Pastuszka Agnieszka, Zamłyński Mateusz, Horzelski Tomasz, Zamłyński Jacek, Horzelska Ewa, Maruniak-Chudek Iwona, Marzec Adrianna, Paprocka Justyna, Gazy Patrycja, Koszutski Tomasz, Olejek Anita
Department of Gynecology, Obstetrics and Oncological Gynecology, Bytom, Medical University of Silesia, 40-752 Katowice, Poland.
Department of Pediatric Surgery and Urology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, 40-752 Katowice, Poland.
Diagnostics (Basel). 2022 Nov 28;12(12):2978. doi: 10.3390/diagnostics12122978.
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.
开放性脊柱裂是中枢神经系统最常见的先天性缺陷之一。开放性胎儿手术作为可用的治疗选择之一，仍然是产前修复的金标准。胎儿镜下闭合术可能会降低与开放性胎儿手术相关的母体并发症数量。无论采用何种方法，脊髓栓系（TSC）综合征的发生都可能影响治疗结果。一名初产妇在妊娠24.2周时因胎儿脊髓脊膜膨出入院，被认为适合进行胎儿镜修复。胎儿手术在妊娠25.0周时进行。这是在胎儿镜下修复脊柱裂过程中首次实现的脊髓完全松解和解剖重建（硬脑膜、脊柱竖脊肌、皮肤）。因胎盘早剥，在妊娠31.1周时进行了剖宫产。产后5周，由于脑室扩大进展，进行了无需再次手术的脑室腹腔分流术。未观察到继发性脊髓栓系的临床或影像学迹象。产后11个月的神经学检查显示颅神经无任何损伤迹象，轴向肌张力减退，下肢肌张力降低，病理反射消失。运动发育略有迟缓。无论采用何种手术方法，都应始终进行神经结构的完全松解，因为这是降低继发性TSC发生风险的唯一措施。
