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Genetic and Rare Diseases Information Center (GARD)

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Meningoencephalocele


Other Names for this Disease
  • Encephalomeningocele
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Overview


Meningoencephalocele is a type of encephalocele characterized by the protrusion of both meninges (membranes that cover the brain and spinal cord) and brain tissue through a defect in the skull. There are two main types of meningoencephalocele, which are named according to the location of the skull defect. The more common frontoethmoidal type is located at the frontal and ethmoid bones while the occipital type is located at the occipital boneHydrocephalus, abnormalities of the eyeball and lacrimal duct (tear duct) and other findings have been associated with the condition.[1][2] Some affected individuals have intellectual disabilities while others have normal psychomotor development.[3] The condition is typically congenital (present at birth) but rarely has been reported to occur spontaneously in older individuals.[4] The underlying cause of the condition is uncertain, but environmental factors are thought to play a role. Treatment depends on the size, location and severity of the defect but mainly includes surgery to repair the defect.[1]
Last updated: 9/21/2011

References

  1. Sitthiporn Agthong and Viroj Wiwanitkit. Encephalomeningocele cases over 10 years in Thailand: a case series. BMC Neurology. 2002; 2:http://www.ncbi.nlm.nih.gov/pmc/articles/PMC113760/?tool=pubmed. Accessed 9/19/2011.
  2. Kiymaz N, Yilmaz N, Demir I, Keskin S. Prognostic Factors in Patients with Occipital Encephalocele. Pediatric neurosurgery 2010. 2010; 46(1):6-11.
  3. Meling TR, Due-Tønnessen BJ, Helseth E, Skjelbred P, Arctander K. [Frontoethmoidal meningoencephaloceles]. Tidsskr Nor Laegeforen. August 20, 2000; 120(19):2250-2252.
  4. Nahas Z. Spontaneous meningoencephalocele of the temporal bone: clinical spectrum and presentation. Arch Otolaryngol Head Neck Surg. May 1, 2008; 134(5):509-518.
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In Depth Information

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