关键词: fetoscopy myelomeningocele prenatal surgery spina bifida

来  源:   DOI:10.3390/diagnostics12122978

Abstract:
Open spina bifida is one of the most common congenital defects of the central nervous system. Open fetal surgery, which is one of the available therapeutic options, remains the gold standard for prenatal repairs. Fetoscopic closure may lower the number of maternal complications associated with open fetal surgery. Regardless of the approach, the outcome may be compromised by the development of tethered spinal cord (TSC) syndrome. At 24.2 weeks of gestation, a primipara was admitted due to fetal myelomeningocele and was deemed eligible for fetoscopic repair. Fetal surgery was performed at 25.0 weeks of gestation. It was the first complete untethering of the spinal cord and anatomic reconstruction (dura mater, spinal erectors, skin) achieved during a fetoscopic repair of spina bifida. Cesarean section due to placental abruption was performed at 31.1 weeks of gestation. VP shunting, with no need for revision, was performed at 5 weeks postdelivery due to progressing ventriculomegaly. No clinical or radiological signs of secondary tethering were observed. Neurological examination at 11 months postdelivery revealed cranial nerves without any signs of damage, axial hypotonia, decreased muscle tone in the lower extremities, and absent pathological reflexes. Motor development was slightly retarded. Complete untethering of the neural structures should always be performed, regardless of the surgical approach, as it is the only course of action that lowers the risk for developing secondary TSC.
摘要:
开放性脊柱裂是中枢神经系统最常见的先天性缺陷之一。开放式胎儿手术,这是可用的治疗选择之一,仍然是产前修复的黄金标准。胎儿镜封堵术可能会降低与开放胎儿手术相关的母体并发症的数量。不管采用哪种方法,脊髓栓系(TSC)综合征的发展可能会损害结果。在妊娠24.2周,一名初产妇因胎儿脊髓膜膨出入院,被认为符合胎儿镜修复的条件。在妊娠25.0周时进行胎儿手术。这是第一次完全解开脊髓和解剖重建(硬脑膜,脊柱勃起,皮肤)在脊柱裂的胎儿镜修复期间实现。妊娠31.1周时因胎盘早剥进行剖宫产。调车副总裁,不需要修改,由于进行性脑室肥大,在分娩后5周进行。未观察到继发性束缚的临床或放射学征象。分娩后11个月的神经系统检查显示颅神经没有任何损伤的迹象,轴向低张力,下肢肌张力下降,和缺乏病理反射。电机的发展略有延迟。神经结构的完全解开应该总是进行,不管手术方法如何,因为这是降低开发二级TSC风险的唯一行动方案。
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