Hydration-induced rapid growth and regression after indirect revascularization of an anterior choroidal artery aneurysm associated with Moyamoya disease: A case report

The prevalence of aneurysm formation in adults with Moyamoya disease (MMD) is higher than that in the general population. The treatment strategy is often individualized based on the patient’s disease characteristics. A 22-year-old man was diagnosed with MMD after presenting a small thalamic intracerebral and subarachnoid hemorrhage in the quadrigeminal cistern. Cerebral angiography revealed a small aneurysm (2.42 mm) in the left anterior choroidal artery. Since the hemodynamics in the left hemisphere was compromised, an indirect bypass surgery was performed. The patient’s condition deteriorated postoperatively because of poor perfusion of the internal carotid artery, and massive hydration was required. During neurocritical care, the aneurysm increased in size (5.33 mm). An observation strategy was adopted because of the distal aneurysmal location and the high risk involved. Subsequently, the patient recovered, and newly developed collateral flow appeared from the external carotid artery. Additionally, a dramatic size reduction of the aneurysm (1.51 mm) was noticed. Our case suggests that MMD-related dissecting aneurysms on a distal cerebral artery, which present a high risk of embolization, could be managed by indirectly reducing the hemodynamic burden. Massive hydration in such cases should be avoided or balanced to avoid the risk of rapid growth and aneurysm rupture.

Factors Affecting Cage Obliquity and the Relationship between Cage Obliquity and Radiological Outcomes in Oblique Lateral Interbody Fusion at the L4-L5 Level

Objective@#: This retrospective study investigated the factors that affect cage obliquity angle despite orthogonal maneuvers performed during oblique lateral interbody fusion (OLIF) and assessed the relationship between cage obliquity angle and radiological outcomes post-surgery. @*Methods@#: Twenty-nine males who underwent L4-L5 OLIF for lumbar degenerative disease between 2019 and 2021 with a followup duration greater than 12 months were analyzed. Radiological parameters were measured including psoas muscle volume, total psoas area index (total psoas muscle area [cm2]/height squared [m2]), distance from the iliac artery to the origin of the psoas muscle (DIAPM), angle between the origin of the psoas muscle and the center of the vertebral disc (APCVD), iliac crest height, disc height, lumbar flexibility (lumbar flexion angle minus extension angle), cage location ratio, cage-induced segmental lumbar lordosis (LL) (postoperative index level segmental LL minus used cage angle), foraminal height changes, fusion grade. @*Results@#: DIAPM, APCVD, iliac crest height, postoperative index level segmental LL, and cage-induced segmental LL were significantly correlated with OLIF cage obliquity angle. However, other radiological parameters did not correlate with cage obliquity. Based on multiple regression analysis, the predictive equation for the OLIF cage obliquity angle was 13.062–0.318×DIAPM+0.325×A PCVD+0.174×iliac crest height. The greater the cage obliquity, the smaller the segmental LL compared to the cage angle used. @*Conclusion@#: At the L4-L5 level, OLIF cage obliquity was affected by DIAPM, APCVD, and iliac crest height, and as the cage obliquity angle increases, LL agnle achievable by the used cage could not be obtained.

Progression of Ossification of Posterior Longitudinal Ligament After Anterior Cervical Discectomy and Fusion in Military Patients Exposed to Minor Trauma / 대한신경손상학회지

Objective@#Ossification of the posterior longitudinal ligament (OPLL) can progress even after cervical spine surgery and may cause neurological injury as a result of minor trauma. The purpose of this study was to investigate the preventive factors associated with OPLL progression after anterior cervical discectomy and fusion (ACDF), a procedure commonly performed in clinical practice. @*Methods@#We retrospectively investigated 295 male soldiers who underwent ACDF surgery between 2012 and 2017. Patients who were followed up for >12 months using dynamic radiography and computed tomography (CT) were included in the study. Radiological parameters investigated included OPLL progression, C2-C7 angles on dynamic radiography, segmental angles, C2-C7 cervical sagittal vertical axis (C2-C7 SVA), and the T1 slope. These parameters were measured preoperatively and 1 year postoperatively. @*Results@#A total of 49 patients were enrolled, and 10 patients were confirmed to have OPLL progression. Comparison between the OPLL progression and non-progression groups showed no statistically significant differences in pre- and postoperative cervical range of motion. However, statistically significant differences were observed in the postoperative neutral C2-C7 angle (progression −3.9°±6.4° vs. non-progression −13.4°±7.9°, p=0.001) and the SVA change (progression 5.8±7.9 mm vs. non-progression −3.7±6.3 mm, p=0.00). The cutoff values were −8.01° for the postoperative neutral C2-C7 angle and 1.4 mm for SVA changes. @*Conclusion@#Increased SVA (>1.4 mm) and a small postoperative neutral C2-C7 angle (>−8.01°) 1 year after ACDF were associated with OPLL progression. It is important to be mindful of these factors during follow-up after ACDF, because additional surgical treatment may be necessary for OPLL progression due to neurological injury caused by minor trauma.

Leptomeningeal Spread at the Diagnosis of Glioblastoma Multiforme: A Case Report and Literature Review

Approximately two-thirds of glioblastoma (GBM) patients progress to leptomeningeal spread (LMS) within two years. While 90% of LMS cases are diagnosed during the progression and/or recurrence of GBM (defined as secondary LMS), LMS presentation at the time of GBM diagnosis (defined as primary LMS) is very rare. 18 F-fluorodeoxy glucose positron emission tomography computed tomography ( 18F-FDG PET/CT) study helps to diagnose the multifocal spread of the malignant primary brain tumor. Our patient was a 31-year-old man with a tumorous lesion located in the right temporal lobe, a wide area of the leptomeninges, and spinal cord (thoracic 5/6, and lumbar 1 level) involvement as a concurrent manifestation. After the removal of the right temporal tumor, the clinical status progressed rapidly, showing signs of increased intracranial pressure and hydrocephalus caused by LMS. He underwent a ventriculoperitoneal shunt a week after craniotomy. During management, progression of cord compression, paraplegia, bone marrow suppression related to radiochemotherapy, intercurrent infections, and persistent ascites due to peritoneal metastasis of the LMS through the shunt system was observed. The patient finally succumbed to the disease nine months after the diagnosis of simultaneous GBM and LMS. The overall survival of primary LMS with GBM in our case was nine months, which is shorter than that of secondary LMS with GBM. The survival period after the diagnosis of LMS did not seem to be significantly different between primary and secondary LMS. To determine the prognostic effect and difference between primary and secondary LMS, further cooperative studies with large-volume data analysis are warranted.

Recurrent Cervical Spontaneous Spinal Epidural Hematoma with Conservative Management: A Case Report / 대한신경손상학회지

Cervical spontaneous spinal epidural hematoma (CSSEH) is a rare condition that can be potentially fatal if not properly diagnosed and managed. While prompt surgical decompression and evacuation of the hematoma are generally considered as the first line of treatment, mild cases that were managed through observation and conservative treatment have been reported. Our patient was a 24-year-old man who experienced two CSSEH events 8 months apart, both of which were managed conservatively. This was a rare case of recurrent CSSEH in which recovery was achieved without surgical intervention. We believe conservative treatment with close observation may be effective in CSSEH patients presenting with mild neurologic symptoms who have a tendency towards spontaneous neurologic improvement.

Posterior Approach in C2–3 Disc Herniation: C1 Laminectomy, C2–3Laminoplasty and Posterior Fixation in C2–3 Disc Herniation / 대한신경손상학회지

C2–3 disc herniation is rare and a definitive treatment of choice has not been established.The purpose of this case report is to suggest posterior approach as one of the best options.A 49-year-old man visited our clinic with a 7-year history of neck pain and occipital headache and a 2-month history of right arm pain. C2–3 intervertebral disc herniation of the central type was diagnosed on magnetic resonance imaging (MRI), and surgery was performed, including C1 laminectomy, C2–3 laminoplasty, and C2–3 posterior fixation. The posterior approach was used because the patient's neck was difficult to operate anteriorly. After 3 months postoperatively, MRI showed widened cerebrospinal fluid space at the C2–3 level.The visual analogue scale score for pain improved in the occipital area and right arm.However, the untouched protruded central disc, subjective weakness in right hand grasping, and numbness persisted. In conclusion, this case highlights posterior decompression and fixation as a good treatment of choice for decompression at the C2–3 level disc herniation, from where it is difficult to remove compressive lesions directly via the anterior corridor.

Long-Term Outcomes and Sequelae Analysis of Intracranial Germinoma: Need to Reduce the Extended-Field Radiotherapy Volume and Dose to Minimize Late Sequelae / Journal of the Korean Cancer Association, 대한암학회지

Purpose@#We aimed to refine the radiotherapy (RT) volume and dose for intracranial germinoma considering recurrences and long-term toxicities. @*Materials and Methods@#Total 189 patients with intracranial germinoma were treated with RT alone (n=50) and RT with upfront chemotherapy (CRT) (n=139). All cases were confirmed histologically. RT fields comprised the extended-field and involved-field only for primary site. The extended-field, including craniospinal, whole brain (WB), and whole ventricle (WV) for cranial field, is followed by involved-field boost. The median follow-up duration was 115 months. @*Results@#The relapses developed in 13 patients (6.9%). For the extended-field, cranial RT dose down to 18 Gy exhibited no cranial recurrence in 34 patients. In CRT, 74 patients (56.5%) showed complete response to chemotherapy and no involved-field recurrence with low-dose RT of 30 Gy. WV RT with chemotherapy for the basal ganglia or thalamus germinoma showed no recurrence. Secondary malignancy developed in 10 patients (5.3%) with a latency of 20 years (range, 4 to 26 years) and caused mortalities in six. WB or craniospinal field rather than WV or involved-field significantly increased the rate of hormone deficiencies, and secondary malignancy. RT dose for extended-field correlated significantly with the rate of hormone deficiencies, secondary malignancy, and neurocognitive dysfunction. @*Conclusion@#De-intensifying extended-field rather than involved-field or total scheme of RT will be critical to decrease the late toxicities. Upfront chemotherapy could be beneficial for the patients with complete response to minimize the RT dose down to 30 Gy. Prospective trials focused on de-intensification of the extended-field RT are warranted.

Clinicopathological Features of Primary Solitary Spinal Cord Tumors in Pediatric Patients : A 32-Year Single Institution Experience

Objective@#: Few studies exist on primary spinal cord tumors (PSCTs) in pediatric patients. The purpose of this study was to perform descriptive analysis and detailed survival analysis for PSCTs. @*Methods@#: Between 1985 and 2017, 126 pediatric patients (male : female, 56 : 70) with PSCTs underwent surgery in a single institution. We retrospectively analyzed data regarding demographics, tumor characteristics, outcomes, and survival statistics. Subgroup analysis was performed for the intramedullary (IM) tumors and extradural (ED) tumors separately. @*Results@#: The mean age of the participants was 6.4±5.04 years, and the mean follow-up time was 69.5±46.30 months. The most common compartment was the ED compartment (n=57, 45.2%), followed by the IM (n=43, 34.1%) and intradural extramedullary (IDEM; n=16, 12.7%) compartments. Approximately half of PSCTs were malignant (n=69, 54.8%). The most common pathologies were schwannomas (n=14) and neuroblastomas (n=14). Twenty-two patients (17.5%) died from the disease, with a mean disease duration of 15.8±15.85 months. Thirty-six patients (28.6%) suffered from progression, with a mean period of 22.6±30.81 months. The 10-year overall survival (OS) rates and progression-free survival (PFS) rates were 81% and 66%, respectively. Regarding IM tumors, the 10-year OS rates and PFS rates were 79% and 57%, respectively. In ED tumors, the 10-year OS rates and PFS rates were 80% and 81%, respectively. Pathology and the extent of resection showed beneficial effects on OS for total PSCTs, IM tumors, and ED tumors. PFS was affected by both the extent of removal and pathology in total PSCTs and ED tumors; however, pathology was a main determinant of PFS rather than the extent of removal in IM tumors. The degree of improvement in the modified McCormick scale showed a trend towards improvement in patients with IM tumors who achieved gross total removal (p=0.447). @*Conclusion@#: Approximately half of PSCTs were malignant, and ED tumors were most common. The most common pathologies were schwannomas and neuroblastomas. Both the pathology and extent of resection had a decisive effect on OS. For IM tumors, pathology was a main determinant of PFS rather than the extent of removal. Radical excision of IM tumors could be a viable option for better survival without an increased risk of worse functional outcomes.

Clinicopathological Features of Primary Solitary Spinal Cord Tumors in Pediatric Patients : A 32-Year Single Institution Experience

Objective@#: Few studies exist on primary spinal cord tumors (PSCTs) in pediatric patients. The purpose of this study was to perform descriptive analysis and detailed survival analysis for PSCTs. @*Methods@#: Between 1985 and 2017, 126 pediatric patients (male : female, 56 : 70) with PSCTs underwent surgery in a single institution. We retrospectively analyzed data regarding demographics, tumor characteristics, outcomes, and survival statistics. Subgroup analysis was performed for the intramedullary (IM) tumors and extradural (ED) tumors separately. @*Results@#: The mean age of the participants was 6.4±5.04 years, and the mean follow-up time was 69.5±46.30 months. The most common compartment was the ED compartment (n=57, 45.2%), followed by the IM (n=43, 34.1%) and intradural extramedullary (IDEM; n=16, 12.7%) compartments. Approximately half of PSCTs were malignant (n=69, 54.8%). The most common pathologies were schwannomas (n=14) and neuroblastomas (n=14). Twenty-two patients (17.5%) died from the disease, with a mean disease duration of 15.8±15.85 months. Thirty-six patients (28.6%) suffered from progression, with a mean period of 22.6±30.81 months. The 10-year overall survival (OS) rates and progression-free survival (PFS) rates were 81% and 66%, respectively. Regarding IM tumors, the 10-year OS rates and PFS rates were 79% and 57%, respectively. In ED tumors, the 10-year OS rates and PFS rates were 80% and 81%, respectively. Pathology and the extent of resection showed beneficial effects on OS for total PSCTs, IM tumors, and ED tumors. PFS was affected by both the extent of removal and pathology in total PSCTs and ED tumors; however, pathology was a main determinant of PFS rather than the extent of removal in IM tumors. The degree of improvement in the modified McCormick scale showed a trend towards improvement in patients with IM tumors who achieved gross total removal (p=0.447). @*Conclusion@#: Approximately half of PSCTs were malignant, and ED tumors were most common. The most common pathologies were schwannomas and neuroblastomas. Both the pathology and extent of resection had a decisive effect on OS. For IM tumors, pathology was a main determinant of PFS rather than the extent of removal. Radical excision of IM tumors could be a viable option for better survival without an increased risk of worse functional outcomes.

Incidentally Found Intramuscular Hemangioma, Mimicking Traumatic Hematoma after Military Training: A Case Report / 대한신경손상학회지

This case report showed a young soldier complained of low back pain during military training. Intramuscular hematoma accompanied by the lumbar compression fracture was observed in computed tomography. However, the possibility of intramuscular tumors could not be ruled out through additional examinations, and thus surgically removed, and was diagnosed as cavernous hemangioma. This report is a rare and instructive case in which a hemangioma mimicked bleeding with the lumbar fracture.

Epilepsy Surgery in 2019: A Time to Change

Epilepsy has been known to humankind since antiquity. The surgical treatment of epilepsy began in the early days of neurosurgery and has developed greatly. Many surgical procedures have stood the test of time. However, clinicians treating epilepsy patients are now witnessing a huge tide of change. In 2017, the classification system for seizure and epilepsy types was revised nearly 36 years after the previous scheme was released. The actual difference between these systems may not be large, but there have been many conceptual changes, and clinicians must bid farewell to old terminology. Paradigms in drug discovery are changing, and novel antiseizure drugs have been introduced for clinical use. In particular, drugs that target genetic changes harbor greater therapeutic potential than previous screening-based compounds. The concept of focal epilepsy has been challenged, and now epilepsy is regarded as a network disorder. With this novel concept, stereotactic electroencephalography (SEEG) is becoming increasingly popular for the evaluation of dysfunctioning neuronal networks. Minimally invasive ablative therapies using SEEG electrodes and neuromodulatory therapies such as deep brain stimulation and vagus nerve stimulation are widely applied to remedy dysfunctional epilepsy networks. The use of responsive neurostimulation is currently off-label in children with intractable epilepsy.

Epilepsy Surgery in 2019: A Time to Change

Epilepsy has been known to humankind since antiquity. The surgical treatment of epilepsy began in the early days of neurosurgery and has developed greatly. Many surgical procedures have stood the test of time. However, clinicians treating epilepsy patients are now witnessing a huge tide of change. In 2017, the classification system for seizure and epilepsy types was revised nearly 36 years after the previous scheme was released. The actual difference between these systems may not be large, but there have been many conceptual changes, and clinicians must bid farewell to old terminology. Paradigms in drug discovery are changing, and novel antiseizure drugs have been introduced for clinical use. In particular, drugs that target genetic changes harbor greater therapeutic potential than previous screening-based compounds. The concept of focal epilepsy has been challenged, and now epilepsy is regarded as a network disorder. With this novel concept, stereotactic electroencephalography (SEEG) is becoming increasingly popular for the evaluation of dysfunctioning neuronal networks. Minimally invasive ablative therapies using SEEG electrodes and neuromodulatory therapies such as deep brain stimulation and vagus nerve stimulation are widely applied to remedy dysfunctional epilepsy networks. The use of responsive neurostimulation is currently off-label in children with intractable epilepsy.

Surgical Outcomes of Thalamic Tumors in Children: The Importance of Diffusion Tensor Imaging, Neuro-Navigation and Intraoperative Neurophysiological Monitoring

BACKGROUND: Recently, modern technology such as diffusion tensor imaging (DTI), neuro-navigation and intraoperative neurophysiological monitoring (IOM) have been actively adopted for the treatment of thalamic tumors. We evaluated surgical outcomes and efficacy of the aforementioned technologies for the treatment of pediatric thalamic tumors. METHODS: We retrospectively reviewed clinical data from 37 children with thalamic tumors between 2004 and 2017. There were 44 operations (27 tumor resections, 17 biopsies). DTI was employed in 17 cases, neuro-navigation in 23 cases and IOM in 14 cases. All diagnoses were revised according to the 2016 World Health Organization Classification of Tumors of the Central Nervous System. Progression-free survival (PFS) and overall survival (OS) rates were calculated, and relevant prognostic factors were analyzed. The median follow-up duration was 19 months. RESULTS: Fifteen cases were gross total resections (GTR), 6 subtotal resections (STR), and 6 partial resections (PR). Neurological status did not worsen after 22 tumor resections. There were statistically significant differences in terms of the extent of resection between the groups with DTI, neuro-navigation and IOM (n=12, GTR or STR=12) and the group without at least one of the three techniques (n=15, GTR or STR=9, p=0.020). The mean PFS was 87.2±38.0 months, and the mean OS 90.7±36.1 months. The 5-year PFS was 37%, and the 5-year OS 47%. The histological grade (p≤0.001) and adjuvant therapy (done vs. not done, p=0.016) were significantly related to longer PFS. The histological grade (p=0.002) and the extent of removal (GTR/STR vs. PR/biopsy, p=0.047) were significantly related to longer OS. CONCLUSION: Maximal surgical resection was achieved with acceptable morbidity in children with thalamic tumors by employing DTI, neuro-navigation and IOM. Maximal tumor resection was a relevant clinical factor affecting OS; therefore, it should be considered the initial therapeutic option for pediatric thalamic tumors.

Hypertensive Encephalopathy with Reversible Brainstem Edema

Presented here is a 36-year-old male with arterial hypertension who developed brainstem edema and intracranial hemorrhage. Magnetic resonance scan revealed diffuse brainstem hyperintensity in T2-weighted and fluid-attenuated inversion-recovery images, with an increase in apparent diffusion coefficient values. After a reduction in blood pressure, rapid resolution of the brainstem edema was observed on follow-up. The patient's condition was thus interpreted as hypertensive brainstem encephalopathy. While many consider this a vasogenic phenomenon, induced by sudden, severe hypertension, the precise mechanism remains unclear. Prompt recognition and aggressive antihypertensive treatment in such patients are essential to prevent permanent or life-threatening neurologic injury.

Indirect revascularization surgery for moyamoya disease in children and its special considerations / 소아과

Moyamoya disease (MMD) is the most common pediatric cerebrovascular disease in Far Eastern countries. In children, MMD frequently manifests as ischemic symptomatology. Cerebral perfusion gradually decreases as the disease progresses, which often leads to cerebral infarction. The benefits of revascularization surgery, whether direct or indirect, have been well established in MMD patients with ischemic symptoms. In adults, the increase in cerebral blood flow achieved with indirect revascularization is often unsatisfactory, and direct revascularization is usually feasible. In children, however, direct revascularization is frequently technically not feasible, whereas the response to indirect revascularization is excellent, although 1 or 2 weeks are required for stabilization of symptoms. The authors describe surgical procedures and perioperative care in indirect revascularization for MMD. In addition, special considerations with regard to very young patients, patients with recent cerebral infarction, and patients with hyperthyroidism are discussed.

Desmoplastic Fibroma of the Cranium in a Young Man

Desmoplastic fibroma, which develops predominantly in long bones and the mandible, is a rare and benign but locally aggressive tumor. Desmoplastic fibroma of the cranium is extremely rare. We report a case of desmoplastic fibroma of the frontal bone in a young man. Because of its locally aggressive behavior, complete surgical excision with a safety margin is essential.

Reduced-dose craniospinal radiotherapy followed by high-dose chemotherapy and autologous stem cell rescue for children with newly diagnosed high-risk medulloblastoma or supratentorial primitive neuroectodermal tumor

BACKGROUND: In this study, we investigated the effects of reduced-dose craniospinal radiotherapy (CSRT) followed by tandem high-dose chemotherapy (HDCT) with autologous stem cell rescue (ASCR) in children with a newly diagnosed high-risk medulloblastoma (MB) or supratentorial primitive neuroectodermal tumor (sPNET). METHODS: Between March 2005 and April 2007, patients older than 3 years with a newly diagnosed high-risk MB or sPNET were enrolled. The patients received two cycles of pre-RT chemotherapy consisting of cisplatin, etoposide, vincristine, and cyclophosphamide (cycle A), and carboplatin, etoposide, vincristine, and ifosphamide (cycle B), followed by CSRT with 23.4 Gy and local RT with 30.6 Gy. After four cycles of post-RT chemotherapy (cycles A, B, A, and B), tandem double HDCT with ASCR was performed. RESULTS: A total of 13 patients (MB=11, sPNET=2) were enrolled. Of these, one patient progressed, one patient died of septic shock after the second cycle of B, and one patient relapsed after the third cycle of B. The 3-year event-free survival (EFS) rate of the patients intended for HDCT was 76.9%, whereas the 3-year EFS rate of the patients who received HDCT was 100%. No treatment-related mortality occurred during HDCT. CONCLUSION: Although the follow-up period was short and the patient cohort was small in size, the results of this study are encouraging. The limited toxicity and favorable EFS rate observed in children treated with reduced-dose CSRT followed by HDCT and ASCR warrant further exploration in a larger study population.

Recent Trend and Transition of Last 10 Years of Ski and Snowboard Injuries / 대한스포츠의학회지

To analysis the trend of ski and snow board injury, and use this study result in designing injuries prevention strategy. We studied 25,292 patients who had the ski and snow board injuries in a ski resort in Pyeongchang Gun, Gangwon Do, Korea during 1999-2001 season, 2005-2007 season, and 2008-2010 season. They were studied with a questionnaires, physical examination and radiograph. And the analysis was based on age, sex, injury type and site and slope level of difficulty. Snow board related injury have increased, injury to teens and thirties increased, injury to female snowboarder increased (p<0.001), contusion type of ski and snow board injury increased (p<0.001) and upper extremity injury increased but beginner grade slope injury (p<0.001) decreased since last 10 years. The overall type and site of ski and snowboard injuries have changed due to increased snowboard injury, gender and age of them have shown the diversity since last 10 years.

Efficacy of High-dose Chemotherapy and Autologous Stem Cell Transplantation in Patients with Relapsed Medulloblastoma: A Report on The Korean Society for Pediatric Neuro-Oncology (KSPNO)-S-053 Study

The efficacy and toxicity of high-dose chemotherapy and autologous stem cell transplantation (HDCT/ASCT) were investigated for improving the outcomes of patients with relapsed medulloblastoma. A total of 15 patients with relapsed medulloblastoma were enrolled in the KSPNO-S-053 study from May 2005 to May 2007. All patients received approximately 4 cycles of salvage chemotherapy after relapse. Thirteen underwent HDCT/ASCT; CTE and CM regimen were employed for the first HDCT (HDCT1) and second HDCT (HDCT2), respectively, and 7 underwent HDCT2. One transplant related mortality (TRM) due to veno-occlusive disease (VOD) occurred during HDCT1 but HDCT2 was tolerable with no further TRM. The 3-yr overall survival probability and event-free survival rates +/-95% confidence intervals (CI) were 33.3+/-12.2% and 26.7% +/-11.4%, respectively. When analysis was confined to only patients who had a complete response (CR) or partial response (PR) prior to HDCT, the probability of 3-yr overall survival rates +/-95% CI was 40.0+/-15.5%. No patients with stable disease (SD) or progressive disease (PD) survived. Survival rates from protocol KSPNO-S-053 are encouraging and show that tumor status prior to HDCT/ASCT is an important factor to consider for improving survival rates of patients with relapsed medulloblastoma.

Chiari 1.5 Malformation : An Advanced Form of Chiari I Malformation

The Chiari 1.5 malformation is defined as a tonsillar herniation within a Chiari I malformation with additional caudal descent of the brainstem through the foramen magnum. We describe a patient with Chiari I malformation who evolved to Chiari 1.5 malformation during longitudinal follow-up. A 15-year-old girl presented with neck pain during exercise for two years. She had been diagnosed with Chiari I malformation with mild hydrocephalus after minor cervical trauma at the age of six years. At that time, she was asymptomatic. After she complained of aggravated neck pain, neuroimaging (nine years after first imaging) revealed caudal descent of the brainstem and syringomyelia in addition to progression of tonsillar herniation. Posterior fossa decompressive surgery resulted in complete resolution of neck pain. Based on neuroimaging and operative findings, she was diagnosed as Chiari 1.5 malformation. Neuroimaging performed seven months after surgery showed an increased anterior-posterior diameter of the medulla oblongata and markedly decreased syringomyelia. This case demonstrates progressive developmental process of the Chiari 1.5 malformation as an advanced form of the Chiari I malformation.