Surgical Management of Chiari Malformation Type I and Instability of the Craniocervical Junction Based on Its Pathogenesis and Classification.

We investigated the mechanism underlying Chiari malformation type I (CM-I) and classified it according to the morphometric analyses of posterior cranial fossa (PCF) and craniocervical junction (CCJ). Three independent subtypes of CM-I were confirmed (CM-I types A, B, and C) for 484 cases and 150 normal volunteers by multiple analyses. CM-I type A had normal volume of PCF (VPCF) and occipital bone size. Type B had normal VPCF and small volume of the area surrounding the foramen magnum (VAFM) and occipital bone size. Type C had small VPCF, VAFM, and occipital bone size. Morphometric analyses during craniocervical traction test demonstrated instability of CCJ. Foramen magnum decompression (FMD) was performed in 302 cases. Expansive suboccipital cranioplasty (ESCP) was performed in 102 cases. Craniocervical posterolateral fixation (CCF) was performed for CCJ instability in 70 cases. Both ESCP and FMD showed a high improvement rate of neurological symptoms and signs (84.4%) and a high recovery rate of the Japanese Orthopaedic Association (JOA) score (58.5%). CCF also showed a high recovery rate of the JOA score (69.7%), with successful joint stabilization (84.3%). CM-I type A was associated with other mechanisms that caused ptosis of the brainstem and cerebellum (CCJ instability and traction and pressure dissociation between the intracranial cavity and spinal canal cavity), whereas CM-I types B and C demonstrated underdevelopment of the occipital bone. For CM-I types B and C, PCF decompression should be performed, whereas for small VPCF, ESCP should be performed. CCF for CCJ instability (including CM-I type A) was safe and effective.

Safety analysis and complications of condylar screws in a single-surgeon series of 250 occipitocervical fusions.

OBJECTIVE: Condylar screw fixation is a rescue technique and an alternative to the conventional configuration of occipitocervical fusion. Condylar screws are utilized when previous surgical bone removal along the supraocciput has occurred which makes anchoring of a traditional barplate technically difficult or impossible. However, the challenging dissection of C0-1 necessary for condylar screw fixation and the concerns about possible complications have, thus far, prevented the acquisition of large surgical series utilizing occipital condylar screws. In the largest case series to date, this paper aims to evaluate the safety profile and complications of condylar screw fixation for occipitocervical fusion. METHODS: A retrospective safety and complication-based analysis of occipitocervical fusion via condylar screws fixation was performed. RESULTS: A total of 250 patients underwent occipitocervical fusions using 500 condylar screws between September 2012 and September 2018. No condylar screw pullouts, or vertebral artery impingements were observed in this series. The sacrifice of condylar veins during the dissection at C0-1 did not cause any venous stroke. Hypotrophic condyles were found in 36.4% (91 of the 250) cases and did not prevent the insertion of condylar screws. Two transient hypoglossal deficits occurred at the beginning of this surgical series and were followed by recovery a few months later. Corrective strategies were effective in preventing further hypoglossal injuries. CONCLUSIONS: This surgical series suggests that the use of condylar screws fixation is a relatively safe and reliable option for OC fusion in both adult and pediatric patients. Methodical dissection of anatomical landmarks, intraoperative imaging, and neurophysiologic monitoring allowed the safe execution of the largest series of condylar screws reported to date. Separate contributions will follow in the future to provide details about the long-term clinical outcome of this series.

Pathogenesis and Classification of Chiari Malformation Type I Based on the Mechanism of Ptosis of the Brain Stem and Cerebellum: A Morphometric Study of the Posterior Cranial Fossa and Craniovertebral Junction.

Introduction We investigated the mechanism of ptosis of the brain stem and cerebellum (hindbrain) in Chiari malformation type I (CM-I) and classified CM-I according to pathogenesis, based on a morphometric study of the posterior cranial fossa (PCF) and craniovertebral junction (CVJ). We discuss the appropriate surgical treatment for hindbrain ptosis. Materials and Methods We examined 500 patients with CM-I and 100 healthy control individuals. We calculated the volume of the PCF (VPCF) and measured the axial length of the enchondral parts of the occipital bone and hindbrain. As statistical analyses, for the multiple analyses, heavy palindromic tests were used. Using three independent objective parameters, we tried to classify CM-I. Results Three independent subtypes were confirmed (CM-I types A, B, and C). CM-I type A (167 cases): normal VPCF, normal volume of the area surrounding the foramen magnum (VSFM), and normal occipital bone size; CM-I type B (178 cases): normal VPCF, small VSFM, and small occipital bone size; and CM-I type C (155 cases): small VPCF, small VSFM, and small occipital bone size. Conclusions Morphometric analyses of PCF and CVJ were very useful for the investigation of the mechanism of hindbrain ptosis and classifying CM-I according to pathogenesis. CM-I type A included mechanisms other than hindbrain ptosis, for example, CVJ instability, tethered cord, and increased intracranial pressure. CM-I types B and C demonstrated underdevelopment of the occipital bone. For CM-I types B and C, posterior decompression should be performed. For CM-I type A, appropriate surgical management should be selected.

Surgical Management of Chiari Malformations: Preliminary Results of Surgery According to the Mechanisms of Ptosis of the Brain Stem and Cerebellum.

Introduction We classified Chiari malformation type I (CM-I) according to the mechanism of ptosis of the brain stem and cerebellum, based on a morphometric study of the posterior cranial fossa (PCF) and craniovertebral junction (CVJ). Surgery was performed to manage the mechanism of the hindbrain ptosis. Materials and Methods We calculated the volume of the PCF (VPCF) and the area surrounding the foramen magnum (VSFM) and measured the axial length of the enchondral parts of the occipital bone (occipital bone size) and the hindbrain. According to these measures, we classified CM-I into type A (normal VPCF, normal VSFM, and normal occipital bone size), type B (normal VPCF, small VSFM, and small occipital bone size), and type C (small VPCF, small VSFM, and small occipital bone size). Foramen magnum decompression (FMD) (280 cases) was performed on CM-I types A and B. Expansive suboccipital cranioplasty (ESCP) was performed on CM-I type C. Posterior craniocervical fixation (CCF) was performed in cases with CVJ instability. Lysis of the adhesion and/or sectioning of the filum terminale were performed on cases with tethered cord syndrome. Results Both ESCP and FMD had a high rate of improvement of neurological symptoms (87%) and recovery rate. There was only small number of complications. CCF had a high rate of improvement of neurological symptoms (88%) and joint stabilization. Conclusion In the management of Chiari malformation, appropriate surgical methods that address ptosis of the hindbrain should be chosen. Each surgical approach resulted in a good improvement of neurological symptoms.

Posterior fossa decompression with duraplasty in Chiari malformation type 1: a systematic review and meta-analysis.

BACKGROUND: Surgery for symptomatic Chiari type I malformation (CM-I) patients include posterior fossa decompression (PFD) involving craniectomy with or without dural opening, and posterior fossa decompression with duraplasty (PFDD). This review aims to examine the evidence to aid surgical decision-making. METHODS: A medical database search was expanded to include article references to identify all relevant published case series. Animal studies, editorials, letters, and review articles were excluded. A systemic review and meta-analysis were performed to assess clinical and radiological improvement, complications, and reoperation rates. RESULTS: Seventeen articles, containing data on 3618 paediatric and adult participants, met the inclusion criteria. In the group, 5 papers included patients that had the dura left open. PFDD is associated with better clinical outcomes (RR 1.24, 95% CI, 1.07 to 1.44; P = 0.004), but has a higher complication rate (RR 4.51, 95% CI, 2.01 to 10.11; P = 0.0003). In adults, clinical outcomes differences did not reach statistical significance (P = 0.07) but re-operation rates were higher with PFD (RR 0.17, 95% CI 0.03 to 0.86; P = 0.03), whilst in children re-operation rates were no different (RR 0.97, 95% CI 0.41 to 2.30; P = 0.94). Patients with a syrinx did better with PFDD (P = 0.02). No significant differences were observed concerning radiological improvement. CONCLUSIONS: In the absence of hydrocephalus and craniocervical region instability, PFDD provides better clinical outcomes but with higher risk. The use of PFD may be justified in some cases in children, and in the absence of a syrinx. To help with future outcome assessments in patients with a CM-I, standardization of clinical and radiological grading systems are required. TRIAL REGISTRATION: not required.

Professional Profiles, Technical Preferences, Surgical Opinions, and Management of Clinical Scenarios from a Panel of 63 International Experts in the Field of Chiari I Malformation.

BACKGROUND: Chiari I Malformation (CMI) and the topics concerning it have been the subject of numerous discussions and polarizing controversies over the course of the past 20 years. METHODS: The opinions of 63 recognized international Neurosurgical CMI experts from 4 continents, with a collective surgical experience of more than 15,000 CMI cases, were gathered through a detailed questionnaire, divided in two parts: diagnostic and therapeutic. The therapeutic part was organized into four sections: Professional Profile, Technical Preferences, Surgical Opinions, and Clinical Scenarios. RESULTS: The data reflected a wide spectrum of opinions, approaches, and expertise. The second part of the questionnaire dealt with the surgical aspects of CMI care and painted a more complex picture: • 81% of the surgeons preferred the Intradural technique. • 88% of the experts agreed that CMI surgery is not indicated for minimal non-debilitating symptoms alone, or as prophylaxis. • In the face of given clinical scenarios, a wide spectrum of therapeutic approaches was chosen by the whole group, but the 4 Surgeons with the largest case series expressed the same opinion. • Eight out of 63 Surgeons had a surgical experience above 600 cases, were responsible for more than half of the total 15,000 declared CMI cases, and shared a similar profile in terms of technical surgical choices, therapeutic opinions, and low complication rate, with a marked preference for Intradural techniques and tonsillar manipulation. • Once large individual case series were accumulated, we did not see any differences in the opinions and preferences between Adult and Pediatric Neurosurgeons. CONCLUSION: Surgeons who have focused on CMI have been able to accumulate large surgical series, have chosen in their practices the more aggressive (and intrinsically more effective) CMI surgical techniques, and have achieved a low complication rate which compares favorably with that one of the extradural techniques.

Chiari I Malformation: Opinions on Diagnostic Trends and Controversies from a Panel of 63 International Experts.

BACKGROUND: Chiari I malformation (CMI) and the topics concerning it have been the subject of numerous discussions and polarizing controversies over the course of the last 20 years. METHODS: The opinions of 63 recognized international CMI experts from 4 continents, with a collective surgical experience of >15,000 CMI cases, were gathered through a detailed questionnaire. RESULTS: Three facts emerged from the analysis of the results: 1) Most of the replies showed a high level of consensus on most CMI-related topics. 2) Several topics, which had been considered controversial as recently as 10 years ago, are now more widely accepted. 3) The so-called 5-mm rule was rejected by 88.5% of the CMI experts who responded to the questionnaire. CONCLUSIONS: Sixty three recognized international CMI experts from 4 continents, with a collective surgical experience of >15,000 CMI cases were polled about a number of CMI topics. The results showed a high level of consensus, as well as a paradigm shift.

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.

Minimally invasive subpial tonsillectomy for Chiari I decompression.

BACKGROUND: A number of different surgical techniques have been used through the years to address Chiari I malformation (CMI). METHODS: This article describes how we surgically manage CMI at two high-volume centers. We call the technique the minimally invasive subpial tonsillectomy (MIST). The technique consists of a minimalistic dissection and craniectomy with a short, linear durotomy for the subpial tonsillar resection. The dura is closed without the use of a duraplasty. CONCLUSIONS: We describe our current methods of surgery for CMI.

Association of Chiari malformation type I and tethered cord syndrome: preliminary results of sectioning filum terminale.

OBJECTIVE: The pathogenesis of CM-I is incompletely understood. We describe an association of CM-I and TCS that occurs in a subset of patients with normal size of the PCF. METHODS: The prevalence of TCS was determined in a consecutively accrued cohort of 2987 patients with CM-I and 289 patients with low-lying cerebellar tonsils (LLCT). Findings in 74 children and 244 adults undergoing SFT were reviewed retrospectively. Posterior cranial fossa size and volume were measured using reconstructed 2D computed tomographic scans and MR images. Results were compared to those in 155 age- and sex-matched healthy control individuals and 280 patients with generic CM-I. The relationships of neural and osseus structures at the CCJ and TLJ were investigated morphometrically on MR images. Intraoperative CDU was used to measure anatomical structures and CSF flow in the lumbar theca. RESULTS: Tethered cord syndrome was present in 408 patients with CM-I (14%) and 182 patients with LLCT (63%). In 318 patients undergoing SFT, there were no significant differences in the size or volume of the PCF as compared to healthy control individuals. Morphometric measurements demonstrated elongation of the brain stem (mean, 8.3 mm; P < .001), downward displacement of the medulla (mean, 4.6 mm; P < .001), and normal position of the CMD except in very young patients. Compared to patients with generic CM-I, the FM was significantly enlarged (P < .001). The FT was typically thin and taut (mean transverse diameter, 0.8 mm). After SFT, the cut ends of the FT distracted widely (mean, 41.7 mm) and CSF flow in the lumbar theca increased from a mean of 0.7 cm/s to a mean of 3.7 cm/s (P < .001). Symptoms were improved or resolved in 69 children (93%) and 203 adults (83%) and unchanged in 5 children (7%) and 39 adults (16%) and, worse, in 2 adults (1%) over a follow-up period of 6 to 27 months (mean, 16.1 months +/- 4.6 SD). Magnetic resonance imaging 1 to 18 months after surgery (mean, 5.7 months +/- 3.8 SD) revealed upward migration of the CMD (mean, 5.1 mm, P < .001), ascent of the cerebellar tonsils (mean, 3.8 mm, P < .001), reduction of brain stem length (mean, 3.9 mm, P < .001), and improvement of scoliosis or syringomyelia in some cases. CONCLUSIONS: Chiari malformation type I/TCS appears to be a unique clinical entity that occurs as a continuum with LLCT/TCS and is distinguished from generic CM-I by enlargement of the FM and the absence of a small PCF. Distinctive features include elongation and downward displacement of the hindbrain, normal position of the CMD, tight FT, and reduced CSF flow in the lumbar theca. There is preliminary evidence that SFT can reverse moderate degrees of tonsillar ectopia and is appropriate treatment for cerebellar ptosis after Chiari surgery in this cohort.

Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and chiari malformation type I in patients with hereditary disorders of connective tissue.

OBJECT: Chiari malformation Type I (CM-I) is generally regarded as a disorder of the paraxial mesoderm. The authors report an association between CM-I and hereditary disorders of connective tissue (HDCT) that can present with lower brainstem symptoms attributable to occipitoatlantoaxial hypermobility and cranial settling. METHODS: The prevalence of HDCT was determined in a prospectively accrued cohort of 2813 patients with CM-I. All patients underwent a detailed medical and neuroradiological workup that included an assessment of articular mobility. Osseous structures composing the craniocervical junction were investigated morphometrically using reconstructed 3D computed tomography and plain x-ray images in 114 patients with HDCT/CM-I, and the results were compared with those obtained in patients with CM-I (55 cases) and healthy control individuals (55 cases). RESULTS: The diagnostic criteria for Ehlers-Danlos syndrome and related HDCT were met in 357 (12.7%) of the 2813 cases. Hereditability was generally compatible with a pattern of autosomal dominant transmission with variable expressivity. The diagnostic features of HDCT/CM-I were distinguished from those of CM-I by clinical stigmata of connective tissue disease, a greater female preponderance (8:1 compared with 3:1, p < 0.001), and a greater incidence of lower brainstem symptoms (0.41 compared with 0.11, p < 0.001), retroodontoid pannus formation (0.71 compared with 0.11, p < 0.001), and hypoplasia of the oropharynx (0.44 compared with 0.02, p < 0.001). Measurements of the basion-dens interval, basion-atlas interval, atlas-dens interval, dens-atlas interval, clivus-atlas angle, clivus-axis angle, and atlas-axis angle were the same in the supine and upright positions in healthy control individuals and patients with CM-I. In patients with HDCT/CM-I, there was a reduction of the basion-dens interval (3.6 mm, p < 0.001), an enlargement of the basion-atlas interval (3.0 mm, p < 0.001), and a reduction of the clivus-axis angle (10.8 degrees, p < 0.001), clivus-atlas angle (5.8 degrees, p < 0.001), and atlas-axis angle (5.3 degrees, p < 0.001) on assumption of the upright position. These changes were reducible by cervical traction or returning to the supine position. CONCLUSIONS: The identification of HDCT in 357 patients with CM-I establishes an association between two presumably unrelated mesodermal disorders. Morphometric evidence in this cohort-cranial settling, posterior gliding of the occipital condyles, and reduction of the clivus-axis angle, clivus-atlas angle, and atlas-axis angle in the upright position-suggests that hypermobility of the occipitoatlantal and atlantoaxial joints contributes to retroodontoid pannus formation and symptoms referable to basilar impression.

Tailored operative technique for Chiari type I malformation using intraoperative color Doppler ultrasonography.

OBJECTIVE: We describe an operative technique for Chiari Type I malformation that uses color Doppler ultrasonography as a guide for performing patient-specific posterior fossa decompressions. The technique has been used since 1999 in more than 300 operations. METHODS: On the basis of real-time anatomic and physiological measurements, the following goals of surgery were monitored: 1) adequate decompression of the cervicomedullary junction; 2) creation of a retrocerebellar space of 8 to 10 cm(3) volume; and 3) establishment of optimal cerebrospinal fluid flow between the cranial and spinal compartments. RESULTS: The size of the craniectomy was tailored to conform to the area of cerebellar impaction as demarcated by compressed subarachnoid spaces. A laminectomy was not performed unless the cerebellar tonsils were herniated below C1. Before opening the dura, color Doppler ultrasonography imaging was invaluable in planning operative strategies. A simple duraplasty without additional steps was found to be appropriate treatment in occasional patients with minimal tonsillar herniation (5-8 mm). In all other cases, it was necessary to perform an internal decompression that included lysis of the arachnoid and shrinkage of the cerebellar tonsils to achieve the goals of surgery. Optimal cerebrospinal fluid flow through the foramen magnum in anesthetized, prone patients was found to have the following characteristics: a peak velocity of 3 to 5 cm/s, bidirectional movement, and a waveform exhibiting vascular and respiratory variations. The attainment of surgical goals was confirmed in most patients by postoperative neuroimaging. CONCLUSION: Color Doppler ultrasonography imaging is an important technological advance that permits the neurosurgeon to tailor the steps of Chiari surgery according to patient-specific variables. The success of this technique depends on the mastery of a new and sophisticated monitoring modality.

Acute syringomyelia: case report.

OBJECTIVE AND IMPORTANCE: Syringomyelia is generally regarded as a chronic, slowly progressive disorder. We describe a case of acute dilation of the central canal of the spinal cord that presented with rapidly progressive segmental signs. CLINICAL PRESENTATION: A 29-year-old female patient who had previously undergone surgical treatment for a Chiari I malformation, syringomyelia, and hydrocephalus presented with an 8-day history of headaches, progressive paraparesis, and urinary retention. Magnetic resonance imaging scans demonstrated panventricular hydrocephalus in association with a holocord syrinx that extended to the obex. Magnetic resonance imaging scans that had been coincidentally obtained just 3 days before the onset of symptoms had revealed no evidence of hydrocephalus or syringomyelia. INTERVENTION: The patient underwent emergency revision of a failed ventriculoperitoneal shunt. Postoperatively, there was prompt resolution of the syringomyelia, hydrocephalus, and associated neurological deficits. CONCLUSION: Among patients with communicating syringomyelia, the central canal of the spinal cord participates as a "fifth ventricle" and can undergo rapid dilation in association with acute hydrocephalus. Appropriate treatment in such cases involves placement of a ventriculoperitoneal shunt.