Chiari Malformation with and without Syringomyelia: Surgical Technique and Outcome in 88 Adult Patients

Samra Majeed, Muhammad Irfan, Muhammad Hammad Nasir, Ajmal Khan, Azam Niaz, Jahangir Khan
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Abstract

Objective:  This study identified the relationship between posterior fossa craniectomy, expansion neuroplasty, and radiological appearances in patients with Chiari malformation with and without clinical syringomyelia with the surgical outcomes in an attempt to correct the lesion. Materials & Methods:  Eighty-eight patients with Chiari malformation (CM) were included in the study where 70 had associated syringomyelia. All underwent posterior fossa craniotomy, expansion duroplasty without fiddling with cerebellar tonsils. Patients were evaluated at 1 month, 3 months, and 12 months. The MRI studies were done at 12 months when symptomatic relief and radiological findings were evaluated and matched. Results:  Most of the patients were young adults between the age range of 25 – 40 years. The most common complication was pseudomeningocele (5.68%) formation followed by CSF leak (4.54%). Patients with a longer history of Chiari malformation or syrinx-related symptoms and signs had partial relief in symptoms and signs. The poor outcome as expected was seen in patients with atrophic changes in upper limbs and hypertonia in lower limbs, especially in patients with loss of joints position sense and poor balance. Patients showed maximum improvement in headaches both suboccipital as well as generalized. Syringomyelia was decreased in size in 49 patients and remained unchanged in 21.  Dysesthesias were improved in 31 patients. Conclusion:  Clinical improvement was related to the expansion of the posterior fossa and subarachnoid cistern and reduction in the size of the syrinx. Surgical decompression of the posterior fossa should create adequate space for its contents and reduce the syrinx cavity. The relationship between symptomatic improvement and radiological findings is not always linear. Keywords:  Chiari Malformation, Tonsillar Herniation, Syringomyelia, Duroplasty.
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伴有和不伴有脊髓空洞的Chiari畸形:88例成人患者的手术技术和结果
目的:本研究确定伴有或不伴有脊髓空洞的Chiari畸形患者后颅窝颅骨切除术、扩张神经成形术和影像学表现与手术结果的关系,试图纠正病变。材料与方法:88例Chiari畸形(CM)患者纳入研究,其中70例伴有脊髓空洞。所有患者均行后颅窝开颅术、硬脑膜扩张成形术,且未损伤小脑扁桃体。患者在1个月、3个月和12个月时进行评估。12个月时进行MRI检查,评估并匹配症状缓解和影像学结果。结果:患者多为25 ~ 40岁的青壮年。最常见的并发症是假性脑膜膨出(5.68%),其次是脑脊液漏(4.54%)。有较长Chiari畸形史或与鼻腔相关症状和体征的患者症状和体征部分缓解。上肢萎缩性改变和下肢强直的患者,尤其是关节位置感丧失和平衡能力差的患者,预后如预期的那样差。患者在枕下头痛和全身头痛方面均表现出最大程度的改善。49例脊髓空洞缩小,21例保持不变。31例患者感觉障碍得到改善。结论:临床改善与后窝和蛛网膜下腔池的扩张和鼻管的缩小有关。手术减压后窝应为其内容物创造足够的空间并缩小喉腔。症状改善与影像学表现之间的关系并不总是线性的。关键词:Chiari畸形扁桃体突出脊髓空洞硬膜成形术
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