nursing Interventions:

Prognosis is poor, and care should be focused on providing comfort measures for the infant. Parents and family will need support during this difficult time. Use of an infant cap may help the parents to feel more comfortable seeing and holding their child. Key nursing interventions will include assisting with anticipatory grieving and end-of-life care.

Sources:

[1] http://www.labtestsonline.org/understanding/conditions/neural_tube.html

[2] http://en.wikipedia.org/wiki/Neural_tube_defect

[3] http://www.chg.duke.edu/diseases/ntd.html

[4] Kyle, T. (2008). Essentials of pediatric nursing. Philadelphia PA: Lippincott Williams & Wilkins.