Proteomic analysis predicts anti-angiogenic resistance in recurred glioblastoma.

BACKGROUND: Recurrence is common in glioblastoma multiforme (GBM) because of the infiltrative, residual cells in the tumor margin. Standard therapy for GBM consists of surgical resection followed by chemotherapy and radiotherapy, but the median survival of GBM patients remains poor (~ 1.5 years). For recurrent GBM, anti-angiogenic treatment is one of the common treatment approaches. However, current anti-angiogenic treatment modalities are not satisfactory because of the resistance to anti-angiogenic agents in some patients. Therefore, we sought to identify novel prognostic biomarkers that can predict the therapeutic response to anti-angiogenic agents in patients with recurrent glioblastoma. METHODS: We selected patients with recurrent GBM who were treated with anti-angiogenic agents and classified them into responders and non-responders to anti-angiogenic therapy. Then, we performed proteomic analysis using liquid-chromatography mass spectrometry (LC-MS) with formalin-fixed paraffin-embedded (FFPE) tissues obtained from surgical specimens. We conducted a gene-ontology (GO) analysis based on protein abundance in the responder and non-responder groups. Based on the LC-MS and GO analysis results, we identified potential predictive biomarkers for anti-angiogenic therapy and validated them in recurrent glioblastoma patients. RESULTS: In the mass spectrometry-based approach, 4957 unique proteins were quantified with high confidence across clinical parameters. Unsupervised clustering analysis highlighted distinct proteomic patterns (n = 269 proteins) between responders and non-responders. The GO term enrichment analysis revealed a cluster of genes related to immune cell-related pathways (e.g., TMEM173, FADD, CD99) in the responder group, whereas the non-responder group had a high expression of genes related to nuclear replisome (POLD) and damaged DNA binding (ERCC2). Immunohistochemistry of these biomarkers showed that the expression levels of TMEM173 and FADD were significantly associated with the overall survival and progression-free survival of patients with recurrent GBM. CONCLUSIONS: The candidate biomarkers identified in our protein analysis may be useful for predicting the clinical response to anti-angiogenic agents in patients with recurred GBM.

Intracranial solitary fibrous tumor/hemangiopericytoma: tumor reclassification and assessment of treatment outcome via the 2016 WHO classification.

PURPOSE: As per the 2016 World Health Organization (WHO) guidelines on the classification of central nervous system tumors, solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) constitute a single disease entity, known as SFT/HPC. This study provides a clinical analysis of these tumors and describes the treatment outcomes of SFT/HPCs. METHODS: This retrospective study included 76 patients with histopathologically proven SFT/HPC. Reclassification according to the 2016 WHO guideline was done for patients who were diagnosed with SFT or HPC based on the 2007 WHO classification. Recurrence-free survival (RFS) and overall survival (OS) were evaluated for all patients and subgroups. RESULTS: The median follow-up period was 77.9 months. The median RFS and OS were 126.5 and 136.8 months, respectively. The 1-, 5-, 10-, and 15-year RFS rates were 93%, 72%, 40%, and 40%, respectively. The 1-, 5-, 10- and 15-year OS rates were 97%, 89%, 54%, and 35%, respectively. In multivariable analyses, stereotactic radiosurgery (SRS; p = 0.009, hazard ratio [HR] 6.986), female sex (p = 0.023, HR 1.76), and age over 45 (p = 0.037, HR 2.74) were associated with shorter RFS. Patients who underwent SRS as initial treatment had a shorter OS than that of patients who underwent primary resection (p < 0.001, HR 12.86). CONCLUSIONS: High-grade tumors tended to have worse OS and occur extracranial metastases earlier than low-grade tumors. The median RFS was not different between grade II and III tumors. Male sex, younger age, and GTR were associated with a better RFS. A history of SRS before tumor resection was associated with a shorter RFS and OS.

Atlantoaxial instability treated with free-hand C1-C2 fusion in a child with Morquio syndrome.

Mucopolysaccharidosis type IVA, also known as Morquio syndrome, is an autosomal recessive lysosomal storage disease. Skeletal dysplasia with short stature, dysplastic-hypoplastic dens (os odontoideum), ligamentous hyperlaxity, and C1-C2 instability are characteristic features. Most patients with Morquio syndrome present with compressive myelopathy at a young age as a result of a combination of C1-C2 instability and extradural soft tissue thickening; treatment generally consists of anterior decompression with occipito-cervical fusion and external orthosis. In this report, we describe the successful treatment of a young child using posterior C1-C2 fusion alone with a free-hand technique. A 3-year-old boy presented at our hospital with a 5-month history of progressive quadriparesis. A whole-body skeletal survey showed skeletal dysplasia with hypoplasia, thoracolumbar kyphosis, and atlantoaxial subluxation. Preoperative cervical imaging showed compressive myelopathy at C1-C2 and atlantoaxial subluxation. C1-C2 fixation and decompression were performed successfully. After the operation, the patient had improved strength and was able to walk independently 8 months postoperatively. Establishment of stability via C1-C2 fusion is challenging in patients with genetic disorders characterized by skeletal dysplasia because of these young patients' small bone size and deficient bone quality. In this unique case, the treatment consisted solely of C1-C2 fusion with a free-hand technique. This case report presents a new approach in the treatment of atlantoaxial instability in Morquio syndrome.

Decreased MEPs during subcutaneous dissection for untethering surgery of a "true" lipomyelomeningocele: aggravated traction of the spinal cord by release of the sac from the original nest.

PURPOSE: It is well known that the preoperative enlarged sac in occult spinal dysraphism, in which the spinal cord is attached to its dome, can be associated with neurological deficits by aggravation of spinal cord traction. We experienced a similar phenomenon during the early stage of untethering surgery for "lipomyelomeningocele in a strict sense (LMMC)." CLINICAL PRESENTATION: We report a case of a 3-month-old girl with LMMC, which showed decreased motor evoked potentials (MEPs) in the lower extremities during the early stage of untethering surgery. The sac was released from the original nest by incision with separation of the overlying skin and dissection from the muscle fascia. The sac became rounder, and the spinal cord attached to the dome of the sac was further stretched. After aspiration of cerebrospinal fluid from the sac, the MEPs recovered. Postoperatively, the patient had no neurological deficits. CONCLUSIONS: Although this phenomenon rarely occurs, release of the herniated sac of LMMC from the original nest during the early stage of untethering surgery may result in neurological changes. Application of intraoperative neurophysiological monitoring helps to detect electrophysiological deterioration and can prevent neurological deficits.

Radical excision of lumbosacral lipoma: an early experience of "followers".

BACKGROUND: Indication, timing, and method for surgical treatment of lumbosacral lipoma are controversial. Radical resection of the lumbosacral lipoma and complete reconstruction of the placode are supported in that better long-term outcome can be achieved without increasing complication rate compared with traditional surgical techniques. OBJECTIVE: We analyzed the early surgical outcomes of lumbosacral lipoma treated with the untethering and radical excision of fat. METHODS: Retrospective analysis of surgically treated 81 fresh lumbosacral lipoma cases with dorsal, transitional, and chaotic types and true lipomyelomeningocele (LMMC) was performed. Caudal and filar types were excluded. RESULTS: Complete untethering was accomplished in 98%. Radical excision of the lipoma was attempted in all cases and achieved in 83%. Postoperative neurological complication was observed in 8 cases (10%). Group of lipoma types (dorsal + transitional vs. chaotic + true LMMC) and availability of radical lipoma excision turn out to be factors related to neurological outcomes in univariate analysis (p < 0.001 and p = 0.027, respectively). Group of lipoma types, availability of radical excision, and postoperative cord/dural sac (C/D) ratio are related factors in multivariate analysis (p = 0.025, p = 0.049, and p = 0.031). CONCLUSIONS: As a follower of untethering and radical excision of fat, careful consideration is required to plan the surgery of lumbosacral lipoma on account of the "underestimated" complication rate. Type of the lipoma is the important factor determining the surgical outcome. Availability of complete radical excision and postoperative C/D ratio are the operative factors related to the neurological outcomes.

Benign extracerebral fluid collection complicated by subdural hematoma and fluid collection: clinical characteristics and management.

INTRODUCTION: Benign extracerebral fluid collection (bECFC) can be complicated by subdural hematoma (SDH) or subdural fluid collection (SDFC). The etiology, natural history, and management strategy for SDH/SDFC in bECFC are not fully understood. We retrospectively reviewed the cases of bECFC patients complicated with SDH/SDFC and tried (1) to confirm the fact that bECFC children are vulnerable to SDH/SDFC, (2) to investigate the clinical significance of 'trauma history' witnessed by a caregiver, and (3) to determine optimal management for them. METHOD: Among 213 bECFC patients identified from January 2000 to August 2015, 20 patients (male:female = 14:6; median age, 6.5 months; range 1-16 months) complicated by SDH/SDFC documented with brain imaging were evaluated for their clinical manifestations, radiologic features, and management outcomes. The median follow-up period was 9.5 months. They were divided into two groups (traumatic group versus non-traumatic group) according to whether objective radiologic evidence of head injury was present or not, and the two groups were analyzed for any clinical differences between them. We also evaluated the clinical significance of witnessed traumatic events by caregivers as an additional independent variable in the analysis. RESULTS: The incidence of SDH/SDFC in bECFC patients was 9.4% (20/213) in our data. In a comparative analysis, the traumatic group is more likely to have 'acute' stage SDH, whereas the non-traumatic group is more likely to have 'chronic' stage SDH. The trauma history witnessed by caregivers did not show clinical significance in the data analysis when included as an independent variable. The prognosis of SDH/SDFC in bECFC patients was favorable without surgery in most of patients regardless of whether the patient has evidence of head trauma or not. CONCLUSION: Benign ECFC is vulnerable to SDH/SDFC development. For the bECFC patients complicated by SDH/SDFC, the trauma history witnessed by a caregiver did not show any clinical significance. A 'wait and watch' strategy is sufficient for the management of SDH/SDFC in bECFC patients.

Visual outcome after endoscopic third ventriculostomy for hydrocephalus.

PURPOSE: Hydrocephalus-related symptoms are mostly improved after successful endoscopic third ventriculostomy (ETV). However, visual symptoms can be different. This study was focused on visual symptoms. We analyzed the magnetic resonance images (MRI) of the orbit and visual outcomes. METHODS: From August 2006 to November 2016, 50 patients with hydrocephalus underwent ETV. The male-to-female ratio was 33:17, and the median age was 61 years (range, 5-74 years). There were 18 pediatric and 32 adult patients. Abnormal orbital MRI findings included prominent subarachnoid space around the optic nerves and vertical tortuosity of the optic nerves. We retrospectively analyzed clinical symptoms, causes of hydrocephalus, ETV success score (ETVSS), ETV success rate, ETV complications, orbital MRI findings, and visual impairment score (VIS). RESULTS: The median duration of follow-up was 59 months (range, 3-113 months). The most common symptoms were headache, vomiting, and gait disturbance. Visual symptoms were found in 6 patients (12%). The most common causes of hydrocephalus were posterior fossa tumor in 13 patients, pineal tumor in 12, aqueductal stenosis in 8, thalamic malignant glioma in 7, and tectal glioma in 4. ETVSS was 70 in 3 patients, 80 in 34 patients, and 90 in 13 patients. ETV success rate was 80%. ETVSS 70 showed the trend in short-term survival compared to ETVSS 90 and 80. ETV complications included epidural hematoma requiring operation in one patient, transient hemiparesis in two patients, and infection in two patients. Preoperative abnormal orbital MRI findings were found in 18 patients and postoperative findings in 7 patients. Four of six patients with visual symptoms had abnormal MR findings. Three patients did not show VIS improvement, including two with severe visual symptoms. CONCLUSIONS: Patients with severe visual impairment were found to have bad outcomes. The visual symptoms related with increased intracranial pressure should be carefully monitored and controlled to improve outcomes.

Extensive brain infarction involving deep structures during an acetazolamide-challenged single-photon emission computed tomography scan in a patient with moyamoya disease.

BACKGROUND: Acetazolamide-challenged brain single-photon emission computed tomography (SPECT) is used for the evaluation of cerebral perfusion in cerebrovascular diseases including moyamoya disease (MMD). Not a few patients experience adverse side effects during the acetazolamide-challenged brain SPECT, but most of the symptoms are mild and transient. To our knowledge, this is the first case report of severe brain infarction leading to death during the examination of an acetazolamide-challenged brain SPECT in a patient with MMD. CASE PRESENTATION: An 11-year-old girl who had been diagnosed of MMD demonstrated sudden tonic movement during an acetazolamide-challenged brain SPECT as the preoperative examination for the second surgery. She had not experienced any adverse effect during the previous SPECT study and her first indirect bypass surgery on both left side and bifrontal area was uneventful. After she had seizures twice, she became unconscious and her pupils were dilated and fixed. Acute infarction involving bilateral occipital lobes, thalami, brainstem, and cerebellum was observed on brain magnetic resonance images which led to brain death. CONCLUSION: We report a mortality case of patient with MMD after the administration of acetazolamide during the examination of brain SPECT that was accompanied by an extensive acute infarction involving the bilateral occipital lobes and thalami, brainstem, and cerebellum. Physicians should be aware of this rare but serious complication.

One-year outcome of postoperative swallowing impairment in pediatric patients with posterior fossa brain tumor.

Impaired swallowing in children who underwent posterior fossa brain tumor (PFBT) resection disrupts development and quality of life, yet its downstream consequences remain unclear. This study explored the risk factors and functional prognosis of postoperative swallowing impairment in pediatric patients (<19 years old) with PFBT. Among 183 patients with PFBT who underwent surgical resection, 39 patients with postoperative swallowing difficulty were analyzed using the videofluoroscopic swallowing study (VFSS). The association between clinical features, swallowing characteristics, and swallowing impairment was explored during the early postoperative phase and 1-year following surgical resection. Duration of tube feeding was investigated using Kaplan-Meier analysis. Twenty-seven (14.8 %) patients needed tube feeding in the early postoperative phase and 11 (6.01 %) at 1-year after surgical resection. Mean duration of tube feeding was 240.2 days and differed by tumor pathologies (P = 0.001), delayed triggering of pharyngeal swallow (DTP) (P = 0.002) and pharyngeal wall coating (P = 0.033). Tumor pathology was associated significantly with the referral for swallowing evaluation (P < 0.001) and 1-year tube feeding (P = 0.019). Tube feeding at 1-year was significantly associated with the tumor's brainstem involvement (P = 0.039), and swallowing abnormalities at early phase including DTP (P = 0.030) and pharyngeal wall coating (P = 0.004). Our results suggest that tumor pathology, brainstem involvement, and specific swallowing abnormalities at early phase are important risk factors for sustained 1-year swallowing impairment following surgical resection. These results can be applied to determine the plan of evaluation, nutrition, and intervention in clinical practice.

Pediatric spinal arteriovenous malformations and fistulas: a single institute's experience.

OBJECTIVE: Pediatric spinal vascular diseases are extremely rare. We aimed to evaluate their clinical characteristics and treatment outcomes in our institute. DESIGN: A total of 10 patient files were retrospectively reviewed, including 3 cases of intramedullary arteriovenous malformations (AVMs), 6 cases of perimedullary arteriovenous fistulas (AVFs), and 1 case of epidural AVF. Clinical features, radiological findings, treatment results, and clinical outcomes were evaluated. The median durations of the radiologic and clinical follow-ups were 17.7 and 107.9 months, respectively. RESULTS: The male to female ratio was 1:1, with a median age at diagnosis of 9 years. All AVMs were juvenile type, all perimedullary AVFs were high flow types (three type IVb and three type IVc), and one epidural AVF was associated with intradural venous drainage. Most cases (90 %) were located in the cervical spine and conus medullaris. Acute neurological deterioration was identified in five patients; however, bleeding was identified in only one patient. Two cases were surgically treated, seven cases underwent embolization, and one case underwent radiosurgery. Three cases were completely obliterated, and their clinical states were improved (n = 2; 66.7 %) and stationary (n = 1; 33.3 %). Meanwhile, seven cases were incompletely obliterated, and their clinical states were improved (n = 2; 28.6 %), stationary (n = 3; 42.8 %), and aggravated (n = 2; 28.6 %). CONCLUSIONS: Pediatric spinal AVMs and AVFs were mostly complex and high flow types, and complete obliteration could not be satisfactorily achieved. Incompletely treated lesions should be closely followed up because they may worsen.

A method of posterior fossa dural incision to minimize hemorrhage from the occipital sinus: the "mosquito" method.

PURPOSE: The posterior fossa dural opening requires the ligation of the occipital sinus to gain successful exposure. However, there could be a prominent occipital sinus which is functioning as the main drainage route and is harboring the risk of unpredictable massive hemorrhage during the dural opening. We introduce a safe method of posterior fossa dural incision to minimize hemorrhage from the occipital sinus using four curved hemostat clamps. METHODS: For the dural incision at the midline part of the posterior cranial fossa, we used four curved hemostat clamps to occlude the prominent occipital sinus: one pair of clamps at the proximal part and the other pair at the distal part to occlude the occipital sinus. Dural incision was made between the two pairs of the curved hemostat clamps. RESULTS: By clamping of the sinus, it allows observation of possible brain swelling after occlusion of the occipital sinus as well as minimizes hemorrhage during incision of the midline dura of the posterior fossa. CONCLUSION: This method allows observation of brain swelling after occipital sinus occlusion and is an easy and safe incision of the midline dura minimizing hemorrhage in selected cases with a prominent occipital sinus.

Surgical Strategy for Petroclival Meningioma-Related Trigeminal Neuralgia: The Role of Porus Trigeminus Opening.

OBJECTIVE: Petroclival meningiomas invade Meckel's cave through the porus trigeminus, leading to secondary trigeminal neuralgia. Microsurgery and stereotactic radiosurgery (SRS) are the typical treatment options. This study investigated symptom control, outcomes, and surgical strategies for PC meningioma-induced TN. METHODS: We retrospectively analyzed 28 TN patients with PC meningiomas who underwent microsurgical nerve decompression between January 2021 and February 2023. In all patients undergoing a transpetrosal approach, the porus trigeminus was opened to enable the removal of the entire tumor within Meckel's cave. Clinical outcomes were assessed using the Barrow Neurologic Institute (BNI) pain intensity scale. Risk factors for poor TN outcomes and poor facial numbness were analyzed. RESULTS: Among 28 patients, 21 (75%) underwent the transpetrosal approach, 5 (17.9%) underwent the retrosigmoid approach, and 2 (7.1%) underwent the Dolenc approach. Following microsurgery, 23 patients (82.1%) experienced TN relief without further medication (BNI I or II). TN recurrence occurred in 2 patients (7.1%), and 3 patients (10.7%) did not achieve TN relief. Cavernous sinus invasion was significantly correlated with poor TN outcomes (P = 0.047). A history of previous SRS (P = 0.011) and upper clivus type tumor (P = 0.018) were significantly associated with poor facial numbness. CONCLUSIONS: Microsurgical nerve decompression is effective in improving BNI scores in patients with TN associated with PC meningiomas. Considering the results of our study, the opening of the porus trigeminus can be considered as a suggested procedure in the treatment of PC meningiomas, especially in cases accompanied by TN.

Long-term outcomes of CNS WHO grade 2 oligodendroglioma in adult patients: a single-institution experience.

PURPOSE: Oligodendrogliomas (ODGs) are a subtype of diffuse lower-grade gliomas with overall survival of > 10 years. This study aims to analyze long-term outcomes and identify prognostic factors in patients with WHO grade 2 ODG. METHODS: We retrospectively reviewed 138 adult patients diagnosed with 1p/19q co-deleted ODG who underwent surgical resection or biopsy between 1994 and 2021, analyzing clinical data, treatment details, and outcomes. Progression-free survival (PFS) and overall survival (OS) were evaluated using Kaplan-Meier analysis. Univariate and multivariate Cox regression analyses were utilized to identify significant prognostic factors. RESULTS: In the gross total resection (GTR) group, 63 (45.7%) underwent observation and 5 (3.6%) received postoperative treatment; in the non-GTR group, 37 (26.8%) were observed and 33 (23.9%) received postoperative treatment. The median PFS and OS were 6.8 and 18.4 years, respectively. Between adjuvant treatment and observation, there was no significant difference in PFS or OS. However, GTR or STR with less than 10% residual tumor exhibited significantly better PFS and OS compared to PR or biopsy (p = 0.022 and 0.032, respectively). Multivariate analysis revealed that contrast enhancement on MRI was associated with worse PFS (HR = 2.36, p < 0.001) and OS (HR = 5.89, p = 0.001). And the presence of seizures at presentation was associated with improved OS (HR = 0.28, p = 0.006). CONCLUSION: This study underscores favorable long-term outcomes for patients with 1p/19q co-deleted ODG WHO grade 2. Our findings indicate that the EOR plays a crucial role as a significant prognostic factor in enhancing PFS and OS outcomes in WHO grade 2 ODG.

The return of an old worm: cerebral paragonimiasis presenting with intracerebral hemorrhage.

Paragonimiasis is caused by ingesting crustaceans, which are the intermediate hosts of Paragonimus. The involvement of the brain was a common presentation in Korea decades ago, but it becomes much less frequent in domestic medical practices. We observed a rare case of cerebral paragonimiasis manifesting with intracerebral hemorrhage. A 10-yr-old girl presented with sudden-onset dysarthria, right facial palsy and clumsiness of the right hand. Brain imaging showed acute intracerebral hemorrhage in the left frontal area. An occult vascular malformation or small arteriovenous malformation compressed by the hematoma was initially suspected. The lesion progressed for over 2 months until a delayed surgery was undertaken. Pathologic examination was consistent with cerebral paragonimiasis. After chemotherapy with praziquantel, the patient was monitored without neurological deficits or seizure attacks for 6 months. This case alerts practicing clinicians to the domestic transmission of a forgotten parasitic disease due to environmental changes.

Head Injury during Childbirth.

Head injuries are the most common type of birth injuries. Among them, most of the injuries is limited to the scalp. and the prognosis is good enough to be unnoticed in some cases. Intracranial injuries caused by excessive forces during delivery are rare. However, since some of them can be fatal, it is necessary to suspect it at an early stage and evaluate thoroughly if there are abnormal findings in the patient.

Clinical Features and Prognosis of Diffuse Midline Glioma: A Series of 24 Cases.

BACKGROUND: Diffuse midline glioma (DMG) which occurs in midline structures and characterized by harboring K27M mutation in genes encoding the histone 3 protein is classified as World Health Organization (WHO) grade IV regardless of histological findings and has a poor prognosis. Nevertheless, because of its relatively rare incidence compared with other high-grade gliomas, a comprehensive description encompassing clinical features and genomic profiles of DMG is still lacking. METHODS: In this study, we analyzed data of 24 patients who were diagnosed as DMG which was confirmed by surgical specimens in both pediatric and adult patients. We described the clinical outcomes of patients with DMG and their genomic profiles through a retrospective analysis of 24 patients with DMG. RESULTS: The clinical characteristics of the 24 patients with DMG were analyzed. Ten patients (41%) underwent tumor resection and 14 patients (59%) underwent tumor biopsy. The median overall survival was 10.4 months (95% confidence interval [CI], 8.4 to 12.5) and progression free survival was 3.9 months (95% CI, 2.6 to 5.2). Fifteen patients (62%) were accompanied by hydrocephalus. None of the patient, tumor, or treatment factors had any significant associated with survival. In both immunohistochemistry staining (n=24) and targeted next generation sequencing (n=15), TP53 mutation was the most common genetic mutation (25% and 46%, respectively) found in the patients except alterations in histone 3 protein. CONCLUSION: Although surgical treatment of patient with DMG does not affect the overall survival prognosis, it can help improve the patient's accompanying neurological symptoms in some limited cases. Hydrocephalus is often accompanied with DMG and treatment for hydrocephalus is often also required. Multidisciplinary therapeutic approach is needed.

Individualized ventricular access using multi-plane brain images.

PURPOSE: Recently, many surgical techniques are applied to accurate ventricular catheterization. However, there are still some limitations in utilizing those methods. We introduce a simple method for the ventricular access and report two illustrative cases using our method. METHODS: We marked the trajectory using the preoperative multi-planar brain images and verifying it with surface markers. RESULTS: Two illustrative cases are shown, in which the surgeries were performed without difficulty and the outcomes were satisfactory. CONCLUSION: Utilizing multi-planar brain images in ventricular access is cost-effective and competent method.

Treatment Outcome of Hydrocephalus Associated with Vestibular Schwannoma.

BACKGROUND AND PURPOSE: Managing hydrocephalus in patients with vestibular schwannoma (VS) is controversial. We evaluated the clinical factors associated with hydrocephalus. METHODS: Between 2000 and 2019, 562 patients with VS were treated at our institute. We applied endoscopic third ventriculostomy (ETV), external ventricular drainage (EVD), and ventriculoperitoneal (VP) shunts to patients with hydrocephalus. The relationships of patient, tumor, and surgical variables with the hydrocephalus outcome were assessed. RESULTS: Preoperative hydrocephalus (Evans ratio ≥0.3) was present in 128 patients. Six patients who received a preresectional VP shunt were excluded after analyzing the hydrocephalus outcome. Seven of the remaining 122 patients had severe hydrocephalus (Evans ratio ≥0.4). Primary tumor resection, VP shunting, ETV, and EVD were performed in 60, 6, 57, and 5 patients, respectively. The hydrocephalus treatment failure rate was highest in the EVD group. Persistent hydrocephalus was present in five (8%) and seven (12%) patients in the primary resection and ETV groups, respectively. Multivariate analysis revealed that severe hydrocephalus, the cystic tumor, and the extent of resection (subtotal resection or partial resection) were associated with hydrocephalus treatment failure. CONCLUSIONS: Larger ventricles and a higher cystic portion are predictive of persistent hydrocephalus. We recommend attempting near-total tumor resection in patients with VS.

Limited Dorsal Myeloschisis: Reconsideration of its Embryological Origin.

BACKGROUND: Limited dorsal myeloschisis (LDM) is postulated to be a result of incomplete dysjunction in primary neurulation. However, clinical experience of LDM located below the first-second sacral (S1-S2) vertebral level, which is formed from secondary neurulation (S2-coccyx), suggested that LDM may not be entirely explained as an error of primary neurulation. OBJECTIVE: To elucidate the location and characteristics of LDM to investigate the possible relation of its pathoembryogenesis to secondary neurulation. METHODS: Twenty-eight patients were surgically treated for LDM from 2010 to 2015. Since the level where the LDM stalk penetrates the interspinous ligament is most clearly defined on the preoperative MRI and operative field, this level was assessed to find out whether the lesions can occur in the region of secondary neurulation. RESULTS: Eleven patients (39%) with typical morphology of the stalk had interspinous defect levels lower than S1-S2. These patients were not different from 17 patients with classic LDMs at a level above or at S1-S2. This result shows that other than the low level of the interspinous level, 11 patients had lesions that could be defined as LDMs. CONCLUSION: By elucidating the location of LDM lesions (in particular, the interspinous level), we propose that LDM may be caused by errors of secondary neurulation. The hypothesis seems more plausible due to the supportive fact that the process of separation between the cutaneous and neural ectoderm is present during secondary neurulation. Hence, incomplete disjunction of the two ectoderms during secondary neurulation may result in LDM, similar to the pathomechanism proposed during primary neurulation.

Postoperative Symptomatic Cerebral Infarction in Pediatric Moyamoya Disease: Risk Factors and Clinical Outcome.

BACKGROUND: Indirect bypass surgery is used to improve the hemodynamic status of pediatric patients with moyamoya disease (MMD). Symptomatic cerebral infarction during the early postoperative period may be the most frustrating complication. This study was conducted to investigate the factors associated with early postoperative symptomatic cerebral infarction. METHODS: Between January 2000 and February 2014, we performed 1241 indirect bypass surgeries in 659 pediatric MMD patients. Symptomatic infarction during the early postoperative period was diagnosed in 63 operations in 61 patients. RESULTS: The overall incidence of symptomatic cerebral infarction after indirect bypass surgery was 5.1%. The median age of the patients with postoperative infarction was 6 years (mean, 6.4 years; range, 1-15 years). The performance of 2 craniotomies in single operation was associated with a higher rate of cerebral infarction. Moreover, the incidence was higher in young patients (age <6 years) compared with older patients. In a matched analysis, an immediate postoperative hemoglobin level >13 g/dL was associated with decreased risk of infarction (odds ratio, 0.144; P = 0.003). Mutation of the methylenetetrahydrofolate reductase (MTHFR) gene occurred in a relatively high proportion of our infarction cohort. CONCLUSIONS: Postoperative symptomatic infarctions can occur despite a unified surgical method and formulaic perioperative management protocol. Patient-centered factors, such as young age, genetic background of MTHFR, and certain medical conditions, including hyperthyroidism, renovascular hypertension, and hemolytic uremic syndrome, as well as management-related factors, including 2 craniotomies and low immediate postoperative hemoglobin level, could be risk factors for early postoperative symptomatic cerebral infarction.