Xuhui Hui

Brainstem gangliogliomas: a retrospective series

OBJECT The authors retrospectively analyzed data on brainstem gangliogliomas treated in their department and reviewed the pertinent literature to foster understanding of the preoperative characteristics, management, and clinical outcomes of this disease. METHODS In 2006, the authors established a database of treated lesions of the posterior fossa. The epidemiology findings, clinical presentations, radiological investigations, pathological diagnoses, management, and prognosis for brainstem gangliogliomas were retrospectively analyzed. RESULTS Between 2006 and 2012, 7 patients suffering from brainstem ganglioglioma were treated at the West China Hospital of Sichuan University. The mean age of the patients, mean duration of symptoms prior to diagnosis, and mean duration of follow-up were 28.6 years, 19.4 months, and 38.1 months, respectively. The main presentations were progressive cranial nerve deficits and cerebellar signs. Subtotal resection was achieved in 2 patients, and partial resection in 5. All tumors were pathologically diagnosed as WHO Grade I or II ganglioglioma. Radiotherapy and adjuvant chemotherapy were not administered. After 21-69 months of follow-up, patient symptoms were resolved or stable without aggravation, and MRI showed that the size of residual lesions was unchanged without progression or recurrence. CONCLUSIONS The diagnosis of brainstem ganglioglioma is of great importance given its favorable prognosis. The authors recommend the maximal safe resection followed by close observation without adjuvant therapy as the optimal treatment for this disease.

Experience of microsurgical treatment of brainstem cavernomas: Report of 37 cases

BACKGROUND Cavernomas are rare in the brainstem and account for 18-35% of central nervous cavernomas and can cause recurrent hemorrhages, devastating neurological deficits and mortality. OBJECTIVES To summarize the experience of microsurgical treatment of brainstem cavernomas and to investigate curative effect of microsurgical treatment of brainstem cavernomas. MATERIALS AND METHODS A retrospective analysis clinical data of 37 patients with brainstem cavernomas seen between 2003 and 2007. The analysis included age distribution, hemorrhage rates, clinical presentation, location of the lesions, and preoperative and postoperative Karnofsky Performance Scale (KPS) scores. The surgical indications, the timing of surgery and the surgical techniques were also assessed. RESULTS All the 37 patients received microsurgical resections, there was no surgery-related mortality. Histopathological examination confirmed the diagnosis of cavernoma. Postoperatively, 20 patients had functional improvement, 15 patients had no change in the neurological status, and two patients deteriorated. Early surgery was associated with better outcomes. Mean followed up period was 21.5 months (range 6-36 months). During the follow-up 20 patients had resumed activities of daily living (KPS scores of 90-100), 10 patients were able to self-care with some efforts (KPS scores of 70-80), five patients needed considerable assistance (KPS score of 50-60) and two patients suffered hemiparesis (KPS scores of 40). None of the patient had recurrent hemorrhage. CONCLUSIONS Brainstem cavernomas can safely be resected. Successful resection of brainstem cavernomas can be achieved by optimal surgical approaches, feasible entry zone and meticulous microsurgical techniques. The goal of surgical intervention should be the total resection of the lesion without any deteriorative in the neurological deficits.

Surgical treatment of hemorrhagic brainstem cavernous malformations

CONTEXT Microsurgery is considered to be the optimal treatment for brainstem cavernous malformations (BCMs); however, the high surgery-related morbidity requires further assessment of therapeutic protocols. AIMS The surgical experience and the optimal surgical strategy for the management of brainstem cavernous malformations is discussed. MATERIALS AND METHODS From September 2007 to August 2014, a total of 120 patients with BCMs underwent surgical treatment in our hospital. The clinical features and neurological outcome of these patients were retrospectively analysed, and our institutional surgical strategy was discussed. RESULTS The preoperative annual hemorrhage and rehemorrhage rates were 4.2% and 42.9%, respectively. Gross total resection was achieved in 116 patients (96.7%) and subtotal resection in 4 (3.3%). After a mean follow-up of 50.7 ± 26.5 months (range: 18-90 months), the neurological status showed improvement in 71 patients (67.0%) and remained stable in 24 (22.6%). The postoperative new-onset or worsened symptoms occurred in 53 cases. During the follow-up period, 58.5% of these symptoms improved and 32.1% remained stable. The mean modified Rankin score (mRS) score was 2.51 ± 0.90 preoperatively, 2.73 ± 0.83 postoperatively, and 1.71 ± 0.98 at the recent follow-up. The surgery-related mortality was 1.7% (n = 2), and two patients suffered from recurrence during the follow-up period. The preoperative mRS was considered to be an independent predictive factor of the neurological outcome (P = 0.003). CONCLUSIONS Safe resection and a favourable outcome can be achieved via a standardized surgical strategy based on appropriate surgical indications, optimal selection of safe trajectories, and application of advanced supplementary techniques in the surgical treatment of BCMs.

Three adolescents with Lhermitte-Duclos disease

Lhermitte-Duclos disease (LDD) is a rare, benign cerebellar disorder, characterized by replacement of granular cells and Purkinje cells by an overgrowth of cerebellar ganglion cells. LDD can be associated with Cowdens syndrome (CS). We studied three adolescent LDD patients, including two with CS. MRI revealed the typical tiger-stripe pattern in all patients. CS was diagnosed on the basis of secondary mucocutaneous lesions and a PTEN/MMAC1 gene mutation. Adolescent-onset LDD appears to have the same phenotype and genotype as adult-onset LDD. Total resection of the lesion is recommended as an initial management strategy. We strongly recommend that patients with LDD undergo genetic screening to find out as early as possible whether CS has developed.

Decompressive craniectomy in hemorrhagic cerebral venous thrombosis: clinicoradiological features and risk factors

OBJECTIVE Decompressive craniectomy (DC) is a life-saving treatment for severe hemorrhagic cerebral venous thrombosis (CVT). However, the correlations between the clinicoradiological features and surgical outcomes of this disease are not well established. Therefore, the authors endeavored to analyze the potential risk factors for this more severe subtype of CVT and to provide more evidence regarding the benefits of DC in patients with hemorrhagic CVT. METHODS The clinical features, radiological findings, and surgical outcomes of patients with severe hemorrhagic CVT who had undergone DC treatment in the period from January 2005 to March 2015 were retrospectively analyzed, and the risk factors for this disease were evaluated. RESULTS Fifty-eight patients, 39 females (67.2%) and 19 males (32.8%), with a mean age of 39.7 ± 12.5 years, were included in this study. The mean duration from symptom onset to surgery was 3.3 ± 1.9 days, and 21 patients experienced acute courses. On neuroimaging, the mean mass lesion volume was 114.7 ± 17.7 ml. Nine patients had bilateral lesions, and 7 patients had deep CVT. According to their hemorrhagic proportion, cases were divided into hemorrhage-dominated (27 [46.6%]) and edema-dominated (31 [53.4%]) groups. After 6 months of follow-up, 56.9% of patients had achieved a favorable outcome, and 8 patients had died. The hemorrhage-dominated lesions (p = 0.026) and deep cerebral venous involvement (p = 0.026) were significantly associated with a poor outcome. CONCLUSIONS In patients suffering from severe hemorrhagic CVT, DC is an effective life-saving treatment that is associated with favorable outcomes. Hemorrhage-dominated lesions and deep cerebral venous involvement have a significant impact on the outcome of this disease.

Surgical treatment of cavernous malformations involving medulla oblongata.

Surgical treatment of cavernous malformations (CMs) involving medulla oblongata is more difficult than the CMs in other sites because of the surrounding vital structures. However, the distinctive features and treatment strategies have not been well illustrated. Therefore, we enrolled a total of 19 patients underwent surgical treatment of CMs involving medulla oblongata in our hospital from August 2008 to August 2014. The clinical features, surgical management and clinical outcome of these patients were retrospectively analyzed, while our institutional surgical indications, approaches and microsurgical techniques were discussed. In our study, gross total resection was achieved in 17 patients and subtotal resection in 2. Two patients underwent emergency surgeries due to severe and progressive neurological deficits. The postoperative new-onset or worsened neurological deficits occurred in 6 patients. After a mean follow-up of 45.8±22.2months, the neurological status was improved in 10 patients and remained stable in 7. The mean modified Rankin Scale (mRS) was 2.58±1.26 preoperatively, 3.11±0.99 postoperatively and 1.84±1.42 at the recent follow-up, respectively. During the follow-up period, no rehemorrhage and recurrence occurred, and the residual lesions remained stable. We recommended surgical resection of symptomatic CMs involving medulla oblongata via optimal approaches, feasible entry zones and meticulous microsurgical techniques in attempting to achieve safe resection and favorable outcome. The clinical features, surgical indications, timing and microsurgical techniques of this special entity should be distinctive from the brainstem cavernous malformations in other sites.

Effects of microvascular decompression on depression and anxiety in trigeminal neuralgia: A prospective cohort study focused on risk factors and prognosis

OBJECTIVE Patients with trigeminal neuralgia (TN) often develop a terrible fear of triggering pain, which may lead to depression and anxiety, exerting a negative effect on their quality of life. This study aimed to investigate the prevalence and risk factors of depression and anxiety in TN patients, and further to investigate the effects of microvascular decompression (MVD) on these psychiatric disorders. PATIENTS AND METHODS A prospective cohort study was conducted, patients with TN who underwent MVD in our department between 2013 and 2015 were included. Visual analogue scale (VAS) score was used to measure the severity of pain. Beck Depression Inventory (BDI) and Beck Anxiety Inventory (BAI) were used to evaluate depression and anxiety disorders before and 6-month after MVD. The clinical data of these patients were collected prospectively and statistically analyzed. RESULTS A total of 128 study subjects comprising 70 women and 58 men with a mean age of 47.5±11.2years were included in this study. The mean VAS score was 7.7±1.5. Eighty-three patients (64.8%) had depression and 24 patients (18.8%) suffered anxiety. Multivariate logistic regression analysis revealed that female gender (OR=2.4, P=0.036), high pain intensity (OR=3.25, P=0.027) and ineffective medicine treatment (OR=1.89, P=0.041) were associated with depression, and female gender (OR=3.45, P=0.034) and high pain intensity (OR=2.88, P=0.022) were also associated with anxiety. There were significant improvements in depression and anxiety symptoms between pre- and postoperative responses. CONCLUSIONS Depression and anxiety are prevalent in patients with idiopathic TN. Female gender, high pain intensity and ineffective medicine treatment are risk factors. MVD not only provides high pain-relief rate, but also leads to significant improvements in the depression and anxiety symptoms.

Expression of angiopoietins in central nervous system hemangioblastomas is associated with cyst formation.

PURPOSE This study aimed to determine the expression of angiopoietin-1 (Ang-1), angiopoietin-2 (Ang-2), and endothelial tyrosine kinase receptor (Tie-2) in central nervous system (CNS) hemangioblastomas (HBs) and explore the correlation with peritumoral cyst formation. METHOD A total of 22 paraffin-embedded specimens derived from 11 cyst-associated and 11 solid solitary CNS HBs were collected for streptavidin-peroxidase immunohistochemical staining. The expression levels of Ang-1, Ang-2 and Tie-2 in cystic and solid HBs were estimated and compared according to the integrated optical density (IOD) of staining in the sections. To further confirm the differential expression of Ang-1 and Ang-2 between cystic and solid HBs, an additional 9 frozen specimens from 5 cyst-associated and 4 solid solitary HBs were collected for Western blot analysis. Clinical histories and radiological records of the patients were reviewed retrospectively. RESULTS Compared to normal cerebellum tissue, Ang-1, Ang-2 and Tie-2 were prominently overexpressed in both the stromal cells and endothelial cells of CNS HBs. The expression of Ang-2 and the ratio of Ang-2/Ang-1 in the cystic group were significantly higher than those in the solid group. The intensity of Ang-2 expression in the cystic group was negatively correlated with age. There was no correlation between the expression level of the proteins and sex, tumor location, tumor volume or cyst volume. CONCLUSIONS Ang/Tie signaling is upregulated in HBs and is likely associated with the process of intense vascularization and cyst formation. Higher Ang-2 levels and a higher Ang-2/Ang-1 ratio may contribute to cyst formation by increasing the vascular permeability. The decrease in Ang-2 expression with advanced age in cystic-associated HBs may be associated with the reduced growth rate of peritumoral cysts among elderly patients. Ang-2 shows great potential as an effective therapeutic target of symptomatic CNS HBs associated with cysts.

Microvascular decompression for trigeminal neuralgia in patients with failed gamma knife surgery: Analysis of efficacy and safety.

OBJECTIVE Though it is usually successful, failure or delayed pain recurrence may occur after gamma knife surgery (GKS) in patients with trigeminal neuralgia (TN), and additional intervention may be required. This study aimed to investigate whether the safety and efficacy of microvascular decompression (MVD) were influenced by prior GKS. PATIENTS AND METHODS The authors retrospectively evaluated 36 consecutive TN patients who underwent MVD after failed GKS from January 2012 to June 2013. The clinical features, operative findings and surgical outcomes were reviewed and statistically analyzed, and the operation results were further compared with a cohort of 60 patients with no prior GKS. RESULTS At surgery, atrophy of the trigeminal nerve was observed in 13 patients (36.1%), arachnoid thickening in 6 patients (16.7%), adhesions between vessels and the trigeminal nerve in 8 patients (22.2%), and atherosclerotic plaque in the offending vessels in 3 patients (8.3%). The complete pain relief rates were 83.3% immediately after MVD and 72.2% at last follow-up, which showed no statistical difference when compared with patients without GKS. New or worsened facial numbness occurred in 7 patients (19.4%), which was significantly higher than those without GKS (p=0.02). Univariate analysis suggested that a positive pain response to the prior GKS correlated with better long-term outcome (p=0.015), and the existence of arachnoid adhesions correlated with higher risk of facial numbness (p=0.03). CONCLUSIONS MVD remains an appropriate and effective alternative therapy for patients with failed GKS, with no added technical difficulty. However, the risk of facial numbness seems to be higher than those with MVD alone.

Alveolar echinococcosis presenting with simultaneous cerebral and spinal involvement

A 37-year-old Tibetan shepherdess presented with headache and weakness of the lower limbs for 10 months. Examination disclosed a positive straight-leg-raising test. Neuroimaging showed a heterogeneous mass in the left temporal lobe (figure 1) and an intradural mass in the lumbosacral canal with L4 vertebral body destruction (figure 2). Chest and abdominal CT scans were negative. Two operations yielded a tissue diagnosis of alveolar echinococcosis and postoperative albendazole therapy was started. Simultaneous involvement of brain and the spinal cord in primary extrahepatic alveolar echinococcosis is rare. Early diagnosis and radical surgery followed by continuous benzimidazole treatment are crucial.1