Division of Orthopaedic Surgery, Oslo University Hospital.
Institute of Clinical Medicine, University of Oslo, Oslo, Norway.
J Pediatr Orthop. 2021;41(5):312-318. doi: 10.1097/BPO.0000000000001785.
The complex syndactyly in Apert syndrome hands is challenging to operate. The synostosis and tightness of skin between third and fourth digits lead to severe coverage problems during ray release. A soft tissue distractor can simplify the release with the aim to keep all 10 fingers.
A retrospective follow-up of 12 patients/24 hands, median age 8 years (6 to 17 y), 6 boys and 6 girls, operated between 2000 and 2013 was done from 2015 to 2016. The surgical management started with syndactyly release of the first and fourth web, and later of the second. The third stage was placing a soft tissue distractor on the third and fourth finger after osteotomy on the synostosis between them. Four weeks of distraction and 2 weeks of rest resulted in regenerated skin between the digits giving much better coverage of the released digits at time of separation 6 weeks later. Assessment of hand function, grip strength and completion of the Patient Reported Outcome Measure CHEQ was performed.
Soft tissue coverage at the time of digit separation was considerably facilitated. We experienced 2 infections in 2 hands. In 18/24 hands median 2 (1 to 3) small full thickness skin grafts were needed, usually for coverage of the base of the digits. All wounds healed well. The children managed different practical tasks well, alternating between best functioning grip depending on the activity. According to CHEQ, the children did median 19 (13 to 27) activities independently and median 8 (2 to 15) nonindependently, of a total of 29. Peak strength values for 10/12 children were for the right hand median 17.8% (9.6% to 40.6%) of normative data and for left hand median 13.6% (2.4% to 20.5%) of normative data.
Soft tissue distraction facilitates the treatment of acrocephalosyndactyly hands, giving 5-fingered hands. Apert children manage many activities independently but struggled with fine motor skills demanding strength.
Level IV evidence.
Apert 综合征手的复杂并指畸形手术难度大。第三和第四指之间的骨融合和皮肤紧绷导致在射线释放时严重的覆盖问题。软组织牵开器可简化释放过程，目的是保留所有 10 个手指。
对 2000 年至 2013 年期间接受手术的 12 名患者/24 只手（中位年龄 8 岁，6 至 17 岁；男 6 例，女 6 例）进行回顾性随访。手术治疗始于第一和第四蹼的并指松解，然后是第二蹼。第三阶段是在第三和第四指之间的骨融合处切开后，在第三和第四指上放置软组织牵开器。4 周的牵伸和 2 周的休息导致手指之间再生的皮肤，在 6 周后手指分离时提供了更好的覆盖。评估手部功能、握力和患者报告的 CHEQ 结果测量。
手指分离时的软组织覆盖得到了极大的改善。我们在 2 只手中发生了 2 例感染。24 只手中的 18 只需要中位数为 2（1 至 3）个小全厚皮片，通常用于覆盖手指基部。所有伤口均愈合良好。孩子们能够很好地完成不同的实际任务，根据活动交替使用最佳功能握持。根据 CHEQ，孩子们独立完成了中位数为 19（13 至 27）项活动，非独立完成了中位数为 8（2 至 15）项活动，总共 29 项。12 名儿童中有 10 名的右手峰值力量值中位数为正常值的 17.8%（9.6%至 40.6%），左手峰值力量值中位数为正常值的 13.6%（2.4%至 20.5%）。
软组织牵开术有助于治疗颅缝早闭并指畸形，提供 5 指手。Apert 综合征儿童能够独立完成许多活动，但在需要力量的精细运动技能方面存在困难。
IV 级证据。
