Rahman Maryam, Perkins Leigh Ann, Pincus David W
Department of Neurological Surgery, McKnight Brain Institute, University of Florida College of Medicine, Gainesville, FL 32610-0265, USA.
Pediatr Neurosurg. 2009;45(5):337-44. doi: 10.1159/000257521. Epub 2009 Nov 11.
BACKGROUND/AIMS: Chiari II malformation represents a group of developmental abnormalities involving the caudal displacement of the cervicomedullary junction, pons, fourth ventricle, and medulla. This constellation of malformations is strongly associated with myelomeningocele (MM) and is a known cause of neurologic deterioration in older MM patients. We describe the evaluation and management of 4 adult MM patients who presented with brainstem compression and a retroflexed odontoid who were subject to aggressive surgical management including occipitocervical (OC) fusion with good clinical results.
Four MM patients who underwent OC fusion for new-onset brainstem dysfunction and myelopathy were identified in the practice of the principal investigator (D.W.P.) from 2003 to 2008.
The initial evaluation of these patients included consideration of other diagnoses, such as hydrocephalus due to shunt malfunction and tethered cord. These patients were treated with aggressive surgical management. In some cases, multiple surgeries were performed. All 4 patients were treated with cervical decompression and OC fixation and initially had good outcomes. No patients required transoral decompression. Three of the 4 had stable improvement at their last follow-up appointment (mean follow-up of 9 months).
Late deterioration in older MM patients may be secondary to brainstem or cervical spinal cord compression from ventral odontoid compression and Chiari II malformation, hydrocephalus, hydromyelia, or tethered cord. These patients may benefit from more than one surgery. Posterior decompression and OC fusion can avoid a morbid transoral odontoid resection, greatly improve patients' symptoms and prevent further neurologic decline.
背景/目的：Chiari II 型畸形是一组发育异常，涉及颈髓交界处、脑桥、第四脑室和延髓的尾端移位。这一系列畸形与脊髓脊膜膨出（MM）密切相关，是老年 MM 患者神经功能恶化的已知原因。我们描述了 4 例成年 MM 患者的评估和治疗情况，这些患者出现脑干受压和齿状突后屈，接受了积极的手术治疗，包括枕颈（OC）融合术，临床效果良好。
在主要研究者（D.W.P.）2003 年至 2008 年的临床实践中，确定了 4 例因新发脑干功能障碍和脊髓病而接受 OC 融合术的 MM 患者。
对这些患者的初步评估包括考虑其他诊断，如分流故障导致的脑积水和脊髓栓系。这些患者接受了积极的手术治疗。在某些情况下，进行了多次手术。所有 4 例患者均接受了颈椎减压和 OC 固定，最初效果良好。没有患者需要经口减压。4 例患者中有 3 例在最后一次随访时病情稳定改善（平均随访 9 个月）。
老年 MM 患者的晚期恶化可能继发于腹侧齿状突压迫和 Chiari II 型畸形、脑积水、脊髓空洞症或脊髓栓系导致的脑干或颈脊髓受压。这些患者可能从不止一次手术中获益。后路减压和 OC 融合可以避免进行病态的经口齿状突切除术，极大地改善患者症状并防止神经功能进一步下降。
