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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Hydranencephaly


Other Names for this Disease

  • Hydroanencephaly
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Overview

Hydranencephaly is a rare condition in which the brain's cerebral hemispheres are absent and replaced by sacs filled with cerebrospinal fluid (CSF). Affected infants may appear and act normal at birth, but irritability and hypertonia often develop within a few weeks. Other signs and symptoms may include seizures, hydrocephalus, visual impairment, lack of growth, deafness, blindness, paralysis, and intellectual disabilities. Prognosis is typically poor with many affected children dying before one year of age. In rare cases, children may survive for several years or more.[1] It has been suspected to be an inherited condition, although some researchers believe it may be caused by prenatal blockage of the carotid artery where it enters the cranium.[2] Treatment is generally symptomatic and supportive; hydrocephalus may be treated with a shunt.[1]
Last updated: 4/11/2012

References

  1. NINDS Hydranencephaly Information Page. NINDS. February 13, 2007; http://www.ninds.nih.gov/disorders/hydranencephaly/hydranencephaly.htm. Accessed 4/11/2012.
  2. Hydranencephaly. NORD. August 7, 2007; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/369/viewAbstract. Accessed 4/11/2012.
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Basic Information

In Depth Information

  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Hydranencephaly. Click on the link to view a sample search on this topic.

Insurance Issues

Other Names for this Disease
  • Hydroanencephaly
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.