Management of complicated syndactyly in Apert’s syndrome: our experience
MK Chira, JN Kortor
Abstract
MK Chira, JN Kortor
Abstract
Background: Management of hand deformities in Apert’s acro-cephalo-syndactyly usually poses a great challenge to the reconstructive hand surgeon due to grossly abnormal anatomy of skeletal and soft tissuestructures associated with a progressive disease process.Patients and methods: We retrospectively reviewed all the patients with Apert’s syndactyly treated in our institution over a four-year period from January 2004 to December 2007.Results: Four patients presented in our centre with Apert’s syndactyly for treatment. There were two boys and two girls. There was no sex predilection. All the cases were sporadic and had complex bilateralsyndactyly. Between the four patients, a total of 24 web spaces were released and only one web space required revision surgery (4.2%).Conclusion: Local flaps and full thickness skin grafts were adequate for soft tissue cover in Apert’s syndactyly. With careful planning and flap designs, four digit hand (three fingers and opposable thumb) wasachievable in all the patients resulting in good functional and aesthetic outcome.Key words: Acrocephalosyndactyly, Apert’s syndrome, brachy-symphalangism,clinodactyly.
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Background: Management of hand deformities in Apert’s acro-cephalo-syndactyly usually poses a great challenge to the reconstructive hand surgeon due to grossly abnormal anatomy of skeletal and soft tissuestructures associated with a progressive disease process.Patients and methods: We retrospectively reviewed all the patients with Apert’s syndactyly treated in our institution over a four-year period from January 2004 to December 2007.Results: Four patients presented in our centre with Apert’s syndactyly for treatment. There were two boys and two girls. There was no sex predilection. All the cases were sporadic and had complex bilateralsyndactyly. Between the four patients, a total of 24 web spaces were released and only one web space required revision surgery (4.2%).Conclusion: Local flaps and full thickness skin grafts were adequate for soft tissue cover in Apert’s syndactyly. With careful planning and flap designs, four digit hand (three fingers and opposable thumb) wasachievable in all the patients resulting in good functional and aesthetic outcome.Key words: Acrocephalosyndactyly, Apert’s syndrome, brachy-symphalangism,clinodactyly.
Key concepts: Syndactyly, Apert syndrome, Medicine, Surgery, Dysostosis, Thumb, Soft tissue, Anatomy