儿童脊髓脊膜膨出115例病理学特征

Pathological features of myelomeningocele:115 children cases

目的探讨儿童脊髓脊膜膨出的病理学形态特征及病理诊断。方法 2010年9月至2016年10月期间在河北省儿童医院神经外科手术治疗的115例患儿脊髓脊膜膨出的病理标本,应用组织学及免疫组化En Vision两法分析其病理特点。结果 115例脊髓脊膜膨出患儿年龄6h～11岁,男性63例,女性52例,男:女为1.2:1,平均年龄(12±4)个月,病变发生在颈部9例,胸背部16例,腰骶部90例。大体病理检查,所有病例均为带皮软组织,皮面见隆起,82例切面囊实性,囊壁光滑,壁厚薄不均;余33例切面实性,局部见半透明胶样区。苏木精-伊红染色光镜检查:皮下纤维组织增生,附属器减少且分布不均,115例均可见神经胶质成分、脊膜成分与胶原纤维混杂分布形成不规则裂隙样结构,局部可见粗大神经纤维及少量平滑肌束;免疫组织化学光镜检查:神经胶质成分GFAP(+),脊膜成分与胶原纤维混杂分布形成裂隙样结构内衬细胞EMA、Vimentin、D2-40及SMA均(+),CD31(-)。结论神经胶质成分及含脊膜成分的裂隙样结构是诊断脊髓脊膜膨出的病理依据。

Objective To investigate the pathological features, histological diagnosis and differential diagnosis of myelomeningocele. Method One hundred and fifteen patients with myelomeningocele after neurosurgery removal of the pathologic specimens, the Children＇s Hospital of Hebei from September 2010 to October 2016, the histological and immunohistochemical En Vision two step analysis its pathological features. Results The one hundred and fifteen patients were aged 6 hous to 11 years, boys were sixty-three and girls were fifty-two, boys, girls ratio of 1.2：1, with mean age as 12.4 months. The site of occurrence included the neck nine cases, the thoracodorsal sixteen cases and lumbosacral ninety cases. General pathological examination： all cases were with skin soft tissue, uplift in all cases, the section were solid-cystic in eighty-two cases, capsule wall were smooth and the wall were uneven. It was with a solid section in the rest of the thirty-three cases and localized traslucent epoxy. HE staining light microscope examined： the pathological characteristics included fibrous tissue hyperplasiaed in the subcutaneous, and diminished and distributed uneven dermal appendages of the skin overlying the lesion. The neuroglia components and the fissure structure, formed by the spinal menings and the collagen, were observed in all one hundred and fifteen cases lesions. Moreover, it was observedbulky nerve fibers and a small amount of smooth muscle bundles. Immunohistochemically light microscope examined：The neuroglial components were positive for GFAP. The fissure structure, formed by the spinal menings and the collagen were positive for EMA, Vimentin, D2-40 and SMA, but negative for CD31. Conclusion The glial components and the fissure structure, formed by the spinal menings and the collagen, are the hallmark structures for the diagnosis of myelomeningocele.