Reversal of Hindbrain Herniation after Maternal-fetal Surgery for Myelomeningocele Subsequently Impacts on Brain Stem Function
Reversal of Hindbrain Herniation after Maternal-fetal Surgery for Myelomeningocele Subsequently Impacts on Brain Stem Function
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DOI：
10.1055/s-0029-1202835
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发表时间：
2008-12-01
期刊：
影响因子：
1.4
通讯作者：
Johnson, M. P.
中科院分区：
文献类型：
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作者：
Danzer, E.;Finkel, R. S.;Johnson, M. P.
我们研究的目的是探讨胎儿脊髓脊膜膨出 (fMMC) 闭合后后脑疝 (HH) 的逆转是否会降低 HH 相关脑干功能障碍 (BSD) 的发生率和严重程度。在 NIH 资助的脊髓脊膜膨出管理研究 (MOMS) 试验之前，我们机构有 54 名儿童接受了 fMMC 封堵术。四十八 (89%) 个家庭参与了一项重点关注 HH 相关 BSD（例如呼吸暂停、神经源性吞咽困难 [ND]、胃食管反流病 [GERD]、神经眼科障碍 [NOD]）的结构化调查。随访时的中位年龄为 72 个月（范围：46-98 岁）。百分之五十需要调车。 15 名（63%）非分流儿童和 10 名（42%）分流儿童完全没有 HH 相关症状（P = 0.15）。没有发生与 HH 相关的死亡，也没有出现严重的持续性紫绀呼吸暂停。 2 名（8%）未分流婴儿和 9 名（38%）分流婴儿报告了 ND（P = 0.03）。未放置分流器的 2 名患者 (8%) 和接受分流器放置的 6 名患者 (25%) 出现轻度 GERD（医疗管理）（P = 0.24）。未分流和分流儿童分别有 6 名 (25%) 和 13 名 (54%) 发现 NOD (P = 0.07)。大多数 fMMC 儿童在随访时没有出现或仅出现轻度 BSD。我们的数据支持这样的假设：与 MMC 相关的神经发育缺陷至少部分是获得性的，并且 fMMC 手术后 HH 的逆转可能有助于降低 BSD 的发生率和严重程度。
The aim of our study was to delineate whether the reversal of hindbrain herniation (HH) following fetal myelomeningocele (fMMC) Closure Subsequently reduces the incidence and severity of HH-associated brainstem dysfunction (BSD). Prior to the NIH-sponsored Management of Myelomeningocele Study (MOMS) trial, 54 children underwent fMMC closure at our institution. Forty-eight (89%) families participated in a structured survey focusing on HH-associated BSD (e.g., apnea, neurogenic dysphagia [ND], gastro-esophageal reflux disease [GERD], neuro-ophthalmologic disturbances [NOD]). Median age at follow-up was 72 months (range: 46-98). Fifty-percent required Shunting. HH-related symptoms were completely absent in 15 (63%) non-shunted and 10 (42%) Shunted children (P = 0.15). No HH-related death occurred and none developed severe persistent cyanotic apnea. ND was reported in 2 (8%) non-shunted and 9 (38%) shunted infants (P = 0.03). Mild GERD (medically managed) developed in 2 (8%) without and 6 (25%) with shunt placement (P = 0.24). NOD was found in 6 (25%) and 13 (54%) of non-shunted and shunted children, respectively (P = 0.07). The majority of fMMC children developed no or only mild BSD at follow-up. Our data Support the hypothesis that neurodevelopmental deficits associated with MMC are at least partially acquired and that reversal of HH following fMMC surgery may help to reduce the incidence and severity of BSD.