Development and validation of a novel nomogram for predicting long-term rebleeding risk among patients with hemorrhagic moyamoya disease: a 10-year multicenter retrospective cohort study.

OBJECTIVE: The aim of this study was to develop and validate a predictive nomogram model for long-term rebleeding events in patients with hemorrhagic moyamoya disease (HMMD). METHODS: In total, 554 patients with HMMD from the Fifth Medical Center of the Chinese PLA General Hospital (5-PLAGH cohort) were included and randomly divided into training (390 patients) and internal validation (164 patients) sets. An independent cohort from the First Medical Center and Eighth Medical Center of Chinese PLA General Hospital (the 1-PLAGH and 8-PLAGH cohort) was used for external validation (133 patients). Univariate Cox regression analysis and least absolute shrinkage and selection operator (LASSO) regression algorithm were used to identify significant factors associated with rebleeding, which were used to develop a nomogram for predicting 5- and 10-year rebleeding. RESULTS: Intraventricular hemorrhage was the most common type of cerebral hemorrhage (39.0% of patients in the 5-PLAGH cohort and 42.9% of the 1-PLAGH and 8-PLAGH cohort). During the mean ± SD follow-up period of 10.4 ± 2.9 years, 91 (16.4%) patients had rebleeding events in the 5-PLAGH cohort. The rebleeding rates were 12.3% (68 patients) at 5 years and 14.8% (82 patients) at 10 years. Rebleeding events were observed in 72 patients (14.3%) in the encephaloduroarteriosynangiosis (EDAS) surgery group, whereas 19 patients (37.3%) experienced rebleeding events in the conservative treatment group. This difference was statistically significant (p < 0.001). We selected 4 predictors (age at onset, number of episodes of bleeding, posterior circulation involvement, and EDAS surgery) for nomogram development. The concordance index (C-index) values of the nomograms of the training cohort, internal validation cohort, and the external validation cohort were 0.767 (95% CI 0.704-0.830), 0.814 (95% CI 0.694-0.934), and 0.718 (95% CI 0.661-0.775), respectively. The nomogram at 5 years exhibited a sensitivity of 48.1% and specificity of 87.5%. The positive and negative predictive values were 38.2% and 91.3%, respectively. The nomogram at 10 years exhibited a sensitivity of 47.1% and specificity of 89.1%. The positive and negative predictive values were 48.5% and 88.5%, respectively. CONCLUSIONS: EDAS may prevent rebleeding events and improve long-term clinical outcomes in patients with HMMD. The nomogram accurately predicted rebleeding events and assisted clinicians in identifying high-risk patients and devising individual treatments. Simultaneously, comprehensive and ongoing monitoring should be implemented for specific patients with HMMD throughout their entire lifespan.

Development and validation of a novel nomogram for predicting good neoangiogenesis after encephaloduroarteriosynangiosis in patients with moyamoya disease and type 2 diabetes mellitus: a case-control study.

OBJECTIVE: Diabetes is often linked to poorer outcomes in patients with moyamoya disease (MMD). However, experience has shown that certain individuals with diabetes have favorable outcomes after encephaloduroarteriosynangiosis (EDAS). The authors aimed to develop a nomogram to predict good neoangiogenesis in patients with MMD and type 2 diabetes mellitus (T2DM) to aid neurosurgeons in the identification of suitable candidates for EDAS. METHODS: Adults with MMD and T2DM who underwent EDAS between June 2004 and December 2018 were included in the analysis. In total, 126 patients (213 hemispheres) with MMD and T2DM from the Fifth Medical Centre of the Chinese PLA General Hospital were included and randomly divided into training (152 hemispheres) and internal validation (61 hemispheres) cohorts at a ratio of 7:3. Univariate logistic and least absolute shrinkage and selection operator regression analyses were used to identify the significant factors associated with good neoangiogenesis, which were used to develop a nomogram. The discrimination, calibration, and clinical utility were assessed. RESULTS: A total of 213 hemispheres in 126 patients were reviewed, including 152 (71.36%) hemispheres with good postoperative collateral formation and 61 (28.64%) with poor postoperative collateral formation. The authors selected 4 predictors (FGD5 rs11128722, VEGFA rs9472135, Suzuki stage, and internal carotid artery [ICA] moyamoya vessels) for nomogram development. The C-indices of the nomogram in the training and internal validation cohorts were 0.873 and 0.841, respectively. The nomogram exhibited a sensitivity of 84.5% and specificity of 81.0%. The positive and negative predictive values were 92.1% and 66.7%, respectively. The calibration curves indicated high predictive accuracy, and receiver operating characteristic curve analysis showed the superiority of the nomogram. The decision-making analysis validated the fitness and clinical application value of this nomogram. Then a web-based calculator to facilitate clinical application was generated. CONCLUSIONS: The nomogram developed in this study accurately predicted neoangiogenesis in patients with MMD and T2DM after EDAS and may assist neurosurgeons in identifying suitable candidates for indirect revascularization surgery.

A long-term study of posterior circulation changes after revascularization in patients with moyamoya disease.

OBJECTIVE: This study aimed to explore the long-term course of posterior circulation changes and predictors in patients with moyamoya disease (MMD). METHODS: The authors retrospectively enrolled patients who were diagnosed with MMD and underwent encephaloduroarteriosynangiosis (EDAS) surgery at the authors' department from December 2002 to September 2011. A comparative study between short-term (6-12 months) and long-term (≥ 9 years) follow-up angiography was conducted. The progression of lesions was defined from lower to higher stages of the posterior cerebral artery (PCA). RESULTS: Eighty-eight patients who received indirect EDAS were enrolled in the study. The mean age at first surgery was 28.1 ± 15.0 years. Among these 88 patients with MMD, 39 (44.3%) exhibited transient ischemic attack and 27 (30.7%) exhibited infarction, comprising 5 with occipital lobe infarction, 14 (15.9%) with hemorrhagic symptoms, and 8 (9.1%) with atypical symptoms as the initial symptoms. Heterozygous mutations occurred significantly more frequently in the cases that presented with PCA involvement. During follow-up, stage progression of PCA was observed in 21 patients (28 hemispheres). At short-term follow-up, 21/176 (11.9%) hemispheres had progression of steno-occlusive lesions in the PCA. At long-term follow-up, 7 (4.0%) hemispheres had progression of steno-occlusive lesions in the PCA. At short-term follow-up, the progression of steno-occlusive lesions in the PCA was associated with progression of the internal carotid artery. Stage progression of PCA occurred significantly more frequently in the cases with PCA involvement on preoperative angiography. Nine strokes (10.2%) occurred in 88 patients during long-term follow-up. Four patients (4.5%) presented with ischemic stroke, including 2 with occipital lobe infarctions. CONCLUSIONS: Progression of PCA stenosis is common in patients with MMD, even if the PCA is normal initially. Mutations of RNF213 p.R4810K may predict PCA involvement or progression. Follow-up of the PCA in MMD patients should be conducted, and timely surgical revascularization is needed.

Feasibility of occipital condyle screw placement in patients with Chiari malformation type I: a computed tomography-based morphometric study.

BACKGROUND: The occipital condyle (OC) screw is an alternative technique for occipitocervical fixation that is especially suitable for revision surgery in patients with Chiari malformation type I (CMI). This study aimed to investigate the feasibility and safety of this technique in patients with CMI. METHODS: The CT data of 73 CMI patients and 73 healthy controls were retrospectively analyzed. The dimensions of OCs, including length, width, height, sagittal angle, and screw length, were measured in the axial, sagittal, and coronal planes using CT images. The OC available height was measured in the reconstructed oblique parasagittal plane of the trajectory. RESULTS: The mean length, width, and height of OCs in CMI patients were 17.79 ± 2.31 mm, 11.20 ± 1.28 mm, and 5.87 ± 1.29 mm, respectively. All OC dimensions were significantly smaller in CMI patients compared with healthy controls. The mean screw length and sagittal angle were 19.13 ± 1.97 mm and 33.94° ± 5.43°, respectively. The mean OC available height was 6.36 ± 1.59 mm. According to criteria based on OC available height and width, 52.1% (76/146) of OCs in CMI patients could safely accommodate a 3.5-mm-diameter screw. CONCLUSIONS: The OC screw is feasible in approximately half of OCs in CMI patients. Careful morphometric analyses and personalized surgical plans are necessary for the success of this operation in CMI patients.

A morphometric study of the atlanto-occipital joint in adult patients with Chiari malformation type I.

BACKGROUND: There are many morphometric studies on Chiari malformation type I (CMI) patients, most of which focus on the posterior cranial fossa (PCF). Less attention has been paid to the atlanto-occipital joint. In this study, we aim to evaluate the morphological characteristics of the atlanto-occipital joint in CMI patients. MATERIALS AND METHODS: The cervical CT imaging data of adult patients diagnosed with CMI but without any bony malformation in craniovertebral junction (CVJ) who were treated by the authors between January 2014 and December 2019 were retrospectively analyzed. The equal number of sex and age-matched healthy individuals were included as the control group. The morphometric analysis was performed by measuring the length and depth of the atlanto-occipital joint, and the depth/length ratio was calculated to evaluate the curvature of the joint. RESULTS: A total of 47 patients (15 males and 32 females) were included. The mean age of patients was 47.49 ± 9.01 years (range 19-62 years). The mean depth/length ratio of the atlanto-occipital joint in CMI patients was 0.141 ± 0.065 (range 0.027 - 0.274), which was smaller than that of control individuals (0.228 ± 0.057, range 0.069 - 0.379). And the difference was statistically significant (p < .01). CONCLUSIONS: The atlanto-occipital joints in CMI patients are significantly flatter compared with those in healthy controls. This morphological variation could lead to differences of the atlanto-occipital stability between CMI patients and normal population, which may be related to the pathogenesis of CMI.

Can Posterior Reduction Replace Odontoidectomy as Treatment for Patients With Congenital Posterior Atlantoaxial Dislocation and Basilar Invagination?

BACKGROUND: For patients with odontoid process protrusion and basilar invagination, posterior screw-rod fixation can usually achieve satisfactory horizontal reduction, but in some cases satisfactory reduction in the vertical direction cannot be achieved at the same time. OBJECTIVE: To propose a method for calculation of the theoretical maximum vertical reduction possible in individual patients. METHODS: The computed tomography imaging data of patients with occipitalization and basilar invagination who were treated at our institute between January 2013 and June 2016 were retrospectively analyzed. The direction of odontoid reduction was decided by the inclination of the lateral joint. The atlanto-dental distance was assumed to be the maximum possible reduction in the horizontal direction. The maximum vertical reduction possible was calculated based on these values. RESULTS: A total of 82 patients (34 males and 48 females) were included. The theoretical vertical reduction value was 4.2 ± 3.0 mm, which was significantly smaller than that of the dental protrusion (14.5 ± 3.8 mm, P = .000). Analysis of follow-up data (29 cases) showed that, the difference between the theoretical vertical reduction value H (4.7 ± 3.5 mm) and the actual vertical reduction value Ha (5.6 ± 3.5 mm) was not significant (P = .139). CONCLUSION: The theoretical calculation method we proposed can well predict the actual degree of vertical reduction. The theoretical vertical reduction value is significantly lower than the odontoid protrusion value, indicating that satisfactory reduction in the vertical direction is difficult with a posterior approach alone.

Usefulness of 3D Printed Models in the Management of Complex Craniovertebral Junction Anomalies: Choice of Treatment Strategy, Design of Screw Trajectory, and Protection of Vertebral Artery.

OBJECTIVE: To evaluate the usefulness of 3-dimensional (3D) printed models as an aid for the treatment of complex CVJ anomalies. METHODS: 3D printed models were fabricated for 21 patients with complex CVJ anomalies, including vertebral artery anomaly, thin C2 pedicle, vertical atlantoaxial facet joint, or rotational dislocation combined with atlantoaxial dislocation and basilar invagination. Preoperative planning, surgical simulation, and intraoperative reference were achieved using the 3D model during the surgical treatment. The usefulness of 3D printed models, and postoperative clinical and radiological outcomes were assessed. RESULTS: Direct posterior reduction and atlantoaxial fixation were achieved in 19 patients. Transoral odontoidectomy followed by posterior fixation was implemented for 2 patients with vertical facet joint and rotational dislocation. All screws were safely inserted with no complication, and 90% patients achieved a >60% reduction of both horizontal and vertical dislocation. Clinical symptoms improved in all patients, with the averaged Japanese Orthopedic Association scores increasing from 11.14 to 14.43 (P < 0.01). CONCLUSIONS: The patient-specific 3D printed model would be an effective tool for evaluation of the reducibility of the atlantoaxial dislocation and basilar invagination, decision making in choosing the optimal surgical approach and way of fixation, and precise placement of the screw while protecting the vertebral artery and spinal cord. The risk of neurovascular injury was minimized, and encouraging outcomes were achieved with the aid of this technique.

Posterior Reduction of Fixed Atlantoaxial Dislocation and Basilar Invagination by Atlantoaxial Facet Joint Release and Fixation: A Modified Technique With 174 Cases.

BACKGROUND: Treatment of fixed atlantoaxial dislocation (AAD) with basilar invagination (BI) is challenging. OBJECTIVE: To introduce a modified technique to reduce fixed AAD and BI through a posterior approach. METHODS: From 2007 to 2013, 174 patients with fixed AAD and BI underwent surgical reduction by posterior atlantoaxial facet joint release and fixation technique. RESULTS: There was 1 death in the series, and 3 patients were lost to follow-up. The follow-up period ranged from 12 to 52 months (mean: 35.2 months) for the remaining 170 patients. Neurological improvement was observed in 168 of 170 patients (98.8%), and was stable in 1 (0.06%) and exacerbated in 1 (0.06%), with the Japanese Orthopedic Association scores increasing from 11.4 preoperatively to 15.8 postoperatively (P < .01). Radiologically, complete or >90% reduction was attained in 107 patients (62.9%), 60% to 90% reduction was attained in 51 patients (30%), and <50% reduction was attained in 12 patients (7.1%), who underwent additional transoral decompression. Complete decompression was demonstrated in all 170 patients. Solid bony fusion was demonstrated in 167 patients at follow-up (98.2%). CONCLUSION: This series showed the safety and efficacy of the posterior C1-2 facet joint release and reduction technique for the treatment of AAD and BI. Most fixed AAD and BI cases are reducible via this method. In most cases, this method avoids transoral odontoidectomy and cervical traction. Compared with the occiput-C2 screw method, this short-segment C1-2 technique exerts less antireduction shearing force, guarantees longer bone purchase, and provides more immediate stabilization.

[Thoracic extramedullary subdural and epidural of inflammatory myofibroblastoma: a case report].

We present here a rare case of thoracic extramedullary subdural and epidural of leisions. The initial diagnosis of preoperation was hypertrophy of ligamentum flavum, however, after excision of the epidural lesions, the symptoms got worse. A second operation found the subdural leisions which were then totally resected. Two pathological examinations confirmed it was inflammatory myofibroblastic tumor (IMT). The incidence of this disease is extremely low. IMT is benign in histology, but it can be aggressive. The exact pathogenesis is still unknown. Tumor resection is the first choice to treat. Long-term follow-up with MRI is required for the patient.

Safety and efficacy of microscope-assisted insertion of screws into fused C1-occipital condyle complex for occipital cervical fixation in atlantal cervical nonsegmentation patients.

OBJECTIVE: To explore the safety and efficacy of the insertion of screws into fused C1-occipital condyle(CC)complex without image guidance in atlantal-cervical nonsegmentation patients. METHODS: The occipital condyle junction was fixed posteriorly in 10 basilar invagination patients with atlantal-cervical nonsegmentation using polyaxial titanium screws(3.5 mm)inserted unicortically into the CC complex and C2 pedicles,followed by fixation to a 3 mm rod. Drilling was guided by anatomic landmarks. The entry point was at the center of posterior surface of the CC complex. The angle of medicalization was 10-15 degrees. In the sagittal plane,the angle for maximal superior screw angulation was also 10-15 degrees. The screw length to obtain unicortical purchase was 16 to 22 mm. CT scans were obtained before and after the surgery. The length,width,and height of CC complex were measured on computed tomography(CT)preoperatively. The position of screws and the condition of fixation were analyzed on postoperative CT scan. Postoperative complications were recorded. The mean follow-up was(30.2±4.38)months(range: 24-36 months). RESULTS: The width,length,height of left side CC complex were(7.96±2.23)mm,(16.06±2.73)mm,and(13.76±2.06)mm,and the width,length,height of right side CC complex were(7.84±1.38)mm,(16.66±2.58)mm,and(12.81±2.62)mm. No fracture was identified. There was no screw malposition or neurovascular complication related to screw insertion. No screw loosening or construct failure was observed during the follow-up. CONCLUSIONS: In patients with atlantal cervical nonsegmentation,the CC complex screws can be safely inserted assisted by microscope without image guidance. Occipital condyle junction fixation using polyaxial CC complex screws is feasible and can be a good alternative where other fixation techniques are not satisfactory.

Posterior realignment of irreducible atlantoaxial dislocation with C1-C2 screw and rod system: a technique of direct reduction and fixation.

BACKGROUND CONTEXT: Treatment of chronic and irreducible atlantoaxial dislocation (AAD) with ventral compression is challenging for surgeons. The main procedures are occipitocervical/C1-C2 fusion after transoral odontoidectomy or release of the periodontoid tissues. These surgical procedures, which are performed simultaneously or intermittently, have many disadvantages that may discount their effectiveness. Therefore, a more effective way to achieve surgical reduction and to keep solid stability with only a single procedure is needed. PURPOSE: We describe a technique to reduce chronic and irreducible AAD with C1 lateral mass and C2 pedicle screw and rod system. STUDY DESIGN: This was a retrospective case series. PATIENT SAMPLE: Our sample comprised 26 patients (9 men and 17 women) with irreducible AAD who ranged in age from 15 to 54 years (mean, 35 years). OUTCOME MEASURES: Patients' neurologic status was evaluated with the Japanese Orthopedic Association (JOA) scale. METHODS: Twenty-six symptomatic patients underwent posterior realignment and reduction through the C1 lateral mass and C2 pedicle screw and rod system. The proposed mechanism of reduction is that the implanted screws and rods between C1 and C2 acting as a lever system drew C1 backward and pushed C2 downward and forward after removing circumambient obstruction and scars and thoroughly releasing the facet joints. The preoperative and postoperative JOA score, the extent of reduction, and the conditions of C1-C2 bony fusion were examined. RESULTS: No neurovascular injury occurred during surgery. Follow-up ranged from 6 to 40 months (mean 20.7 months). Radiographic evaluation showed that solid bony fusion was achieved in all patients, and that complete reduction was attained in 18 patients and partial reduction (>60% reduction) in 8 patients. The mean postoperative JOA score at last follow-up was 15.7, compared with the preoperative score of 12.1 (p<.01). CONCLUSIONS: This C1-C2 screw and rod system provides reliable stability and sufficient reduction of the anatomic malalignment at the craniovertebral junction and meanwhile retains the mobility of atlanto-occipital joints in the treatment of chronic and irreducible AAD. Sophisticated skills, thorough release of the facet joints, and intraoperative protection of the vertebral artery are key points to accomplish this technique.

[High-field intraoperative magnetic resonance imaging suite with neuronavigation system: implementation and preliminary experience in the pituitary adenoma operation with transsphenoidal approach].

OBJECTIVES: To review the preliminary clinical experience with high-field-strength intraoperative magnetic resonance imaging (iMRI) suite with neuronavigation system in the pituitary adenoma operation with transsphenoidal approach. METHODS: From March 2009 to December 2010, 31 patients [range, 29 - 76 years, mean age (47 ± 11) years]of pituitary adenoma were operated with transsphenoidal approach and intraoperatively with a movable 1.5 T high-field-strength iMRI suite in combination with neuronavigation system. Tumor size was 1.8 - 7.3 cm, mean (3.5 ± 1.2) cm. Twenty-five cases were non-functional pituitary adenoma, 4 cases were prolactin-secreting pituitary adenoma, 2 cases were growth hormone-secreting pituitary adenoma. Thirty patients' resection with transnasal transsphenoidal approach were performed, one patient with transoral transsphenoidal approach was performed. RESULTS: In 12 cases of 30 patients who planed to totally remove tumor, iMRI had revealed residual lesions and resulted in the change of the surgical strategy, 2 invasive cavernous sinus cases no further resection of the tumor because of internal carotid artery encasement, the other 10 cases resected further, eventually. Finally, 8 cases were totally removed. The ratio of total removal tumor was enhanced to 86.7% (26/30) from 60.0% (18/30). There was no perioperative mortality. CONCLUSIONS: High-field-strength iMRI suite with neuronavigation system provides valuable information of tumor resection that allows intraoperative modification of the surgical strategy. It could be very helpful to maximize the resection of the pituitary adenoma and minimize the injury to neurological function.