The role of cervicomedullary decompression (CMD) in the care of hydrocephalic achondroplastic children who present with simultaneous foramen magnum stenosis is not well understood. We sought to determine the percentage of symptomatic achondroplastic children with foramen magnum stenosis who had stabilization or improvement in ventriculomegaly following CMD. The authors retrospectively reviewed the records of pediatric patients at Cedars‐Sinai Medical Center with achondroplasia and signs of progressive ventriculomegaly who underwent CMD for symptomatic foramen magnum stenosis between the years 2000 and 2018. Clinical outcomes included changes in fontanelle characteristics, head circumference (HC) percentile, and incidence of ventriculoperitoneal (VP) shunting. Radiographic outcomes measured included changes in Evans ratio. We excluded individuals who were shunted before CMD from our study. Sixteen children presented with symptomatic foramen magnum stenosis and full anterior fontanelle or jump in the HC percentiles. Two children underwent placement of a VP shunt before decompressive surgery and were excluded from further analysis. Of the remaining 14 children who underwent CMD, 13 (93%) showed softening or flattening of their fontanelles post‐operatively. Ten of these 14 children had both pre‐ and post‐operative HC percentile records available, with 8 showing increasing HC percentiles before surgery. Seven of those eight children (88%) showed a deceleration or stabilization of HC growth velocity following decompression of the foramen magnum. Among 10 children with available pre‐ and post‐operative brain imaging, ventricular size improved in 5 (50%), stabilized in 2 (20%), and slightly increased in 3 (30%) children after decompression. Two children (14%) required a shunt despite decompression of the foramen magnum. A significant proportion of children with concomitant signs of raised intracranial pressure or findings of progressive ventriculomegaly and foramen magnum stenosis may have improvement or stabilization of these findings following CMD. CMD may decrease the need for VP shunting and its associated complications in the select group of hydrocephalic children with achondroplasia presenting with symptomatic foramen magnum stenosis.

中文翻译：

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患有软骨发育不良和孔大口狭窄的患儿进行子宫颈减压手术后脑室扩大的改善。

子宫颈髓腔减压术（CMD）在同时伴有孔眼大口狭窄的脑积水性软骨发育不良儿童的护理中的作用尚不清楚。我们试图确定在CMD后有症状或稳定的或有改善的脑室扩大的有孔眼狭窄的症状性软骨发育不全儿童的百分比。作者回顾性回顾了Cedars-Sinai医学中心的小儿患者的记录，这些患者在2000年至2018年之间因有症状的大孔狭窄而接受了CMD的软骨发育不良和进行性脑室扩大的征兆。临床结果包括font门特征，头围（HC）的变化百分位数和心室腹膜（VP）分流的发生率。所测量的放射线结果包括埃文斯比率的变化。我们排除了在CMD之前被分流的个体。十六名患儿出现症状性孔眼大狭窄，前full完全肿胀或HC百分位数跳高。两名儿童在进行减压手术之前接受了VP分流，并被排除在进一步分析之外。其余14名接受CMD的儿童术后有13例（93％）的font门变软或变平。这14名儿童中有10名有术前和术后HC百分位数记录，其中8名表明术前HC百分位数增加。在八个大孔减压后，这八个孩子中的七个（88％）显示出HC生长速度减慢或稳定。在有术前和术后脑影像学检查的10名儿童中，有5名（50％）改善了心室大小，减压后，有2名儿童（20％）稳定下来，有3名（30％）儿童略有增加。尽管有孔大瓶减压，但仍有两个孩子（14％）需要分流。伴有颅内压升高迹象或进行性脑室扩大和大孔狭窄的伴发症状的儿童中，有很大比例的儿童在CMD后可改善或稳定这些发现。在有症状性孔眼狭窄的伴有软骨发育不良的脑积水精选儿童中，CMD可以减少VP分流及其相关并发症的发生。伴有颅内压升高迹象或进行性脑室扩大和大孔狭窄的伴发症状的儿童中，有很大比例的儿童在CMD后可改善或稳定这些发现。在有症状性孔眼狭窄的伴有软骨发育不良的脑积水精选儿童中，CMD可以减少VP分流及其相关并发症的发生。伴有颅内压升高迹象或进行性脑室扩大和大孔狭窄的伴发症状的儿童中，有很大比例的儿童在CMD后可改善或稳定这些发现。在有症状性孔眼狭窄的伴有软骨发育不良的脑积水精选儿童中，CMD可以减少VP分流及其相关并发症的发生。