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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Hydrocephalus due to congenital stenosis of aqueduct of sylvius


Other Names for this Disease
  • Aqueductal stenosis, X-linked
  • Bickers-Adams syndrome
  • HSAS
  • HSAS1
  • HYCX
Related Diseases
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Treatment

Newline Maker

How might hydrocephalus due to congenital stenosis of aqueduct of sylvius be treated?

The treatment of hydrocephalus due to congenital stenosis of aqueduct of sylvius (HSAS) is based on the signs and symptoms present in each person. For example, hydrocephalus is typically treated with shunt surgery. Special education and early intervention may be recommended for children with intellectual disability. Although intervention is rarely necessary for adducted thumbs (bent towards the palms), tendon transfer surgery or splinting may be suggested in some cases.[1][2]
Last updated: 4/26/2015

References
  1. Stumpel C & Vos YJ. L1 syndrome. GeneReviews. March 5, 2015; http://www.ncbi.nlm.nih.gov/books/NBK1484/. Accessed 4/24/2015.
  2. L1 syndrome. NORD. August 2012; https://www.rarediseases.org/rare-disease-information/rare-diseases/byID/1097/viewAbstract.


Management Guidelines

  • GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions. Click on the link to view the article on this topic.

Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Hydrocephalus due to congenital stenosis of aqueduct of sylvius. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
Other Names for this Disease
  • Aqueductal stenosis, X-linked
  • Bickers-Adams syndrome
  • HSAS
  • HSAS1
  • HYCX
Related Diseases
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.