Lee Ji Yeoun, Chong Sangjoon, Choi Young Hun, Phi Ji Hoon, Cheon Jung-Eun, Kim Seung-Ki, Park Sung Hye, Kim In-One, Wang Kyu-Chang
Divisions of 1 Pediatric Neurosurgery and.
Department of Anatomy, Seoul National University College of Medicine, Seoul, Korea.
J Neurosurg Pediatr. 2017 May;19(5):616-619. doi: 10.3171/2016.12.PEDS16171. Epub 2017 Feb 17.
OBJECTIVE Since the entity limited dorsal myeloschisis (LDM) was proposed, numerous confusing clinical cases have been renamed according to the embryopathogenesis. However, clinical application of this label appears to require some clarification with regard to pathology. There have been cases in which all criteria for the diagnosis of LDM were met except for the presence of a neural component in the stalk, an entity the authors call "probable" LDM. The present study was performed to meticulously review these cases and suggest that a modified surgical strategy using limited laminectomy is sufficient to achieve the surgical goal of untethering. METHODS The authors retrospectively reviewed the imaging findings, operative notes, and pathology reports of spinal dysraphism patients with subcutaneous stalk lesions who had presented to their institution between 2010 and 2014. RESULTS Among 33 patients with LDM, 13 had the typical nonsaccular lesions with simple subcutaneous stalks connecting the skin opening to the spinal cord. Four cases had "true" LDM meeting all criteria for diagnosis, including pathological confirmation of CNS tissue by immunohistochemical staining with glial fibrillary acidic protein. There were also 9 cases in which all clinical, imaging, and surgical findings were compatible with LDM, but the "neural" component in the resected stalk was not confirmed. For all the cases, limited exposure of the stalk was done and satisfactory untethering was achieved. CONCLUSIONS One can speculate based on the initial error of embryogenesis that if the entire stalk were traced to the point of insertion on the cord, the neural component would be proven. However, this would require an extended level of laminectomy/laminotomy, which may be unnecessary, at least with regard to the completeness of untethering. Therefore, the authors propose that for some selected cases of LDM, a minimal extent of laminectomy may suffice for untethering, although it may be insufficient for diagnosing a true LDM.
目的 自从提出实体性有限性脊髓脊膜膨出(LDM)以来,许多令人困惑的临床病例已根据胚胎发病机制重新命名。然而,这一命名在病理学方面的临床应用似乎需要一些澄清。曾有一些病例,除了茎中存在神经成分外,满足LDM诊断的所有标准,作者将这种实体称为“可能的”LDM。本研究旨在仔细回顾这些病例,并表明采用有限椎板切除术的改良手术策略足以实现脊髓松解的手术目标。方法 作者回顾性分析了2010年至2014年期间在其机构就诊的伴有皮下茎病变的脊髓发育异常患者的影像学检查结果、手术记录和病理报告。结果 在33例LDM患者中,13例有典型的非囊状病变,有简单的皮下茎将皮肤开口与脊髓相连。4例符合所有诊断标准的“真正”LDM,包括通过胶质纤维酸性蛋白免疫组化染色对中枢神经系统组织进行病理确认。还有9例患者,其所有临床、影像学和手术结果均与LDM相符,但切除的茎中的“神经”成分未得到证实。对于所有病例,均对茎进行了有限暴露,并实现了满意的脊髓松解。结论 基于胚胎发育的初始错误可以推测,如果将整个茎追溯到其在脊髓上的插入点,神经成分将得到证实。然而,这需要扩大椎板切除术/椎板切开术的范围,这可能是不必要的,至少就脊髓松解的完整性而言。因此,作者建议,对于某些选定的LDM病例,最小范围的椎板切除术可能足以实现脊髓松解,尽管这可能不足以诊断真正的LDM。