Monday, March 25, 2013

Hydranencephaly: Definition, Information, Diagnosis and Prognosis

Information by Global Hydranencephaly Foundation from Disabled World towards tomorrow

Definition causes and treatment of Hydranencephaly, a condition where the brain cerebral hemispheres are absent and replaced by sacs filled with cerebrospinal fluid.

Hydranencephaly, synonym hydroanencephaly (NORD, 2007), is one of the various cephalic classification types of disorders.

These disorders are congenital conditions that derive from either damage to, or abnormal development of, the fetal nervous system in the earliest stages of development in utero. Cephalic is the medical term for “head” or “head end of body.” These conditions do not have any definitive identifiable cause factor; instead generally attributed to a variety of hereditary or genetic conditions, but also by environmental factors such as maternal infection, pharmaceutical intake, or even exposure to high levels of radiation (NINDS, 2007). This condition possesses isolated occurrences, affecting less than 1 in 10,000 births worldwide (Kurtz & Johnson, 1999) and officially classifying hydranencephaly as a rare disorder by affecting fewer than 1 in 200,000 in the United States (Rare, 2011). Even rarer are cases of hemihydranencephaly, in which the effects of damage are unilateral and existing only on one hemisphere of the brain (Dubey, Gobinathan, et.al, 2002).

Many children live for an extended period prior to diagnosis due to no obvious symptomatic presentation at birth.

Though hydranencephaly is typically a congenital disorder, it can occur as a postnatal diagnosis in the aftermath of meningitis, intracerebral infarction, and ischemia (stroke), or other traumatic brain injury (Dubey, Gobinathan, et.al, 2002).


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