Imaging characteristics, misdiagnosis and microsurgical outcomes of patients with spinal dural arteriovenous fistula: a retrospective study of 32 patients.

Background: Spinal dural arteriovenous fistula (SDAVF) is an extremely rare spinal vascular malformation. As SDAVF exhibits no specific clinical manifestations nor diverse imaging results, it is easily misdiagnosed, resulting in delayed treatment and irreversible neurological damage. Most patients were initially misdiagnosed, but there were few reports on reducing misdiagnosis. Methods: A total of 32 consecutive patients, who presented to our institution (Shanghai Deji Hospital) with SDAVF between June 2013 and January 2016 were retrospectively analyzed. Data were collected on demographics, clinical presentation, imaging findings, follow-up, and clinical outcomes. The Aminoff-Logue scale (ALS) was used to assess clinical outcomes. Results: Of the 32 enrolled patients (3 females, mean age 59.1±3.8 years), 23 patients (71.9%) were misdiagnosed as acute myelitis (11 patients), intramedullary tumors (6 patients), lumbar disc herniation (4 patients), and other conditions (2 patients). All patients underwent surgical procedures under electrophysiological monitoring. Fistulas were found in all 32 patients and were successfully occluded. The mean follow-up period was 19.22±8.21 months (ranging from 2 weeks to 30 months). One year later, 20 patients underwent magnetic resonance imaging (MRI), and 14 showed no T2 edema, and the edema was relieved in 6 patients. A total of 10 patients underwent enhancement MRI and no enhancement signs were detected. Among the 27 patients with long-time follow-up, the fistula had no residual or recurrence, 21 patients showed decreased ALS scores (P<0.05). Six patients exhibited nonsignificant improvement. No aggravating patient was found. Prognosis differed significantly between patients with ALS <6 and those with ALS ≥6 (P<0.05). Conclusions: Spinal angiography should be performed with full intubation, and microcatheter angiography can reduce misdiagnosis. SDAVF must be differentiated from acute myelitis, intramedullary tumor, and other spinal vascular malformations. Microsurgical treatment is effective with a low recurrence rate.

A novel functional polymorphism of GFAP decrease glioblastoma susceptibility through inhibiting the binding of miR-139.

Glioblastoma (GBM) is the most commonly diagnosed solid tumor outside the central nervous system. However, genetic factors underlying GBM remain largely unclear. Previous studies indicated that Glial fibrillary acidic protein (GFAP) might play an important role in the aggressiveness of GBM and also contributed to its poor overall survival. The present study aims to test (1) the associations between GFAP single nucleotide polymorphisms (SNPs) and GBM cells chemoresistance and metastasis, and (2) the molecular mechanism accounting for their effects. Four tagging SNPs of GFAP were initially genotyped in 667 subjects and the significant SNP was further analyzed via online bioinformatical tools. SNP rs11558961 was found to be significantly associated with GBM susceptibility. It was predicted to influence microRNA(miR)-139 binding to 3'UTR of GFAP gene. In functional experiments, we found that cells transfected with rs11558961 G-allele constructs had lower baseline luciferase activities and were more responsive to miR-139 changes, compared to C-allele constructs. Moreover, rs11558961 C>G variant reduced the chemoresistance of GBM cells and migration capability. In conclusion, rs11558961 might influence the chemoresistance and progression of GBM cells via promoting the binding of miR-139, ultimately decrease the susceptibility of GBM. This investigation will shed light on the optimizing for clinical trial design and individualizing of therapeutic plans.

Extraventricular neurocytoma of the sellar region: case report and literature review.

INTRODUCTION: The extraventricular neurocytoma of the sellar region (EVNSR) is a rare disease, it is difficult to make exact diagnosis of and operate on patients. Retrospectively analysed the clinical manifestations, image features, therapy methods and outcomes among patients with EVNSR, to investigate the epidemiological characteristics, image features, diagnosis, treatment and prognosis. CASE DESCRIPTION: A 25-year-old man man with 7-month worsening vision of left eye, was confirmed EVNSR after subtotally resection from the neurosurgical department of Deji hospital. DISCUSSION AND EVALUATION: Nine cases of EVNSR were reported from this article and elsewhere. Ages of these patients were ranging from 25 to 66 (with an average of 45.67). The male-female ratio was 1-2. All EVNSR patients had visual damage. Images showed the tumors were in the sellar and suprasellar regions. Preoperatively, all patients were misdiagnosed as other diseases: such as pituitary tumor, craniopharyngioma, and meningioma. For tumor removal treatment, five patients received transpterional approach, one received subfrontal approach and three received transnostril-transsphenoidal approach. EVNSR was confirmed by pathological tests. The tumor was completely removed in one patient. During the 12-24 month postoperative follow up period, the recurrence or metastasis of the tumor was found in two patients. CONCLUSIONS: EVNSR is a rare disease. It occurs mostly in middle-aged women. EVNSR is likely to be misdiagnosed as pituitary adenoma preoperatively. The histological examination would help confirm the diagnosis. Using transpterional approach to remove tumor will help the prognosis, especially among patients with normal pituitary function. Both postoperative radiotherapy and long-term follow-up are recommended.

A spinal tumor showing mixed features of ependymoma and hemangioblastoma: a case report and literature review.

We report an intramedullary spinal tumor consisting of an ependymoma and a hemangioblastoma (HB). A 37-year-old woman presented with progressive bilateral lower limb sensory and motor deficits. Magnetic resonance imaging showed a single intramedullary mass in the thoracic cord (T4-T6 level). Clinically, the patient had no von Hippel-Lindau disease and neurofibromatosis type 2. Metastatic carcinomas including renal cell carcinoma were altogether negative. Complete surgical resection was performed. Histologically, the tumor consisted of a mixed ependymoma and HB. Tumor cells of ependymoma displayed a rather uniform appearance with round to oval nuclei having salt-and-pepper-like chromatin, forming perivascular pseudorosette structures with radially arranged, tapering cell processes extending to intratumoral blood vessels. Stromal cells of HB had vacuolated or homogeneously eosinophilic cytoplasm and variable sized hyperchromatic nuclei within a background of capillaries. Immunohistochemically, tumor cells of ependymoma were strongly positive for glial fibrillary acidic protein (GFAP), focally positive for epithelial membrane antigen (EMA) and D2-40 in a dot-like or ring-like pattern. Stromal cells of HB showed immunoreactivity for S100, vimentin, inhibin-α, D2-40, EMA and cytokeratins (CK: AE1/AE3, CK19). A review of the literature, in conjunction with the present case, shows that ependymomas and HBs may have a close relationship with each other.

Anatomical study of suboccipital vertebral arteries and surrounding bony structures using virtual reality technology.

BACKGROUND: This work aimed to evaluate the efficacy of virtual reality (VR) technology in neurosurgical anatomy through a comparison of the virtual 3D microanatomy of the suboccipital vertebral arteries and their bony structures as part of the resection of tumors in the craniovertebral junction (CVJ) of 20 patients compared to the actual microanatomy of the vertebral arteries of 15 cadaveric headsets. MATERIAL AND METHODS: The study was conducted with 2 groups of data: a VR group composed of 20 clinical cases and a physical body group (PB group) composed of 15 cadaveric headsets. In the VR group, the dissection and measurements of the vertebral arteries were simulated on a Dextroscope. In the PB group, the vertebral arteries in the cadaver heads were examined under a microscope and anatomical measurements of VA and bony structures were performed. The length and course of the vertebral arteries and its surrounding bony structures in each group were compared. RESULTS: The distances from the inferior part of the transverse process foramen (TPF) of C1 to the inferior part of TPF of C2 were 17.68±2.86 mm and 18.4±1.82 mm in the PB and VR groups, respectively. The distances between the middle point of the posterior arch of the atlas and the medial intersection of VA on the groove were 17.35±2.23 mm in the PB group and 18.13±2.58 mm in the VR group. The distances between the middle line and the entrance of VA to the lower rim of TPF of Atlas were 28.64±2.67 mm in PB group and 29.23±2.89 mm in VR group. The diameters of the vertebral artery (VA) at the end of the groove and foramen of C2 transverse process were 4.02±046 mm and 4.25±0.51 mm, respectively, in the PB group and 3.54±0.44 mm and 4.47±0.62 mm, respectively, in VR group. The distances between the VA lumen center and midline of the foramen magnum at the level of dural penetration was 10.4±1.13 mm in the PB group and 11.5±1.34 mm in the VR group (P>0.05). CONCLUSIONS: VR technology can accurately simulate the anatomical features of the suboccipital vertebral arteries and their bony structures, which facilitates the planning of individual surgeries in the CVJ.

Anatomical studies on the temporal bridging veins with Dextroscope and its application in tumor surgery across the middle and posterior fossa.

OBJECTIVE: To evaluate the application of virtual reality technology in neurosurgical anatomy we compared the virtual three-dimensional (3D) microanatomy of the temporal bridging veins as part of the resection of tumors across the petrosal crest in 25 patients against the actual microanatomy of the temporal bridging veins on 20 cadaveric head sets. PATIENTS AND METHODS: The experiment was carried out by two groups of data: a virtual group made of 25 clinical cases and a physical body group made of 20 cadaveric head sets. In the physical body group, the temporal bridging veins on the cadaveric heads were examined under the microscope from the number of their tributaries to termination and measure the distance between emptying point on sinus of labbé vein and sigmoid transverse sinus. In the virtual group, the dissection of the temporal bridging veins was simulated on Dextroscope. We compared the anatomical features of temporal bridging veins in two groups. Virtual reality technology was used in the planning of 25 cases for which the anatomy of temporal bridging veins needed to be studied carefully by the neurosurgical team. RESULTS: Four basic configurations of veins were found: venous lakes running in the tentorium before emptying the sinuses 17.5% (7 hemispheres), candelabra of veins uniting to form one large drainage 40% (16 hemispheres), single independent draining veins 20% (8 hemispheres) and multiple independent draining veins 22.5% (9 hemispheres) in physical body group, venous lake16% (8 hemispheres), candelabra of veins uniting to form one large drainage 42% (21 hemispheres), single independent draining veins 18% (9 hemispheres) and multiple independent draining veins 24% (12 hemispheres) in virtual group. 213 tributaries (85 drainage to labbé veins) and 87 terminations of temporal bridging veins were found in cadaveric heads, whereas 167 tributaries (80 to labbé veins) and 81 terminations of temporal bridging veins were found in the virtual group. The distribution of anatomical terminations of temporal bridging veins were divided into three groups: transverse area 52.87% (46) tentorium area 24.13% (21) and petrosal area 23.10% (20) in physical body group, whereas 54.35% (50) 23.91% (22) and 21.74% (20) in virtual group, respectively. The proportion of fore-placed type veins of labbé is 7.69% in physical body group and 8.33% in virtual group (P>0.05). The distance from the emptying point of the labbé veins to the sigmoid transverse point in the virtual group was 18.75 ± 1.95 mm, in the physical body group was 20.12 ± 2.51 mm (P>0.05). The anatomical features of labbé veins found during the operation of the 25 patients with tumors extended from middle fossa to posterior fossa and were identical to what was seen in presurgical planning. CONCLUSIONS: Virtual reality technology can accurately simulate the anatomical feature of the temporal bridging veins which facilitates the planning of individual operations in neurosurgery.

Temporal base intradural transpetrosal approach to the petoclival region: an appraisal of anatomy, operative technique and clinical experience.

BACKGROUND: Tumours in the petroclival region have been a challenge to neurosurgeons. We present a cohort of 24 patients with petroclival meningioma (PCM) and trigeminal schwannoma (TS) in the petroclival region with extension to the middle fossa which were removed with the temporal base intradural transpetrosal (TBIT) approach. METHODS: To avoid damage to the important surrounding structures in the petrosal bone, a morphometric analysis in the TBIT approach was performed in 15 cadaveric heads, and the 'safe area of intradural petrosectomy' was identified in the TBIT approach. Subsequently, 14 patients with PCM and 10 patients with TS in the petroclival region were operated on with the TBIT approach. RESULTS: There were no operative deaths in this cohort related to the surgery. Common complications included light hemiparesis in two patients (8.0%), new cranial nerve paresis in nine (37.5%), post-operative pneumonia in one (4.0%) and transient cerebrospinal fluid leak in one (4.0%). Total tumour resection was achieved in 20 patients (83.3%) and subtotal resection in 4 (16.7%). There was no tumour recurrence in all patients at follow-up with a mean duration of 37 months. CONCLUSIONS: Surgical strategy for PCM and TS in the petroclival region should be tailored to individual patients. The TBIT approach may improve the exposure of tumours in the petroclival region. A clear description of the 'safe area of intradural petrosectomy' appears to decrease the risk associated with petrosectomy procedure in the TBIT approach.

Surgical treatment for hemangioblastomas in the medulla oblongata.

PURPOSE: The purpose of this study was to evaluate the outcome of surgical treatment of hemangioblastomas in the medulla oblongata. METHODS: Between January 2006 and December 2007, 18 patients who underwent surgery for hemangioblastomas in the medulla oblongata in the Neurosurgical Department of Huashan Hospital were retrospectively reviewed. RESULT: The study population was 13 males and five females. The main symptoms were headache, cervical pain, and dizziness. All patients had preoperative and postoperative examination by MRI. There were five cystic tumors and 13 solid tumors. Tumor diameter ranged from 1 to 4.3 cm (mean, 2.6 cm). Complete tumor resection was achieved in all patients, but one patient died. Embolization was done in three patients. According to McCormick scale, postoperative condition was worse in one patient, unchanged in 14 patients, and improved in three patients. In follow-up assessments, no surviving patients remained in a worse condition. Compared with the preoperative condition, 11 patients were unchanged, and six patients exhibited improvement. Tumor recurrence was not observed during follow-up. CONCLUSION: Surgery is the first-line treatment for symptomatic patients with hemangioblastomas in the medulla oblongata. Good results can be achieved for the cystic or small solid tumors. Large solid tumors remain a surgical challenge due to arteriovenous malformation-like vascularization. Preoperative embolization is useful for large solid tumors. For asymptomatic tumors, careful long-term observation or radiosurgery could be chosen.

Trigeminal neurinomas: clinical features and surgical experience in 84 patients.

Trigeminal neurinomas are the second most common intracranial neurinomas next to the vestibular neurinomas. Eighty-four patients with trigeminal neurinomas were treated between 2003 and 2007. There were 40 women and 44 men (mean age 43 years). The most frequent symptoms were headache or numbness of the ipsilateral hemiface. There were 24 type A, nine type B, 45 type C, and six type D tumors. Dextroscope virtual reality technology was used for preoperative planning in recent eight cases. Gross total resection was achieved in 63 patients. We found that the major impediments to complete removal were adherent to the brainstem and skull base vascular structure, the frontotemporal approach with zygomatic or orbitozygomatic osteotomy or subtemporal approach could offer excellent exposure of the middle fossa and access to the posterior fossa, and Dextroscope virtual reality technology was a very useful tool to identify surgical and anatomic nuances and enhance preoperative planning in trigeminal neurinomas resection.

Anatomy of the petrosphenoidal and petrolingual ligaments at the petrous apex.

The petrous apex is a complex area surrounded by the cavernous sinus, Dorello's canal and Meckel's cave. The petrosphenoidal ligament (PSL) and the petrolingual ligament (PLL) are important structures located in the region. These two ligaments were examined under a surgical microscope in 10 specimens of five adult cadaveric heads fixed in formalin. They were found to span from the petrous apex to the posterior clinoid process, and the lingula of the sphenoid bone, respectively. The dural sleeve of the abducens nerve, the dorsal meningeal artery or its medial branch, and the venous blood space were located below the PSL in all specimens, and the petrous or sphenoidal insertion of the PSL varied in five specimens. The PLL invariably surrounded part of the dorsal and lateral walls of the lacerum segment of the internal carotid artery (ICA), just under the anteroinferior portion of the anteromedial wall of Meckel's cave in all specimens. The PSL and PLL are valuable anatomical landmarks for identifying the ICA and the nerves in this region. A thorough understanding of the relationship of the two ligaments with neurovascular structures is a prerequisite for surgery in and around the petrous apex.

[The diagnosis and surgical management for intramedullary spinal cord cavernous angioma].

OBJECTIVE: The clinical diagnosis and surgical management of intramedullary spinal cord cavernous angioma were discussed. METHOD: Total 19 patients with intramedullary cavernous angioma were analyzed retrospectively on the clinical manifestation, radiographic feature, diagnosis and differentiation, surgical technique and caution. Of all the 19 patients, averaging 38.7 years old, 14 were male and 5 were female. Nine patients were followed. RESULT: All the 19 patients pathologically diagnosed with spinal cord cavernous angioma got good surgical results, besides one patient showed loss of proprioception. Nine patients were followed up and all demonstrated improvement on neurological function. CONCLUSION: The clinical symptom of most of the patients with spinal cord cavernous angioma presented mildly at onset, but deteriorated gradually because of repeated prehemorrhage. Since the lesion showed some characteristic in MRI, MRI examination was regarded as an important diagnostic tool. Dissection should be done between the tumor and the gliosis during the surgical procedure, special attention should be paid to avoiding tumor residual. It was not necessary to aggressively evacuate the hematoma derived from tumor hemorrhage, which extended along the central canal up and down, except obvious occupied syndrome exited.

[Diagnosis and treatment of spinal cord hemangioblastoma].

OBJECTIVE: To study the diagnosis and treatment of spinal cord hemangioblastoma. METHOD: The clinical data of 42 patients with spinal cord hemangioblastoma who were operated on between 1997 and 2005 were analyzed. RESULT: Spinal cord hemangioblastoma mostly showed space occupying lesions with clear boundary and Dd-DTPA homogenous enhancement by MRI. All the 42 patients underwent complete excision. 27 patients showed improvement of their symptoms, the 6 patients failed to show any change, and neurological deficits were aggravated in the 5 patients. CONCLUSION: MRI and DSA are helpful in qualitative and localized diagnoses of the spinal cord hemangioblastoma. Surgical outcomes are favorable.

[Choice of the surgical approach to petroclival tumor].

OBJECTIVE: To discuss the reasonable choice of the surgical approach to petroclvial tumors. METHODS: The clinical data of consecutive 53 patients with the petroclival tumors, treated from June 2002 to June 2004, were reviewed to compare the different surgical approaches to pertroclival region. RESULT: Subtemporal transtentorial approach were used in 11 patients, suboccipital retrosigmoid approach in 12 patients, (transzygomatic or orbitozygomatic) frontotemporal (pterional) approach in 12 patients, presigmoid sinus approach in 2 patients, subtemporal and retrosigmoid sinus combined approach in 7 patients, subtemporal anterior petrosal extradural approach in 7 patients and extended transfrontal base extradural approach in 2 patients. Of all patients in this group, total tumor removal was achieved in 32 patients, subtotal in 9, largely partial in 12. The new cranial nerve deficit took place in 16 patients postoperatively, two patients died from coma and serious pneumonia. CONCLUSIONS: Using perfect microsurgical technique, conventional surgical approaches on petroclival region such as suboccipital retrosigmoid approach, subtemporal transtentorial approach can be suitable for most petroclival tumor with the help of neuro-navigation and neuro-endoscopy. To the epidural tumor on petroclival region, the epidural approach should be used with less invasion to the brain tissue. And to the giant petroclival tumor, the combined-tentorial approach can provide an excellent access and exposure to the tumor.

[Diagnosis and treatment of intracranial hemangiopericytoma].

OBJECTIVE: To summarize the experience in diagnosis and treatment of intracranial hemangiopericytoma (HPC). METHODS: The clinical data of 63 patients, 37 males and 26 females, aged 41 (14 - 77), with intracranial HPC were studied retrospectively. RESULTS: Fifty-eight patients underwent total excision of tumor and 5 patients underwent subtotal excision. Thirty-eight patients were followed up for 3 to 84 months (on average: 25 months), of which 34 underwent conventional external beam radiotherapy as an adjuvant measure after the first operation. Six patients who had local recurrence received second operation. Two patients had metastases to the spine. CONCLUSION: Complete excision followed with radiotherapy can delay the recurrence of HPC, a malignant tumor with rich blood supply, and improve the prognosis. Pre-operative embolization of the feeding artery may be helpful to the surgery and abundant transfusion should be ready intra-operatively.