Anesthetic management of a patient with Holt-Oram syndrome undergoing right radial head excision

Holt-Oram syndrome also known as heart and hand syndrome, first reported in 1960. It is a rare, inherited, an autosomal dominant disorder with mutation in TBX5. As the name suggests, the feature involves skeletal abnormality mainly involving upper limb, that is, upper-extremity malformations involvi...

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Bibliographic Details
Published in:Anesthesia: essays and researches 2014-01, Vol.8 (1), p.83-85
Main Authors: Shetti, Akshaya N., Dhulkhed, Vithal K., Panchgar, Vinayak, Prakash, Lokesh
Format: Article
Language:English
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Case Report
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Summary:Holt-Oram syndrome also known as heart and hand syndrome, first reported in 1960. It is a rare, inherited, an autosomal dominant disorder with mutation in TBX5. As the name suggests, the feature involves skeletal abnormality mainly involving upper limb, that is, upper-extremity malformations involving radial, thenar, or carpal bones; congenital heart diseases like, atrial-septal defect and ventricular septal defect and conduction problems. This syndrome may also involve other part of skeletal structure. We hereby report a case of a patient who was suffering from this syndrome scheduled for right radial head excision.
ISSN:0259-1162
2229-7685
DOI:10.4103/0259-1162.128917