824 Congenital Hand Deformities and Associated Syndromes

Abstract

Background: Congenital Hand Deformity is an upper limb abnormality which present at the time of birth. Only 5% of congenital hand deformities occur as part of a recognized syndrome. Objective: To retrospectively review all congenital hand deformities treated in Children University Hospital from January 2003 to October 2009 and correlate with syndromes associated. Methods: Getting the list of hand surgeries from theatre. Retrieve charts from Chart Library. Compiled and enter data in Microsoft Excel. Results: 20% patients have an associated syndrome. 29.3% presented with polydactyly. 7 of them are associated with syndromes. 59 out of 317 has syndactyly and 15% are associated with Apert Syndrome. 70 out of 317 are associated with radial ray abnormality and 24%has a syndromic background. None of the radial ray abnormality are associated with fanconi anaemia. 45 out of 317 presented with multiple ulnar deformities and 3 are associated with syndromes. 38 out of 317 presented with symbrachydactyly and 21 % are associated with various syndrome. 10 out of 317 are associated with multiple hand deformity and mostly they are associated with amniotic band syndrome. There are 2 patients that present with macrodactyly, however they are no medical association noted. Discussion: In conclusion, 19.9% of the patients has associated medical background. None of them has proven Fanconi Anaemia manifestation. Fanconi Anaemia is commonly associated with radial aplasia. Hence in the future, we will retrospectively screen all patient that has radial ray abnormality and prospectively screen them for fanconi anemia screening test.