Focal Spinal Nondisjunctional Disorders: Including a Discussion on the Embryogenesis of Cranial Focal Nondisjunctional Lesions.

The publication of a comprehensive report on limited dorsal myeloschisis by the senior author (DP) in 2010 has brought full attention to the concept of limited myeloschisis that he first formulated in 1992 and ignited interests in the whole spectrum of focal spinal nondisjunctional disorders. Now that focal nondisjunctional disorders have become well known, new clinical reports on these conditions or relevant subjects are frequently seen. Here we present an updated review on the full spectrum of focal spinal nondisjunctional disorders and extend the scope to include a discussion on the embryogenesis of cranial focal nondisjunctional malformations.

Radiotherapy-induced glioblastoma: distinct differences in overall survival, tumor location, pMGMT methylation and primary tumor epidemiology in Hong Kong chinese patients.

INTRODUCTION: Radiotherapy-induced glioblastomas (RIGB) are a well-known late and rare complication of brain irradiation. Yet the clinical, radiological and molecular characteristics of these tumors are not well characterized. METHODS: This was a retrospective multicentre study that analysed adult patients with newly diagnosed glioblastoma over a 10-year period. Patients with RIGB were identified according to Cahan's criteria for radiation-induced tumors. A case-control analysis was performed to compare known prognostic factors for overall survival (OS) with an independent cohort of IDH-1 wildtype de novo glioblastomas treated with standard temozolomide chemoradiotherapy. Survival analysis was performed by Cox proportional hazards regression. RESULTS: A total of 590 adult patients were diagnosed with glioblastoma. 19 patients (3%) had RIGB. The mean age of patients upon diagnosis was 48 years ± 15. The mean latency duration from radiotherapy to RIGB was 14 years ± 8. The mean total dose was 58Gy ± 10. One-third of patients (37%, 7/19) had nasopharyngeal cancer and a fifth (21%, 4/19) had primary intracranial germinoma. Compared to a cohort of 146 de novo glioblastoma patients, RIGB patients had a shorter median OS of 4.8 months versus 19.2 months (p-value: <.001). Over a third of RIGBs involved the cerebellum (37%, 7/19) and was higher than the control group (4%, 6/146; p-value: <.001). A fifth of RIGBs (21%, 3/19) were pMGMT methylated which was significantly fewer than the control group (49%, 71/146; p-value: .01). For RIGB patients (32%, 6/19) treated with re-irradiation, the one-year survival rate was 67% and only 8% for those without such treatment (p-value: .007). CONCLUSION: The propensity for RIGBs to develop in the cerebellum and to be pMGMT unmethylated may contribute to their poorer prognosis. When possible re-irradiation may offer a survival benefit. Nasopharyngeal cancer and germinomas accounted for the majority of original malignancies reflecting their prevalence among Southern Chinese.

Epidural multi-slitted microporous non-absorbable patch in decompressive craniectomy to facilitate cranioplasty: a preliminary study.

Developing a surgical plane between the temporalis muscle and the dura is the most technically challenging step when performing cranioplasty for post-decompressive craniectomy defects. The authors report a simple technique to demarcate this surgical plane by laying a multi-slitted, microporous polyesterurethane (MPU) patch during decompressive craniectomy. Specifically, they tried to avoid creating potential spaces around the patch, which is the inherent drawback of published anti-adhesive techniques. In 21 patients undergoing decompressive craniectomy, and in 11 of them subsequently undergoing cranioplasty, there was no wound related complications. During cranioplasty, no epidural fluid collection was found; the patch could be separated from the temporalis muscle and the dura with blunt dissection leaving the muscle intact. They conclude that their epidural MPU patch technique is a safe technique, and appears useful to facilitate cranioplasty by helping the surgeon in developing the surgical plane between the temporalis muscle and the dura during cranioplasty.

Innervation of the brain, intracerebral Schwann cells and intracerebral and intraventricular schwannomas.

The cerebral vasculature and the choroid plexus are innervated by peripheral nerves. The anatomy of the vascular supply to the brain and its related perivascular nerves is reviewed. Intracerebral and intraventricular schwannomas most likely come from neoplastic transformation of Schwann cells investing the perivascular nerves and nerves within the choroid plexus.

AB011. A propensity-score matched prospective study on the impact of tumour treating fields (TTF) on overall survival and quality of life in newly diagnosed WHO grade 4 astrocytoma patients.

BACKGROUND: World Health Organization (WHO) grade 4 astrocytoma is a high-grade brain tumour in adults. Tumour treating fields (TTF) has been shown to improve overall survival (OS). Few studies have explored quality-of-life (QoL) in these patients. This study aims to assess the QoL of TTF patients and OS. METHODS: This was a prospective multicenter study of adult patients diagnosed with WHO grade 4 astrocytoma from 2018 to 2023 receiving TTF for >1 month after completing standard therapy. A propensity-score matched comparison with a 1:2 ratio with historical control was performed for OS analysis. The patients completed European Organisation for Research and Treatment of Cancer (EORTC) QLQ-30/BN20 questionnaires before TTF and at 3-month interval. Primary outcomes included OS, and secondary outcomes included QoL and TTF-associated adverse effects at 3 months. RESULTS: A total of 141 patients were reviewed, with TTF patients (n=47, 33%) and propensity-score matched controls (n=94). The mean duration of TTF use was 10±8 months. The mean age of the TTF group was 54±13 years, and for the control group 52±13 years. Sixty percent (n=28) were male, similar to the control group with 71% (n=67) (P=0.16). Seventy-two percent of TTF patients had preoperative Karnofsky Performance Scale (KPS) score ≥80, while controls had 70% (P=0.79). Five (11%) TTF patients and 8 (9%) controls were IDH1 mutant (P=0.70). Twenty (43%) TTF patients and 42 (45%) controls were O6-methylguanine-DNA methyltransferase promoter (pMGMT) methylated (P=0.81). Twenty-one (45%) of TTF patients and 55 (59%) of controls had gross total resection (P=0.72). After adjusting for independent predictors for OS, the median OS of the TTF group was 22.4 months [interquartile range (IQR): 18.6-26.5 months], significantly longer than the control group (17.2 months; IQR: 12.1-22.3 months) (log-rank test: P=0.01). Forty-seven TTF patients and 40 control patients completed EORTC questionnaires. There was no difference for EORTC functional and symptom scores between the TTF and control group [P=0.45, analysis of variance (ANOVA)] at 3 months. Thirty-two (67%) of TTF patients reported associated RTOG grade I scalp dermatitis. CONCLUSIONS: TTF for WHO grade 4 astrocytoma patients is an independent predictor for OS. QoL between the groups was similar, and overall QoL over time for TTF patients was not affected. TTF is a novel and effective outpatient treatment with minimal adverse effects.

Limited dorsal myeloschisis: a not-so-rare form of primary neurulation defect.

PURPOSE: Limited dorsal myeloschisis (LDM) is a distinctive form of spinal dysraphism characterized by two constant features: a focal "closed" midline skin defect and a fibroneural stalk that links the skin lesion to the underlying cord. The embryogenesis is hypothesized to be incomplete disjunction between cutaneous and neural ectoderms, thus preventing complete midline skin closure and allowing persistence of a physical link (fibroneural stalk) between the disjunction site and the dorsal neural tube. OBJECTIVE: We utilize the experience gained from the management of 63 patients with LDM to illustrate these features. METHODS: All patients were studied with MRI or CT myelogram, operated on, and followed for a mean of 9.4 years. RESULTS: There were 11 cervical, 16 thoracic, 8 thoracolumbar, and 28 lumbar lesions. Two main types of skin lesion were: saccular (26 patients; consisting of a skin base cerebrospinal fluid sac topped with squamous epithelial dome or a thin membranous sac) and nonsaccular (37 patients; with a flat or sunken squamous epithelial crater or pit). The internal structure of a saccular LDM could be a basal neural nodule, a stalk that inserts on the dome, or a segmental myelocystocoele. In nonsaccular LDMs, the fibroneural stalk has variable thickness and complexity. All fibroneural stalks contain glioneuronal tissues accompanied by variable quantities of nerves and mesodermal derivatives. In all LDMs, the fibroneural stalk was tethering the cord. Twenty-nine patients had neurological deficits. There was a positive correlation between neurological grade and age suggesting progression with chronicity. Treatment consisted of detaching the stalk from the cord. Most patients improved or remained stable. LDMs were associated with three other dysraphic malformations in more than coincidental frequencies: six LDMs were contiguous with dorsal lipomas, four LDMs shared the same tract or traveled in parallel with a dermal sinus tract, and seven LDMs were related to a split cord malformation. The embryogenetic implications of these associations are discussed. CONCLUSION: LDM is a distinctive clinicopathological entity and a tethering lesion with characteristic external and internal features. We propose a new classification incorporating both saccular and flat lesions.

Surgical treatment of complex spinal cord lipomas.

PURPOSE: This paper shows the long-term benefits of total/near-total resection of complex spinal cord lipomas and meticulous reconstruction of the neural placode, and specifically, its advantage over partial resection, and over non-surgical treatment for the subset of children with asymptomatic virgin lipomas. METHODS: The technique of total resection and placode reconstruction, together with technical nuances, are described in detail. We added 77 patients with complex lipomas to our original lipoma series published in 2009 and 2010, to a total of 315 patients who had had total or near-total resection and followed for a span of 20 years. Long-term outcome is measured by overall progression-free survival (PFS) with the Kaplan-Meier analysis, and by subgroup Cox proportional recurrence hazard analysis for the influence on outcome of 4 predictor variables of lipoma type, presence of symptoms, prior surgery, and post-operative cord-sac ratio. These results are compared to an age-matched, lesion-matched series of 116 patients who underwent partial lipoma resection over 11 years. The results for total resection is also compared to two large published series of asymptomatic lipomas followed without surgery over 9 to 10 years, to determine whether prophylactic total resection confers better long-term protection over conservative treatment for children with asymptomatic lipomas.. RESULTS: The PFS after total resection for all lipoma types and clinical subgroups is 88.1 % over 20 years versus 34.6 % for partial resection at 10.5 years (p < 0.0001). Culling only the asymptomatic patients with virgin (previously unoperated) lipomas, the PFS for prophylactic total resection for this subgroup rose to 98.8 % over 20 years, versus 67 % at 9 years for one group of non-surgical treatment and 60 % at 10 years for another group of conservative treatment. Our own as well as other published results of partial resection also compare poorly to non-surgical treatment for the subset of asymptomatic virgin lipomas. Multivariate subgroup analyses show that cord-sac ratio is the only independent variable that predicts outcome, with a 96.9 % PFS for ratio < 30 % (loosest sac), 86.2 % for ratio between 30 and 50 %, and 78.3 % for ratio > 50 % (tightest sac), and a threefold increase in recurrence hazard for high ratios (p = 0.0009). Pre-operative patient profiling using multiple correspondence analysis shows the ideal patient for total resection is a child less than 2 years old with a virgin asymptomatic lipoma, who, with a PFS of 99.2 %, is virtually cured by total resection. CONCLUSION: Total/near-total resection of complex lipomas and complete reconstruction of the neural placode achieves far better long-term protection against symptomatic recurrence than partial resection for all lesions; and for the subset of asymptomatic virgin lipomas, also better than non-surgical treatment. Partial resection in many cases produces worse outcome than conservative treatment for asymptomatic lesions.

Parallel angulated frontal bone slat cuts for treatment of metopic synostosis and other frontal skull deformities: the "cathedral dome procedure".

PURPOSE: This study aims to describe a new procedure for the treatment of metopic synostosis and other frontal skull deformities. METHOD: The procedure comprises a supraorbital bandeau widened with an interpositional graft and rounded laterally to eliminate the acute angle, and parallel angulated slat cuts in the frontal bones. Greenstick fracturing of the medial bases of these slats along a parasagittal hinge line causes fanning of the slats and expansion of the frontal flap both anteriorly and laterally making the forehead contour wider and more rounded. We performed this procedure on six infants (four with severe trigonocephaly from metopic synostosis, one with brachycephaly from bicoronal synostosis, and one with multiple suture synostosis and parietal flattening) for whom only the angulated slat cuts (without bandeau) were used. Each patient had preoperative three-dimensional computed tomography (3D-CT) and postoperative 3D-CT at 1 week, 3 months, and 12 months, to follow the result. RESULT: The cosmetic improvements are dramatic in eliminating the midfrontal keel, hypotelorism, frontal-lateral retrusion, and temporal hollowing seen in severe metopic synostosis. In coronal synostosis, the procedure corrects the brachycephaly and gives a balanced, well-rounded frontal contour. The end results of the fronto-orbital correction resemble the ribbed dome of a cathedral; hence, the moniker the "cathedral dome procedure". No patient needed a second procedure to fill in cranial defects or recorrect deficient areas. CONCLUSION: The parallel angulated frontal slat cuts technique (the "cathedral dome procedure") is a straightforward and easily mastered method that reliably produces excellent result for the correction of trigonocephaly and other frontal skull deformities.

Frameless versus frame-based stereotactic radiosurgery for intracranial arteriovenous malformations: A propensity-matched analysis.

Objective: The frameless linear accelerator (LINAC) based stereotactic radiosurgery (SRS) has been evolving with a reduction in patient discomfort. However, there was limited evidence comparing frame-based and frameless SRS for intracranial arteriovenous malformations (AVM). We aimed to compare the treatment outcomes between frame-based and frameless LINAC SRS. Materials and Methods: This retrospective cohort compared the outcomes of frame-based LINAC SRS (1998-2009) with frameless LINAC SRS (2010-2020). The primary outcome was the obliteration rate. The other outcomes included the neurological, radiological, and functional outcomes after SRS. A matched cohort was identified by propensity scores for further comparisons. Results: A total of 65 patients were included with a mean follow-up time of 13.2 years (158.5 months). There were 40 patients in the frame-based group and 25 patients in the frameless group. The overall obliteration rate was comparable (Frame-based 82.5% vs Frameless 80.0%, p = 0.310) and not significantly different over time (log-rank p = 0.536). The crude post-SRS hemorrhage rate was 1.5% and the incidence was 0.3 per 100 person-years. There were 67.7% of patients with AVM obliteration without new persistent neurological deficits at the last visit and 56.9% of patients with AVM obliteration without any deficits (transient or persistent) during the entire follow-up period. Four patients (8.0%) developed late onset persistent adverse radiation effects (more than 96 months after SRS) among 50 patients with more than 8-year surveillance. In the propensity-matched cohort of 42 patients, there was no significant difference in AVM obliteration (Frame-based vs Frameless, log-rank p = 0.984). Conclusion: Frameless and frame-based LINAC SRS have comparable efficacy in intracranial AVM obliteration. A longer follow-up duration may further characterize the rate of late adverse radiation effects in frameless SRS.

Reducing radiation exposure from computed tomography of the brain in children--report of a practical approach.

PURPOSE: To reduce radiation exposure to paediatric neurosurgical patients from computed tomography (CT), a CT scanning protocol - lower radiation dose and selective scan segment (LDSS) protocol was used for CT brain at the authors' hospital. To evaluate the amount of reduction in radiation exposure by using this LDSS protocol compared to their usual protocol, the authors prospectively documented their findings. METHODS: From May 2010 to June 2011, paediatric neurosurgical patients requiring CT brain, and when it was not a clinical emergency and there was baseline CT or MRI brain available, were evaluated for the LDSS protocol. The LDSS protocol used a lower tube current-time product and a shorter scan length to attain a lower total radiation dose. The CT scanning parameters of the patients' usual CT brain and LDSS CT were recorded and compared. Adverse events were also recorded. RESULTS: A total of 24 paediatric patients were included. Using the LDSS protocol, the effective doses were between 9% and 80% of the usual protocol and, in 20 patients, ≤50% of the usual protocol. The tube voltage was 120 kV. For patients below 10 years old, 100 mA s was adequate for the purposes of their CTs; in some patient categories, it was lower than 100 mA s. For patients aged 10 or above, 150 mA s was used. The scan length varied. CONCLUSIONS: Radiation exposure from CT brain in paediatric neurosurgical patients could be reduced by adopting a CT scanning protocol, which aimed dynamically at a lower tube current-time product and a shorter scan length than the usual settings at a hospital.

Multidimensional Outcomes of Selective Dorsal Rhizotomy for Children With Spastic Cerebral Palsy: Single-Level Laminectomy vs Multiple-Level Laminotomy Techniques.

BACKGROUND: Selective dorsal rhizotomy (SDR) reduces lower limb spasticity, improves gait patterns, and restores normal physical and social activity in children with spastic cerebral palsy. Single-level laminectomy (SLL) and multiple-level laminotomy (MLL) are 2 surgical approaches for SDR with limited clinical data comparing their postoperative outcomes. OBJECTIVE: To compare the differences in multidimensional outcomes after SDR between SLL and MLL for children with spastic cerebral palsy. METHODS: We retrospectively reviewed children who underwent SDR in our hospital from 1997 to 2016. The multidimensional outcomes in spasticity, joint range of motions, gait kinetics, gross motor activities, functional outcomes, and urological outcomes were assessed 1 year postoperatively. Hip dysplasia and scoliosis rate were compared as long-term outcomes. RESULTS: Sixty children underwent SDR, including 34 SLL patients and 26 MLL patients. Most improvements in multidimensional outcomes were comparable between SLL and MLL. Patients in the SLL group had larger improvements in ankle dorsiflexion in the midstance phase (SLL 7.59° ± 11.48° vs MLL 0.29° ± 11.30°, P = .027). The rate of scoliosis was similar between the 2 surgical approaches (SLL 12.1% vs MLL 15.4%, P = .722). CONCLUSION: SDR for children with spastic cerebral palsy could provide physical, functional, and urological improvements. SLL achieved a higher degree of improvement in ankle dorsiflexion in the midstance phase. The rate of scoliosis was not significantly increased by multiple-level laminotomy.

Focal Spinal Nondisjunction in Primary Neurulation : Limited Dorsal Myeloschisis and Congenital Spinal Dermal Sinus Tract.

Spinal dysraphic lesions due to focal nondisjunction in primary neurulation are commonly encountered in paediatric neurosurgery, but the "fog-of-war" on these conditions was only gradually dispersed in the past 10 years by the works of the groups led by the senior author and Prof. Kyu-Chang Wang. It is now clear that limited dorsal myeloschisis and congenital spinal dermal sinus tract are conditions at the two ends of a spectrum; and mixed lesions of them with various configurations exist. This review article summarizes the current understanding of these conditions' embryogenetic mechanisms, pathological anatomy and clinical manifestations, and their management strategy and surgical techniques.

Ruptured brain arteriovenous malformations in children: correlation of clinical outcome with admission parameters.

AIMS: To gain a better understanding of how clinical outcome in children with ruptured brain arteriovenous malformations (bAVMs) correlates with clinical and imaging parameters on admission. METHODS: The authors retrospectively reviewed patients with bAVMs, aged 18 or below, managed at their hospital between January 1992 and December 2008. Clinical outcome was assessed using the modified Rankin Scale (mRS). Patients with ruptured bAVMs were analyzed; their clinical parameters and computerized tomography findings on admission were recorded. Clinical outcome was then evaluated against admission scores using the Glasgow Coma Scale (GCS), the World Federation of Neurosurgical Societies Grading System of Subarachnoid Hemorrhage (WFNS-SAH), the Spetzler-Martin grade, the intracerebral hemorrhage score and 2 other independent parameters, namely pupillary response and significant focal neurological injuries. Spearman's correlation coefficient, linear regression analysis and multivariate logistic regression analysis were used for data analysis. RESULTS: 40 pediatric patients with bAVMs were found and 32 of them presented with hemorrhage (80%). In the 32 children with ruptured bAVMS, follow-up ranged between 7 and 204 months (median 100.5 months). The complete excision/obliteration rate as confirmed by digital subtraction angiography was 73.3%. The mRS scores at the last follow-up were: grades 0-II in 87.5%; grades IV-V in 6.25%, and grade VI in 6.25%. The Spetzler-Martin grade, the intracerebral hemorrhage score, the WFNS-SAH grade, the GCS scores, and a combined scoring scale consisting of the GCS, pupillary response and significant focal neurological injuries correlated significantly with clinical outcome 6 months after hemorrhage. However, on the scatter diagrams, it appeared that only the combined scoring scale might be valid for clinical practice. Multivariate logistic regression analysis showed that the combined scoring scale was a statistically significant independent predictor of clinical outcome 6 months after hemorrhage. CONCLUSION: In this series of pediatric patients with ruptured bAVMs, although various grading scales correlated significantly with clinical outcome 6 months after hemorrhage, only the combined scoring scale might have the potential to be applied to predict clinical outcome in these children.

Newly-Diagnosed, Histologically-Confirmed Central Nervous System Tumours in a Regional Hospital in Hong Kong : An Epidemiological Study of a 21-Year Period.

OBJECTIVE: To investigate the epidemiology of newly-diagnosed, histologically-confirmed (NDHC) central nervous system (CNS) tumours and its changes over a 21-year period in a regional hospital in Hong Kong. METHODS: This is a single-institute retrospective descriptive study of patients undergoing surgery for CNS tumours in a regional hospital of Hong Kong in the period from January 1996 to December 2016. The histological definition of CNS tumours was according to the World Health Organization classification, while the site definition for case ascertainment of CNS tumours was as set out by the Central Brain Tumour Registry of the United States. Patients of any age, who had NDHC CNS tumours, either primary or secondary, were included. The following parameters of the patients were retrieved : age at diagnosis, gender, tumour location, and histological diagnosis. Population data were obtained from sources provided by the Government of Hong Kong. The incident rate, estimated by the annual number of cases per 100000 population, for each histology grouping was calculated. Statistical analyses, both including and excluding brain metastases, were performed. Statistical analysis was performed with Microsoft Excel, 2016 (Microsoft, Redmond, WA, USA). RESULTS: Among the 2134 cases of NDHC CNS tumours, there were 1936 cases of intracranial tumours and 198 cases of spinal tumours. The annual number of cases per 100000 population of combined primary intracranial and spinal CNS tumours was 3.6 in 1996, and 11.1 in 2016. Comparing the 5-year average annual number of cases per 100000 population of primary CNS tumours from the period 1996-2000 to 2011-2015, there was an 88% increase, which represent an increase in the absolute number of cases by 4.52 cases/100000 population. This increase was mainly contributed by benign histologies. In the aforementioned periods, meningiomas increased by 1.45 cases/100000 population; schwannomas by 1.05 cases/100000 population, and pituitary adenomas by 0.91 cases/100000 population. While gliomas had a fluctuating 5-year average annual number of cases per 100000 population, it only had an absolute increase of 0.51 cases/100000 population between the 2 periods, which was mainly accounted for by the change in glioblastomas. CONCLUSION: This retrospective study of CNS tumour epidemiology revealed increasing trends in the incidences of several common CNS tumour histologies in Hong Kong, which agrees with the findings in large-scale studies in Korea and the United States. It is important for different geographic locations to establish their own CNS tumour registry with well-defined and structured data collection and analysis system to meet the international standards.

Redistribution of hematoma to spinal subdural space as a mechanism for the rapid spontaneous resolution of posttraumatic intracranial acute subdural hematoma: case report.

BACKGROUND: Rapid spontaneous resolution of posttraumatic intracranial ASDH has been reported in the literature since 1986. We report a case to demonstrate that redistribution of hematoma to the spinal subdural space is a mechanism for the rapid spontaneous resolution of posttraumatic intracranial ASDH. CASE DESCRIPTION: A 73-year-old woman with a slipped-and-fell injury had a worst GCS score of 8/15. Computerized tomography of the brain demonstrated a large intracranial ASDH with mass effect. Conservative management was decided because of her poor premorbid general condition. Rapid clinical improvement was observed within 5 hours after the CT. Progress CT of the brain at 45 hours postinjury showed that the size of the intracranial ASDH was markedly diminished. The CT findings apparently demonstrated a caudal distribution of the intracranial ASDH over the tentorium and then into the posterior fossa. To investigate this further, an MRI of the spine was performed, which showed that there was spinal SDH in the cervical and thoracic spine. CONCLUSION: This is the first report demonstrating that redistribution of posttraumatic intracranial ASDH to the spinal subdural space is one of the mechanisms behind the rapid spontaneous resolution of posttraumatic intracranial ASDH in the acute phase.

Pathophysiological mechanism of ipsilateral cerebral and brainstem hemiatrophy in basal ganglia germ cell tumors: case report.

INTRODUCTION: The basal ganglia is an uncommon location for germ cell tumors. It has been reported that basal ganglia germinomas and mixed germ cell tumors are associated with ipsilateral cerebral and brainstem hemiatrophy on presentation. Several pathophysiological mechanisms including autoimmune process and direct tumor infiltration of the thalamus or the internal capsule have been postulated to explain this association. CASE REPORTS: The authors report two boys, aged 7 and 10, with basal ganglia germ cell tumors. Both of them presented with gradual onset of hemiparesis and had features of ipsilateral cerebral and brainstem hemiatrophy on imaging studies. They underwent chemotherapy followed by reduced dose radiotherapy with good response. DISCUSSION: Pathophysiological mechanisms of the associated ipsilateral cerebral and brainstem hemiatrophy are discussed. The authors postulate that the gradual obliteration of the perforating arteries to the diencephalon especially the lenticulostriate arteries of the prebifurcation middle cerebral artery may be the major mechanism of the associated hemiatrophy.

Determining a cut-off residual tumor volume threshold for patients with newly diagnosed glioblastoma treated with temozolomide chemoradiotherapy: A multicenter cohort study.

Standard-of-care treatment of glioblastomas involves maximal safe resection and adjuvant temozolomide chemo-radiotherapy. Although extent of resection (EOR) is a well-known surgical predictor for overall survival most lesions cannot be completely resected. We hypothesize that in the event of incomplete resection, residual tumor volume (RTV) may be a more significant predictor than EOR. This was a multicenter retrospective review of 147 adult glioblastoma patients (mean age 53 years) that underwent standard treatment. Semiautomatic magnetic resonance imaging segmentation was performed for pre- and postoperative scans for volumetric analysis. Cox proportional hazards regression and Kaplan-Meier survival analyses were performed for prognostic factors including: age, Karnofsky performance score (KPS), O(6)-methylguanine methyltransferase (MGMT) promoter methylation status, EOR and RTV. EOR and RTV cut-off values for improved OS were determined and internally validated by receiver operator characteristic (ROC) analysis for 12-month overall survival. Half of the tumors had MGMT promoter methylation (77, 52%). The median tumor volume, EOR and RTV were 43.20 cc, 93.5%, and 3.80 cc respectively. Gross total resection was achieved in 52 patients (35%). Cox proportional hazards regression, ROC and maximum Youden index analyses for RTV and EOR showed that a cut-off value of <3.50 cc (HR 0.69; 95% CI 0.48-0.98) and ≥84% (HR 0.64; 95% CI 0.43-0.96) respectively conferred an overall survival advantage. Independent overall survival predictors were MGMT promoter methylation (adjusted HR 0.35; 95% CI 0.23-0.55) and a RTV of <3.50 cc (adjusted HR 0.53; 95% CI 0.29-0.95), but not EOR for incompletely resected glioblastomas.

A Comparative Analysis of the Usefulness of Survival Prediction Models for Patients with Glioblastoma in the Temozolomide Era: The Importance of Methylguanine Methyltransferase Promoter Methylation, Extent of Resection, and Subventricular Zone Location.

OBJECTIVE: Several survival prediction models for patients with glioblastoma have been proposed, but none is widely used. This study aims to identify the predictors of overall survival (OS) and to conduct an independent comparative analysis of 5 prediction models. METHODS: Multi-institutional data from 159 patients with newly diagnosed glioblastoma who received adjuvant temozolomide concomitant chemoradiotherapy (CCRT) were collected. OS was assessed by Cox proportional hazards regression and adjusted for known prognostic factors. An independent CCRT patient cohort was used to externally validate the 1) RTOG (Radiation Therapy Oncology Group) recursive partitioning analysis (RPA) model, 2) Yang RPA model, and 3) Wee RPA model, Chaichana model, and the RTOG nomogram model. The predictive accuracy for each model at 12-month survival was determined by concordance indices. Calibration plots were performed to ascertain model prediction precision. RESULTS: The median OS for patients who received CCRT was 19.0 months compared with 12.7 months for those who did not (P < 0.001). Independent predictors were: 1) subventricular zone II tumors (hazard ratio [HR], 1.6; 95% confidence interval [CI], 1.0-2.5); 2) methylguanine methyltransferase promoter methylation (HR, 0.36; 95% CI, 0.2-0.6); and 3) extent of resection of >85% (HR, 0.59; 95% CI, 0.4-0.9). For 12-month OS prediction, the RTOG nomogram model was superior to the RPA models with a c-index of 0.70. Calibration plots for 12-month survival showed that none of the models was precise, but the RTOG nomogram performed relatively better. CONCLUSIONS: The RTOG nomogram best predicted 12-month OS. Methylguanine methyltransferase promoter methylation status, subventricular zone tumor location, and volumetric extent of resection should be considered when constructing prediction models.

Traumatic rupture of the neurocentral synchondrosis of the axis in a child.

The authors report the first case of unilateral traumatic rupture of the C-2 neurocentral synchondrosis. A 26-month-old child was in a vehicular collision that caused his head to be rotated sharply to the left with the neck flexed. He had severe neck pain but was neurologically normal. Computerized tomography scanning showed rupture of the left C-2 neurocentral synchondrosis, a right C-2 pars interarticularis fracture, and anterior angulation of C-2 on C-3. The neck injury was unrecognized until postinjury Day 9 when an MRI study showed a tear of the posterior longitudinal ligament at C2-3 and separation of the C-2 body from the inferior anular epiphysis. A second CT showed widening of the synchondrosis fracture, increased angulation of C-2 on C-3, and distraction of the right C-2 pars fracture. The mechanism of the neurocentral synchondrosis fracture is thought to be hyperflexion-axial loading combined with leftward rotation, which provided the lateral force that overcame the cartilaginous synchondrosis and extruded the lateral mass. The patient underwent open reduction and posterior fusion of C1-3, and was maintained in a halo jacket for 4 months, when CT scans demonstrated solid C1-C3 fusion and ossification of the injured synchondrosis. Unilateral traumatic rupture of the C-2 neurocentral synchondrosis is one component of several injuries involving C-2 sustained before synchondrosis closure. The resulting C2-3 relationship is highly unstable. Reduction and C1-C3 fusion are necessary in patients with significant displacement of the adjacent bony units.

Isolated unilateral rupture of the alar ligament.

Only 6 cases of isolated unilateral rupture of the alar ligament have been previously reported. The authors report a new case and review the literature, morbid anatomy, and pathogenesis of this rare injury. The patient in their case, a 9-year-old girl, fell head first from a height of 5 feet off the ground. She presented with neck pain, a leftward head tilt, and severe limitation of right rotation, extension, and right lateral flexion of the neck. Plain radiographs and CT revealed no fracture but a shift of the dens toward the right lateral mass of C-1. Magnetic resonance imaging of the cervical spine showed signal hyperintensity within the left dens-atlas space on both T1- and T2-weighted sequences and interruption of the expected dark signal representing the left alar ligament, suggestive of its rupture. After 12 weeks of immobilization in a Guilford brace, MRI showed lessened dens deviation, and the patient attained full and painless neck motion. Including the patient in this case, the 7 patients with this injury were between 5 and 21 years old, sustained the injury in traffic accidents or falls, presented with marked neck pain, and were treated with external immobilization. All patients had good clinical outcome. The mechanism of injury is hyperflexion with rotation. Isolated unilateral alar ligament rupture is a diagnosis made by excluding associated fracture, dislocation, or disruption of other major ligamentous structures in the craniovertebral junction. CT and MRI are essential in establishing the diagnosis. External immobilization is adequate treatment.