Vetrano Ignazio G, Sconfienza Luca Maria, Albano Domenico, Chianca Vito, Nazzi Vittoria
Dipartimento di Neurochirurgia, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Unità Operativa di Radiologia Diagnostica ed Interventistica, IRCCS Istituto Ortopedico Galeazzi, Via Riccardo Galeazzi 4, 20161, Milano, Italy.
Skeletal Radiol. 2019 Jun;48(6):989-993. doi: 10.1007/s00256-018-3098-y. Epub 2018 Oct 20.
Macrodystrophia lipomatosa, a hamartomatous enlargement of soft tissues leading to gigantism of a part or a whole extremity, generally affects the territory of distribution of a single nerve. In some cases, this condition may cause an entrapment neuropathy. We report the clinical, radiological, and surgical findings of a patient with isolated non-syndromic macrodactyly and giant median nerve presenting recurrent carpal tunnel syndrome (CTS). In this case, conventional magnetic resonance imaging (MRI) and diffusion tensor imaging (DTI) supported the presence of fibrohamartomatous infiltration, determining an enlargement of the median nerve and consequently an increased susceptibility to compression damage. A 57-year-old man presenting macrodactyly of the second and third finger of the right hand showed recent onset of severe hypoesthesia of the first three fingers of the right hand. He also underwent surgery for right CTS 23 years before. The electromyography/electroneurography confirmed the diagnosis of CTS. The ultrasonography showed a massive enlargement of the median nerve within the carpal tunnel, while MRI confirmed the enlargement of the median nerve with thickened hypointense bundles and interposed tissue, with increased mean diffusivity and decreased fractional anisotropy at DTI. We discuss about the use of a relatively novel imaging technique, investigating for the first time an uncommon cause of a very common entrapment neuropathy.
巨指脂肪瘤病是一种软组织错构瘤样增大，可导致部分或整个肢体的巨大畸形，通常影响单一神经的分布区域。在某些情况下，这种情况可能导致卡压性神经病变。我们报告了一例孤立性非综合征性巨指症和巨大正中神经伴复发性腕管综合征（CTS）患者的临床、放射学和手术结果。在该病例中，传统磁共振成像（MRI）和扩散张量成像（DTI）支持纤维错构瘤样浸润的存在，确定了正中神经增粗，因此增加了对压迫损伤的易感性。一名57岁男性，右手第二和第三指出现巨指症，近期右手前三指出现严重感觉减退。他23年前还接受过右侧CTS手术。肌电图/神经电图确诊为CTS。超声检查显示腕管内正中神经明显增粗，而MRI证实正中神经增粗，低信号束增粗且有插入组织，DTI显示平均扩散率增加，各向异性分数降低。我们讨论了一种相对新颖的成像技术的应用，首次研究了一种非常常见的卡压性神经病变的罕见病因。
