Encephalocele overview?
Encephalocele is a rare disorder in which an infant is born with a gap in the skull; that is, a part of one or more of the plates that form the skull does not seal. The membranes that cover the brain (meninges), and brain tissue, protrude through this gap. It is likely thatthis disorder is caused by the failure of the neural tube to close during development of the fetus.
An encephalocele is caused by a defect in the fusion of the cranial bone. This occurs in the midline, and is most common in the occipital region. Encephalocele is an extension of intracranial contents (meninges and brain tissue) outside the normal confines of the skull.
Synonyms of encephalocele?
- Bifid Cranium
- Cephalocele
- Cranial Meningoencephalocele
- Craniocele
- Cranium Bifidu
What is the treatment for encephaloceles?
Surgical excision of the sac and its contents with a water tight closure is the treatment of choice. The brain substance within the cavity is abnormal and resection of it will not lead to any additional neurological signs. However, normal vascular structures may protrude into the sac and should not be removed. Hydrocephalus is often present and may need to be treated separately.
How are encephaloceles diagnosed?
Encephaloceles that protrude to the surface are usually diagnosed at birth by visual inspection. However, diagnosis often may be delayed if the encephalocele is not clearly visible at birth. This is particularly true of basal or fronto-ethmoidal encephaloceles that may be discovered because of a nasal mass, nasal congestion or snoring, wide spaced eyes, or a facial abnormality. These children are often neurologically normal and have a normal physical exam. CT and MRI scan can be used to identify encephaloceles.
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