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Bo Xiao, Fang-Fang Wu, Hong Zhang and Yan-Bin Ma

Object

When treating patients with a spontaneous supratentorial massive (≥ 70 ml) intracerebral hemorrhage (ICH), the results of surgery are gloomy. A worsening pupil response has been observed in patients preoperatively, despite blood pressure control and diuretic administration. Because open surgery needs time for decompression to occur, the authors conducted a prospective randomized study to determine whether patients who have suffered a massive ICH can benefit from a more urgently performed decompressive procedure.

Methods

Overall, 36 eligible patients admitted 6 or fewer hours post-ictus were enrolled in the study. In Group A, 12 patients underwent CT-based hematoma puncture and partial aspiration in the emergency department (ED) and subsequent evacuation via a craniectomy; in Group B, 24 patients underwent hematoma evacuation via a craniectomy only. Pupil responses were categorized into 5 grades (Grade 0, bilaterally fixed; Grade 1, unilaterally fixed with the fixed pupil > 7 mm; Grade 2, unilaterally fixed with the fixed pupil ≤ 7 mm; Grade 3, a unilaterally sluggish response; and Grade 4, a bilaterally brisk response). Grades were obtained on admission, at surgical decompression (defined as the point at which liquid hematoma began to flow out in Group A and at dural opening in Group B), and at completion of craniectomy. The Barthel Scale was used to assess survivors' functional outcome at 12 months. Comparisons were made between Groups A and B. Logistic regression analysis was used to evaluate the positive likelihood ratio of all variables for survival and function (Barthel Scale score of ≥ 35 at 12 months).

Results

Decompressive surgery was undertaken approximately 60 minutes earlier in Group A than B. A worsening pupil reflex before decompression was observed in no Group A patient and in 9 Group B patients. At the time of decompression pupil response was better in Group A than B (p < 0.05). Although only approximately one-third of the hematoma volume documented on initial CT scanning had been drained before the craniectomy in Group A, when partial aspiration was followed by craniectomy, better pupil-response results were obtained in Group A at the completion of craniectomy, and survival rate and 12-month Barthel Scale score were better as well (p < 0.05). Logistic regression analysis revealed that one variable, a minimum pupil grade of 3 at the time of decompression, had the highest predictive value for survival at 12 months (8.0, 95% CI 2.0–32.0), and a pupil grade of 4 at the same time was the most valuable predictor of a Barthel Scale score of 35 or greater at 12 months (15.0, 95% CI 1.9–120.9).

Conclusions

Patients with massive spontaneous supratentorial ICHs may benefit from more urgent surgical decompression. The results of logistic regression analysis implied that, to improve long-term functional outcome, decompression should be performed in patients before herniation occurs. Due to the fact that most of these patients have signs of herniation when presenting to the ED and because conventional surgical decompression requires time to take effect, this combination of surgical treatment provides a feasible and effective surgical option.

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Qian Zhang, Yaping Liu, Dong Zhang, Rong Wang, Yan Zhang, Shuo Wang, Lanbing Yu, Chaoxia Lu, Fang Liu, Jian Zhou, Xue Zhang and Jizong Zhao

OBJECTIVE

Moyamoya disease (MMD) is a rare, genetically heterogeneous cerebrovascular disease. The authors conducted a genetic study of really interesting new gene (RING) finger protein 213 (RNF213); actin alpha 2 (ACTA2); BRCA1/BRCA2-containing complex subunit 3 (BRCC3); and guanylate cyclase 1, soluble, alpha 3 (GUCY1A3) as well as a clinical phenotype analysis in Chinese MMD patients to determine whether genetic differences are responsible for the different clinical features that appear in MMD in different ethnicities.

METHODS

A panel was designed to identify disease-causing mutations in MMD genes and those involved in related disorders (RNF213, ACTA2, BRCC3, and GUCY1A3). The panel was used to detect disease-causing mutations in 255 Chinese MMD patients. Genotype and allele frequencies were compared between patients and 300 controls. A mutation segregation analysis was performed in 34 families, and genotype-phenotype correlations were made.

RESULTS

Twenty-seven rare missense variants of RNF213 were identified and were not found in controls. Among them, p.R4810K was identified in 31.4% of patients (80 of 255) with MMD. Significantly higher frequencies of the A allele and G/A genotype of p.R4810K were observed in MMD patients compared with controls (χ2 = 104.166, p < 0.000). Twenty-five rare variants were identified in 10.6% of patients (27 of 255) without p.R4810K variants. Segregation analysis supported an association between MMD and 3 variants. No possible disease-causing mutations were identified in ACTA2, BRCC3, or GUCY1A3. Compared with patients without the rare variants in RNF213, the p.R4810K heterozygous patients were younger at diagnosis (25 vs 29 years old, p = 0.049) and had more familial cases (24% vs 4.4%, p = 0.000), ischemic cases (81.3% vs 67.5%, p = 0.037), and involvement of the posterior cerebral artery (52% vs 32.5%, p = 0.007).

CONCLUSIONS

RNF213 is the major susceptibility gene in Chinese MMD patients. The spectrum of rare variants identified in Chinese MMD patients was diverse. Compared to patients without the rare variants in RNF213, the p.R4810K heterozygous patients exhibited different clinical features.

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Jiang Liu, Yue Yuan, Ying Fang, Li Zhang, Xiao-Li Xu, Hong-Ju Liu, Zhe Zhang and Yan-Bing Yu

OBJECT

Typical hemifacial spasm (HFS) commonly initiates from the orbicularis oculi muscle to the orbicularis oris muscle. Atypical HFS (AHFS) is different from typical HFS, in which the spasm of muscular orbicularis oris is the primary presenting symptom. The objective of this study was to analyze the sites of compression and the effectiveness of microvascular decompression (MVD) for AHFS.

METHODS

The authors retrospectively analyzed the clinical data for 12 consecutive patients who underwent MVD for AHFS between July 2008 and July 2013.

RESULTS

Postoperatively, complete remission of facial spasm was found in 10 of the 12 patients, which gradually disappeared after 2 months in 2 patients. No recurrence of spasm was observed during follow-up. Immediate postoperative facial paralysis accompanied by hearing loss occurred in 1 patient and temporary hearing loss with tinnitus in 2. All 3 patients with complications had gradual improvement during the follow-up period.

CONCLUSIONS

The authors conclude that most cases of AHFS were caused by neurovascular compression on the posterior/rostral side of the facial nerve distal to the root entry zones. MVD is a safe treatment for AHFS, but the incidence of postoperative complications, such as facial paralysis and decrease in hearing, remains high.

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Lingling Fang, Jia Huang, Qian Zhang, Raymond C. K. Chan, Rong Wang and Weiqing Wan

OBJECTIVE

Dysexecutive syndrome is common in patients with moyamoya disease (MMD), a chronic cerebrovascular disease that is characterized by stenosis of the bilateral internal carotid arteries and progressive collateral revascularization, and MMD can be classified as ischemic or hemorrhagic according to the disease presentation and history. In this study, the authors aimed to determine which aspects of executive function are impaired in patients with MMD, in addition to the specific dysexecutive functions present among its clinical subtypes and the mechanisms underlying dysexecutive function in these patients.

METHODS

The authors administered 5 typical executive function tests (the Stroop test, the Hayling Sentence Completion Test [HSCT], the verbal fluency [VF] test, the N-back test, and the Sustained Attention to Response Task [SART]) to 49 patients with MMD and 47 IQ-, age-, education-, and social status–matched healthy controls. The dysexecutive questionnaire (DEX) was also used to assess participants' subjective feelings about their executive function. A total of 39 of the patients were evaluated by CT perfusion (CTP) before the assessments were performed, and the correlations among the performances of the patients on the above tests with the parameters of cerebral blood volume, cerebral blood flow (CBF), mean transit time (MTT), and time-to-peak (TTP) in the frontal lobes of these patients were also analyzed.

RESULTS

Many aspects of executive function in the patients with MMD were significantly poorer than those in the healthy controls, and the patients performed particularly poorer on the VF test, HSCT, N-back test, and SART. The patients with hemorrhagic MMD exhibited worse executive inhibition, executive processing, and semantic inhibition compared with those with ischemic MMD, but the latter group presented a worse working memory and poorer sustained attention. There were no significant differences in the DEX scores between the patients with MMD and healthy controls. The other findings were as follows: CBF was significantly positively correlated with the number correct on part B of the HSCT (r = 0.481, p = 0.01) and accuracy on the 0-back task of the N-back (r = 0.346, p = 0.031); MTT was significantly positively correlated with accuracy on the 2-back task of the N-back (r = 0.349, p = 0.034) and factor 5 of the DEX (r = 0.359, p = 0.032); and TTP was significantly positively correlated with the number correct on part B of the HSCT (r = 0.402, p = 0.034) and the 1-back reaction time of the N-back (r = 0.356, p = 0.026).

CONCLUSIONS

The patients with MMD exhibited impairments in semantic inhibition, executive processing, working memory, and sustained attention, but they were not aware of these deficits. Moreover, differences in dysexecutive function existed between the different subtypes of MMD. Hypoperfusion of the frontal lobe may be related to working memory and semantic inhibition impairments in patients with MMD.

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Zhong Yang, Yuan Xue, Qin Dai, Chao Zhang, H. Fang Zhou, J. Feng Pan and Dan Sheng

Object

The authors introduce a novel technique to treat thoracic myelopathy caused by ossification of the ligamentum flavum (OLF): upper facet joint en bloc resection. This surgical procedure avoids surgery to the most heavily compressed cord surface, contact with the cord, and cord injury. The epidural venous plexus bleeding point can be directly seen and easily controlled during the decompression.

Methods

Between January 2007 and January 2009, thoracic myelopathy caused by OLF was diagnosed in 38 patients using plain radiography, CT, and MRI, and diagnoses were confirmed by postoperative pathological examination. All upper facet joint en bloc resection procedures were performed in 2 steps. First, the bony structures above the upper facet joint surfaces were resected and the upper facet joints were isolated. Second, en bloc resection of the upper facet joint was performed by dissection of the junction between the pedicle and upper facet joint. Intraoperative neurological monitoring was performed in all cases. The modified Japanese Orthopaedic Association (mJOA) scoring system was used to assess neurological status. The degree of postoperative expansion of the spinal cord was calculated on axial MR images. The pre- and postdecompression Cobb angle was applied to assess the magnitude of local kyphosis.

Results

Of the 38 cases of OLF, 6 were single level, 12 were double level, and 20 were multilevel. Of the 92 ossified segments in this study, 23 (25.0%) were located in the upper thoracic spine (T1–4), 13 (14.1%) were located in the midthoracic spine (T5–8), and 56 (60.9%) were located in the lower thoracic spine (T9–L1). The mean intraoperative blood loss was 340 ± 54 ml. The neurological status improved during follow-up (mean 46.1 months) from a preoperative mean mJOA score of 5.39 ± 1.52 to 8.97 ± 1.22 points (t = 18.39, p < 0.05). The neurological function recovery rate ranged from 28.6% to 100%. The mean increase in pre- and postoperative kyphosis of the involved vertebrae was only 1.3° ± 1.6°. The increase in the cross-sectional area of the dural sac at the level of maximum compression suggested that decompression was complete.

Conclusions

Upper facet joint en bloc resection is effective and may be a reasonable alternative treatment choice for thoracic myelopathy caused by OLF.

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Ming Li, Suxi Gu, Jianqiang Ni, Xiutong Fang, Xiaodong Zhu and Zhiyu Zhang

Object

The authors evaluated the effectiveness of Lenke Type 2 criteria in scoliosis correction with the segmental pedicle screw (PS) technique, with emphasis on shoulder balance.

Methods

Twenty-five consecutive patients with Lenke Type 2 scoliosis (structural double thoracic curves, sidebending Cobb angle > 25°, or T2–5 kyphosis > 20°) who underwent segmental PS instrumentation were included in this study. At surgery, the patients were an average of 14.1 years of age, and the average duration of follow-up was 2.9 years. For radiological evaluation of the patients, preoperative, postoperative, and the latest available follow-up radiographs were used. The difference between right and left shoulder heights was determined to assess shoulder balance. All patients were treated with fusion of both the proximal and distal curves.

Results

The mean preoperative proximal thoracic curve of 43° was corrected to 21° postoperatively, a 51.2% correction. The preoperative lower thoracic curve of 61° was corrected to 23°, for a 62.3% correction. The preoperative shoulder height difference of −5.92 ± 12.52 mm (range: −31 to +14 mm, negative designating a lower left shoulder) was improved to 1.52 ± 8.12 mm. Postoperatively, no patient had significant or moderate shoulder imbalance, 4 patients had minimal shoulder imbalance, and 21 patients had balanced shoulders.

Conclusions

Although Lenke Type 2 criteria were developed wth Cotrel-Dubousset instrumentation, they are successfully applied to determining thoracic fusion when segmental PS instrumentation is used.

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Chen Wang, Chien-Min Chen, Fang Shen, Xiao-Dong Fang, Guang-Yu Ying, Yu-Cheng Ren, Dan-Feng Yu, Liang-Liang Zhu, Yong-Jian Zhu and Jian-Min Zhang

Spinal dural arteriovenous fistulas (SDAVFs) are the most common type of spinal arteriovenous malformations, and microsurgical ligation is the treatment modality most frequently used for these lesions. Developments in endoscopic techniques have made endoscopy an even less invasive alternative to routine microsurgical approaches in spine surgery, but endoscopic management of SDAVF or other intradural spinal lesions has not been reported to date.

The authors describe the use of a microscope-assisted endoscopic interlaminar approach for the ligation of the proximal draining vein of an L-1 SDAVF in a 58-year-old man. A complete cure was confirmed by postoperative angiography. The postoperative course was uneventful, and short-term follow-up showed improvements in the patient's neurological function. The authors conclude that the endoscopic interlaminar approach with microscope assistance is a safe, minimally invasive, innovative technique for the surgical management of SDAVFs in selected patients.

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Nikhil G. Thaker, Fang Zhang, Peter R. McDonald, Tong Ying Shun, John S. Lazo and Ian F. Pollack

Glioblastoma multiforme (GBM) is a high-grade brain malignancy arising from astrocytes. Despite aggressive surgical approaches, optimized radiation therapy regimens, and the application of cytotoxic chemotherapies, the median survival of patients with GBM from time of diagnosis remains less than 15 months, having changed little in decades. Approaches that target genes and biological pathways responsible for tumorigenesis or potentiate the activity of current therapeutic modalities could improve treatment efficacy. In this regard, several genomic and proteomic strategies promise to impact significantly on the drug discovery process. High-throughput genome-wide screening with short interfering RNA (siRNA) is one strategy for systematically exploring possible therapeutically relevant targets in GBM. Statistical methods and protein-protein interaction network databases can also be applied to the screening data to explore the genes and pathways that underlie the pathological basis and development of GBM. In this study, we highlight several genome-wide siRNA screens and implement these experimental concepts in the T98G GBM cell line to uncover the genes and pathways that regulate GBM cell death and survival. These studies will ultimately influence the development of a new avenue of neurosurgical therapy by placing the drug discovery process in the context of the entire biological system.

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Hua-Qiao Tan, Ming-Hua Li, Yue-Qi Zhu, Chun Fang, Chun-Geng Wu, Ying-Sheng Cheng, Jue Wang, Jian Xie and He Zhang

Object

The development and preclinical assessment of new endovascular devices necessitate readily available and reproducible animal models. The purpose of this study was to develop an in vivo carotid siphon model for testing the properties of covered stents specially designed for the intracranial vasculature.

Methods

Six carotid siphon–shaped devices were created. Six dogs underwent surgery to expose and isolate both common carotid arteries (CCAs). The right CCA origin was ligated and incised distal to the ligation point after temporary constriction of the distal right CCA. The distal left CCA was ligated and incised proximal to the ligation point after the left CCA origin was temporarily clamped. The proximal isolated left CCA was passed through the shaped device and then anastomosed end-to-end to the distal isolated right CCA. Finally, the shaped device was fixed and embedded in the neck. Intraarterial digital subtraction angiography was performed at 7 days, 2 weeks, and 1 month postprocedure. All models underwent endovascular interventional simulation. The carotid siphon models were evaluated.

Results

The animals tolerated the surgical procedure well. The mean time for surgical construction of the model was 90 minutes. The morphology and endovascular manipulation of the siphon models were similar to those in humans. Stenosis of anastomotic stoma occurred in 2 models, and mural thrombosis of anastomotic stoma occurred in 1 model; however, all models were patent at postprocedural follow-up angiography.

Conclusions

Surgical construction of an in vivo carotid siphon model in dogs with carotid siphon–shaped devices is feasible and potentially useful for testing neurovascular devices.

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Yong-Jian Zhu, Guang-Yu Ying, Ai-Qin Chen, Lin-Lin Wang, Dan-Feng Yu, Liang-Liang Zhu, Yu-Cheng Ren, Chen Wang, Peng-Cheng Wu, Ying Yao, Fang Shen and Jian-Min Zhang

OBJECT

Posterior midline laminectomy or hemilaminectomy has been successfully applied as the standard microsurgical technique for the treatment of spinal intradural pathologies. However, the associated risks of postoperative spinal instability increase the need for subsequent fusion surgery to prevent potential long-term spinal deformity. Continuous efforts have been made to minimize injuries to the surrounding tissue resulting from surgical manipulations. The authors report here their experiences with a novel minimally invasive surgical approach, namely the interlaminar approach, for the treatment of lumbar intraspinal tumors.

METHODS

A retrospective review was conducted of patients at the Second Affiliated Hospital of Zhejiang University School of Medicine who underwent minimally invasive resection of lumbar intradural-extramedullary tumors. By using an operative microscope, in addition to an endoscope when necessary, the authors were able to treat all patients with a unilateral, paramedian, bone-sparing interlaminar technique. Data including preoperative neurological status, tumor location, size, pathological diagnosis, extension of resections, intraoperative blood loss, length of hospital stay, and clinical outcomes were obtained through clinical and radiological examinations.

RESULTS

Eighteen patients diagnosed with lumbar intradural-extramedullary tumors were treated from October 2013 to March 2015 by this interlaminar technique. A microscope was used in 15 cases, and the remaining 3 cases were treated using a microscope as well as an endoscope. There were 14 schwannomas, 2 ependymomas, 1 epidermoid cyst, and 1 enterogenous cyst. Postoperative radiological follow-up revealed complete removal of all the lesions and no signs of bone defects in the lamina. At clinical follow-up, 14 of the 18 patients had less pain, and patients' motor/sensory functions improved or remained normal in all cases except 1.

CONClUSIONS

When meeting certain selection criteria, intradural-extramedullary lumbar tumors, especially schwannomas, can be completely and safely resected through a less-invasive interlaminar approach using a microscope, or a microscope in addition to an endoscope when necessary. This approach was advantageous because it caused even less bone destruction, resulting in better postoperative spinal stability, no need for facetectomy and fusion, and quicker functional recovery for the patients. Individualized surgical planning according to preoperative radiological findings is key to a successful microsurgical resection of these lesions through the interlaminar space.