Jain A, Rehman S, Smith G
Department of Plastic Surgery, Great Ormond Street Hospital for Sick Children, London, UK.
J Hand Surg Eur Vol. 2010 May;35(4):296-301. doi: 10.1177/1753193409354523. Epub 2009 Dec 23.
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.
鲁宾斯坦-泰比综合征是一种罕见疾病，发病率为1:125,000。它与短而宽且向桡侧偏斜的拇指有关，继发于拇指近端指骨呈三角形。我们对7例患有鲁宾斯坦-泰比综合征的儿童（13根拇指）进行了回顾性研究，这些儿童的拇指在13年期间接受了针对三角形指骨的矫正截骨术治疗。该系列中使用的截骨术类型包括反向楔形截骨术、开口楔形截骨术和圆顶形截骨术。术前拇指的平均桡侧偏斜度为68度（范围为45 - 85度）。随访时，13根拇指中有5根显示有一些残余的桡侧偏斜。所有复发均发生在圆顶形截骨术组。我们的数据表明，手术在矫正畸形方面是有效的，但存在矫正不完全或复发的风险。尽管有复发情况，但术后平均畸形程度明显优于术前，大多数患者家属主观报告功能良好。我们系列中的患者均未接受进一步的矫正手术。
