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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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Encephalocele
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Sincipital encephalocele in a 6-year-old boy.

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Encephalocele
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Huge occipital meningoencephalocystocele in an infant.

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Encephalocele
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Three-dimensional MRI reconstruction of the head of a patient with a large occipital encephalocele revealing the impressive underlying bone lesion associated with the encephalocele.

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Encephalocele
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Transilluminated nuchal encephalocele in a newborn baby positioned in the prone position for surgery.

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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 ...

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