Mayer Steffi, Weisser Margit, Till Holger, Gräfe Gerd, Geyer Christian
Department of Pediatric Surgery, University Hospital Leipzig, Liebigstrasse 20a, 04103 Leipzig, Germany.
Cerebrospinal Fluid Res. 2010 Oct 14;7:17. doi: 10.1186/1743-8454-7-17.
Eagerly awaiting the results of the Management of Myelomeningocele Study (MOMS) and with an increasing interest in setting up intrauterine myelomeningocele repair (IUMR), the optimal management of patients suffering from congenital myelomeningocele (MMC) has become a matter of debate again. We performed a cross-sectional study at our referral-center for MMC to determine the outcome for our expectantly managed patients.
A computed chart review at our institution revealed 70 patients suffering from MMC. Forty-three patients were eligible for the study and analyzed further. A retrospective analysis was performed only in patients that underwent MMC repair within the first two days of life and were seen at our outpatient clinic between 2008 and 2009 for a regular multidisciplinary follow-up. Data were collected on: gestational age (GA) and weight at birth, age at shunt placement and shunt status after the first year of life, radiological evidence for Arnold-Chiari malformation (ACM) and tethered cord (TC), need for surgery for TC, bladder function, lower leg function and educational level. Data were compared to published results for IUMR and to studies of historical controls.
Patients were born with MMC between 1979 and 2009 and are now 13.3 ± 8.9 (mean ± SD) years of age. At birth, mean GA was 37.8 ± 2.3 weeks and mean weight was 2921.3 ± 760.3 g, both significantly higher than in IUMR patients. Shunt placement in our cohort was required in 69.8% at a mean age of 16.0 ± 10.7 days, which was less frequent than for historical controls. Amongst our cohort, radiological observations showed 57.1% had ACM II and 41.9% had TC. Only two of our patients underwent a surgical correction for TC. Clean intermittent catheterization was performed in 69.7% of our patients, 56.4% were (assisted) walkers and 64.1% attended regular classes, both comparable to historical controls.
With a close and interdisciplinary management by pediatric surgeons, neurologists and urologists, the long-term outcome of patients suffering from MMC can currently be considered satisfactory. With respect to the known drawbacks of fetal interventions for mother and child, especially preterm delivery, the results of the MOMS trial should be awaited with caution before proceeding with a complex intervention like IUMR.
热切期待脊髓脊膜膨出症治疗研究（MOMS）的结果，且对开展宫内脊髓脊膜膨出症修复术（IUMR）的兴趣与日俱增，先天性脊髓脊膜膨出症（MMC）患者的最佳治疗方法再次成为争论焦点。我们在本院的MMC转诊中心进行了一项横断面研究，以确定期待治疗患者的预后情况。
对本院病历进行计算机检索，发现70例MMC患者。43例患者符合研究条件并进一步分析。仅对出生后前两天内接受MMC修复术且于2008年至2009年期间在我院门诊接受定期多学科随访的患者进行回顾性分析。收集的数据包括：孕周（GA）和出生体重、分流置入年龄及1岁后的分流状况、Arnold-Chiari畸形（ACM）和脊髓栓系（TC）的影像学证据、TC手术需求、膀胱功能、小腿功能及教育水平。将数据与已发表的IUMR结果及历史对照研究进行比较。
患者于1979年至2009年期间出生时患有MMC，目前年龄为13.3±8.9（均值±标准差）岁。出生时，平均孕周为37.8±2.3周，平均体重为2921.3±760.3克，均显著高于IUMR患者。我们队列中69.8%的患者需要分流置入，平均年龄为16.0±10.7天，这一比例低于历史对照。在我们的队列中，影像学观察显示57.1%有ACM II，41.9%有TC。我们仅2例患者接受了TC手术矫正。69.7%的患者进行了清洁间歇性导尿，56.4%的患者可（辅助）行走，64.1%的患者参加常规课程，这些均与历史对照相当。
通过小儿外科医生、神经科医生和泌尿外科医生密切的多学科管理，目前MMC患者的长期预后可认为是令人满意的。鉴于胎儿干预对母婴已知的不利之处，尤其是早产，在开展IUMR等复杂干预之前，应谨慎等待MOMS试验的结果。
