Fibrous Dysplasia with Aneurysmal Bone Cyst Presenting as Painful Solitary Skull lesion

We report a rare case of fibrous dysplasia with the development of a secondary aneurysmal bone cyst presenting as solitary tumor of calvarium. Although fibrous dysplasia with aneurysmal bone cyst is rare, it should be taken into account in differential diagnosis of the osteolytic solitary skull lesion.     

Clinical and radiological observation in a surgical series of 36 cases of fibrous dysplasia of the skull

Objectives

To describe the clinical and radiological findings in a consecutive series of patients diagnosed with fibrous dysplasia of the skull.

Patients and methods

A retrospective analysis of collected data for 36 patients with histopathologically confirmed fibrous dysplasia involving the skull is presented. The demographic data, clinical presentation, radiographic characteristics, and the management of these patients were reviewed.

Results

All 36 patients in this review were diagnosed with fibrous dysplasia involving at least part of the skull. In this study, the most commonly involved area of the skull was the frontal bone (52.78% of patients). The next most common area of skull was the temporal bone (30.56% of patients), followed by the sphenoid bone (25% of patients), the parietal bone (19.44% of patients), and orbital bone (13.89% of patients). The principal clinical presentation included headache, local lump, exophthalmos, visual disorder, cranial nerve paralysis, and facial malformation. These patients were treated by surgical treatment, and several of our patients underwent various degrees of reconstruction to optimize function.

Conclusions

Effective surgical treatment may improve the short-term outcome in these patients, and a “tailored” surgical approach is necessary.     

Cervical Fibrous Dysplasia Presenting as a Pathologic Fracture in an Older Patient

Vertebral involvement of fibrous dysplasia (FD) is rare, especially in the cervical spine. Moreover, cervical FD presenting as a pathologic fracture in older patients is extremely rare. We report a case of symptomatic cervical FD associated with pathologic fracture in a 63-year-old man. The patient presented with progressive weakness of the left arm and pain in the shoulder and arm. Radiologic studies revealed a collapsed and typical ''ground glass'' radiolucency of C4. Multiple lytic lesions involved the odontoid process of C2 and the body, left pedicle, and posterior elements of C4. Combined anterior and posterior decompression and reconstruction were performed. Post-operatively, the histopathologic examination confirmed FD. On the post-operative follow-up examination, the neurologic deficits had completely resolved.     

经颅显微手术治疗额-眶骨纤维结构不良致视神经管狭窄

目的评价经颅显微视神经管减压术在治疗及预防额-眶骨纤维结构不良致视神经管狭窄引起视力下降中的作用及其手术适应证和时机。方法对18例（20侧）因额-眶骨纤维结构不良致视神经管狭窄的患者,进行单侧视神经管减压术16例,双侧视神经管减压术2例,其中治疗性手术14例（15侧）,预防性手术4例（5侧）。结果18例均于术后随访1年9个月至10年,平均34个月。治疗性手术后视力改善3只眼,视力稳定在术前水平10只眼,视力下降2只眼,手术有效率为87%（13/15）;预防性手术后视力均稳定在术前水平,手术有效率为100%（5/5）。结论经颅显微视神经管减压术是治疗及预防额-眶骨纤维结构不良致视力下降的有效方法,对于治疗性手术而言,早期手术视力可望改善。     

前入路的斜坡侧壁显微解剖研究与临床应用

目的 为前方经斜坡入路提供斜坡侧壁重要结构的显微解剖参数和显露方法。方法 对20具成人头颅标本，仿Janecka的标准面部移位入路切口(左侧)行两侧斜坡侧壁结构显微解剖观察和测量，并初步探讨颞肌瓣转移重建斜坡硬膜的可行性。结果 上斜坡侧壁主要结构有视神经管隆起和颈内动脉隆起；中斜坡侧壁重要结构有颈内动脉岩骨段、岩下窦和外展神经经过的Dorello管，循翼管向后方可安全确认颈内动脉岩骨段；下斜坡侧壁首要结构为椎动脉和后组颅神经。颞肌瓣转移可有助于斜坡硬膜重建。结论 掌握斜坡侧壁的显微解剖和重要结构的显露方法，有助于经斜坡入路的侧方扩展。颞肌瓣转移重建斜坡硬膜具有可行性。     

Kawase入路切除上斜坡区肿瘤

目的　评价经Kawase入路切除上斜坡区肿瘤的适用性。方法　应用Kawase入路对 2 1例上斜坡区肿瘤患者进行了显微外科手术。结果　 2 1例中有 6例脑膜瘤和 8例三叉神经鞘瘤获得全切 ,4例脑膜瘤和 3例三叉神经鞘瘤获得次全切。术后 2例患者出现永久性动眼神经瘫 ,12例出现暂时性颅神经损害。结论　Kawase入路适用于处理上斜坡区肿瘤。该入路具有如下优点 :(1)可直接进入桥前池及其上方 ,无需牵拉小脑 ;(2 )可同时处理中、后颅凹的病变 ,路径较短 ;(3)在切除肿瘤之前即可分离出小脑幕动脉 ,阻断肿瘤部分血运 ;(4)保留听力 ;(5 )Labb啨静脉并发症较少     

Relief of severe retro-orbital pain and vision improvement after optic-nerve decompression in polyostotic fibrous dysplasia: case report and review of the literature

INTRODUCTION: We describe a case of a 9-year-old girl who developed progressive severe retro-orbital pain and partial visual loss in association with left optic-nerve compression due to polyostotic fibrous dysplasia of the skull. MATERIALS AND METHODS: Intradural decompression of the optic nerve resulted in immediate and complete resolution of the pain as well as a vast visual improvement. CONCLUSION: In cases of fibrous dysplasia of the skull with evidence of optic-nerve compression, relief of retro-orbital pain should be an additional consideration when deciding to proceed with surgical management.     

Sixth Cranial Nerve Palsy Caused by Gastric Adenocarcinoma Metastasis to the Clivus

Tumors of the clivus and metastases to the clivus are very rare. Metastasis involving the clivus has previously been described in only two case reports. In skull metastasis, the breast and prostate are the most common primary foci, while metastasis from gastric carcinoma is extremely rare. A review of the English literature revealed only one published case of clivus metastases from gastric adenocarcinoma. There is no literature thoroughly explaining the differential diagnosis between chordoma and metastasis. Here we report a rare case of metastasis to the clivus from a gastric adenocarcinoma in a 42-year-old female patient with sudden blurry vision, presenting as bilateral cranial nerve VI palsy.     

枕后极外侧经髁入路术式和显微解剖

目的：探讨枕下极外侧经髁手术对颅颈交界腹侧区肿瘤切除的适应证及手术方法。方法：结合临床显微解剖讨论分析我科6例颅颈交界区肿瘤手术的情况。结果：6例颅颈交界区肿瘤采用枕下远外侧经髁术式,除一例髓内肿瘤右上肢肌力无恢复外,余5例均取得满意临床效果。结论：对比其他传统入路,经髁术式有入路短,显露清楚,脑干牵拉轻,对周围结构保护好等优点,是此区大部分肿瘤切除的首选入路。     

Anatomical progression of the Chiari II malformation

To evaluate whether anatomic change of the relationship of the Chiari II malformation and the cranial base was occurring, 22 children with meningomyelocele had serial MRI scans reviewed. A ratio (B/A) was established between the distance from the foramen magnum to the caudalmost portion of herniated cerebellum (B) and the diameter of the foramen magnum (A) and this ratio was compared on serial MRI scans. Eighteen children had an increase in the B/A ratio, two children had a decrease, and two had no change. This indicates that continuous anatomic change of the Chiari II malformation and the skull base is occurring. Clinical deterioration in the older child may be explained by a combination of compressive and traction forces due to this change.     

The retrosigmoid approach to petroclival meningioma surgery

Petroclival meningiomas are technically challenging lesions. The authors retrospectively analyzed their experience between 2000 and 2010 in 82 patients with petroclival meningioma to evaluate changes in management strategy. A total of 42 patients (51%) were treated via the retrosigmoid approach. The patients received postoperative neurological and neuroradiological follow-up. The maximum diameter of the tumors ranged from 1.5 cm to 6.5 cm (mean, 3.8 cm). Gross total resection (Simpson Grade II) was achieved in 27 patients (64%), subtotal resection (Simpson Grade III) in 11 (26%), and partial removal (Simpson Grade IV) in four (9.5%). Ten patients (24%) had new neurological deficits or worsening of pre-existing deficits. One patient (2%) died because of brainstem dysfunction after surgery. The retrosigmoid approach is suitable for treatment of selected petroclival meningioma if the main part of the tumor is located in the posterior fossa in the cerebellopontine angle and the low clivus, and only a minor part of the tumor extends to the posterior wall of the cavernous sinus. This approach provides a low degree of surgical difficulty and a low complication rate.     

显微手术治疗额-眶-蝶骨纤维异常增殖症

目的 探讨额-眶-蝶骨纤维异常增殖症的手术指征,评价一期进行病变骨切除+患侧视神经管减压+计算机塑形钛网颅骨缺损修补术的手术效果.方法 回顾性分析2007~2010年收治的15例额-眶-蝶骨纤维异常增殖症患者的临床资料,均通过一期进行病变骨切除+患侧视神经管减压+计算机塑形钛网颅骨缺损修补术治疗.结果 全部病例的病变骨质病理学检查结果均为骨纤维性结构不良.术后随访1~4年,15例患者20只视力受影响的眼中9只视力改善,11只视力稳定,无视力减退及明显副损伤;患者面容皆明显改观,效果满意.结论 累及额-眶-蝶骨的颅骨纤维异常增殖症伴视力下降的患者应尽早手术治疗,一期行病变骨切除+患侧视神经管减压+计算机塑形钛网颅骨缺损修补术,可有效防止视力下降、美观面容.     

经鼻蝶入路至斜坡区的内镜解剖学研究

目的 通过对经鼻蝶入路至斜坡区的内镜解剖研究，为临床提供形态学依据。方法测量10例成人干颅骨标本的解剖数据；在10例经甲醛固定的成人尸头标本上模拟经鼻蝶手术入路，用内镜对斜坡区进行解剖观察。结果 前鼻棘至蝶窦口、鞍底、鞍结节中心、鞍背中心、颈动脉管、视神经管颅口和枕骨大孔前端的距离分别为（55．62±4．23）mm、（71．50±6．21）mm、（79．02±4．97）mm、（81．04±8．02）mm、（68．39±6．42）mm、（67．96±645）mm、（91．08±5．20）mm；以鼻腔底平面为基线，与上述结构之间矢状面夹角分别为（32．09±3．90）°、（31．34±6．02）°、（35．02±4．03）°、（34．65±3．23）°、（32．01±3．02）°（36．55±5．20）°、（39．04±8．02）°；卵圆孔、破裂孔、颈内动脉管外口、舌下神经管外口内侧缘距正中线的距离分别为（23．30±1．97）mm、（10．56±2．39）mm、（25．07±2．77）mm、（16．98±1．99）mm。经鼻蝶入路至斜坡区在内镜下硬膜外可显露从鞍底到斜坡、枕骨大孔的中线附近结构。在硬膜下可显露基底动脉分叉、动眼神经、脑干腹侧等结构。结论 内镜下行经蝶入路可良好显露鞍后-斜坡解剖结构，适用于鞍后-斜坡区病变的手术。     

Endoscopic repair technique for traumatic penetrating injuries of the clivus

Unlike basilar skull fractures, penetrating traumatic injuries to the clivus are uncommon. We present two novel and interesting cases of traumatic crossbow arrow injury and penetrating screwdriver injury to the clivus. A review of the literature describing methods to repair these injuries was performed. A careful, systematic approach is required when working up and treating these injuries, as airway preservation is critical. An adaptation to the previously described “gasket-seal” method for skull base repair was utilized to repair the traumatic cerebrospinal fluid (CSF) fistulas. This repair technique is unique in that it is tailored to a much smaller defect than typical post-surgical defects. Two patients are presented, one with a post-traumatic CSF fistula after penetrating crossbow injury to the clivus and one with a penetrating screwdriver injury to the clivus. The patients were treated successfully with transnasal endoscopic repair with fascia lata graft and a nasoseptal flap, a novel adaptation to the previously described “gasket-seal” technique of skull base repair.     

原发颅底脊索瘤的临床误诊病例分析

目的探讨原发颅底脊索瘤临床误诊的临床特点及影像学特征,以期提高原发颅底脊索瘤的临床诊断准确率。方法回顾性纳入2005年2月至2014年12月首都医科大学附属北京天坛医院神经外科连续收治的155例颅底脊索瘤患者。测量磁共振他加权和T1增强序列中肿瘤信号强度与脑干信号强度的比值(RT2和REN),比较误诊组(50例)和对照组(105例)患者的一般资料、影像学及病理学特征,进而依据肿瘤的部位和常见的误诊肿瘤特征进行横向比较分析。结果误诊组的50例颅底脊索瘤中,19例误诊为神经鞘瘤,12例误诊为表皮样囊肿,8例误诊为岩斜脑膜瘤,5例误诊为其他骨肿瘤,3例误诊为垂体腺瘤,3例误诊为海绵窦海绵状血管畸形。误诊组的RT2和REN中位数(四分位间距)分别为2.49(0.79)和0.99(0.77),与对照组相比差异元统计学意义(P>0.05);但误诊组和对照组间肿瘤的部位和骨质浸润程度的差异均有统计学意义(均P<0.001)。多因素logistic回归显示,与蝶枕部相比,蝶岩部(P=0.001)和岩枕部(P=0.008)的脊索瘤易导致误诊;与弥散内生型相比,外生型脊索瘤更容易导致误诊(P<0.001)。结论颅底脊索瘤的影像学表现异质性较大,发生于侧颅底和外生型脊索瘤容易被临床误诊,最常见的易混淆肿瘤为侧颅底神经鞘瘤和环池表皮样囊肿。磁共振信号强度比值可以提供定量参考,但其在脊索瘤的诊断和鉴别诊断中的意义仍需进一步探索。     

颅骨骨纤维结构不良的诊断和治疗

目的探讨颅骨骨纤维结构不良的诊断和治疗。方法回顾性分析33例经病理证实为颅骨骨纤维结构不良病例的临床资料．探讨其手术原则及其疗效。结果33例颅骨骨纤维结构不良患者中,男13例,女20例;年龄6-58岁,其中小于18岁者15例。该病常见累及额骨、筛骨、蝶骨,其主要临床表现为头痛、局部肿物、眼球突出、视力障碍、面部畸形等。X线平片及CT检查主要表现为局部骨密度增高,出现占位效应。病灶位于颅骨一侧皮质者,边界清楚,可有硬化;而病灶在骨髓腔内者,则多边界不清。CT增强扫描可见病灶明显强化,密度不均匀。MRI检查骨纤维结构不良信号呈多样性,无特异性,因此MRI对其的诊断意义不大。术后随访．33例患者经手术治疗后临床症状均有改善,2例复发。结论该病多见于儿童和青少年,女性较男性多见。外科手术为颅骨骨纤维结构不良的主要治疗手段,早期积极手术可使骨质愈合良好,缓解症状,改善预后。     

Radiological approach to basilar invagination type B: Reliability and accuracy

ObjectivesThis study aims to determine the reliability of the radiological tests used in the diagnosis of basilar invagination (BI).MethodsPatients diagnosed with type B basilar invagination, who had both magnetic resonance (MR) and computed tomography (CT) imaging between January 2014 and November 2019 were included in this retrospective reliability study. In this study, distance from odontoid apex to Chamberlain’s line (OA-CL) was accepted as a reference method for the diagnosis. Forty-two BI cases and 79 controls were included. Three radiologists with different levels of experience individually evaluated OA-CL, Boogard’s angle (BoA), clivo-axial angle (CXA), clivo-dens angle (CDA), and clivo-palate angle (CPA) on midsagittal CT and MR images. Statistical analysis was made with the intraclass correlation coefficient (ICC), t-test, and receiver operating characteristic (ROC) curve.ResultsThe ICC for CT and MR were; 0.977?0.973 (OA-CL), 0.912?0.882 (BoA), 0.845?0.846 (CXA), 0.862?0.864 (CDA), and 0.762?0.747 (CPA) respectively (P < 0.001). The areas under the ROC curve were 0.977 (BoA), 0.832 (CXA), 0.852 (CDA), and 0.719 (CPA) (P < 0.001). The cut-off measures were ≥137.84° (BoA), ≤149.25° (CXA), ≤129.58° (CDA), and ≤61.83° (CPA). The diagnostic accuracies were 0.954 (BoA), 0.664 (CXA), 0.704 (CDA), 0.438 (CPA) (P < 0.001).ConclusionsOA-CL and BoA express excellent inter-rater agreement than CXA, CDA, and CPA, which are limited due to morphological variations and head spatial position. BoA is the second most reliable diagnostic test. CXA, CDA, should only be used for complementary information. CPA was found inadequate for the diagnosis of BI.?     

Fibrous dysplasia in combination with aneurysmal bone cyst presenting as a subarachnoid haemorrhage

Abstract Fibrous dysplasia (FD) is a rare tumour, representing 2.5% of all bone tumours and 7% of benign tumours. Aneurysmal bone cyst (ABC) is also an uncommon pathology, usually associated with a secondary vascular lesion consisting of an arteriovenous malformation. In this article, we relate a case report of a young female with a rare combination of FD with aneurysmal bone cyst presenting as a subarachnoid haemorrhage (SAH). Despite the possibility of clinical treatment of these lesions, this report demonstrates that symptomatic lesions may be successfully managed by surgical resection. The authors’ opinion is that this treatment should be individualised depending on particularities of each case, such as localisation of the lesion, biopsy results and image exams features. We also present a critical literature review of diagnostic methods and therapeutical options for both ABC and FD, with emphasis on controversial topics surrounding these issues.     

ASA-610V导航系统在微侵袭神经外科手术中的应用

目的 探讨ASA-610V导航系统在微侵袭神经外科手术中的应用效果。方法 应用ASA-610V神经外科导航系统施行各类显微神经外科手术89例,其中颅内肿瘤70例,炎性病灶12例,血管畸形7例。10例运动功能区肿瘤患者施行功能性磁共振引导下肿瘤切除导航手术。结果 系统注册误差为2.5mm左右。病灶全切除者59例(66.29％),次全及大部切除者18例(20.22％),部分切除者12例(13.48％)。80例患者预后良好,9例术后出现轻微并发症。10例运动功能区肿瘤患者均行全切除手术,术后无一例神经功能障碍加重。结论 ASA-610V导航系统在神经外科手术中定位准确,尤其对于位于颅底和功能区附近的病变,有助于提高手术切除率,降低手术并发症。