Kunz Mathias, Lehner Markus, Heger Alfred, Armbruster Lena, Weigand Heike, Mast Gerson, Peraud Aurelia
Department of Neurosurgery, Klinikum Großhadern, Ludwig-Maximilians-University Munich, Marchioninistrasse 15, 81377, Munich, Germany.
Childs Nerv Syst. 2014 Jun;30(6):1075-82. doi: 10.1007/s00381-013-2340-0. Epub 2013 Dec 13.
Metopic suture synostosis leading to trigonocephaly is considered the second most frequent type of craniosynostosis. Besides esthetic results, we present 25 consecutive pediatric cases operated upon metopic suture synostosis with a focus on the child's motor, speech, and neurocognitive development.
Twenty-five children (aged 6 to 33 months; median 9.2 months) with trigonocephaly were operated upon between 2002 and 2012 with fronto-orbital advancement including frontal bone cranioplasty and fronto-orbital bandeau remodeling. Neurodevelopmental deficits were evaluated by a standardized questionnaire including gross motor function, manual coordination, speech, and cognitive function performed by independent pediatric/developmental neurologists before surgery and at 6 and 12 months of time interval postoperatively.
Twenty-one (84 %) boys and four (16 %) girls were included in this study. Mean follow-up period was 33 ± 28 months. Outcome analysis for esthetic results showed a high degree of satisfaction by the parents and treating physicians in 23 cases (92 %). Preoperative evaluation revealed neurodevelopmental deficits in 10 children (40 %; six mild, four moderate degree). Twelve children (48 %) were proven to have a normal preoperative neuropediatric development. Mild or moderate developmental restraints were no longer apparent in 6/13, improved but still apparent in 3/13, and stable in 4/13, 6 months after cranial vault reconstruction. At 12 months of follow-up, deficits were no longer present in 9/13 and improved in 4/13. Apart from this cohort, two children were diagnosed with a syndromic form, and one child had a fetal valproate syndrome. In these three children, neurodevelopmental deficits were more pronounced. Neurocognitive progress was obvious, but was comparably slower, and major deficits were still apparent at last follow-up. All children with proven mild/moderate/severe deficits received intensive physiotherapy, logopedic, or neurobehavioral support.
As shown in a single-center observation, surgical correction of metopic suture synostosis not only refines esthetic appearance but also might improve neurodevelopmental outcome if deficits are apparent, even in syndromic forms of the deformity under additional physiotherapy, logopedic, or neurobehavioral support.
导致三角头畸形的额缝早闭被认为是第二常见的颅缝早闭类型。除了美观效果外,我们报告了连续25例接受额缝早闭手术的儿科病例,重点关注儿童的运动、语言和神经认知发育。
2002年至2012年间,对25例(年龄6至33个月;中位年龄9.2个月)三角头畸形患儿进行了额眶前移手术,包括额骨颅骨成形术和额眶带重塑。术前及术后6个月和12个月,由独立的儿科/发育神经科医生通过标准化问卷对神经发育缺陷进行评估,问卷包括粗大运动功能、手动协调、语言和认知功能。
本研究纳入21名(84%)男孩和4名(16%)女孩。平均随访期为33±28个月。美观效果的结果分析显示,23例(92%)患儿的家长和治疗医生满意度较高。术前评估发现10名儿童(40%;6名轻度,4名中度)存在神经发育缺陷。12名儿童(48%)术前经神经儿科检查发育正常。在颅盖重建术后6个月,13名儿童中有6名不再有轻度或中度发育受限,3名有所改善但仍有受限,4名保持稳定。随访12个月时,13名儿童中有9名缺陷不再存在,4名有所改善。除了这个队列外,有2名儿童被诊断为综合征形式,1名儿童患有胎儿丙戊酸综合征。在这3名儿童中,神经发育缺陷更为明显。神经认知有明显进展,但相对较慢,在最后一次随访时主要缺陷仍然明显。所有经证实有轻度/中度/重度缺陷的儿童均接受了强化物理治疗、言语治疗或神经行为支持。
如单中心观察所示,额缝早闭的手术矫正不仅能改善美观外观,而且如果存在缺陷,即使是综合征形式的畸形,在额外的物理治疗、言语治疗或神经行为支持下,也可能改善神经发育结局。