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Anesthetic management of a child with Langer-Giedion (TRPS II) syndrome

We describe the anesthetic and perioperative management of a child with Langer-Giedion syndrome (trichorhinophalangeal syndrome type II). This is a very rare genetic syndrome caused by 8q chromosome deletion. The clinical features of this syndrome include craniofacial and urogenital abnormities, var...

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Bibliographic Details
Published in:Journal of anesthesia 2009, Vol.23 (3), p.456-459
Main Authors: Michalek, Pavel, Doherty, John T., Vesela, Michaela Michalkova
Format: Article
Language:English
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Summary:We describe the anesthetic and perioperative management of a child with Langer-Giedion syndrome (trichorhinophalangeal syndrome type II). This is a very rare genetic syndrome caused by 8q chromosome deletion. The clinical features of this syndrome include craniofacial and urogenital abnormities, variable postnatal growth deficiency with mental retardation, multiple exostoses, hyperflexible joints, and recurrent respiratory tract infections. Potential perioperative problems are highlighted.
ISSN:0913-8668
1438-8359
DOI:10.1007/s00540-009-0779-7