Carpal tunnel syndrome in the mucopolysaccharidoses and mucolipidoses
Carpal tunnel syndrome in the mucopolysaccharidoses and mucolipidoses
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DOI：
10.1302/0301-620x.79b4.7547
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发表时间：
1997-07-01
影响因子：
--
通讯作者：
Pitt, MC
中科院分区：
文献类型：
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作者：
Haddad, FS;Jones, DHA;Pitt, MC
患有粘多糖沉积症或粘脂沉积症的儿童会出现手部进行性残疾，特别是与正中神经功能障碍有关，这是一个日益严重的问题，因为骨髓移植显着提高了生存率，而没有明显改变肌肉骨骼表现，我们回顾了 48 名患有这些综合征的儿童，他们需要腕管减压，记录症状、体征、放射学、电生理学和手术结果、组织学和上肢功能，在这些儿童中，腕管综合征与成人不同，症状很少见，但出汗减少、牙髓萎缩、鱼际消耗和手法笨拙等体征更为常见。手术时，屈肌支持带增厚，大量白色腱鞘吞没屈肌腱，大多数患者有一些明确的神经收缩，神经外膜增厚。早期减压后可见功能改善，同步肌腱松解有一定益处，定期物理治疗有助于维持增加的手部运动。我们描述了我们的评估方案、物理治疗和手术方案以及标准功能审查，有助于最大限度地发挥这些儿童的手和上肢的功能。
Children with a mucopolysaccharidosis or mucolipidosis suffer progressive disability of the hands, particularly in relation to dysfunction of the median nerve, This is an increasing problem because bone-marrow transplantation has dramatically improved survival without apparently changing the musculoskeletal manifestations, We have reviewed 48 children with these syndromes who required carpal tunnel decompression, recording symptoms, signs, radiological, electrophysiological and operative findings, histology and upper-limb function, In these children the carpal tunnel syndrome differs from that seen in adults, Symptoms are rare but signs such as decreased sweating, pulp atrophy, thenar wasting and manual clumsiness are much more common, At operation, the flexor retinaculum was thickened and a mass of white tenosynovium engulfed the flexor tendons, Most patients had some definite nerve constriction with a thickened epineurium.Functional improvement was seen after early decompression, with some benefit from simultaneous tendon release, Regular physiotherapy helped to maintain increased hand movement.We describe our assessment protocol, the physiotherapy and operative regime and the standard functional review which helps to maximise function in the hands and upper limbs of these children.