Encinas Hernández Jose Luis, Soto C, García-Cabezas M A, Pederiva F, Garriboli M, Rodríguez R, Peiró J L, Carceller F, López-Santamaría M, Tovar J A
Department of Pediatric Surgery, Hospital Universitario La Paz, Paseo de la Castellana 261, 28046, Madrid, Spain.
Pediatr Surg Int. 2008 Dec;24(12):1335-40. doi: 10.1007/s00383-008-2276-8.
To examine if brain malformations, similar to those which account for cognitive disorders seen in human disease, are present in an ovine model of myelomeningocele (MMC).
An MMC-like lesion was surgically created in 16 fetal lambs between 60 and 80 days of gestation. Ten did not undergo fetal repair (group A), 2 were repaired with an open two-layer closure (group B), 2 with open bioglue coverage (group C) and 2 with fetoscopic coverage (group D). Lambs were killed and their brains were examined. Two brains from normal unoperated lambs served as controls.
Thirteen lambs died in utero (81%). Two lambs in group A and 1 in group B were delivered at term. Group A brains showed hydrocephalus and extensive areas of polymicrogyria. There was also an extensive denudation of the ependymal lining under the polymicrogyric areas and the corpus callosum was thinner than normal. No hindbrain herniation was observed. Brains from group B and the control did not show any of these abnormalities.
Some of the central nervous system abnormalities associated to MMC in human patients are also found in the uncorrected fetal lamb model of MMC but not in the only survivor to intrauterine coverage. Further studies are necessary to ascertain if these abnormalities can be prevented by coverage of the defect.
研究在脊髓脊膜膨出（MMC）的绵羊模型中是否存在与人类疾病中导致认知障碍的脑畸形相似的情况。
在妊娠60至80天的16只胎羊中通过手术制造出类似MMC的损伤。10只未进行胎儿修复（A组），2只采用开放式双层缝合修复（B组），2只采用开放式生物胶水覆盖（C组），2只采用胎儿镜覆盖（D组）。处死羔羊并检查其大脑。取2只未手术的正常羔羊的大脑作为对照。
13只羔羊死于子宫内（81%）。A组的2只羔羊和B组的1只羔羊足月分娩。A组的大脑显示脑积水和广泛的多小脑回区域。在多小脑回区域下方还存在室管膜内衬的广泛剥脱，胼胝体比正常的薄。未观察到后脑疝。B组和对照组的大脑未显示出这些异常。
在未矫正的MMC胎羊模型中发现了一些与人类患者MMC相关的中枢神经系统异常，但在子宫内覆盖治疗的唯一存活者中未发现。需要进一步研究以确定这些异常是否可以通过覆盖缺损来预防。
