Secondary Aneurysmal Bone Cyst in a Craniofacial Fibrous Dysplasia: Case Report

Aneurysmal bone cyst (ABC) is a rare non-neoplastic bone lesion that involves mostly the long bones and vertebrae and may occur very rarely in the craniofacial bones. ABCs may occur as secondary bony pathologies in association with various benign and malignant bone tumors and with fibrous dysplasia (FD). FD is a common non-neoplastic bony pathology mostly affecting craniofacial bones. Secondary ABC occurring in craniofacial FD is extremely rare, with only approximately 20 cases reported in the literature to date. Here, we report on a case of secondary ABC in a 25-year-old woman who has had a craniofacial deformity for over 10 years and who presented to us with a rapidly growing painful pulsatile mass in the right frontal region that began over 2 months prior to admission. On thorough examination of computed tomography and magnetic resonance imaging brain scans taken at two-month interval, an aggressive, rapidly enlarging ABC, arising from the right frontal FD, was diagnosed. The patient underwent preoperative embolization followed by gross total resection of the ABC and cranioplasty. The 6-month follow up showed no recurrence of the ABC, nor was any progression of the FD noticed.

Complications Following Burr Hole Craniostomy and Closed-System Drainage for Subdural Lesions / 대한신경손상학회지

OBJECTIVE: Burr hole craniostomy and closed-system drainage (BCD) is a common surgical procedure in the field of neurosurgery. However, complications following BCD have seldom been reported. The purpose of this study was to report our experiences regarding complications following BCD for subdural lesions. METHODS: A retrospective study of all consecutive patients who underwent BCD for presumed subdural lesions at one institute since the opening of the hospital was performed. RESULTS: Of the 395 patients who underwent BCD for presumed subdural lesions, 117 experienced surgical or nonsurgical complications. Acute intracranial hemorrhagic complications developed in 14 patients (3.5%). Among these, 1 patient died and 5 patients had major morbidities. Malposition of the drainage catheter in the brain parenchyma occurred in 4 patients, and opposite-side surgery occurred in 2 patients. Newly developed seizures after BCD occurred in 8 patients (2.0%), five of whom developed the seizures in relation to new brain lesions. Eighty-eight patients (22.3%) suffered from nonsurgical complications after BCD. Pulmonary problems (7.3%) were the most common nonsurgical complications, followed by urinary problems (5.8%), psychologic problems (4.3%), and cognitive impairments (3.8%). CONCLUSION: The incidence of complications after BCD for subdural lesions is higher than previously believed. In particular, catastrophic complications such as acute intracranial hematomas and surgical or management errors occur at rates that cannot be ignored, possibly causing medico-legal problems. Great caution must be taken during surgery and the postoperative period, and these complications should be listed on the informed consent form before surgery.

Dynamic Susceptibility Contrast (DSC) Perfusion MR in the Prediction of Long-Term Survival of Glioblastomas (GBM): Correlation with MGMT Promoter Methylation and 1p/19q Deletions

PURPOSE: To investigate the surgical, perfusion, and molecular characteristics of glioblastomas which influence long-term survival after treatment, and to explore the association between MR perfusion parameters and the presence of MGMT methylation and 1p/19q deletions. MATERIALS AND METHODS: This retrospective study was approved by our institutional review board. A total 43 patients were included, all with pathologic diagnosis of glioblastoma with known MGMT methylation and 1p/19q deletion statuses. We divided these patients into long-term (≥ 60 months, n = 7) and short-term (< 60 months, n = 36) survivors, then compared surgical extent, molecular status, and rCBV parameters between the two groups using Fisher's exact test or Mann-Whitney test. The rCBV parameters were analyzed according to the presence of MGMT methylation and 1p/19q deletions. We investigated the relationship between the mean rCBV and overall survival using linear correlation. Multivariable linear regression was performed in order to find the variables related to overall survival. RESULTS: Long-term survivors (100% [7 of 7]) demonstrated a greater percentage of gross total or near total resection than short-term survivors (54.5% [18 of 33]). A higher prevalence of 1p/19q deletions was also noted among the long-term survivors (42.9% [3 of 7]) than the short-term survivors (0.0% [0 of 36]). The rCBV parameters did not differ between the long-term and short-term survivors. The rCBV values were marginally lower in patients with MGMT methylation and 1p/19q deletions. Despite no correlation found between overall survival and rCBV in the whole group, the short-term survivor group showed negative correlation (R2 = 0.181, P = 0.025). Multivariable linear regression revealed that surgical extent and 1p/19q deletions, but not rCBV values, were associated with prolonged overall survival. CONCLUSION: While preoperative rCBV and 1p/19q deletion status are related to each other, only surgical extent and the presence of 1p/19q deletion in GBM patients may predict long-term survival.

Palliative Resection of Metastatic Brain Tumors Previously Treated by Stereotactic Radiosurgery

BACKGROUND: Therapeutic approaches to brain metastases include surgery, whole-brain radiotherapy, stereotactic radiosurgery (SRS), and combination therapy. Recently, postoperative or preoperative SRS draws more attention to reduce postoperative recurrence in brain metastases. The goal of this study is to review surgical outcome of patients who had been treated by SRS, and to discuss the effectiveness of preoperative SRS. METHODS: From 2009 to 2015, 174 patients were treated by SRS for brain metastases, and among these 50 patients underwent surgery. Eighteen patients underwent surgery after SRS, and 14 had oligometastases. The patients' median age at the time of surgery was 56 years (range, 34–84 years). The median follow-up duration was 16.5 months (range, 4–47 months). Pathological findings were classified as follows; radiation necrosis (Group I, n=3), mixed type (Group II, n=2), and tumor-dominant group (Group III, n=9). We compared surgical outcome in respect of steroid, mannitol dosage, Karnofsky performance scale, and pathological subgroups. RESULTS: The median overall survival was 11 months (range, 2–40 months). Six, 12 and 24 months survival rate was 64.3, 42.9, and 28.6%, respectively. Improvement of Karnofsky performance score was achieved in 50% after surgery. The overall survival of Group I (26.6 months) was longer than the other groups (11.5 months). Additionally the patients were able to be weaned from medications, such as steroid administration after surgery was reduced in 10 cases, and mannitol dosage was reduced in 6 cases. Time interval within 3 months between SRS and surgery seemed to be related with better local control. CONCLUSION: Surgical resection after radiologically and symptomatically progressed brain metastases previously treated with SRS seems to be effective in rapid symptom relief and provides an improvement in the quality of life. A short time interval between SRS and surgical resection seems to be associated with good local tumor control.

Metastatic Intracranial Hemangiopericytoma to the Spinal Column: A Case Report

Intracranial hemangiopericytoma (HPC) is a rare brain tumor with aggressive biologic behavior associated with high recurrence rate and often with extracranial metastasis. The most common sites of extracranial metastasis of the intracranial HPC are the long bones, lung, liver and abdominal cavity in the order of frequencies. Extracranial metastases usually occur long after the initial diagnosis of the primary tumor. Metastatic intracranial HPC to the vertebra has been rarely reported. We present a case of intracranial HPC metastasized to the L2 vertebral body 13 years after multiple surgical resections and radiotherapy of the primary intracranial HPC.

Serial Mini-Mental Status Examination to Evaluate Cognitive Outcome in Patients with Traumatic Brain Injury / 대한신경손상학회지

OBJECTIVE: This study was aimed at finding out the changes in cognitive dysfunction in patients with traumatic brain injury (TBI) and investigating the factors limiting their cognitive improvement. METHODS: Between January 2010 and March 2014, 33 patients with TBI participated in serial mini-mental status examination (MMSE). Their cognitive functions were statistically analyzed to clarify their relationship with different TBI status. Patients who developed hydrocephalus were separately analyzed in regards to their cognitive function depending on the placement of ventriculoperitoneal shunt (VPS). RESULTS: Bi-frontal lobe injury (beta=-10.441, p<0.001), contre-coup injury (beta=-6.592, p=0.007), severe parenchymal injury (beta=-7.210, p=0.012), temporal lobe injury (beta=-5.524, p=0.027), and dominant hemisphere injury (beta=-5.388, p=0.037) significantly lowered the final MMSE scores. The risk of down-grade in the prognosis was higher in severe parenchymal injury [odds ratio (OR)=13.41, 95% confidence interval (CI)=1.31-136.78], temporal lobe injury (OR=12.3, 95% CI=2.07-73.08), dominant hemisphere injury (OR=8.19, 95% CI=1.43-46.78), and bi-frontal lobe injury (OR=7.52, 95% CI=1.31-43.11). In the 11 post-traumatic hydrocephalus patients who underwent VPS, the final MMSE scores (17.7+/-6.8) substantially increased from the initial MMSE scores (11.2+/-8.6). CONCLUSION: Presence of bi-frontal lobe injury, temporal lobe injury, dominant hemisphere injury, and contre-coup injury and severe parenchymal injury adversely influenced the final MMSE scores. They can be concluded to be poor prognostic factors in terms of cognitive function in TBI patients. Development of hydrocephalus aggravates cognitive impairment with unpredictable time of onset. Thus, close observation and routine image follow-up are mandatory for early detection and surgical intervention for hydrocephalus.

Metachronous schwannoma in the colon with vestibular schwannoma

We experienced a case of vestibular schwannoma and metachronous schwannoma in the colon. A 59-year-old female presented with a 1-month history of hematochezia. She had undergone suboccipital craniectomy resulting in radical subtotal resection, followed by gamma knife radiosurgery for a large left vestibular schwannoma 4 years prior to admission. On preoperative colonoscopy, a huge mass through which the colonoscope could not be passed was detected. CT scans showed colo-colonic intussusception with a 4.8-cm-sized mass in the descending colon. PET/CT revealed hypermetabolism of the descending colon tumor and pericolic lymph nodes. We performed left hemicolectomy under the preoperative impression of colon cancer with intussusception. A pathological diagnosis of benign schwannoma of the colon was made in this patient.

The Clinico-Oncologic Outcomes of Elderly Patients with Glioblastoma after Surgical Resection Followed by Concomitant Chemo-Radiotherapy

BACKGROUND: There have been controversies in the treatment of elderly patients with glioblastoma. We introduce the outcome of the treatment of elderly patients with glioblastoma comparing with younger patients. METHODS: The author's hospital database was used to identify patients with histologically confirmed glioblastoma after surgery between January 2006 and December 2013. Forty-eight patients (control group) were under age 65 and 16 patients (elderly group) were aged 65 years or over at the time of surgery. RESULTS: The median age of the elderly group was 71 years and control group was 50 years. Mean number of medical comorbidities was 1.8 in the elderly group vs. 0.5 in the control group. The median progression free survival (PFS) was 5.6 months and the median overall survival (OS) was 19.9 months in all patients. The elderly group had a median PFS of 4.2 months vs. 8 months for the control group (log-rank test, p=0.762). Median OS was 8.2 months in the elderly group vs. 20.9 months in the control group (log-rank test, p=0.457). Major complications occurred in 5 cases (7.8%) for all patients. The ratio of completion of concomitant chemo-radiotherapy (CCRT) was 81.3% and was the same between the two groups. In multivariable analysis, extent of resection (p=0.034) and completion of CCRT (p=0.023) were statistically significant, independent prognostic factors only for PFS in all patients by Cox proportional hazards model. Age was not an independent prognostic factor. As for OS, there was no significant factor. CONCLUSION: Surgical resection and CCRT were well tolerated in elderly patients with glioblastoma, and maximal safe resection followed by timely CCRT could improve clinic-oncologic outcomes.

Clinical Characteristics of Cerebral Venous Thrombosis in a Single Center in Korea

OBJECTIVE: The purpose of this study is to investigate the clinical characteristics of cerebral venous thrombosis (CVT) in a single center in Korea. METHODS: A total of 36 patients were diagnosed with CVT from August 2005 to May 2013. The patient data regarding age, sex, disease stage, pathogenesis, location, laboratory findings, radiological findings, and treatment modalities were retrospectively collected. The results were compared with those of previous studies in other countries. RESULTS: The patient group comprised 21 men and 15 women with a mean age of 46.9 years (ranging from three months to 77 years). The most common cause was a prothrombotic condition (8 patients, 22.2%). Within the patient group, 13 patients (36.1%) had a hemorrhagic infarction, whereas 23 (63.9%) had a venous infarction without hemorrhage. By location, the incidence of hemorrhagic infarction was the highest in the group with a transverse and/or sigmoid sinus thrombosis (n=9); however, the proportion of hemorrhagic infarction was higher in the cortical venous thrombosis group (75%) and the deep venous thrombosis group (100%). By pathogenesis, the incidence of hemorrhagic infarction was the highest in the prothrombotic group (n=6), which was statistically significant (p=0.016). CONCLUSION: According to this study, CVT was more prevalent in men, and the peak age group comprised patients in the sixth decade. The most common cause was a prothrombotic condition. This finding was comparable with reports from Europe or America, in which CVT was more common in younger women. Hemorrhagic infarction was more common in the prothrombotic group (p=0.016) than in the non-prothrombotic group in this study.

Primary Intracranial Fibrosarcoma Presenting with Hemorrhage

Primary intracranial fibrosarcomas (PIFs) are extremely rare and the origin of these tumors is still controversial. The rarity of primary intracranial fibrosarcomas makes it difficult to diagnose them correctly and establish a standard treatment. The pathologic diagnosis is made by distinguishing findings from light microscopic and immunohistochemistry analysis. PIFs have been known to be very aggressive neoplasms. The extra-axial location of the tumor could provide an opportunity to perform a total resection even if it does not mean a cure. We present a case of PIFs mimicking a falx meningioma in a 17-year-old man.

Extremely Delayed Brain Metastasis from Renal Cell Carcinoma

Brain metastasis occurs in 3.9-24% of patients with renal cell carcinoma (RCC), with an average interval from nephrectomy to brain metastasis of 1 to 3 years. A few cases have been reported where brain metastasis occurred after a delay of more than 10 years from the initial onset of renal cell carcinoma. This long interval for central nervous system metastasis from the primary cancer has been recognized as an indicator of better prognosis. Histopathological confirmation and aggressive treatment must be considered in these delayed brain metastases cases, since the patients usually show long survival and good prognosis. We present a case of a 76-year-old woman who developed extremely late multiple brain metastases 18 years after a nephrectomy for RCC.

Galeal Tack-Up Sutures to Prevent Subgaleal Cerebrospinal Fluid Collection

OBJECTIVE: Postoperative subgaleal cerebrospinal fluid (CSF) collection is considered as one of the common minor surgical complication which can lead to prolonged hospitalization. We introduce "galeal tack-up suture" to prevent postoperative subgaleal CSF collection. METHODS: Galeal tack-up suture consists of various surgical techniques which aim to fix galea to cranium in order to prevent CSF pooling in subgaleal space. A total of 87 patients who underwent craniotomy were divided into two groups while closing the wound : group A with galeal tack-up suture and group B with routine wound closure without galeal tack-up suture. The patients were observed for postoperative subgaleal CSF collection. RESULTS: Among 87 cranitomy cases, galeal tack-up suture was performed in 32 cases and routine wound closure was done in 55 cases. Postoperative subgaleal CSF collection occurred in 13 cases (15%) in which 12 cases occurred in group B patients and 1 case occurred in group A patients (p=0.026). CONCLUSION: Galeal tack-up suture is an easy and effective technique in wound closure to prevent postoperative CSF collection.

Endovascular Management of Great Vessel Injury Following Lumbar Microdiscectomy

Great vessel injury is a rare but well-known complication of lumbar disc surgery, which may result in acute or fatal outcomes of delayed diagnosis. Thus, early detection and proper management is vital. The authors report a case of retroperitoneal hemorrhage with arteriovenous fistula and pseudoaneurysm after lumbar microdiscectomy. The patient was successfully managed by endovascular intervention using a stent graft. Endovascular repair is a minimally invasive and efficient treatment modality with considerably low morbidity and mortality.

Grading of Intracerebral Hemorrhage in Ruptured Middle Cerebral Artery Aneurysms

OBJECTIVE: To propose grading of intracerebral hemorrhage (ICH) in ruptured middle cerebral artery (MCA) aneurysms, which helps to predict the prognosis more accurately. METHODS: From August 2005 to December 2010, 27 cases of emergent hematoma evacuation and aneurysm clipping for MCA aneurysms were done in the author's clinic. Three variables were considered in grading the ICH, which were 1) hematoma volume, 2) diffuse subarachnoid hemorrhage (SAH) that extends to the contralateral sylvian cistern, and 3) the presence of midline shifting from computed tomography findings. For hematoma volume of greater than 25 mL, we assigned 2 points whereas 1 point for less than 25 cc. We also assigned 1 point for the presence of diffuse SAH whereas 0 point for the absence of it. Then, 1 point was assigned for midline shifting of greater than 5 mm whereas 0 point for less than 5 mm. RESULTS: According to the grading system, the numbers of patients from grade 1 to 4 were 4, 6, 8 and 9 respectively and 5, 7, 8, 4 and 3 patients belonged to Glasgow Outcome Scale (GOS) 5 to 1 respectively. It was found that the patients with higher GOS had lower ICH grade which were confirmed to be statistically significant (p<0.01). Preoperative Hunt and Hess grade and absence of midline shifting were the factors to predict favorable outcome. CONCLUSION: The ICH grading system composed of above three variables was helpful in predicting the patient's outcome more accurately.

A Consideration of MGMT Gene Promotor Methylation Analysis for Glioblastoma Using Methylation-Specific Polymerase Chain Reaction and Pyrosequencing

BACKGROUND: O6-methylguanine-DNA methyltransferase (MGMT) gene promoter methylation is currently the most promising predictive marker for the outcome and benefit from temozolomide treatment in patients with glioblastoma, but there is no consensus on the analysis method for assessing the methylation status in the molecular diagnostic field. The objective of this study was to evaluate methylation-specific polymerase chain reaction (MSP) and pyrosequencing methods for assessing MGMT gene promotor methylation of glioblastoma as well as assessing the MGMT protein expression by immunohistochemistry. METHODS: Twenty-seven cases of glioblastoma from the archives at the Department of Pathology Konkuk University Hospital were selected. MGMT promoter methylation was evaluated by MSP and the pyrosequencing methods. The MGMT expression was also measured at the protein level by immunohistochemistry. RESULTS: Overall, MGMT hypermethylation was observed in 44.4% (12/27 cases) of the case of glioblastoma using either MSP or pyrosequencing. The concordant rate was 70.3% (19/27 cases) between MSP and pyrosequencing for MGMT methylation. There was no correlation between MGMT methylation and the protein expression. No significant differences in progression free survival and overall survival were seen between the methylated group and the unmethylated group by using either MSP or pyrosequencing. The status of the MGMT protein expression was correlated with progression free survival (p=0.026). CONCLUSIONS: In this study the concordance rate between MSP and the pyrosequencing methods for assessing MGMT gene promotor methylation was relatively low for the cases of glioblastoma. This suggests that more reliable techniques for routine MGMT methylation study of glioblastoma remain to be developed because of quality control and assurance issues.

Fluoroscopy and Sonographic Guided Injection of Obliquus Capitis Inferior Muscle in an Intractable Occipital Neuralgia

Occipital neuralgia is a form of headache that involves the posterior occiput in the greater or lesser occipital nerve distribution. Pain can be severe and persistent with conservative treatment. We present a case of intractable occipital neuralgia that conventional therapeutic modalities failed to ameliorate. We speculate that, in this case, the cause of headache could be the greater occipital nerve entrapment by the obliquus capitis inferior muscle. After steroid and local anesthetic injection into obliquus capitis inferior muscles under fluoroscopic and sonographic guidance, the visual analogue scale was decreased from 9-10/10 to 1-2/10 for 2-3 weeks. The patient eventually got both greater occipital neurectomy and partial resection of obliquus capitis inferior muscles due to the short term effect of the injection. The successful steroid and local anesthetic injection for this occipital neuralgia shows that the refractory headache was caused by entrapment of greater occipital nerves by obliquus capitis inferior muscles.

A Case of Moyamoya Disease in a Girl with Thyrotoxicosis

Moyamoya disease is a cerebrovascular disorder of unknown cause, characterized by slowly progressive bilateral stenosis or occlusion of the internal carotid arteries and produces collateral vessels. Moyamoya syndrome has rarely been reported in association with Graves' disease, especially in children. Several reports suggest that a cerebral infarction might have occurred in patients with clinical and laboratory evidence of hyperthyroid function. We report a case of Moyamoya disease in a girl with Down syndrome and thyrotoxicosis, and we review the relevant literature. To our best knowledge, this is the first report of Moyamoya disease associated with thyrotoxicosis in a young person in Korea.

Traumatic Entrapment of the Vertebrobasilar Junction Due to a Longitudinal Clival Fracture: A Case Report

Vertebrobasilar junction entrapment due to a clivus fracture is a rare clinical observation. The present case report describes a 54-yr-old man who sustained a major craniofacial injury. The patient displayed a stuporous mental state (Glasgow Coma Scale [GCS]=8) and left hemiparesis (Grade 3). The initial computed tomography (CT) scan revealed a right subdural hemorrhage in the frontotemporal region, with a midline shift and longitudinal clival fracture. A decompressive craniectomy with removal of the hematoma was performed. Two days after surgery, a follow-up CT scan showed cerebellar and brain stem infarction, and a CT angiogram revealed occlusion of the left vertebral artery and entrapment of vertebrobasilar junction by the clival fracture. A decompressive suboccipital craniectomy was performed and the patient gradually recovered. This appears to be a rare case of traumatic vertebrobasilar junction entrapment due to a longitudinal clival fracture, including a cerebellar infarction caused by a left vertebral artery occlusion. A literature review is provided.

Efficacy of Laparoscopy Guided Ventriculo-Peritoneal Shunt : Analysis of Data in 102 Consecutive Patients / 대한뇌혈관외과학회지

OBJECTIVE: This study compare outcomes in patients with laparoscopic guided ventriculo-peritoneal shunt placement with the nonlaparoscopic approach (conventional mini-laparotomy technique) in patients with hydrocephalus. METHODS: The study enrolled 102 adult patients who had undergone surgery between August 2005 and May 2008 for the treatment of hydrocephalus at our department. Seventy-six patients (38 men and 38 women) received laparoscopy-assisted distal catheter placement, and 26 patients (14 men and 12 women) received shunt surgery by a conventional mini-laparotomy approach. The median follow-up period of the non-laparoscopic group was 26 months (14-33 months) and laparoscopic group was 12 months (1-30 months). We analyzed age, sex, cause of hydrocephalus, type of shunt valve, operation time, shunt infection rate, and shunt distal catheter malfunction during follow-up. RESULTS: There was no statistically significant difference in age, sex, cause of hydrocephalus, or type of shunt valve. Shunt infections were observed in 10 cases (13.2%) in the laparoscopic group and in 4 cases (15.4%) in the mini-laparotomy group, which was not statistically significant. Distal shunt malfunction rates were significantly lower in the laparoscopic group (1.3%) compared to the mini-laparotomy group (11.5%, P<0.05). The mean operation time was also shorter in the laparoscopic group (108 min, 45-190 min) than of the mini-laparotomy group (146 min, 75-255 min, P<0.005). CONCLUSION: Laparoscopic guided placement of the VP shunt in hydrocephalus patients reduces shunt distal catheter malfunction and operation time.

CT Angiography in Acute Ischemic Stroke: Clinical Research / 대한뇌혈관외과학회지

OBJECTIVE: This study was designed to evaluate the feasibility and clinical implications of CT angiography (CTA) in patients with acute ischemic stroke. METHODS: From August 2004 to July 2005, 24 cases of acute ischemic stroke were prospectively included in this study. We checked location of ischemic parenchymal lesion, location of vascular occlusion, degree of collateral supply, and presence of other accompanying vascular lesions on CT and CTA, and assessed the usefulness of CTA by comparing the findings with those of diffusionweighted MR imaging and digital subtraction angiography. RESULTS: Average time required for performing CT and CTA and getting reconstructed images was 30 minutes. Location of the parenchymal lesions and the corresponding occluded or stenosed artery could be clarified in 16 cases (67%) and 20 cases (83%), respectively. There were 13 cases of severe stenosis and 7 cases of occlusion. In 7 cases of major arterial occlusion, degree of collateral circulation could be assessed as good in 5, and moderate in 2. Incidental unruptured intracranial aneurysms were identified in 5 cases. CONCLUSION: CTA could provide valuable information regarding locations of parenchymal lesion and vascular occlusion, degree of collateral supply, and presence of accompanying intracranial aneurysm in cases of acute ischemic stroke without significant time delay, thereby guiding therapeutic plan.