Pang D, Dias M S
Department of Neurosurgery, Children's Hospital of Pittsburgh, Pennsylvania.
Neurosurgery. 1993 Sep;33(3):363-72; discussion 372-3. doi: 10.1227/00006123-199309000-00003.
Cervical myelomeningoceles are rare dysraphic lesions. Nine cases of cervical myelomeningoceles are reported. The external features of all nine myelomeningoceles were strikingly similar: They were sturdy, tubular protuberances from the back of the infants' necks, covered at the base by full-thickness skin and covered on the dome by thick squamous epithelium. Internally, these were tethered cord lesions in which fibroneural bands or sagittal midline fibrous septa were tightly tethering the cervical spinal cord to the adjacent dural or intrasaccular soft tissues. Six of our early cases (Group 1) were initially treated with simple subcutaneous resection of the sac and ligation of the dural fistula without release of the internal tethering structures. Five of these children subsequently deteriorated 13 months to 8 years later, all with worsening hand function and spastic legs. All five were reexplored, and the tethering bands and septa were excised; all showed improvement. The other three neonates (Group 2) treated in the last 4 years underwent initial intradural exploration of the lesions; in one case, the tethering fibrous elements were only partially eliminated and the patient deteriorated 4 years later, but improved after a second operation for resection of a missed ventral fibrous septum. The other two Group 2 infants had a thorough release of the fibroneural stalks initially, and both were neurologically stable 3 years later. We recommend that cervical myelomeningoceles should be studied preoperatively with magnetic resonance imaging and computed tomographic myelography to identify the internal structures. The minimum initial surgical treatment should be a two-level laminectomy, intradural exploration, and excision of all tethering bands and septa, in addition to resection of the sac. If a split cord is revealed by imaging studies, both the ventral and dorsal surfaces of the hemicords must be carefully inspected to locate the median septum.
颈髓脊膜膨出是罕见的神经管闭合不全性病变。本文报告了9例颈髓脊膜膨出病例。所有9例髓脊膜膨出的外部特征极为相似：它们是从婴儿颈部后方突出的坚实管状肿物，基部被全层皮肤覆盖，顶部被厚层鳞状上皮覆盖。在内部，这些是脊髓栓系病变，其中纤维神经束或矢状中线纤维间隔将颈脊髓紧密栓系于相邻的硬脑膜或囊内软组织。我们早期的6例病例（第1组）最初采用单纯的囊皮下切除术和硬脑膜瘘管结扎术，未松解内部栓系结构。其中5名儿童在13个月至8年后病情恶化，均出现手部功能恶化和腿部痉挛。所有5例均再次进行探查，并切除栓系带和间隔；所有病例均有改善。在过去4年中治疗的另外3例新生儿（第2组）最初接受了病变的硬脊膜内探查；1例中，栓系纤维成分仅部分切除，患者在4年后病情恶化，但在第二次手术切除遗漏的腹侧纤维间隔后病情改善。另外2例第2组婴儿最初对纤维神经束进行了彻底松解，3年后神经功能均稳定。我们建议术前应通过磁共振成像和计算机断层脊髓造影对颈髓脊膜膨出进行检查，以识别内部结构。最初的最小手术治疗应包括两级椎板切除术、硬脊膜内探查、切除所有栓系带和间隔，以及切除囊。如果影像学检查显示脊髓分裂，则必须仔细检查半脊髓的腹侧和背侧表面以定位正中间隔。
