Tulipan N, Bruner J P
Department of Neurosurgery, Vanderbilt University Medical Center, Nashville, Tenn., USA.
Pediatr Neurosurg. 1998 Apr;28(4):177-80. doi: 10.1159/000028645.
Evidence accumulating over the last 10 years suggests that the exposed spinal cord tissue in a myelomeningocele sustains a secondary injury as the result of prolonged exposure to the intrauterine environment. These data suggest that early closure of the myelomeningocele sac might prevent such injury and in turn improve the neurologic outcome in the affected infant.
Three patients with fetuses carrying the ultrasonic diagnosis of myelomeningocele elected to enter a study of the feasibility of repairing myelomeningocele in utero. At approximately 28 weeks of gestation each patient underwent laparotomy and hysterotomy, thus exposing the myelomeningocele defect. The defect was closed in a routine surgical fashion, and the hysterotomy was then closed.
The 3 patients recovered from surgery without incident. Early premature contractions subsided, and they were discharged by the 5th postoperative day. At between 33 and 36 weeks of gestation, each infant was delivered via cesarean section. The observed neurologic deficits were within the range expected from the anatomic level of the defects. Two of the infants have not required ventriculoperitoneal shunting.
This limited series of patients suggests that myelomeningocele can be repaired in utero with minimal morbidity to either the mother or her fetus. A larger study will be needed to substantiate this low morbidity and to determine the extent of any neurologic benefit of early surgery.
过去10年积累的证据表明,脊髓脊膜膨出中暴露的脊髓组织由于长期暴露于子宫内环境而遭受继发性损伤。这些数据表明,早期闭合脊髓脊膜膨出囊可能预防此类损伤,进而改善受影响婴儿的神经学预后。
3例胎儿经超声诊断为脊髓脊膜膨出的患者选择参加一项关于宫内修复脊髓脊膜膨出可行性的研究。在妊娠约28周时,每位患者均接受剖腹术及子宫切开术,从而暴露脊髓脊膜膨出缺损。以常规手术方式闭合缺损,然后闭合子宫切开术。
3例患者术后顺利恢复。早期的早产宫缩缓解,术后第5天出院。在妊娠33至36周之间,每个婴儿均通过剖宫产分娩。观察到的神经功能缺损在根据缺损解剖水平预期的范围内。其中2例婴儿无需进行脑室腹腔分流术。
这一有限的患者系列表明,脊髓脊膜膨出可在宫内修复,对母亲或其胎儿的发病率均极低。需要进行更大规模的研究来证实这种低发病率,并确定早期手术在神经学方面的获益程度。