Surgery for basilar invagination with and without Chiari I malformation.

Objective: The surgical treatment for Chiari I malformation and basilar invagination has been discussed with great controversy in recent years. This paper presents a treatment algorithm for these disorders based on radiological features, intraoperative findings, and analyses of long-term outcomes.

Methods: Eight-five operations for 82 patients (mean ± SD age 40 ± 18 years; range 9-75 years) with basilar invagination were evaluated, with a mean follow-up of 57 ± 55 months.

Relevance of C1/2 Facet Configurations and Clivus-Canal-Angles for Adult Patients with Chiari I Malformation with and without Basilar Invagination.

Objective: C1/2 facet configurations and clivus-canal-angles (CXAs) have been proposed as criteria for posterior fusion in Chiari I malformation (CMI).

Methods: Three-hundred and forty adults with CMI without basilar invagination (BI), 111 with CMI with BI, and 100 age- and sex-matched controls were studied using sagittal T2-weighted magnetic resonance imaging scans analyzing preoperative and postoperative values with their impact on progression-free survival rates.

Results: For CMI without BI, C1/2 facet configurations and CXA were similar to controls (142 ± 11 degrees and 144 ± 10 degrees, respectively) with low rates for posterior C1 displacements (7.

Diagnosis and treatment of Chiari malformation and syringomyelia in adults: international consensus document.

Background: Syringomyelia and Chiari malformation are classified as rare diseases on Orphanet, but international guidelines on diagnostic criteria and case definition are missing.

Aim Of The Study: to reach a consensus among international experts on controversial issues in diagnosis and treatment of Chiari 1 malformation and syringomyelia in adults.

Methods: A multidisciplinary panel of the Chiari and Syringomyelia Consortium (4 neurosurgeons, 2 neurologists, 1 neuroradiologist, 1 pediatric neurologist) appointed an international Jury of experts to elaborate a consensus document.

Histological and intraoperative evaluations of the arachnoid in patients with Chiari I malformation.

Background: Arachnoid dissection for decompression of Chiari I malformation is controversial. Whether arachnoid changes have an impact on the clinical course is not established. This paper documents the histological spectrum of arachnoid changes and evaluates correlations with preoperative, intraoperative, and postoperative data.

Long-term course of anterior spinal cord herniation presenting with an upper motor neuron syndrome: case report illustrating diagnostic and therapeutic implications.

Background: Anterior spinal cord herniation (aSCH) is a rare cause of myelopathy which may present as pure motor syndrome and mimic other degenerative diseases of the spinal cord. In slowly progressive cases, diagnosis may be impeded by equivocal imaging results and mistaken for evolving upper motor neuron disease. As early imaging studies are lacking, we aimed to provide a detailed description of imaging and neurophysiology findings in a patient with aSCH, focusing on the early symptomatic stages.

How Should Syringomyelia be Defined and Diagnosed?

Objective: Syringomyelia is considered as a fluid-filled cavitation inside the spinal cord. However, there is no agreement whether a dilated central canal should be included under this heading or how glioependymal cysts, myelomalacias, or cystic tumors should be distinguished from syringomyelia. This article provides a definition of syringomyelia and guidelines for its diagnosis.

A New Classification for Pathologies of Spinal Meninges-Part 2: Primary and Secondary Intradural Arachnoid Cysts.

Background: Spinal intradural arachnoid cysts are rare causes of radiculopathy or myelopathy. Treatment options include resection, fenestration, or cyst drainage.

Objective: To classify intradural spinal arachnoid cysts and present results of their treatment.

A New Classification for Pathologies of Spinal Meninges, Part 1: Dural Cysts, Dissections, and Ectasias.

Background: The clinical significance of pathologies of the spinal dura is often unclear and their management controversial.

Objective: To classify spinal dural pathologies analogous to vascular aneurysms, present their symptoms and surgical results.

Methods: Among 1519 patients with spinal space-occupying lesions, 66 patients demonstrated dural pathologies.

Surgical treatment of multilevel cervical spondylosis in patients with or without a history of syringomyelia.

Purpose: Cervical spondylotic myelopathy (CSM) is the commonest spinal cord disease in adults. This paper compares patients who developed CSM after successful treatment of syringomyelia to those with CSM exclusively related to degenerative spinal disease.

Methods: In this prospective study, 70 consecutive patients with CSM and spondylotic changes in at least three levels underwent 73 operations between 2005 and 2015 (mean follow-up: 39 ± 36 months).

Spinal ependymomas. Part 2: Ependymomas of the filum terminale.

OBJECT Ependymomas of the filum terminale provide specific surgical challenges due to their often enormous size, contact with nerve roots of the cauda equina and conus, and potential for subarachnoid dissemination. This study presents treatment results for these tumors over a 30-year period. METHODS Among 1447 patients with tumors of the spinal canal treated between 1980 and 2014, 618 patients presented with extramedullary tumors.

Spinal ependymomas. Part 1: Intramedullary ependymomas.

OBJECT Ependymomas represent the most common intramedullary tumor in adults. Despite their usually well-defined dissection plane, surgical morbidity has been documented to be considerably higher compared with other intramedullary entities. This study presents an analysis of risk factors for surgical morbidity and data on long-term results for intramedullary ependymomas.

Chiari I malformation with and without basilar invagination: a comparative study.

Object: Chiari I malformation is the most common craniocervical malformation. Its combination with basilar invagination in a significant proportion of patients is well established. This study presents surgical results for patients with Chiari I malformation with and without additional basilar invagination.

Treatment of basilar invagination.

Purpose: Basilar invagination is a rare craniocervical malformation which may lead to neurological deficits related to compression of brainstem and upper cervical cord as well as instability of the craniocervical junction. This study presents results of a treatment algorithm developed over a 20-year period focussing on anatomical findings, short-term and long-term outcomes.

Methods: 69 patients with basilar invagination (mean age 41 ± 18 years, history 64 ± 85 months) were encountered.

Treatment of intramedullary tumors: analysis of surgical morbidity and long-term results.

Object: Surgery of intramedullary tumors is established as the treatment of choice for these challenging lesions. This study presents a detailed analysis of risk factors for surgical morbidity and data on long-term results for intramedullary tumors.

Methods: Among 1317 patients with tumors of the spinal canal treated between 1980 and 2012, 278 patients with intramedullary tumors are presented.

Treatment of syringomyelia related to nontraumatic arachnoid pathologies of the spinal canal.

Background: Disturbances of cerebrospinal fluid (CSF) flow are the commonest cause of syringomyelia. Spinal arachnopathies may lead to CSF flow obstructions but are difficult to diagnose. Consequently, associated syringomyelias are often categorized as idiopathic.

Neurological deterioration after foramen magnum decompression for Chiari malformation type I: old or new pathology?

Object: Decompression of the foramen magnum is widely accepted as the procedure of choice for patients with Chiari malformation Type I (CM-I). This study was undertaken to determine the mechanisms responsible for neurological deterioration after foramen magnum decompression and the results of secondary interventions.

Methods: Between 1987 and 2010, 559 patients with CM-I presented, 107 of whom had already undergone a foramen magnum decompression, which included a syrinx shunt in 27 patients.

Treatment of posttraumatic syringomyelia.

Object: This paper presents results of a prospective study for patients undergoing surgery for posttraumatic syringomyelia between 1991 and 2010.

Methods: A group of 137 patients with posttraumatic syringomyelia were evaluated (mean age 45 ± 13 years, mean follow-up 51 ± 51 months) with pre- and postoperative MRI and clinical examinations presenting in this period and followed prospectively by outpatient visits and questionnaires. Surgery was recommended for symptomatic patients with a progressive course.

Surgical treatment of Chiari I malformation--analysis of intraoperative findings, complications, and outcome for 371 foramen magnum decompressions.

Background: Foramen magnum decompression is widely accepted as the treatment of choice for Chiari I malformation. However, important surgical details of the procedure are controversial.

Objective: This study analyzes 371 decompressions focusing on intraoperative findings, analysis of complications, and long-term outcomes.

Tethered cord syndrome in adults.

Object: The treatment of tethered cord syndromes in adults is discussed regarding the natural history and surgical indications. The author analyzes data obtained in patients who were diagnosed with a tethered cord in adulthood and either underwent surgical or conservative therapy between 1991 and 2009.

Methods: Since 1991, data obtained in 2515 patients with spinal cord pathologies were entered into the spinal cord database, and prospective follow-up was performed through outpatient visits and questionnaires.

Syringomyelia associated with foramen magnum arachnoiditis.

Object: Syringomyelia is often linked to pathological lesions of the foramen magnum. The most common cause is hindbrain herniation, usually referred to as Chiari I or II malformation. Foramen magnum arachnoiditis without either Chiari I or II malformation is a rare cause of syringomyelia.