Progressive nerve territory overgrowth after subtotal resection of lipomatosis of the median nerve in the palm and wrist: a case, a review and a paradigm
Progressive nerve territory overgrowth after subtotal resection of lipomatosis of the median nerve in the palm and wrist: a case, a review and a paradigm
复制标题
手掌和腕部正中神经脂肪瘤次全切除后进行性神经区域过度生长：病例、回顾和范例
作者：
M. Mahan;K. Amrami;B. Niederhauser;R. Spinner
背景神经脂肪瘤病（Lipomatosis of the nerve，LN）是一种罕见的以周围神经大量增大为特征的疾病，常伴有全身性纤维脂肪增生和骨骼过度生长。这种疾病的治疗仍然存在争议，部分原因是罕见和表现的可变性。一些作者主张完全切除这种良性病变，包括功能性神经，而另一些作者则建议单独进行症状性减压。方法我们一直在定期跟踪一名10岁男孩的手腕正中神经脂肪过多症，出生后不久就发现了这种情况。他接受正中神经切除术，并在另一个研究所进行腓肠神经移植。我们回顾了有关LN的文献和神经移植术的疗效。结果临床上，他恢复良好，鱼际功能轻度丧失，感觉相对保留。5年的连续磁共振成像显示两个接合部位的LN进展，神经移植物内以及远端指神经内的纤维脂肪增生，以及接合部位的纤维瘢痕扩大。这从来没有被报道在9个十年的研究，这种diseases.ConclusionWe目前的第一个中期随访的病人谁经历了神经牺牲，试图治愈LN的治疗的历史回顾。我们认为，宏观大体全切除术（即，显微镜下次全切除术）不足以阻止这种错构瘤性病变的潜在进展，因为营养因子的持续作用。
BackgroundLipomatosis of the nerve (LN) is a rare disorder characterized by the massive enlargement of peripheral nerves, frequently accompanied by generalized fibroadipose proliferation and skeletal overgrowth. The treatment of this disorder remains controversial, in part because of the rarity and the variability of presentation. Some authors have advocated total resection of this benign lesion including the functioning nerve, while others recommend symptomatic decompression alone.MethodsWe have been routinely following a 10-year-old boy for lipomatosis of the median nerve at the wrist noted shortly after birth. He underwent median nerve resection accompanied by sural nerve grafting at another institute. We review the literature on LN and the efficacy of nerve grafting.ResultsClinically, he made a good recovery, with mild loss of thenar function and relatively preserved sensation. Serial magnetic resonance imaging over 5 years has revealed progression of the LN at both coaptation sites, fibrofatty proliferation within the nerve grafts as well as distal digital nerves, and enlargement of a fibrous scar at the coaptation sites. This has never been reported in the 9 decades of study of this disease.ConclusionWe present the first medium-term follow-up of a patient who underwent nerve sacrifice to attempt to cure the LN alongside a historical review of treatment. We believe that macroscopic gross total resection (i.e., microscopic subtotal resection) is insufficient in stopping the potential progression of this hamartomatous lesion because of the persistent effect of trophic factors.