Department of Surgery, Division of Pediatric Surgery, University of CO Denver Anschutz Medical Campus, Aurora, CO, USA.
University of Colorado School of Medicine, Anschutz Medical Campus, Aurora, CO, USA.
Fetal Pediatr Pathol. 2024 May-Jun;43(3):225-233. doi: 10.1080/15513815.2024.2326834. Epub 2024 Apr 18.
Myelomeningocele or spina bifida is an open neural tube defect that is characterized by protrusion of the meninges and the spinal cord through a deformity in the vertebral arch and spinous process. Myelomeningocele of post-natal tissue is well described; however, pre-natal tissue of this defect has no known previous histologic characterization. We compared the histology of different forms of pre-natal myelomeningocele and post-natal myelomeningocele tissue obtained via prenatal intrauterine and postnatal surgical repairs. Pre-and post-natal tissues from spina bifida repair surgeries were obtained from lipomyelomeningocele, myeloschisis, and myelomeningocele spina bifida defects. Tissue samples were processed for H&E and immunohistochemical staining (KRT14 and p63) to assess epidermal and dermal development. Prenatal skin near the defect site develops with normal epidermal, dermal, and adnexal structures. Within the grossly cystic specimens, histology shows highly dense fibrous connective tissue with complete absence of a normal epidermal development with a lack of p63 and KRT14 expression. Tissues harvested from prenatal and postnatal spina bifida repair surgeries appear as normal skin near the defect site. However, cystic tissues consist of highly dense fibrous connective tissue with complete absence of normal epidermal development.
脊膜脊髓膨出或脊柱裂是一种开放性神经管缺陷,其特征是脑膜和脊髓通过椎弓和棘突的畸形突出。已充分描述了产后组织的脊膜脊髓膨出;然而,该缺陷的产前组织以前没有已知的组织学特征。我们比较了通过产前宫内和产后手术修复获得的不同形式的产前脊膜脊髓膨出和产后脊膜脊髓膨出组织的组织学。从脂肪脊膜脊髓膨出、脊髓脊膜膨出和脊膜脊髓裂脊柱裂缺陷的脊柱裂修复手术中获得了产前和产后组织。对组织样本进行 H&E 和免疫组织化学染色(KRT14 和 p63),以评估表皮和真皮的发育情况。 缺陷部位附近的产前皮肤发育正常,具有正常的表皮、真皮和附属结构。在大体囊性标本中,组织学显示出高度密集的纤维结缔组织,完全缺乏正常的表皮发育,缺乏 p63 和 KRT14 表达。 从产前和产后脊柱裂修复手术中采集的组织在缺陷部位附近表现为正常皮肤。然而,囊性组织由高度密集的纤维结缔组织组成,完全缺乏正常的表皮发育。