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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Hallermann-Streiff syndrome


Other Names for this Disease

  • Francois dyscephalic syndrome
  • Hallermann Streiff Francois syndrome
  • Hallermann Streiff syndrome
  • HSS
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Treatment

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How might Hallermann-Streiff syndrome be treated?

Treatment for Hallermann-Streiff syndrome depends on the specific signs and symptoms present in each affected individual. Early disease management for infants may include monitoring of breathing, consideration of tracheostomy, and various measures to improve feeding and ensure sufficient intake of nutrients. Although early surgical removal of cataracts may be recommended to help preserve vision, some studies have suggested that spontaneous cataract absorption may occur in up to 50% of untreated patients. Regular appointments with an ophthalmologist are strongly recommended to identify and treat other eye abnormalities, some of which may require surgical intervention.

With respect to dental anomalies, natal/neonatal teeth (teeth present at birth) may be incorrectly diagnosed as extra teeth and there may be a tendency to extract them. However, the loss of teeth may worsen glossoptosis (posteriorly location of the tongue) or cause other complications. It has thus been recommended to preserve prematurely erupting teeth to facilitate eating until the existence of successional permanent teeth can be confirmed. Ensuring good dental hygiene is also important.

Management of the condition may also include surgical reconstruction of certain craniofacial malformations (particularly the mandibular and nasal region) at the appropriate age. For some affected infants and children with heart defects, medical treatment and/or surgical intervention may be recommended.[1]
Last updated: 7/24/2012

References
  1. Hallermann Streiff Syndrome. NORD. April 11, 2012; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/498/viewAbstract. Accessed 7/23/2012.


Management Guidelines

  • Project OrphanAnesthesia is a project whose aim is to create peer-reviewed, readily accessible guidelines for patients with rare diseases and for the anesthesiologists caring for them. The project is a collaborative effort of the German Society of Anesthesiology and Intensive Care, Orphanet, the European Society of Pediatric Anesthesia, anesthetists and rare disease experts with the aim to contribute to patient safety.

Clinical Trials & Research for this Disease

  • The Centers for Mendelian Genomics program is working to discover the causes of rare genetic disorders. For more information about applying to the research study, please visit their website.
Other Names for this Disease
  • Francois dyscephalic syndrome
  • Hallermann Streiff Francois syndrome
  • Hallermann Streiff syndrome
  • HSS
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.