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Rubinstein-Taybi Syndrome is a rare condition affecting 1:125,000 children. It is associated with short broad radially deviated thumbs, secondary to a delta proximal phalanx of the thumb. We undertook a retrospective review of seven children (13 thumbs) with Rubinstein-Taybi syndrome whose thumbs were treated using a corrective osteotomy to the delta phalanx over a 13 year period. The types of osteotomy used in the series were reverse wedge osteotomy, opening wedge osteotomy and dome shaped osteotomy. The mean preoperative radial deviation of thumbs was 68 degrees (range 45-85 degrees ). At follow up five of the 13 thumbs demonstrated some residual radial deviation. All recurrences occurred in the dome shaped osteotomy group. Our data suggest that surgery is effective in correcting the deformity, but there is a risk of incomplete correction or recurrence. Despite the recurrence the mean postoperative deformity was significantly better than preoperatively and the majority of patients families subjectively reported good function. No patient in our series has yet undergone further corrective surgery.

Original publication

DOI

10.1177/1753193409354523

Type

Journal article

Journal

The Journal of hand surgery, European volume

Publication Date

05/2010

Volume

35

Pages

296 - 301

Addresses

Department of Plastic Surgery, Great Ormond Street Hospital for Sick Children, London, UK. AJainuk@aol.com

Keywords

Thumb, Humans, Rubinstein-Taybi Syndrome, Hand Deformities, Congenital, Hand Strength, Treatment Outcome, Osteotomy, Retrospective Studies, Cohort Studies, Recovery of Function, Child, Child, Preschool, Infant, Female, Male, Finger Phalanges