Department of Neurosurgery, University Hospital of Tlemcen, Tlemcen, Algeria.
Qena Faculty of Medicine, South Valley University, Qena, Egypt.
Pediatr Neurosurg. 2023;58(2):97-104. doi: 10.1159/000530726. Epub 2023 Apr 19.
Myelomeningocele (MMC) is a malformation resulting from the neural tube's failure to close during embryonic development, and the majority of the cases of neural tube defects (NTDs) were prevalent as single location lesions along the spine; however, multiple NTDs (MNTDs) are a very rare condition. Only a few cases of MNTDs were found in the literature.
We report the case of a 2-month-old male infant prenatally diagnosed with MMC, presented with two unconnected lumbar and lumbosacral epidermal, soft, dome-shaped swellings located on both sides of the midline (paravertebral) covered by intact skin. MRI revealed double MMC at the level of L4-L5, with spinal nerve roots. The patient underwent surgical repair of the defects by replacing the spinal cord and its nerve roots inside the thecal sac and recreating a covering layer around the neural structures to resemble thecal sac. The outcome was favorable, and postoperative head CT scan did not show any complication.
Our case report is considered the first from Algeria to report the condition and the first to report the occurrence of double lesions in the same spine region. MMC can be associated with neurological deficits or other congenital anomalies, thus it is necessary to thoroughly examine such patients. However, there was no antenatal folic acid deficiency in our case. We recommend antenatal care with adequate folic acid supplementation given that its deficiency during pregnancy is considered a ubiquitous risk factor for the condition. The optimal timing for surgery of MMC cases is 8 ± 5 days. Prenatal intrauterine repair of the condition provides favorable outcomes but carries high fetal and maternal risks. Surgical repair should include the sac removal, the reconstruction of the placode, and the closure of the overlying meninges. With early diagnosis and proper repair of such cases, MMC has good prognosis and favorable outcomes.
脊髓脊膜膨出（MMC）是一种由胚胎发育过程中神经管闭合失败引起的畸形，大多数神经管缺陷（NTD）病例以脊柱单一位置病变为主；然而，多发性 NTD（MNTD）是一种非常罕见的情况。只有少数 MNTD 病例在文献中被报道。
我们报告了一例 2 个月大的男性婴儿，产前诊断为 MMC，表现为两个未连接的腰和腰骶部表皮、柔软、圆顶状肿胀，位于中线两侧（椎旁），覆盖完整皮肤。MRI 显示 L4-L5 水平的双 MMC，伴有脊神经根。患者接受了手术修复缺陷，将脊髓和其神经根置于硬脊膜内，并在神经结构周围重建覆盖层，以模拟硬脊膜。结果良好，术后头部 CT 扫描未显示任何并发症。
我们的病例报告被认为是阿尔及利亚首例报告该病例，也是首例报告同一脊柱区域发生双病变的病例。MMC 可伴有神经功能缺损或其他先天性异常，因此有必要对这类患者进行彻底检查。然而，我们的病例中没有产前叶酸缺乏。我们建议进行产前护理并充分补充叶酸，因为在妊娠期间缺乏叶酸被认为是该病的普遍危险因素。MMC 病例的最佳手术时机为 8±5 天。该疾病的产前宫内修复可提供良好的结果，但存在较高的胎儿和母体风险。手术修复应包括囊袋切除、板层重建和脑膜覆盖的闭合。早期诊断和适当修复此类病例，MMC 具有良好的预后和良好的结果。
