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Malformation characterized by cerebral anomalies and a
median skull gap originating from the nasal root, orbits, or forehead
(sincipital encephalocele), or the skull base or occiput (occipital
encephalocele), allowing cerebrospinal fluid and/or brain to herniate. The
encephalocele itself is defined as a herniation of part of the cranial
contents through this skull defect. It may contain meninges (meningocele),
meninges and brain (meningoencephalocele), or meninges, brain, and ventricle
(meningoencephalocystocele).
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Cephalocele; Craniocele; Cranial Meningoencephalocele.
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Defect of neural tube development resulting in a
osseous defect in the midline of the skull that allows herniation of the
meninges and portions of the brain.
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1:5000-10,000 live births. No sexual predilection.
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Multifactorial inheritance pattern.
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Dysraphism of the skull with a defect that may
occur anywhere from the nasal cavity to the foramen magnum. Herniation of
cerebral structures with (encephalocystocele) or without ventricular
components (cenencephalocele) are distinguished. If meningeal structures are
involved in the herniation, the terms encephalomenigocele and
meningoencephalocele have been used. Another classification distinguishes
just between meningocele or encephalocele. The herniated brain is often
abnormal, and there are frequently associated structural and functional
abnormalities that also affect the nonherniated brain areas.
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Based on the obvious clinical findings and confirmation
by computed tomography scanning or magnetic resonance imaging. In utero
diagnosis can be made by ultrasound, elevated maternal (and patient)
alpha-fetoprotein, and amniocentesis.
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The clinical picture depends on the type of
encephalocele:
Sincipital encephaloceles (25% of all encephaloceles) most often originate from the orbits, nose,
or forehead and occur most commonly in Asians. (1) Anterior sincipital encephaloceles present as a mass in the
nasopharynx. They are often diagnosed late and may present because of
persistent nasal discharge or recurrent meningitis. (2) Basal sincipital encephaloceles may extend into the
upper pharynx and present with feeding difficulties and/or upper airway
obstruction. The content of the encephalocele may include hypothalamus or
pituitary gland, which may result in neuroendocrine disturbances and
preclude resection. The associated mortality is high.
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Notencephaloceles (75% of all encephaloceles) extend from the occipital region aspect of
the skull. Portions of ...