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[上肢主要神经的纤维脂肪瘤样肥大]

[The fibrolipomatous hypertrophy of the main nerves of the upper extremity].

作者信息

Narakas A

出版信息

Handchirurgie. 1976;8(2):89-95.

PMID:186366
Abstract

10 cases of lipofibromatous hypertrophy of the main nerves in the upper limb in 7 female and 3 male patients are described. In 1 case at the level of the middle third of the upper arm and in 6 cases at the level of the wrist and neighboring areas. In 2 patients these pathological changes were found in the ulnar nerve, in 1 case the hypertrophy was limited to the upper arm, in the other it extended all along the extremity from below the axilla to the wrist. Finally, in 1 case a 20 cm long hypertrophy was found at the level of the deep branch of the radial nerve (posterior interosseus nerve) in the lower arm below the elbow. The fibrous and lipomatous tissue snugly surrounds the fascicles and cannot be separated from them without damaging them, even if the finest microsurgical techniques are used. As the disease has been diagnosed in children as young as five years a congenital etiology has to be considered. The condition is distinct though from the disease of DEJERINE-SOTTAS or from that of von RECKLINGHAUSEN. The hypertrophic nerve is progressively submitted to a chronic compression in the naturally narrow passages (carpal tunnel, ulnar grove at the elbow, arcade of FROHSE) or it becomes painful and paretic after even minor trauma which leads probably to local oedema, intraneural bleeding and epineural compression. An external and internal decompression has to be carried out. Resection or defatting of the hypertrophic fascicles, however, should be avoided. In 2 of our cases the disease was anamnestically present for 45-50 years and clinically followed in another case for 18 years. The disease does not seem progressive. These cases show, that this disorder, which first appeared in the medical literature in 1964 and have been described by several authors in the median nerve, exists as well in the two other main nerves of the upper extremity.

摘要

本文描述了10例上肢主要神经的脂肪纤维瘤性肥大病例,其中7例为女性,3例为男性。1例位于上臂中1/3水平,6例位于腕部及邻近区域。2例患者的尺神经出现这些病理改变,1例肥大局限于上臂,另1例则从腋窝以下一直延伸至腕部。最后,1例在肘部以下的前臂桡神经深支(骨间后神经)水平发现了20厘米长的肥大。纤维和脂肪组织紧密包裹着神经束,即使使用最精细的显微外科技术,也无法在不损伤神经束的情况下将其分离。由于该疾病在年仅5岁的儿童中就已被诊断出来,因此必须考虑先天性病因。不过,这种情况与德热里纳 - 索塔斯病或冯·雷克林豪森病不同。肥大的神经在自然狭窄通道(腕管、肘部尺神经沟、弗罗伊泽弓)中逐渐受到慢性压迫,或者即使受到轻微创伤后也会变得疼痛和麻痹,这可能导致局部水肿、神经内出血和神经外膜受压。必须进行内外减压。然而,应避免切除或去除肥大的神经束。在我们的2例病例中,该疾病在既往史中已存在45 - 50年,另一例临床随访了18年。该疾病似乎不会进展。这些病例表明,这种疾病于1964年首次出现在医学文献中,已有多位作者对正中神经的情况进行过描述,在上肢的另外两条主要神经中也存在。

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