Grant Ryan A, Heuer Gregory G, Carrión Geneive M, Adzick N Scott, Schwartz Erin S, Stein Sherman C, Storm Phillip B, Sutton Leslie N
Department of Neurosurgery, Yale-New Haven Medical Center, New Haven, Connecticut, USA.
J Neurosurg Pediatr. 2011 Apr;7(4):362-8. doi: 10.3171/2011.1.PEDS10234.
Myelomeningocele (MMC) is characterized by a defect in caudal neurulation and appears at birth with a constellation of neuroanatomical abnormalities, including Chiari malformation Type II. The authors investigated the effects of antenatal versus postnatal repair of MMC through a quantitative analysis of morphometric changes in the posterior fossa (PF).
The authors retrospectively reviewed the records of 29 patients who underwent in utero MMC repair, 24 patients who underwent postnatal repair, and 114 fetal and pediatric controls. Tonsillar displacement, cerebellum length, pons length, clivus-supraocciput (CSO) angle, and PF area were compared in antenatal and postnatal MMC repair groups as well as in controls without neural tube defects by using t-tests and correlation coefficients.
Initially, the in utero CSO angle was significantly more acute in all patients with MMC--prenatally and postnatally repaired--as compared with controls (57.8° vs 75.4°, p < 0.001); however, the angle rapidly changed and became similar to that in controls between 30 and 31 weeks' gestation to approximately 80°, with antenatal repair having little effect. Postnatally, the CSO angle decreased in controls (R = -0.58) and in the antenatal repair group (R = -0.17). The cerebellum and pons length demonstrated no significant differences in any group. Overall, tonsil descent was corrected in the antenatal repair group as compared with postnatal repair (p < 0.001), and the PF area increased in all 3 groups in utero. Growth was less rapid in patients with MMC compared with controls, but this was corrected by antenatal repair (p = 0.015).
Myelomeningocele was associated with tonsillar herniation and a smaller PF than in control fetuses. Antenatal surgical repair corrected both abnormalities. The CSO angle began significantly more acutely in patients with MMC, but normalized with development regardless of when surgery was performed. Determining the clinical effects of antenatal repair requires further follow-up.
脊髓脊膜膨出（MMC）的特征是尾部神经管闭合缺陷，出生时伴有一系列神经解剖学异常，包括Ⅱ型Chiari畸形。作者通过对后颅窝（PF）形态学变化的定量分析，研究了产前与产后修复MMC的效果。
作者回顾性分析了29例行宫内MMC修复术的患者、24例行产后修复术的患者以及114例胎儿和儿童对照的记录。通过t检验和相关系数，比较了产前和产后MMC修复组以及无神经管缺陷对照组的扁桃体移位、小脑长度、脑桥长度、斜坡-枕骨大孔（CSO）角和PF面积。
最初，所有产前和产后修复的MMC患者的宫内CSO角均明显比对照组更尖锐（57.8°对75.4°，p<0.001）；然而，该角度迅速变化，在妊娠30至31周时变得与对照组相似，约为80°，产前修复几乎没有影响。产后，对照组（R=-0.58）和产前修复组（R=-0.17）的CSO角均减小。小脑和脑桥长度在任何组中均无显著差异。总体而言，与产后修复相比，产前修复组的扁桃体下移得到纠正（p<0.001），所有3组在宫内的PF面积均增加。与对照组相比，MMC患者的生长速度较慢，但产前修复可纠正这一情况（p=0.015）。
脊髓脊膜膨出与扁桃体疝和比对照胎儿更小的PF相关。产前手术修复纠正了这两种异常。MMC患者的CSO角最初明显更尖锐，但无论何时进行手术，随着发育均会恢复正常。确定产前修复的临床效果需要进一步随访。
