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Anencephaly is the absence of a major portion of the brain, skull, and scalp that occurs during embryonic development. [1] 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. [ 2 ]
As of 2008, treatments of NTDs depends on the severity of the complication. No treatment is available for anencephaly and infants usually do not survive more than a few hours. Aggressive surgical management has improved survival and the functions of infants with spina bifida, meningoceles and mild myelomeningoceles. [66]
The fetus in this case had developed rachischisis without any indications of acrania. This is not typical of most cases, so this treatment would not be possible in the majority of cases of the condition. [citation needed] Due to the lack of treatments available for rachischisis, there is a focus on prevention of the disease.
Little genetic counseling can be offered for acrania because the genetic origins are not fully understood. In order to make genetic counseling for families easier this disease is often differentially diagnosed with other diseases that can occur at the same time such as anencephaly and acalvaria, though these diseases may not always occur simultaneously. [1]
The skull does not close all the way in this deformity (especially at the occipital region). As a result, the amniotic fluid enters the brain, leading to eventual brain degeneration and anencephaly. [1] Craniorachischisis is on the extreme end of the dysraphism spectrum, wherein the entire length of the neural tube fails to close. [2]
Diprosopus often occurs in combination with other congenital disorders, particularly anencephaly, neural tube defect and cardiac malformations. [14] When present, the brain may show abnormalities ranging from partial to complete duplication of brain structures, and/or underdevelopment of brain tissues.
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As the irreversibility of anencephaly is widely understood in the medical community, he argued that the decision to continue futile care only resulted in the repetitive diversion of medical equipment. [6] Keene's cardiac death occurred on April 5, 1995, at Fairfax Hospital, at age of 2 years 174 days. [1]