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目的探讨脊髓脊膜膨出的病理形态学特征、病理诊断标准和鉴别诊断。方法回顾分析14例脊髓脊膜膨出患者的临床资料、组织学特征并行免疫组化研究。结果患儿年龄1天～4岁,平均11.4个月,男女各7例。病变发生在胸、腰椎部位的2例、骶尾部12例。病理形态特征包括:病变中央部位的皮肤附件数量减少或缺如,真皮层内可见多量血管增生,病灶边界不清;14例均可见神经胶质组织、脊膜组织与胶原纤维混合形成的裂隙样结构,局部可见丰富的小神经束和平滑肌束;6例病变可见奇异形细胞和多核细胞散在分布。免疫组化:神经胶质组织GFAP(+);裂隙样结构内衬的细胞EMA、vimentin、D2-40和SMA(+),而CD31(-);奇异形细胞和多核细胞vimentin和SMA(+)。结论神经胶质组织和内衬脊膜细胞的裂隙样结构是脊髓脊膜膨出病理诊断依据,病灶中平滑肌束的出现对诊断有提示意义。奇异形细胞和多核细胞可能是退变的肌纤维母细胞。熟悉脊髓脊膜膨出的形态特征对病理诊断和鉴别诊断十分重要。
Objective To investigate the pathomorphological features, pathological diagnostic criteria and differential diagnosis of myelomeningocele. Methods The clinical data of 14 patients with myelomeningocele were retrospectively analyzed. The histological features were studied in parallel with immunohistochemistry. Results Children aged 1 day to 4 years, an average of 11.4 months, 7 men and women each. Lesions occurred in the chest, lumbar spine in 2 cases, 12 cases of sacrococcygeal. Pathological features include: the lesion in the central part of the reduction or absence of skin appendages, visible in the dermis a large number of vascular hyperplasia, the lesion border is unclear; 14 cases were seen glial, meningitis and collagen fibers form a fissure-like Structure, partially visible rich small nerve bundle and smooth muscle bundle; 6 cases of lesions can be seen in the scattered distribution of singular cells and polymorphonuclear cells. Immunohistochemistry: Glial GFAP (+); fission-like structures were lined with EMA, vimentin, D2-40 and SMA (+), while CD31 (-); singular and multinucleated cells vimentin and SMA ). Conclusions The fissure-like structures of glia and lining of meningococcal cells are the basis for the diagnosis of myelomeningocele. The presence of smooth muscle bundles in the lesion is suggestive of the diagnosis. Odontoblastic and multinucleated cells may be degenerative myofibroblasts. Familiar with the morphological features of spinal meningocele is very important for pathological diagnosis and differential diagnosis.