C4 to C7 Laminoplasty for Resection of an Intradural Intramedullary Ependymoma: 2-Dimensional Operative Video.

A 28-year-old woman with a history of a prolactinoma presented with about 5 months of neck pain and left upper extremity numbness and tingling in the C7 and C8 dermatomal distribution. She had no weakness on examination though notable increased deep tendon reflexes in the lower extremities. She had a homogenously enhancing lesion with an associated syrinx on MRI of her cervical spine. She was offered a C4-7 laminoplasty for resection of the tumor and ultimate tissue diagnosis. The patient consented to the procedure. Her postoperative imaging was notable for gross total resection and significant improvement of the syrinx and cervical alignment. Clinically, her paresthesias and gait improved significantly. The pathology confirmed a diagnosis of World Health Organization grade II ependymoma. The management of ependymomas in adults often starts with surgical resection for cytoreduction followed by postoperative radiotherapy for World Health Organization grade II and grade III at least.1-4 Spinal cord ependymomas tend to have favorable prognosis, except when carrying an MYCN gene amplification.5,6.

Chiari Type I Revision Decompressive Surgery Indications and Operative Technique: Experience in a Large Adult Cohort.

BACKGROUND: Chiari malformation is characterized by inferior displacement of the cerebellar tonsils through the foramen magnum, frequently resulting in strain related headaches, and motor/sensory dysfunction. Chiari decompression technique varies significantly, possibly contributing to frequent revisions. We reviewed revision Chiari decompressions at our institution to determine the primary indications for revision and outcomes after revision. METHODS: We retrospectively reviewed patients who underwent revision of Chiari decompression at our institution from 2005 to 2020. Demographics, indications for revision surgery, operative techniques, imaging findings, and preoperative/postoperative symptoms were collected. χ2 test was performed to determine statistical significance using a P < 0.05. Independent predictors of operative outcomes were identified. RESULTS: A total of 46 patients (91% females, mean age 38.8 years) were included for analysis. The median time to revision surgery was 69.1 months (range 0-364 months) with headache (n = 37, 80%) being the most commonly recurring symptom. Large craniectomy (n = 28, 61%) was the most frequent indication for revision surgery. Thirty-two (70%) patients underwent cranioplasty, 20 (43%) required duraplasty, 15 (33%) required arachnoid dissection, and 15 (33%) required tonsillar reduction during revision surgery. Postrevision follow-up (at 8.9 ± 5.2 months average, range 1-18 months), revealed an average reduction in all Chiari-related symptoms relative to symptoms before the revision. CONCLUSIONS: The most common indication for revision Chiari decompression was a large craniectomy resulting in cerebellar ptosis. We found that tonsillar reduction paired with modest craniectomy achieved near-complete resolution of symptoms with minimal complications. For patients with recurrent or persistent sequelae of Chiari malformation after decompression, revision may reduce symptom severity.

Tandem resection of multiple spinal schwannomas.

This video depicts the resection of three separate intradural extramedullary spinal tumors performed under the same anesthetic. Neuromonitoring was used to identify motor nerve roots, and laminoplasty was performed at the thoracolumbar junction to preserve alignment and minimize the risk of postoperative CSF leak.

A Novel Untethering and Duraplasty Technique for Postsurgical Tethered Spinal Cord.

Progressive post-traumatic postsurgical myelopathy (PPPM) is a known entity that can occur months to years after the initial insult. Symptomatic patients can become myelopathic and have rapid and progressive neurological decline. Surgical correction of PPPM usually involves intradural exploration and lysis of adhesions that carries the risk of further injury to the spinal cord. In this manuscript, we provide a report of a patient presenting more than 50 years after the initial resection of an intramedullary tumor. Additionally, we present and describe a novel surgical technique for managing this difficult problem and restoring normal CSF dynamics.

Short-Term and Long-Term Complications Associated with Posterior Fossa Decompression for Chiari Malformation.

Surgery is the treatment of choice for symptomatic patients with Chiari anomalies Although the surgical treatment of Chiari anomalies in adults is a straightforward procedure, complications and less than satisfactory outcomes do occur. Understanding these complications is important for correcting the problem as well as preventing the recurrence of similar problems. In this article, the author review the short-term and long-term complications associated with posterior fossa decompression for Chiari malformation.

Structural Changes in the Cervicomedullary Junction in Adult Chiari Patients.

OBJECTIVE: To assess volumetric changes in the spinal cord at the cervicomedullary junction, diameter of the cervicomedullary cord, and width of the brainstem following posterior fossa decompression (PFD). METHODS: A retrospective analysis of adult patients with Chiari malformation who underwent PFD was performed. Segmentations were done on clinical quality T2-weighted cervical magnetic resonance images obtained before and after decompression using ITK-SNAP. Volumes of neural tissue within the cervicomedullary junction were evaluated from 10 mm cranial to the medullary beak to the cervical spinal cord at the level of the caudal endplate of the second cervical vertebra. The diameter of the cervicomedullary cord was calculated perpendicular to the spinal cord. The width of the brainstem was measured perpendicular to the clivus at the level of the basion. RESULTS: Twenty adult patients, a mean age of 49.55 years, were included. The cervical cord increased in volume by 13 mm3 to 338 mm3, with an average increase of 155 mm3 (P-value of 0.00002). The diameter of the cervicomedullary cord increased 10.30% 7 mm superior to the beak (P-value of 0.00074), 11.49% at the apex of the beak (P-value of 0.00082), 8.29% 7 mm inferior to the beak (P-value of 0.00075), and the brainstem increased 14.46% perpendicular to the clivus (P-value of 0.00109). The spinal cord at the inferior aspect of the C3 vertebra changed insignificantly (P-value of 0.10580). CONCLUSION: The volume of the cervical cord at the cervical-medullary junction, width of the cervicomedullary cord, and diameter of the brainstem increase following PFD.

Clinical and surgical management of holocervical spinal cord ependymomas.

BACKGROUND: Spinal ependymomas are rare tumors of the central nervous system, and those spanning the entire cervical spine are atypical. Here, we present two unusual cases of holocervical (C1-C7) spinal ependymomas. CASE DESCRIPTION: Two patients, a 32-year-old female and a 24-year-old male presented with neck pain, motor, and sensory deficits. Sagittal MRI confirmed hypointense lesions on T1 and hyperintense regions on T2 spanning the entire cervical spine. These were accompanied by cystic cavities extending caudally into the thoracic spine and rostrally to the cervicomedullary junction. Both patients underwent gross total resection of these lesions and sustained excellent recoveries. CONCLUSION: Two holocervical cord intramedullary ependymomas were safely and effectively surgically resected without incurring significant perioperative morbidity.

Chiari malformation and syringomyelia.

Chiari malformation was first described over a century ago, and consists of posterior fossa anomalies that generally share the feature of cerebellar tonsillar descent through the foramen magnum. Our understanding of this disorder was initially based on autopsy studies, and has been greatly enhanced by the advent of MRI. The surgical management of Chiari anomalies has also evolved in a parallel fashion. Although the exact surgical technique varies among individual surgeons, the goals of surgery remain constant and consist of relieving brainstem compression and cranial nerve distortion, restoring the normal flow of CSF across the foramen magnum, and reducing the size of any associated syrinx cavity. Syrinx cavities are most commonly associated with Chiari anomalies, yet primary spinal syringomyelia (PSS) can be caused by traumatic, infectious, degenerative, and other etiologies that cause at least a partial CSF flow obstruction in the spinal subarachnoid space. As with syringomyelia associated with Chiari anomalies, the main goal of PSS surgery is to reestablish CSF flow across the area of obstruction. In addition to MRI, myelography with CT can be very helpful in the evaluation and management of these patients by identifying focal regions of CSF obstruction that may be amenable to surgical intervention. Future directions for the treatment of Chiari anomalies and syringomyelia include the application of advanced imaging techniques, more widespread use of genetic evaluation, large-scale outcome studies, and the further refinement of surgical technique.

Development of Common Data Elements for Use in Chiari Malformation Type I Clinical Research: An NIH/NINDS Project.

The management of Chiari I malformation (CMI) is controversial because treatment methods vary and treatment decisions rest on incomplete understanding of its complex symptom patterns, etiologies, and natural history. Validity of studies that attempt to compare treatment of CMI has been limited because of variable terminology and methods used to describe study subjects. The goal of this project was to standardize terminology and methods by developing a comprehensive set of Common Data Elements (CDEs), data definitions, case report forms (CRFs), and outcome measure recommendations for use in CMI clinical research, as part of the CDE project at the National Institute of Neurological Disorders and Stroke (NINDS) of the US National Institutes of Health. A working group, comprising over 30 experts, developed and identified CDEs, template CRFs, data dictionaries, and guidelines to aid investigators starting and conducting CMI clinical research studies. The recommendations were compiled, internally reviewed, and posted online for external public comment. In October 2016, version 1.0 of the CMI CDE recommendations became available on the NINDS CDE website. The recommendations span these domains: Core Demographics/Epidemiology; Presentation/Symptoms; Co-Morbidities/Genetics; Imaging; Treatment; and Outcome. Widespread use of CDEs could facilitate CMI clinical research trial design, data sharing, retrospective analyses, and consistent data sharing between CMI investigators around the world. Updating of CDEs will be necessary to keep them relevant and applicable to evolving research goals for understanding CMI and its treatment.

Clinical Presentation and Alternative Diagnoses in the Adult Population.

This article describes the presentation of tussive headaches in the adult population. Posterior headaches can also occur in patients with basilar invagination, and they may require occipital cervical fusion. Lower cranial nerve dysfunction is another common presenting symptom in adult Chiari patients. Almost 25% of symptomatic adult Chiari patients had a recent episode of trauma. Syringomyelia is not present in all Chiari patients possibly because of the involution of the central canal. Adults must also be evaluated for other causes of acquired Chiari malformations such as pseudotumor cerebri.

Johann Conrad Brunner (1653-1727) and the first description of syringomyelia.

INTRODUCTION: Johann Conrad Brunner (1653-1727) was a European anatomist and physician whose research concerning the pancreas and duodenum has made him a prominent figure in medicine. However, Brunner should also be recognized for his descriptions of syringomyelia, which were originally published in 1688 and included in the second edition of Theophilus Boneti's compendium of postmortem examinations, the Sepulchretum, which was published in 1700. CONCLUSIONS: Our current understanding of syringomyelia is based on the early observations of researchers such as Johann Conrad Brunner.

Posterior fossa reconstruction using titanium plate for the treatment of cerebellar ptosis after decompression for Chiari malformation.

OBJECTIVE: We describe our use of a perforated titanium plate to perform a partial posterior fossa cranioplasty in the treatment of cerebellar ptosis and dural ectasia after posterior fossa decompression (PFD). METHODS: Twelve patients who had undergone PFD underwent posterior fossa reconstruction using a titanium plate. Symptoms were related to either descent of the cerebellum into the decompression or to dural ectasia into the craniectomy defect. RESULTS: Twelve patients who had undergone large suboccipital craniectomies and who presented with persistent headaches and some with neurological symptoms related to syringomyelia, underwent reoperation with placement of a small titanium plate. Ten of 12 patients showed symptomatic improvement after reoperation. CONCLUSIONS: Placement of a titanium plate appears to be an effective method of treatment of cerebellar ptosis and dural ectasia after PFD for Chiari malformation.

Surgical treatment of Chiari malformation with and without syringomyelia: experience with 177 adult patients.

OBJECT: This study aims to show the relationship between clinical outcome in patients who underwent surgical decompression for Chiari malformation (CM) and postoperative imaging studies, with particular emphasis on the subarachnoid cisterns of the posterior fossa. METHODS: One hundred seventy-seven patients with CM, including 97 with syringomyelia, underwent posterior fossa decompressive surgery. Both the dura and arachnoid were opened in 150 of these patients, and 135 underwent reduction of the cerebellar tonsils. The patients' clinical signs and symptoms were evaluated at 2 time points after surgery. Their imaging studies were analyzed specifically for the size of the retrotonsillar and subtonsillar cisterns and the syringomyelic cavities. The authors evaluated the relationship between these imaging findings and clinical parameters. RESULTS: Clinical improvement correlated strongly with enlargement of the subarachnoid cisterns, and enlargement of the cisterns also correlated with reduction in size of the syrinx cavities. Symptoms related to syringomyelia responded to reduction in size of the syrinx cavities. CONCLUSIONS: Surgical decompression of the posterior fossa should aim to create relatively large subarachnoid cisterns and reduce the size of the syrinx cavity. Reduction of the cerebellar tonsils by surgical means, together with duraplasty, achieves this goal and thereby improves the clinical outcome for patients with CM. An incidental observation of the study is that obesity increases the likelihood of headache in patients with CM.

Idiopathic thoracic spinal cord herniation: report of 10 patients and description of surgical approach.

STUDY DESIGN: Ten patients with idiopathic spinal cord herniation were treated surgically to reduce the herniation. In 9 patients, a sling was passed between the spinal cord and the anterior dural hiatus. The results were analyzed with respect to the neurological symptoms and imaging. OBJECTIVE: The study calls attention to an unusual condition with similar clinical presentation and characteristic imaging findings in the hope of improving early recognition and treatment. We present the outcome of treating patients with a safe technique that avoids the risks of primary dural closure and of cerebrospinal fluid leakage. SUMMARY OF BACKGROUND DATA: Over 100 patients with this disorder have been reported in the literature. Treatment in the past has included potentially hazardous attempts at primary dural closure of the dural hiatus, and the contraindicated step of biopsy, or even resection of herniated tissue. Various different surgical approaches have been used and the technique described by us has proved to be safe and effective. METHOD: : Nine patients underwent treatment by a similar sling technique; 1 patient was treated by a 2-stage more complex approach, which we have since abandoned. We begin with a laminectomy over the suspected level of cord herniation, followed by dural opening. The dentate ligaments are sectioned and the dural defect may have to be enlarged to safely disengage the herniated cord. The cord is elevated by the dentate ligaments and a bovine pericardial sling is passed under the cord, thereby occluding the dural hiatus. The epidural cavity and site of herniation may be filled with a variety of material, and an expansile duraplasty is performed. RESULTS: Sensory symptoms, weakness and spasticity, among the most common findings, improved in approximately one third of our patients. A long time interval between the appearance of symptoms, correct diagnosis, and appropriate treatment may reduce the chance of significant recovery. Severe pain may be a poor prognostic sign. The dural hiatus was located in the upper thoracic region in all of our patients. CONCLUSIONS: Idiopathic spinal cord herniation presents with symptoms and signs of spinal cord dysfunction and a very characteristic imaging appearance. The condition can be treated safely, with the expectation of neurological stabilization and some improvement. The technique described minimizes cord manipulation. Postoperative imaging often reflects the long-standing incarceration of the spinal cord.

Curriculum-based neurosurgery digital library.

BACKGROUND: Recent work-hour restrictions and the constantly evolving body of knowledge are challenging the current ways of teaching neurosurgery residents. OBJECTIVE: To develop a curriculum-based digital library of multimedia content to face the challenges in neurosurgery education. METHOD: We used the residency program curriculum developed by the Congress of Neurological Surgeons to structure the library and Microsoft Sharepoint as the user interface. RESULTS: This project led to the creation of a user-friendly and searchable digital library that could be accessed remotely and throughout the hospital, including the operating rooms. CONCLUSION: The electronic format allows standardization of the content and transformation of the operating room into a classroom. This in turn facilitates the implementation of a curriculum within the training program and improves teaching efficiency. Future work will focus on evaluating the efficacy of the library as a teaching tool for residents.

Thecal shunt placement for treatment of obstructive primary syringomyelia.

OBJECT: The most commonly reported treatment of primary syringomyelia has been laminectomy with duraplasty or direct shunting from the syrinx cavity. Diversion of cerebrospinal fluid (CSF) from the spinal subarachnoid space to peritoneal, atrial, or pleural cavities has been described previously in only a few case reports. Shunting of the CSF from the subarachnoid space rostral to the level of myelographic blockage may reduce the filling force of the syrinx cavity and avoids myelotomy and manipulation of the spinal cord parenchyma. The authors report on 7 patients who underwent thecal shunt placement for primary spinal syringomyelia. METHODS: This study is a retrospective review of a consecutive series. The authors reviewed the medical records and neuroimaging studies of 7 adult patients with posttraumatic, postsurgical, or postinflammatory syringomyelia treated with thecoperitoneal, thecopleural, or thecoatrial shunt placement at the University of California Los Angeles Medical Center. Myelographic evidence of partial or complete CSF flow obstruction was confirmed in the majority of patients. The mean duration of follow-up was 33 months (range 6-104 months). RESULTS: Six (86%) of 7 patients showed signs of clinical improvement, whereas 1 remained with stable clinical symptoms. Of the 6 patients with available postoperative imaging, each demonstrated a reduction in syrinx size. Three patients (43%) had > or = 1 complication, including shunt-induced cerebellar tonsillar descent in 1 patient and infections in 2. CONCLUSIONS: If laminectomy with duraplasty is not possible for the treatment of primary syringomyelia, placement of a thecoperitoneal shunt (or thecal shunt to another extrathecal cavity) should be considered. Although complications occurred in 3 of 7 patients, the complication rate was outweighed by a relatively high symptomatic and imaging improvement rate.

Esophageal injury associated with anterior cervical spine surgery.

BACKGROUND: Anterior cervical spinal surgery has been used to treat a variety of conditions including spondylosis, fracture, tumor, infection, trauma, and instability. Esophageal perforation, a rare and unusual complication of anterior cervical procedures, has been largely relegated to only incidental case reports with few large retrospective studies performed to determine true incidence, treatment, etiology, and outcome. METHODS: More than 3000 anterior cervical spine surgeries conducted over a 30-year period by 5 active practicing surgeons were reviewed. There were 3 cases of esophageal injury identified with subsequent critical evaluation to determine presentation, diagnosis, risk factors, management, and outcomes. In addition, incidence rates were calculated based on overall occurrence and antecedent risk factors. RESULTS: Two of the patients with esophageal injury had predisposing risk factors, including diverticula or cervical spine trauma. The third patient had no antecedent risk factors. Symptoms included axial spine pain, odynophagia, dysphagia, purulent spondylitis, and sepsis. Treatment consisted of one or more of the following: reoperation with exploration and repair, esophageal diversion, esophageal rest, antibiotic administration, and wound drainage. Functional outcomes were achieved in all cases with no deaths. CONCLUSIONS: Esophageal injury incidence based on overall occurrence in this study was 0.1%. Patients with no antecedent risk factors had an incidence of 0.03%. Our results compare favorably with those of the Cervical Spine Research Society survey from 1989, which predicted an incidence of 0.25% based on questionnaires filed by surgeons, representing 1 of only 2 reports that included more than 1000 patients.

Observations on spine deformity and syringomyelia.

OBJECTIVE: Spine deformities, notably scoliosis, are known to occur in conjunction with syringomyelia. This study aims to analyze the effect of laminectomies performed in the course of treatment of syringomyelia. It examines the incidence, severity, and type of spine deformity as it relates to the extent and location of laminectomies performed. METHODS: Records of 169 patients were analyzed for evidence of spinal deformity on imaging studies for the extent of the syringomyelic cavities and for previous surgical procedures on the spine. This analysis included patients with syringomyelia related to Chiari malformation, as well as patients with primary spinal pathology. RESULTS: Spinal deformities were encountered in 41% of Chiari-syringomyelia patients who had not undergone previous surgery and in 57% of such patients who underwent reoperation. Scoliosis, the most common type of deformity encountered, was likely to be mild in patients who had not undergone previous surgery and severe in reoperated patients. Spine deformity was significantly more common in those patients who had more extensive bone removal. CONCLUSION: Complete laminectomy should be avoided whenever possible in patients with syringomyelia because local denervation of the axial musculature, added to loss of medial anterior horn cells from syringomyelia, favors the development of spine deformities. This is particularly true of laminectomies performed at the junctional areas of the spine, i.e., cervical-thoracic and thoracolumbar. Hemilaminectomy usually suffices for shunt placement; instrumented stabilization should be considered in patients undergoing full laminectomy, especially those considered to be at high risk of developing deformity.

Phenotypic definition of Chiari type I malformation coupled with high-density SNP genome screen shows significant evidence for linkage to regions on chromosomes 9 and 15.

Chiari type I malformation (CMI; OMIM 118420) is narrowly defined when the tonsils of the cerebellum extend below the foramen magnum, leading to a variety of neurological symptoms. It is widely thought that a small posterior fossa (PF) volume, relative to the total cranial volume leads to a cramped cerebellum and herniation of the tonsils into the top of the spinal column. In a collection of magnetic resonance imagings (MRIs) from affected individuals and their family members, we measured correlations between ten cranial morphologies and estimated their heritability in these families. Correlations between bones delineating the PF and significant heritability of PF volume (0.955, P = 0.003) support the cramped PF theory and a genetic basis for this condition. In a collection of 23 families with 71 affected individuals, we performed a genome wide linkage screen of over 10,000 SNPs across the genome to identify regions of linkage to CMI. Two-point LOD scores on chromosome 15 reached 3.3 and multipoint scores in this region identified a 13 cM region with LOD scores over 1 (15q21.1-22.3). This region contains a biologically plausible gene for CMI, fibrillin-1, which is a major gene in Marfan syndrome and has been linked to Shprintzen-Goldberg syndrome, of which CMI is a distinguishing characteristic. Multipoint LOD scores on chromosome 9 maximized at 3.05, identifying a 40 cM region with LOD scores over 1 (9q21.33-33.1) and a tighter region with multipoint LOD scores over 2 that was only 8.5 cM. This linkage evidence supports a genetic role in Chiari malformation and justifies further exploration with fine mapping and investigation of candidate genes in these regions.