Campiglio G L, Pajardi G, Rafanelli G
Ann Chir Main Memb Super. 1997;16(2):91-100; discussion 101. doi: 10.1016/s0753-9053(97)80025-7.
The recessive form of dystrophic epidermolysis bullosa creates severe hand deformities with disabling functional limitations in the main daily activities. Typically, the thumb is contracted in adduction, the first web space is obliterated, the palm and digits are contracted in flexion and interdigital spaces are lost (pseudo-syndactyly). In this paper, we present our experience with a protocol based on the association of various internationally developed techniques: brachial plexus anesthesia with ketamine sedation, dynamic splinting and coverage of the wounds with allogenic keratinocytes sheets. The overall results obtained in the first 13 patients showed a good tolerance of the procedure, no anesthesiologic complication and marked improvement of the hand deformities. The long-term follow-up revealed a recurrence before 2 years in 2 hands, between 2 and 4 years in 7 hands and after 4 years in 6 hands. The conclusion is that an aggressive surgical attitude, along with an adequate intra and post-operative rehabilitation, ensures a good restoration of hand function and a satisfying delay of inevitable recurrence.
营养不良性大疱性表皮松解症的隐性形式会导致严重的手部畸形，在主要日常活动中功能受限，致残严重。典型表现为拇指内收挛缩，第一掌指关节间隙消失，手掌和手指屈曲挛缩，指间间隙丧失（假性并指）。在本文中，我们介绍了我们采用的一种方案的经验，该方案基于多种国际上开发的技术的联合应用：氯胺酮镇静下的臂丛神经麻醉、动态夹板固定以及同种异体角质形成细胞片覆盖伤口。前13例患者的总体结果显示，该手术耐受性良好，无麻醉并发症，手部畸形明显改善。长期随访发现，2只手在2年之前复发，7只手在2至4年之间复发，6只手在4年之后复发。结论是，积极的手术态度以及充分的术中和术后康复，可确保手部功能良好恢复，并令人满意地延迟不可避免的复发。
