本文選題：Chiari畸形 + 寰樞關(guān)節(jié)脫位�。� 參考：《西南醫(yī)科大學(xué)》2017年碩士論文

【摘要】：目的:探討后路減壓復(fù)位內(nèi)固定術(shù)對Chiari畸形I型(ACM I)并不穩(wěn)定型顱底凹陷的療效;方法:回顧西南醫(yī)科大學(xué)附屬醫(yī)院2013年4月至2016年1月經(jīng)后路減壓及復(fù)位枕頸內(nèi)固定術(shù)治療的ACMI并不穩(wěn)定型顱底凹陷患者15例,男性4例,女性11例,年齡19-62歲,平均43.3歲;病程1月-10余年;15例患者術(shù)前均行全脊柱/脊髓MRI及頭頸部3D-CT檢查,并行血管成像檢查,了解周圍血管走形。所有患者均合并寰樞椎脫位,齒狀突陷入顱內(nèi),13例合并脊髓空洞。根據(jù)術(shù)前臨床癥狀及影像學(xué)表現(xiàn)是否存在小腦扁桃體下疝超過10mm,明顯延髓變性;MRI顯示四腦室流出道顯著狹窄或合并腦積水者及頭頸交界區(qū)蛛網(wǎng)膜下腔閉塞,后組顱神經(jīng)癥狀嚴(yán)重或呼吸心跳異常考慮與腦干受壓相關(guān)等情況采取不同減壓方式。其中6例采用枕大池成形(包括寰枕減壓,下疝小腦扁桃體切除及枕大池硬/脊膜擴大成形),9例采用后顱窩減壓術(shù)(PFD)。所有患者均采用直接復(fù)位內(nèi)固定術(shù)對不穩(wěn)定型顱底凹陷進行復(fù)位、固定。術(shù)后1年定期隨訪了解患者入院自覺癥狀恢復(fù)情況,以日本骨科協(xié)會(JOA)評分對患者對神經(jīng)功能進行評分,通過寰齒間距(ADL),齒狀突超過錢氏線距離(CL),延髓頸髓角(CMA)及脊髓空洞最大層面空洞大小等復(fù)查指標(biāo)對手術(shù)效果進行綜合評價。結(jié)果:本組所有患者均采用后路減壓及復(fù)位內(nèi)固定術(shù),術(shù)后無患者出現(xiàn)神經(jīng)功能惡化,無死亡病例。患者主要自覺癥狀頭痛消失7例(7/10,70%),3例(3/7,30%)好轉(zhuǎn);頭暈消失6例(6/7,85.7%),好轉(zhuǎn)1例(1/7,14.3%);肢體乏力消失2例(2/11,18.2%),好轉(zhuǎn)8例(8/11,72.7%),無改善1例(1/11,9.1%);肢體麻木消失4例,4/10,40%),好轉(zhuǎn)6例(6/10,60%);10(66.7%)例神經(jīng)功能預(yù)后良好,4(26.7%)例中效,1(6.7%)例無效。JOA評分由術(shù)前10.47±2.23分提高到術(shù)后13.67±1.54分,差異有統(tǒng)計學(xué)意義(P0.05)。齒狀突超過Chamberlain線距離術(shù)后平均6.07±1.36mm較術(shù)前11.19±2.70下降,寰齒間隙術(shù)后3.44±1.90mm較術(shù)前4.76±2.11mm縮窄,延頸角術(shù)后136.94±7.46°較術(shù)前126.43±7.90°增大,差異有統(tǒng)計學(xué)意義(P0.05)。13例合并脊髓空洞病例中2例(15.4%)脊髓空洞消失,9例(69.2%)較前縮小,2例(15.4%)無明顯變化。并發(fā)癥:1例行枕大池成形術(shù)患者術(shù)后傷口積液,腦脊液漏,經(jīng)持續(xù)腰池引流及加強換藥后傷口愈合出院,隨訪無復(fù)發(fā)。結(jié)論:1.后路減壓復(fù)位內(nèi)固定術(shù)在ACM I型并不穩(wěn)定顱底凹陷癥治療中能改善患者預(yù)后;2.直接后路撐開復(fù)位內(nèi)固定能改善患者寰樞椎不穩(wěn),有效擴大頭頸交界區(qū)椎管容積,緩解蛛網(wǎng)膜下腔受壓,改善局部腦脊液循環(huán);3.根據(jù)ACM I型并不穩(wěn)定型顱底凹陷患者術(shù)前臨床癥狀及影像學(xué)表現(xiàn)區(qū)別手術(shù)方式能在既改善患者預(yù)后同時減小手術(shù)并發(fā)癥的發(fā)生率;
[Abstract]:Objective: to investigate the effect of posterior decompression and reduction and internal fixation on unstable cranial base depression of type I Chiari malformation. Methods: from April 2013 to January 2016, 15 patients with unstable cranial base depression of ACMI were treated by posterior decompression and occipitocervical internal fixation from April 2013 to January 2016, including 4 males and 11 females, aged 19-62 years with an average of 43.3 years. Fifteen patients with disease course from 1 month to more than 10 years underwent MRI of whole spine / spinal cord and 3D-CT of head and neck before operation. Angiography was performed to understand the shape of peripheral blood vessels. All patients were complicated with atlantoaxial dislocation and 13 cases with syringomyelia. According to the clinical symptoms and imaging findings before operation, there was a cerebellar subtonsillar hernia more than 10 mm. The MRI showed that the outflow tract of the fourth ventricle was significantly narrow or complicated with hydrocephalus and subarachnoid space occlusion in the junction of head and neck. In the posterior group, severe cranial nerve symptoms or abnormal respiration and heartbeat were related to brainstem compression, and different decompression methods were adopted. Among them, 6 cases were treated with occipital cisternoplasty (including atlantooccipital decompression, resection of inferior cerebellar tonsillectomy and enlarged occipital cistern dura / meningoplasty). 9 cases were treated with posterior cranial fossa decompression. All patients were treated with direct reduction and internal fixation. One year after operation, the patients were followed up regularly to find out the recovery of the patients' conscious symptoms, and the neurologic function was evaluated by the Japanese Orthopaedic Association (JOAA) score. The operative results were evaluated comprehensively by means of atlantoid distance (ADL), distance of odontoid process beyond Qian's line (CLA), medullary cervical spinal cord angle (CMA) and the size of syringomyelia. Results: all the patients were treated with posterior decompression and internal fixation. The main symptoms of the patients disappeared. 7 cases had 7 / 10 / 70% of headache disappeared and 3 cases had 3 / 7 / 7 / 30% of the symptoms. Dizziness disappeared in 6 / 7 / 85.7 cases, improvement in 1 / 7 / 714.3C in 1 / 1; loss of limb fatigue in 2 / 1118.2J; improvement in 8 / 8 / 1172.7m; no improvement in 1 / 1 / 1172.7m; loss of limb numbness in 4 / 4 / 10 / 1040m; improvement in 6 / 61066.7m / 61066.7a) in which the neurologic function was good (426.66.7e).) the prognosis of the patients was 10.47 鹵2.23 (n = 10.47 鹵2.23). (JOA score was 10.47 鹵2.23% before operation, and the prognosis of the patients was as follows: 10.47 鹵2.23%; 10.47 鹵2.23%; 10.47 鹵2.23 cases; 10.47 鹵2.23 cases) The score increased to 13.67 鹵1.54 after operation. The difference was statistically significant (P 0.05). The average distance between odontoid process and Chamberlain line was 6.07 鹵1.36mm, 3.44 鹵1.90mm, 136.94 鹵7.46 擄and 126.43 鹵7.90 擄, respectively. The difference was statistically significant (P 0.05. 05%. 13 cases with syringomyelia: 2 cases (15. 4) the syringomyelia disappeared in 9 cases (69.2%), and there was no significant change compared with the former 2 cases (15. 4%). Complications: 1 case of occipital cisternoplasty received wound effusion and cerebrospinal fluid leakage. The wound healed and discharged after continuous lumbar cistern drainage and enhanced dressing change. No recurrence was found during follow-up. Conclusion 1. Posterior decompression and reduction and internal fixation can improve the prognosis of patients with ACM I unstable skull base depression. Direct posterior open reduction and internal fixation can improve the atlantoaxial instability, enlarge the volume of spinal canal effectively, relieve the pressure of subarachnoid space, and improve the circulation of local cerebrospinal fluid (CSF). According to the preoperative clinical symptoms and imaging findings of patients with unstable skull base depression of ACM type I, the operative methods can not only improve the prognosis of patients but also reduce the incidence of surgical complications.
【學(xué)位授予單位】：西南醫(yī)科大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2017
【分類號】：R651.1

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