目的:宫颈囊状有限背侧骨髓分裂(LDM),以前所谓的“宫颈脊髓膜膨出”,“是一种罕见的脊柱发育不良。尽管真性脊髓脊膜膨出的发病机制是原发性神经形成失败,LDM是由神经形成最后阶段的延迟异常引起的。该研究的目的是评估这些患者的预后,并评估预后与病变程度和类型的相关性。此外,我们系统回顾了有关类似病变的文献汇总数据.
方法:在儿童医疗中心(CMC)进行了一项回顾性研究,德黑兰,伊朗。2004年至2020年期间接受手术的患者信息(即,最近的系列)是从2000年至2003年之间从同一中心获得的先前发布的系列(CMC系列)中提取并结合数据。对所有已发表病例的文献进行了回顾,结合CMC系列进行进一步分析。
结果:最近的系列包括22例患者。结合以前发表的16个案例,CMC系列包括38例手术时平均±SD年龄为11.75±28.64个月的患者。神经功能缺损的发生率,脑积水,CMC系列中Chiari畸形II型为26.32%,39.47%,28.95%,分别。病变在17例(44.7%)中处于上水平,在21例(55.3%)中处于下水平,诊断为茎型病变31例(81.58%),骨髓囊肿型病变7例(18.42%)。在最后的后续行动中,31例患者(81.57%)实现括约肌失禁,所有36名无障碍患者都被送上了床,由28名(73.68%)独立和8名(21.05%)依赖性步行患者组成。Chiari畸形II型和脑积水的发生率在上一级病变患者中并不明显高,但是那些神经缺陷,步行,括约肌失禁与水平无关。脑积水的发生率(p<0.01),ChiariII型畸形(p<0.01),脊髓囊肿组的神经功能缺损(p=0.04)明显更高。在系统审查中,24.77%的患者有神经功能缺损。二元逻辑回归显示,手术年龄较大(p=0.03)和相关的脊柱异常(p=0.04)是缺陷的重要预测因素。ChiariII型畸形显着(p<0.001),脑积水在骨髓囊肿型病变患者中更为常见(p=0.06)。Chiari畸形II型在上层病变患者中的发生率较高(p=0.02)。
结论:与远端区域的真性脊髓膜膨出患者相比,宫颈囊状LDM患者的预后更好。根据目前的系列,大多数患者获得了步行和排尿失禁,无论病变的级别或类型。脑积水,ChiariII型畸形,并且神经功能缺损在骨髓囊肿型病变患者中更为常见。
OBJECTIVE: Cervical saccular limited dorsal myeloschisis (LDM), previously so-called \"cervical myelomeningocele,\" is a rare spinal dysraphism. Although the pathogenesis of true myelomeningocele is primary neurulation failure, LDM results from a delayed abnormality during the final stages of neurulation. The aim of the study was to evaluate the outcome of these patients and to assess the correlation of outcomes with the level and type of lesion. Also, pooled data from the literature on similar lesions were systematically reviewed.
METHODS: A retrospective study was conducted at Children\'s Medical Center (CMC), Tehran, Iran. Information of patients who underwent surgery between 2004 and 2020 (i.e., the recent series) was extracted and combined with data from a previously published series from the same center that were obtained between 2000 and 2003 (CMC series). The literature was reviewed for all published cases, to be combined with the CMC series for further analyses.
RESULTS: Twenty-two patients were included in the recent series. Combined with 16 previously published cases, 38 patients with a mean ± SD age at surgery of 11.75 ± 28.64 months were included in the CMC series. The rates of neurological deficit, hydrocephalus, and Chiari malformation type II in the CMC series were 26.32%, 39.47%, and 28.95%, respectively. The lesions were at the upper levels in 17 (44.7%) and lower cervical levels in 21 (55.3%) patients, with 31 cases (81.58%) diagnosed with stalk-type lesions and 7 cases (18.42%) with myelocystocele-type lesions. At final follow-up, 31 patients (81.57%) achieved sphincter continence, and all 36 accessible patients were ambulated, consisting of 28 (73.68%) independent and 8 (21.05%) dependent ambulation patients. The rates of Chiari malformation type II and hydrocephalus were insignificantly higher in patients with upper-level lesions, but those of neurological deficit, ambulation, and sphincter continence were not associated with level. The rates of hydrocephalus (p < 0.01), Chiari type II malformation (p < 0.01), and neurological deficit (p = 0.04) were significantly higher in the myelocystocele group. In the systematic
review, 24.77% of patients had neurological deficit. Binary logistic regression showed that older age at surgery (p = 0.03) and associated spinal anomalies (p = 0.04) were significant predictors of deficits. Chiari type II malformation was significantly (p < 0.001) and hydrocephalus was marginally (p = 0.06) more common in patients with myelocystocele-type lesions. The rate of Chiari malformation type II was higher in patients with upper-level lesions (p = 0.02).
CONCLUSIONS: Patients with cervical saccular LDM had better outcome compared with those patients with true myelomeningocele in more distal areas. According to the current series, most patients obtained ambulation and voiding continence, regardless of the level or type of lesion. Hydrocephalus, Chiari type II malformation, and neurological deficit were more common in patients with myelocystocele-type lesions.