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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Hanhart syndrome


Other Names for this Disease
  • Aglossia adactylia
  • Hypoglossia-hypodactylia syndrome
  • Peromelia with micrognathia
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Overview



What is Hanhart syndrome?

What are the signs and symptoms of Hanhart syndrome?


What is Hanhart syndrome?

Hanhart syndrome is a rare birth defect in which the main signs are a short, incompletely developed tongue (hypoglossia); absent or partially missing fingers and/or toes (hypodactylia); malformed arms and/or legs (peromelia); and an extremely small jaw (micrognathia).The severity of these physical abnormalities varies greatly among individuals, and children with this disorder often have some, but not all, of the symptoms. The cause of Hanhart syndrome is not fully understood.[1] It appears to occur sporadically,[2] being unlikely to recur in a family. Treatment may involve orthopedic and/or plastic surgery for limb abnormalities.[3]
Last updated: 3/23/2011

What are the signs and symptoms of Hanhart syndrome?

The main features of Hanhart syndrome include a short, incompletely developed tongue (hypoglossia); absent or partially missing fingers and/or toes (hypodactylia); malformed arms and/or legs (peromelia); and an extremely small jaw (micrognathia).[1] Retrognathia (receding jaw) is also a common feature of Hanhart syndrome. Other features that have been reported include hypodontia (missing teeth) and the absence of major salivary glands.[2] The limb abnormalities in individuals with Hanhart syndrome have ranged from shortened fingers or toes and oligodactyly (missing fingers or toes) to more severe abnormalities such as absent or malformed limbs. In an affected individual, any limb may be affected.[2] The severity of the physical abnormalities associated with Hanhart syndrome varies greatly among affected individuals, and children with this disorder often have some, but not all, of the symptoms.[1]
Last updated: 3/23/2011

References
  1. Hanhart syndrome. NORD. 2004; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Hanhart%20Syndrome. Accessed 3/22/2011.
  2. Raoul Hennekam, Judith Allanson, Ian Krantz. Gorlin's Syndromes of the Head and Neck. USA: Oxford University Press; February 5, 2010;
  3. E. Robert-Gnansia. Hypoglossia - hypodactyly. Orphanet. July 2005; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=989. Accessed 3/22/2011.