Higashida Tetsuhiro, Sasano Mari, Sato Hironobu, Sekido Ken'ichi, Ito Susumu
Department of Neurosurgery, Kanagawa Children's Medical Center, Yokohama, Kanagawa.
Neurol Med Chir (Tokyo). 2010;50(5):426-30. doi: 10.2176/nmc.50.426.
Three neonates presented with split cord malformation (SCM) associated with myelomeningocele (MMC), complicated with various coexisting anomalies. All patients were female and classified as SCM type I. All patients had a syrinx located rostral to the SCM. One patient had hydrocephalus and Chiari malformation causing serious respiratory problems. Two patients had partial hypertrichosis located close to the MMC, suggesting association with SCM. One patient had sacral hypoplasty and right kidney agenesis, suggesting that some embryologic errors may affect not only neural but also mesodermal development. All patients underwent surgical treatment for SCM after detailed evaluation and management of concomitant anomalies, and developed no new neurological deficits. Delayed surgery is an alternative treatment strategy for SCM in patients with both SCM and MMC with similar complications.
三名新生儿出现了与脊髓脊膜膨出(MMC)相关的脊髓分裂畸形(SCM),并伴有各种并存的异常情况。所有患者均为女性,归类为I型SCM。所有患者在SCM头侧均有一个脊髓空洞。一名患者患有脑积水和Chiari畸形,导致严重的呼吸问题。两名患者在靠近MMC处有局部多毛症,提示与SCM有关。一名患者有骶骨发育不全和右肾缺如,提示一些胚胎学错误可能不仅影响神经发育,还影响中胚层发育。所有患者在对并存异常进行详细评估和处理后均接受了SCM手术治疗,且未出现新的神经功能缺损。对于患有SCM和MMC且有类似并发症的患者,延迟手术是SCM的一种替代治疗策略。