Hangman's fracture in an osteogenesis imperfecta patient.

BACKGROUND: Traumatic spondylolisthesis of the axis, also known as a hangman's fracture, is a well-described entity but is relatively uncommon. There are only two published reports of these fractures occurring in pediatric patients with osteogenesis imperfecta, a disorder that predisposes patients to long bone fractures. CASE DESCRIPTION: We present a unique case of an adult woman with osteogenesis imperfecta who sustained an Effendi type II hangman's fracture. CONCLUSION: Effendi type II fractures can be conservatively managed via rigid or nonrigid cervical orthosis.

Craniocervical arachnoid cyst in a patient with Klippel-Feil syndrome: a unique case: case report.

Klippel-Feil syndrome, or brevicollis, is a complex congenital disorder caused by the improper segmentation of the cervical vertebrae. The authors present the very rare case of a patient with Klippel-Feil syndrome who presented with an intradural arachnoid cyst at the craniocervical junction. They also examine possible factors contributing to this association. A 46-year-old woman presented with complaints of progressively worsening headaches and dizziness of 18 months' duration. She also demonstrated mild bilateral upper-extremity weakness. Magnetic resonance imaging revealed fused cervical vertebrae and a dorsal intradural arachnoid cyst at the craniocervical junction, extending down to the fourth cervical level. Because of worsening myelopathy and the presence of brainstem compression, the patient underwent surgical excision of the arachnoid cyst, which was approached via a midline posterior suboccipital/upper cervical route. An endoscope was introduced through a gap between the occiput and fused upper cervical vertebrae, and the arachnoid cyst was widely fenestrated. Postoperatively, the patient has remained symptom free for more than 2 years with evidence of good radiological decompression. The authors report a unique association between craniocervical arachnoid cyst and Klippel-Feil syndrome. To their knowledge, no other cases of this association have been reported in the literature. Arachnoid cysts should be part of the differential diagnosis in the presence of worsening myelopathic symptoms or pain in patients with Klippel-Feil syndrome.

Primary calvarial angiosarcoma: A case report and review of the literature.

BACKGROUND: Angiosarcomas are high-grade endothelial tumors remarkable for their rarity and malignant behavior. Primary calvarial angiosarcoma is an extremely rare entity and its behavior usually sets it apart from other angiosarcoma types. We highlight the successful management of cranial angiosarcoma using a multidisciplinary approach. CASE DESCRIPTION: We present a 16-year-old male who was first noted to have a right-sided parietal cranial mass that was biopsied in 2008. Pathology was initially thought to be Kaposiform hemangioendothelioma. The patient subsequently underwent chemotherapy with vincristine. The patient did well until early 2010, when he suffered a right-sided intraparenchymal intratumoral hemorrhage. At this time, the original pathologic diagnosis was revisited and the diagnosis was upgraded to an angiosarcoma. The patient underwent a second round of chemotherapy using vincristine, cyclophosphamide, and actinomycin. The tumor continued to progress despite this treatment and he developed extensive skull deformity. At this point more definitive surgical intervention was reconsidered. Preoperative embolization of the mass was performed followed by aggressive surgical resection of the bony disease. The patient tolerated the procedure well and was discharged 6 days postoperatively without any new deficits. The patient is currently in the process of completing radiation therapy to entire tumor bed. He has clinically done well with no neurologic deterioration and has demonstrated long-term survival (>3 years). CONCLUSION: With the combined efforts of pediatric oncology, radiation oncology, interventional neuroradiology, and neurosurgery, a survival of greater than 3 years is possible with this aggressive pathology.

An update on unilateral sporadic small vestibular schwannoma.

Advances in neuroimaging have increased the detection rate of small vestibular schwannomas (VSs, maximum diameter < 25 mm). Current management modalities include observation with serial imaging, stereotactic radiosurgery, and microsurgical resection. Selecting one approach over another invites speculation, and no standard management consensus has been established. Moreover, there is a distinct clinical heterogeneity among patients harboring small VSs, making standardization of management difficult. The aim of this article is to guide treating physicians toward the most plausible therapeutic option based on etiopathogenesis and the highest level of existing evidence specific to the different cohorts of hypothetical case scenarios. Hypothetical cases were created to represent 5 commonly encountered scenarios involving patients with sporadic unilateral small VSs, and the literature was reviewed with a focus on small VS. The authors extrapolated from the data to the hypothetical case scenarios, and based on the level of evidence, they discuss the most suitable patient-specific treatment strategies. They conclude that observation and imaging, stereotactic radiosurgery, and microsurgery are all important components of the management strategy. Each has unique advantages and disadvantages best suited to certain clinical scenarios. The treatment of small VS should always be tailored to the clinical, personal, and social requirements of an individual patient, and a rigid treatment protocol is not practical.

Do cystic vestibular schwannomas have worse surgical outcomes? Systematic analysis of the literature.

OBJECT: The goal of this study was to perform a systematic quantitative comparison of the surgical outcomes between cystic vestibular schwannomas (CVSs) and solid vestibular schwannomas (SVSs). METHODS: A review of English-language literature published between 1990 and 2011 was performed using various search engines including PubMed, Google Scholar, and the Cochrane database. Only studies that reported surgical results of CVSs in comparison with SVSs were included in the analysis. The primary end point of this study was surgical outcomes, defined by the following: 1) facial nerve outcomes at latest follow-up; 2) mortality rates; or 3) non-facial nerve complication index. Secondary end points included extent of resection and brainstem adherence. RESULTS: Nine studies comprising 428 CVSs and 1287 SVSs were included in the study. The mean age of patients undergoing surgery was 48.3 ± 6.75 and 47.1 ± 9 years for CVSs and SVSs, respectively (p = 0.8). The mean tumor diameter for CVSs was 3.9 ± 0.84 cm and that for SVSs was 3.7 ± 1.2 cm (p = 0.7). There was no significant difference in the extent of resection among CVSs and SVSs (81.2% vs 80.7%, p = 0.87) Facial nerve outcomes were significantly better in the cohort of patients with SVSs than in those with CVSs (52.1% vs 39%, p = 0.0001). The perioperative mortality rates for CVSs and SVSs were not significantly different (3% and 3.8%, respectively; p = 0.6). No significant difference was noted between the cumulative non-facial nerve complication rate (including mortality) among patients with CVSs and SVSs (24.5% and 25.6%, respectively; p = 0.75) CONCLUSIONS: Facial nerve outcomes are worse in patients undergoing resection for CVSs than in patients undergoing resection for SVSs. There were no significant differences in the extent of resection or postoperative morbidity and mortality rates between the cohorts of patients with vestibular schwannomas.

Repair of middle fossa cerebrospinal fluid leaks using a novel combination of materials: technical note.

OBJECT: Methods for repairing middle fossa CSF (MFCSF) leaks have varied and yielded mixed results. The objective of this study was to evaluate the safety and durability of the authors' repair technique using a novel combination of 3 synthetic materials. METHODS: The authors performed a retrospective case review of patients treated for CSF leaks between January 2009 and September 2011. Eight patients were found to have undergone middle fossa craniotomies for CSF leaks. Inclusion criteria for the study included age greater than 18 years, neuroimaging-documented temporal bone defect, and symptoms consistent with CSF leaks or gross CSF otorrhea. Seven patients, 3 men and 4 women, met the inclusion criteria, and their charts were reviewed. Hydroxyapatite cement, collagen-based dural substitute matrix, and polyethylene glycol hydrogel sealant were used in all patients for the repair. RESULTS: In all patients the MFCSF leaks were successfully repaired. Initial presenting symptoms included CSF otorrhea in 4 patients (57.1%), hearing loss in 3 (42.9%), and CSF rhinorrhea in 1 (14.3%). The mean follow-up duration was 12 months (range 5-33 months). In 1 patient an epidural hematoma developed at the operative site on postoperative Day 2, and in another patient a superficial wound dehiscence occurred on postoperative Day 48. During the follow-up period, the authors found no evidence of wound infections, neurovascular damage, or CSF leakage requiring reoperation. CONCLUSIONS: The middle fossa approach involving a combination of hydroxyapatite cement, collagen-based dural substitute matrix, and polyethylene glycol hydrogel sealant is a safe, effective method for repairing MFCSF leaks. The combination of synthetic materials provides an alternative to existing materials for skull base surgeons.

The "agnes fast" craniotomy: the modified pterional (osteoplastic) craniotomy.

The "Agnes Fast" craniotomy is a fast and simple way of performing the pterional craniotomy while preserving the temporalis muscle, together with its fascia and bony attachment. Using this technique, the surgeon need not divide the temporalis muscle, separate it from its bony attachment, or perform an interfacial dissection. With a little practice, this craniotomy can be performed in less than 5 minutes and is highly recommended in emergent settings. The modified pterional craniotomy was performed in 10 cadaveric specimens, preserving the temporalis muscle with its attachment. An interfascial dissection was not performed while exposing the frontozygomatic process. The exposure gained, the length of the procedure, and the ease of application were recorded for all heads studied. In all heads studied, the Agnes Fast craniotomy was performed, with complete preservation of the temporalis muscle and its attachments. This procedure was performed quickly, with complete preservation of the fascial nerve and its branches. The muscle was put back in its natural place following the craniotomy. The Agnes Fast craniotomy offers a fast way of performing a pterional craniotomy while preserving the temporalis muscle, with its blood supply, neural innervation, bony attachment, and fascia intact. Replacing the muscle is also fast and simple and involves placement of two CranioFix (Aesculap, Inc., Center Valley, PA) holders to the bone, with no suture material. This approach does not limit the exposure gained and offers the same exposure as the "usual" pterional craniotomy.

Intracerebral metastasis of a sinonasal teratocarcinosarcoma: a case report.

Sinonasal teratocarcinosarcoma (SNTCS) is a rare, malignant neoplasm that contains both mesenchymal and epithelial components. The mortality rate for this tumor is ∼60% within 3 years, with the average survival rate being 1.7 years. Usually, this neoplasm presents with symptoms of nasal obstruction and epistaxis. Neurological symptoms from intracranial extension and dural invasion are rare presentations for this neoplasm. We present the first known case of an intracerebral metastasis of a previously resected SNTCS.

Glioblastoma multiforme in two non-nuclear family members.

BACKGROUND: In spite of traditional and current epidemiological research, there have been few environmental risk factors identified for malignant brain tumors. It has been an equally difficult challenge to identify genetic causes for brain tumors because of the rarity of families with multiple affected individuals, which prevents the use of traditional methods of genetic analysis such as genetic linkage, sib-pair, or even population-based association studies. Thus, it is important to take advantage of rare occasions of familial brain tumors. METHODS: Identification and careful study of such families may provide important clues about the etiology of brain malignancies. We studied one family of which two nonnuclear family members were affected with pathologically diagnosed glioblastoma multiforme. Fluorescence in situ hybridization (FISH) assays were used on archival sections from each patient's tumor to investigate the loss and/or gain of important allelic endpoints. Tissue sections were prepared and processed for FISH. DNA probes for targeted gene loci were used to assess allelic gain/loss. FISH probes targeted regions including 19q13, 1p36, 10q/phosphate and tensin homolog (PTEN), chromosome 3, chromosome 7, chromosome 17/17q and p53/17p. RESULTS: FISH analyses identified distinct abnormalities in the two patients, suggesting that despite the familial connections and histologically similar tumors, genetic abnormalities are abundant and heterogeneous among these malignancies. CONCLUSION: These abnormalities, however, serve to contribute to valuable information regarding patient outcomes, albeit their precise roles in the etiology of this malignancy are yet to be determined.

Survival trends in elderly patients with glioblastoma multiforme: resective surgery, radiation, and chemotherapy.

BACKGROUND: It is appropriate to investigate and to determine survival trends following glioblastoma multiforme treatment using resective surgery, radiation therapy, and/or chemotherapy in patients aged 59 years and higher. METHODS: We retrospectively reviewed 30 elderly patients (> or =59 years old) who were treated for histopathologically confirmed glioblastoma multiforme at our tertiary care institution from 1990 through 2002. All patients were treated with steroids. In addition, 22 patients underwent resective surgery (RS), 17 patients underwent radiation therapy (RT), and 10 patients underwent chemotherapy (C). Many patients underwent these treatments in various combinations: 6 underwent biopsy only, 7 RS only, 6 RS+RT only, and 9 RS+RT+C. For each case, pre-treatment Karnofsky performance scores (KPS), tumor location, presenting symptoms and signs, associated surgical morbidity, and pre-existing medical conditions were also recorded. Patients were categorized into one of four treatment subgroups: Biopsy only, RS only, RS+RT, and RT+RS+C. For each of these subgroups, pretreatment KPS and post-treatment survival were compared. RESULTS: Post-treatment survival following biopsy only was 3.2 +/- 0.8 months (mean +/- SE); RS 2.2 +/- 0.5; RS+RT 5.5 +/- 1.2; RS+RT+C 13.6 +/- 2.1. A longer survival trend was noted for the RS+RT versus RS group (two-tailed unpaired t test, p = 0.02;), as well as the RS+RT+C group, which showed consistently higher survival in comparison to most of the other groups (p = 0.0021, 0.00039, 0.013 vs. the biopsy only, RS only, and RS+RT groups, respectively). No significant difference was found in KPS, comparing all individual groups versus each other (p > or = 0.06). Remarkably, 6 patients survived over 14 months (range, 14.1-22.7 months), all of which received RS+RT+C. CONCLUSIONS: This study suggests a significant improvement in elderly patients treated with the combination of resective surgery, radiation therapy, and chemotherapy, rather than either treatment alone or other combination. This significant improvement does not appear to be biased by pretreatment KPS, as mean KPS values did not significantly differ between any of these groups. However, a greater number of patients in each group must be considered to achieve the power to make more definitive treatment guidelines.

Multicentric glioblastoma multiforme determined by positron emission tomography: a case report.

UNLABELLED: The actual incidence of true multicentric glioblastoma multiforme (GBM) varies between 2.4 and 4.9% of all GBMs. True multicentric tumors are described as widespread lesions in different lobes or hemispheres, which cannot be explained by spreading along the cerebrospinal fluid or blood pathways. We present here a case of multicentric GBM identified with positron emission tomography. CASE REPORT: A 73-year-old woman with sudden onset headaches, balance problems, and one episode of syncope was diagnosed as having an irregular, contrast-enhancing, space-occupying lesion in the left-temporal-parietal region on magnetic resonance imaging (MRI). The tissue diagnosis was confirmed as GBM, and she received stereotactic radiosurgery using the Leksell Gamma Knife (Elekta Instruments, Atlanta, GA). A 3-month, follow-up, MRI scan showed a remarkable decrease in the size of the contrast-enhancing area that was targeted during radiosurgery. A suspicious area of enhancement was detected on the right side, although no surrounding edema was evident. Fluorodeoxyglucose (FDG)-PET scanning revealed a large irregular neoplasm extending from the inferior left-temporal lobe into the deep parietal lobe with extremely intense FDG uptake, suggesting a very aggressive tumor. A smaller lesion was also discovered in the deep right-frontal lobe, representing a second neoplastic focus. The patient refused any further treatment. CONCLUSION: PET scans, in conjunction with MRI scans, allow for the best possible and most comprehensive diagnosis and treatment plans.