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Xue-song Liu, Chao You, Ma Lu and Jia-gang Liu

Object

A growing skull fracture (GSF) is a rare but significant late complication of skull fractures, usually occurring during infancy and early childhood. Delayed diagnosis and improper treatment could exacerbate this disease. The aim of this study was to introduce a new hypothesis about, describe the stages of, and discuss the treatment strategy for GSF.

Methods

The authors performed a retrospective review of 27 patients with GSF, who were grouped according to 3 different GSF stages.

Results

Over a period of 20 years, 27 patients with GSF (16 males and 11 females) were treated in the authors' department. The mean follow-up period was 26.5 months. Six patients were in the prephase of GSF (Stage 1), 10 patients in the early phase (Stage 2), and 11 in the late phase (Stage 3). All patients underwent duraplasty. All 6 patients at Stage 1 and 5 patients at Stage 2 underwent craniotomy without cranioplasty. Five patients at Stage 2 and all of the patients at Stage 3 underwent cranioplasty with autologous bone and alloplastic materials, respectively. Among all patients, 5 underwent ventriculoperitoneal shunt placement.

Symptoms in all patients at Stages 1 and 2 were alleviated or disappeared, and the cranial bones developed without deformity during follow-up. Among patients with Stage 3 GSF, no obvious improvement in neurological deficits was observed. Three patients underwent additional operations because of cranial deformation or infection.

Conclusions

The authors identify the stages of GSF according to a new hypothesis. They conclude that accurately diagnosing and treating GSF during Stages 1 and 2 leads to a better prognosis.

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Jian-Bin Chen, Ding Lei, Min He, Hong Sun, Yi Liu, Heng Zhang, Chao You and Liang-Xue Zhou

OBJECT

The present study aimed to clarify the incidence and clinical features of disease progression in adult moyamoya disease (MMD) patients with Graves disease (GD) for better management of these patients.

METHODS

During the past 18 years, 320 adult Chinese patients at West China Hospital were diagnosed with MMD, and 29 were also diagnosed with GD. A total of 170 patients (25 with GD; 145 without GD) were included in this study and were followed up. The mean follow-up was 106.4 ± 48.6 months (range 6–216 months). The progression of the occlusive lesions in the major intracranial arteries was measured using cerebral angiography and was evaluated according to Suzuki's angiographic staging. Information about cerebrovascular strokes was obtained from the records of patients' recent clinical visits. Both angiographic progression and strokes were analyzed to estimate the incidences of angiographic progression and strokes using Kaplan-Meier analysis. A multivariate logistic regression model was used to test the effects of sex, age at MMD onset, disease type, strokes, and GD on the onset of MMD progression during follow-up.

RESULTS

During follow-up, the incidence of disease progression in MMD patients with GD was significantly higher than in patients without GD (40.0% vs 20.7%, respectively; p = 0.036). The interval between initial diagnosis and disease progression was significantly shorter in MMD patients with GD than in patients without GD (p = 0.041). Disease progression occurred in both unilateral MMD and bilateral MMD, but the interval before disease progression in patients with unilateral disease was significantly longer than in patients with bilateral disease (p = 0.021). The incidence of strokes in MMD patients with GD was significantly higher than in patients without GD (48% vs 26.2%, respectively; p = 0.027). The Kaplan-Meier survival curve showed significant differences in the incidence of disease progression (p = 0.038, log-rank test) and strokes (p = 0.031, log-rank test) between MMD patients with GD and those without GD. Multivariate analysis suggested that GD may contribute to disease progression in MMD (OR 5.97, 95% CI 1.24–33.76, p = 0.043).

CONCLUSIONS

The incidence of disease progression in MMD patients with GD was significantly higher than that in MMD patients without GD, and GD may contribute to disease progression in MMD patients. The incidence of strokes was significantly higher in MMD patients with GD than in patients without GD. Management guidelines for MMD patients with GD should be developed.

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Jian-Bin Chen, Yi Liu, Liang-Xue Zhou, Hong Sun, Min He and Chao You

OBJECT

This study explored whether there were differences between the autoimmune disease prevalence rates in unilateral and bilateral moyamoya disease (MMD).

METHODS

The authors performed a retrospective review of data obtained from the medical records of their hospital, analyzing and comparing the clinical characteristics and prevalence rates of all autoimmune diseases that were associated with unilateral and bilateral MMD in their hospital from January 1995 to October 2014.

RESULTS

Three hundred sixteen patients with bilateral MMD and 68 with unilateral MMD were identified. The results indicated that patients with unilateral MMD were more likely to be female than were patients with bilateral MMD (67.6% vs 51.3%, p = 0.014, odds ratio [OR] 1.99). Overall, non-autoimmune comorbidities tended to be more prevalent in the unilateral MMD cases than in the bilateral MMD cases (17.6% vs 9.8%, p = 0.063, OR 1.97, chi-square test). Autoimmune thyroid disease and other autoimmune diseases also tended to be more prevalent in the unilateral MMD cases than in the bilateral MMD cases (19.1% vs 10.8%, p = 0.056, OR 1.96 and 8.8% vs 3.5%, p = 0.092, OR 2.77, respectively, chi-square test). The overall autoimmune disease prevalence in the unilateral MMD cases was significantly higher than in the bilateral MMD cases (26.5% vs 13.6%, p = 0.008, OR 2.29, 95% CI 1.22–4.28, chi-square test). Multiple logistic regression analysis showed that autoimmune disease was more likely to be associated with unilateral than with bilateral MMD (p = 0.039, OR 10.91, 95% CI 1.13–105.25).

CONCLUSIONS

This study indicated a higher overall autoimmune disease prevalence in unilateral than in bilateral MMD. Unilateral MMD may be more associated with autoimmune disease than bilateral MMD. Different pathogenetic mechanisms may underlie moyamoya vessel formation in unilateral and bilateral MMD.

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Kai Shen, Zhongliang Deng, Junsong Yang, Chao Liu and Ranxi Zhang

OBJECTIVE

Atlantoaxial instability is usually corrected by anterior and/or posterior C1–2 fusion. However, fusion can lead to considerable loss of movement at the C1–2 level, which can adversely impact a patient’s quality of life. In this study, the authors investigated the stability and function of a novel posterior artificial atlanto-odontoid joint (NPAAJ) by using cadaveric cervical spines.

METHODS

The Oc–C7 regions from 10 cadaveric spines were used for anteroposterior (AP) translation and range of motion (ROM) tests while intact and after destabilization, NPAAJ implantation, and double-rod fixation.

RESULTS

The mean AP C1–2 translational distances in the intact, destabilization, and double-rod groups were 6.53 ± 1.07 mm, 11.54 ± 1.59 mm, and 3.24 ± 0.99 mm, respectively, and the AP translational distance in the NPAAJ group was significantly different from that in the intact group (p < 0.05). The AP translational distance in the NPAAJ group was not significantly different from that in the double-rod group (p = 0.24). The mean flexion, extension, and axial rotation ROM values of the NPAAJ group were 9.87° ± 0.91°, 8.75° ± 0.99°, and 61.93° ± 2.93°, respectively, and these were lower than the corresponding values in the intact group (p < 0.05). The mean lateral bending ROM in the NPAAJ group (9.26° ± 0.86°) was not significantly different from that in the intact group (p = 0.23), and the flexion, extension, and rotation ranges in the NPAAJ group were 79.5%, 85.2%, and 82.3%, respectively, of those in the intact group.

CONCLUSIONS

Use of NPAAJ for correction of atlantoaxial instability disorders caused by congenital odontoid dysplasia, odontoid fracture nonunion, and C-1 transverse ligament disruption (IA, IB, and IIB) may restore the stability and preserve most of the ROM of C1–2. Additionally, the NPAAJ may prevent soft tissue from embedding within the joint. However, additional studies should be performed before the NPAAJ is used clinically.

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Liu Xue-Song, You Chao, Yang Kai-Yong, Huang Si-Qing and Zhang Heng

Object

An extensive sacrococcygeal chordoma is considered a challenge for neurosurgeons. Because of the complex anatomy of the sacral region, the risk of uncontrollable intraoperative hemorrhage, and the typically large tumor size at presentation, complete resections are technically difficult and the tumor recurrence rate is high. The aim of this study was to assess the value of using occlusion of the abdominal aorta by means of a balloon dilation catheter and electrophysiological monitoring when an extensive sacrococcygeal chordoma is removed.

Methods

Between 2004 and 2008, 9 patients underwent resection of extensive sacrococcygeal chordomas in the authors' department with the aid of occlusion of the abdominal aorta and electrophysiological monitoring. All of these operations were performed via the posterior approach. The records of the 9 patients were reviewed retrospectively.

Results

Wide resections were performed in 6 cases and marginal excisions in the other 3. Five patients underwent postoperative radiotherapy. Intraoperative hemorrhage was controlled at 100–400 ml. Postoperatively, none of the patients had any new neurological dysfunction, and 2 patients regained normal urinary and bowel function. The mean follow-up period was 31.4 months (range 10–57 months). No patient developed local recurrence or had metastatic spread of tumor during follow-up.

Conclusions

Occlusion of the abdominal aorta and electrophysiological monitoring are useful methods for assisting in resection of sacrococcygeal chordoma. They can reduce intraoperative hemorrhage and entail little chance of tumor cell contamination. They can also help surgeons to protect the organs in the pelvic cavity and neurological function. Use of these methods could give patients better quality of life.

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Hong-Qi Zhang, Ling-Qiang Chen, Shao-Hua Liu, Di Zhao and Chao-Feng Guo

Object

The object of this study was to evaluate the efficacy and safety of posterior decompression with kyphosis correction for thoracic myelopathy due to ossification of the ligamentum flavum (OLF) and ossification of the posterior longitudinal ligament (OPLL) at the same level.

Methods

Between January 2003 and December 2005, 11 patients (8 men and 3 women) with thoracic myelopathy due to OLF and OPLL at the same level underwent posterior decompressive laminectomy and excision of OLF. Posterior instrumentation was also performed for stabilization of the spine and reducing the thoracic kyphosis angle by approximately 5–15° (kyphosis correction), and spinal fusion was performed in all cases. The follow-up period ranged from 2 to 4 years (mean 2.8 years). The outcomes were evaluated using a recovery scale based on the Japanese Orthopaedic Association classification. The score of each patient was calculated before surgery, 1 year after surgery, and at the final follow-up visit.

Results

After surgery, the thoracic kyphosis in the stabilization area was reduced from 30.0 ± 4.02° to 20.8 ± 2.14° on average. The mean score on the Japanese Orthopaedic Association scale improved from 3.5 ± 1.69 preoperatively to 8.5 ± 1.63 at the final follow-up, with a recovery rate of 68.0%. The results were good in 9 patients and fair in 2 patients. Postoperative MR imaging showed that the spinal cord was shifted posteriorly and decompressed completely in all cases. Myelopathy was not aggravated in any case after surgery.

Conclusions

A considerable degree of neurological recovery was observed after posterior decompression and kyphosis correction. The procedure is easy to perform with a low risk of postoperative paralysis. The authors therefore suggest that the procedure is useful for patients whose spinal cords are severely impinged by OLF and OPLL at the same level.

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Tzu-Hsien Chao, Cheng-Jung Lin, Hunghui Liu, Chen-Chih Chu and Dueng-Yuan Hueng

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Wen-Chao Liu, Liang Wen, Tao Xie, Hao Wang, Jiang-Biao Gong and Xiao-Feng Yang

OBJECTIVE

Erythropoietin (EPO) exerts a neuroprotective effect in animal models of traumatic brain injury (TBI). However, its effectiveness in human patients with TBI is unclear. In this study, the authors conducted the first meta-analysis to assess the effectiveness and safety of EPO in patients with TBI.

METHODS

In December 2015, a systematic search was performed of PubMed, Web of Science, MEDLINE, Embase, the Cochrane Library databases, and Google Scholar. Only English-language publications of randomized controlled trials (RCTs) using EPO in patients with TBI were selected for analysis. The assessed outcomes included mortality, favorable neurological outcome, hospital stay, and associated adverse effects. Continuous variables were presented as mean difference (MD) with a 95% confidence interval (CI). Dichotomous variables were presented as risk ratio (RR) or risk difference (RD) with a 95% CI. Statistical heterogeneity was examined using both I2 and chi-square tests.

RESULTS

Of the 346 studies identified in the search, 5 RCTs involving 915 patients met the inclusion criteria. The overall results demonstrated that EPO significantly reduced mortality (RR 0.69, 95% CI 0.49–0.96, p = 0.03) and shortened the hospitalization time (MD −7.59, 95% CI −9.71 to −5.46, p < 0.0001) for patients with TBI. Pooled results of favorable outcome (RR 1.00, 95% CI 0.88–1.15, p = 0.97) and deep vein thrombosis (DVT; RD 0.00, 95% CI −0.05 to 0.05, p = 1.00) did not show a significant difference.

CONCLUSIONS

The authors suggested that EPO is beneficial for patients with TBI in terms of reducing mortality and shortening hospitalization time without increasing the risk of DVT. However, its effect on improving favorable neurological outcomes did not reach statistical significance. Therefore, more well-designed RCTs are necessary to ascertain the optimum dosage and time window of EPO treatment for patients with TBI.

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Cheng-Siu Chang, Chun-Chao Chuang, Ming-Fan Wu, Wen-Shan Liu, Hsien-Tang Tu and Chuan-Fu Huang

Object

Most cases of tumor-related hemifacial spasm (HFS) are treated by open surgery. The authors report the effects of Gamma Knife surgery (GKS) on benign tumor–related HFS at a mean follow-up time of 84 months.

Methods

Between 2000 and 2011, 6 patients (5 women and 1 man) harboring single tumors of the cerebellopontine angle (4 meningiomas and 2 vestibular schwannomas [VSs]) and experiencing HFS underwent GKS as a primary treatment. The mean age of the patients at the time of radiosurgery was 52.7 years (range 45–60 years).

The patients' tumors lay within the radiosurgical target area. In the 4 cases of meningioma, the mean radiosurgical treatment volume was 5.3 cm3 (range 1.2–9.6 cm3), and the mean radiosurgical tumor margin dose was 14.1 Gy (range 12–18 Gy); in the 2 cases of VS, the treatment volume was 2.5 cm3 in 1 patient and 11.2 cm3 in the other, and the margin doses were 11.5 and 12 Gy, respectively. The mean duration of HFS symptoms was 15.5 months (range 3–36 months).

Results

The mean follow-up period was 84 months (range 40–110 months). Overall, 4 (66%) of the 6 patients experienced complete relief from HFS without medication after GKS and 1 patient obtained a good outcome. The mean time for improvement to be realized was 12.6 months (range 3–24 months). Only 1 patient failed to experience relief from HFS, and coincidentally, the tumor did not shrink in that case. In all 6 patients (100%), tumor growth was controlled at a mean follow-up of 56 months after GKS: in 5 patients the tumor had decreased in size and in the other patient the tumor size remained unchanged. No new neurological deficit was noted after GKS, and 1 patient with facial numbness reported improvement after tumor shrinkage.

Conclusions

Gamma Knife surgery appears to be effective in treating benign tumor–related HFS and in controlling tumor growth. A reduction in tumor volume is related to spasm improvement. Although a time latency for spasm relief is associated with GKS, minimal side effects are expected.

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Fang-Chen Liu, Yin-Hsien Liao, Che-Hsien Chang, Chao-Ming Chang, Yu-Chi Tsai and Dueng-Yuan Hueng