Long-term developmental outcome of children with a fetal diagnosis of isolated inferior vermian hypoplasia.
Long-term developmental outcome of children with a fetal diagnosis of isolated inferior vermian hypoplasia.
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DOI：
10.1136/archdischild-2013-304054
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发表时间：
2014-01
期刊：
影响因子：
--
通讯作者：
du Plessis AJ
中科院分区：
文献类型：
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作者：
Tarui T;Limperopoulos C;Sullivan NR;Robertson RL;du Plessis AJ
孤立性下蚓部发育不全（iiVH）是最常见的胎儿小脑异常之一，需要进行胎儿神经学咨询，其产后神经发育结果存在争议。在本研究中，我们描述了学龄期产前诊断的 iiVH 的长期神经发育结果。我们前瞻性地跟踪了 20 名胎儿 MRI 诊断为 iiVH 的儿童，包括他们的产后 MRI 结果和学龄（平均 6.1 岁±1.9 岁标准差）的发育结果，使用一套全面的适龄发育测试，涵盖认知、语言、社交和行为领域。还评估了父母的压力水平和社会经济状况。所有产后确诊的 iiVH 儿童均具有正常的神经发育结果。表现出认知迟缓和行为障碍的儿童亚组（2/20）患有更广泛的小脑畸形。尽管发育结果正常，但与产后 MRI 正常的孩子的父母相比，产后确诊 iiVH 的孩子的父母有更高的父母压力。产后确诊的 iiVH 儿童在学龄时表现出与年龄相适应的功能。产后 MRI 对于确认 iiVH 的诊断并排除影响神经发育结果的相关异常非常重要。 iiVH 的诊断与父母压力持续升高有关，尽管这些儿童的发育结果正常，表明需要持续的父母支持。
Isolated inferior vermian hypoplasia (iiVH) is one of the most common fetal cerebellar anomalies presenting for fetal neurological counselling with controversial postnatal neurodevelopmental outcome. In the present study, we characterised the long-term neurodevelopmental outcome of prenatally diagnosed iiVH at school age. We prospectively followed 20 children with fetal MRI diagnosis of iiVH including their postnatal MRI result and developmental outcome at school age (mean 6.1 years±1.9 years SD) using a comprehensive age-appropriate developmental testing battery, which encompassed cognitive, language, social and behavioural domains. Parental stress level and socioeconomic status were also evaluated. All children with postnatally confirmed iiVH had a normal neurodevelopmental outcome. A subgroup of children (2/20) who demonstrated cognitive delays and behavioural impairments had more extensive cerebellar malformation. Despite a normal developmental outcome, the parents of children with postnatally confirmed iiVH had higher parental stress compared with those parents whose children had normal postnatal MRI. Children with postnatally confirmed iiVH show age appropriate functioning at school age. Postnatal MRI is important to confirm the diagnosis of iiVH and to exclude associated anomalies that impact neurodevelopmental outcome. A diagnosis of iiVH is associated with persistent elevated parental stress despite normal developmental outcomes in these children suggesting the need for ongoing parental support.