Tag: Syringomyelia

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Single-stage correction of severe scoliosis with syringomyelia: performed with traction assistance without prophylactic neurosurgical decompression

J Neurosurg Spine 41:316–324, 2024

There is still controversy about whether it is necessary to perform prophylactic neurosurgical decompression for severe scoliosis (SS) with syringomyelia (SM) to reduce the risk of neurological complications during subsequent spinal correction. This study aimed to explore the safety and effectiveness of using traction-assisted single-stage spinal correction as a treatment for patients who had SS with SM (SS-SM).

METHODS The patients who had SS-SM without previous neurosurgical intervention and who underwent traction-assisted single-stage posterior spinal correction at a single center were included, and the initial, posttraction, and postoperative clinical data were reviewed. Based on preoperative MRI, the included patients were divided into two categories: those with versus those without Chiari malformation type I (CM-I–related SM [CS] vs idiopathic SM [IS]), and those with a moderate syrinx (MS) versus those with a large syrinx (LS). Different groups’ traction and operation contributions were calculated for comparisons (CS vs IS, MS vs LS).

RESULTS A total of 28 patients were included. The initial mean major scoliosis was 101.0° with a mean flexibility of 21.4%. After the operation, the mean total correction rate for scoliosis was 63.9%. The mean traction and operation contributions were 61.5% and 38.5%, respectively. Most of the patients (75%) underwent spinal corrections without 3-column osteotomies, and only 1 patient reported postoperative regional numbness without motor deficits. No differences were found in the mean total correction rates, traction, and operation contributions when comparing CS versus IS and MS versus LS with the comparable initial clinical data (p > 0.05). More than 50% of the total corrections were achieved by preoperative traction in all groups.

CONCLUSIONS Traction-assisted single-stage spinal correction can safely and effectively correct SS-SM without prophylactic neurosurgical decompression under strict patient selection. Additionally, traction can achieve more than half of the final spinal correction, even for patients with varying sizes of SMs.

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Familial Chiari malformation: a systematic review and illustrative cases

J Neurosurg Spine 41:105–114, 2024

Chiari malformations (CMs) are a group of congenital or acquired disorders characterized by hindbrain overcrowding into an underdeveloped posterior cranial fossa. CM is considered largely sporadic—however, there exists growing evidence of transmissible genetic underpinnings. The purpose of this systematic review of all familial studies of CM was to investigate the existence of an inherited component and provide recommendations to manage and monitor at-risk family members.

METHODS This paper includes the following: 1) a unique case report of dizygotic twins who presented at the Toronto Western Hospital Spinal Cord Clinic with symptomatic CM type 1 (CM-1) and syringomyelia; and 2) a systematic review of familial CM. The EMBASE and MEDLINE databases were searched on June 27, 2023, in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Only articles in the English language concerning the diagnosis of CM in > 1 human family member presented as a case study, case series, or literature review were included.

RESULTS Among the 29 articles included in the final analysis, a total of 34 families with CM were analyzed. An average of 3 cases of CM were found per family among all generations. Eighty-one cases (88%) reported CM-1, whereas the other 11 (12%) cases reported either CM-0, CM-1.5, or tonsillar ectopia. A syrinx was present in 37 (54%) cases, with 14 (38%) of these patients also reporting a skeletal abnormality, the most common comorbidity. Most family members diagnosed with CM were siblings (18; 35%), followed by monozygotic twins/triplets (12; 23%).

CONCLUSIONS Patients most often presented with headaches, sensory disturbances, or generalized symptoms. Overall, there exists mounting evidence for a hereditary component of CM. It is unlikely to be explained by a classic mendelian inheritance pattern, but is rather a polygenic architecture influenced by variable penetrance, cosegregation, and entirely nongenetic factors. For first-degree relatives of those affected by CM, the authors’ findings may influence clinicians to conduct closer clinical and radiographic monitoring, promote patient education, and consider earlier genetic testing.

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Syringopleural shunt for refractory syringomyelia

Acta Neurochirurgica (2023) 165:3039–3043

Surgical treatment of syringomyelia isdirected at the reconstruction of the subarachnoid space and restoration normal cerebrospinal fluid flow. Direct intervention on the syrinx is a rescue procedure and should be offered to patients with refractory syringomyelia.

Methods We provide an overview on indications and technique of syringopleural shunt (SPS). The procedure involves the connection of syrinx with the pleural space using a lumboperitoneal shunt. The occurrence of a negative pressure inside the pleural compartment offers an appropriate gradient for drainage from the syrinx.

Conclusions The SPS allows for a safe and effective treatment of persistent syringomyelia when management of the underlying cause does not yield substantial improvement.

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Dura Management Strategies in the Surgical Treatment of Adult Chiari Type I Malformation

Operative Neurosurgery 23:304–311, 2022

Symptomatic Chiari I malformation is treated with suboccipital decompression and C1 laminectomy. However, whether the dura should be opened (durotomy) or enlarged with a graft (duraplasty) remains unclear.

OBJECTIVE: To compare outcomes in adult Chiari I malformation patients treated with duraplasty, durotomy, or without dural opening (“mini-decompression”).

METHODS: A retrospective, multicenter, population-based cohort study was performed of all adult patients surgically treated for a Chiari I malformation at 3 regional neurosurgical centers between 2005 and 2017. Three different dura management strategies were favored by the participating hospitals, with data stratified accordingly. The primary outcome was measured using the Chicago Chiari Outcome Scale (CCOS), dichotomized into favorable (CCOS ≥13) or unfavorable (CCOS ≤12). Propensity score matching was used to adjust for potential confounders in outcome comparisons.

RESULTS: In total, 318 patients were included, of whom 52% were treated with duraplasty, 37% with durotomy, and 11% with mini-decompression. In total, 285 (90%) showed a favorable surgical outcome (CCOS ≥13). Duraplasty was associated with more favorable CCOS and shorter hospital stay compared with durotomy, both in unadjusted (93% vs 84%. P = .018 and 6.0 vs 8.0 days, P < .001) and adjusted analyses (92% vs 84%, P = .044 and 6.0 vs 8.0 days, P < .001). Mini-decompression was excluded from the adjusted analyses because of its small sample size.

CONCLUSION: In this study of adult Chiari I malformation, posterior fossa decompression with duraplasty was associated with more favorable postoperative outcome, as determined by the CCOS, compared with posterior fossa decompression with durotomy alone.

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Posterior cranial fossa and cervical spine morphometric abnormalities in symptomatic Chiari type 0 and Chiari type 1 malformation patients with and without syringomyelia

Acta Neurochirurgica (2021) 163:3051–3064

To better understand how anatomical features of Chiari malformation type 0 (CM0) result in the manifestation of Chiari malformation type 1 (CM1) signs and symptoms, we conducted a morphometric study of the posterior cranial fossa (PCF) and cervical canal in patients with CM1 and CM0.

Methods This retrospective study had a STROBE design and included 120 adult patients with MRI evidence of a small PCF (SPCF), typical clinical symptoms of CM1, and a diagnosis of CM1, CM0, or SPCF-TH0-only (SPCF with cerebellar ectopia less than 2 mm and without syringomyelia). Patients were divided by MRI findings into 4 groups: SPCF-TH0-only, SPCF-TH0-syr (CM0 with SPCF and syringomyelia), SPCF-CM1-only (SPCF with cerebellar ectopia 5 mm or more without syringomyelia), and SPCF-CM1-syr (CM1 with syringomyelia). Neurological examination data and MRI parameters were analyzed.

Results All patient cohorts had morphometric evidence of a small, flattened, and overcrowded PCF. The PCF phenotype of the SPCF-TH0-only group differed from that of other CM cohorts in that the length of clivus and supraocciput and the height of the PF were longer, the upper CSF spaces of PCF were taller, and the area of the foramen magnum was smaller. The SPCF-TH0 groups had a more significant narrowing of the superior cervical canal and a smaller decrease in PCF height than the SPCF-CM1 groups.

Conclusions Patients with SPCF-TH0 with and without syringomyelia developed Chiari 1 symptoms and signs. Patients with SPCF-TH0-syr (Chiari 0) had more constriction of their CSF pathways in and around the foramen magnum than patients with SPCF-TH0-only.

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Cerebrospinal fluid area and syringogenesis in Chiari malformation type I

J Neurosurg 134:825–830, 2021

Syringogenesis in Chiari malformation type I (CM-I) is thought to occur secondary to impaction of the cerebellar tonsils within the foramen magnum (FM). However, the correlation between the CSF area and syringogenesis has yet to be elucidated. The authors sought to determine whether the diminution in subarachnoid space is associated with syringogenesis. Further, the authors sought to determine if syrinx resolution was associated with the degree of expansion of subarachnoid spaces after surgery.

METHODS The authors performed a retrospective review of all patients undergoing posterior fossa decompression for CM-I from 2004 to 2016 at the University of Virginia Health System. The subarachnoid spaces at the FM and at the level of the most severe stenosis were measured before and after surgery by manual delineation of the canal and neural tissue area on MRI and verified through automated CSF intensity measurements. Imaging and clinical outcomes were then compared.

RESULTS Of 68 patients, 26 had a syrinx at presentation. Syrinx patients had significantly less subarachnoid space at the FM (13% vs 19%, p = 0.0070) compared to those without syrinx. Following matching based on degree of tonsillar herniation and age, the subarachnoid space was significantly smaller in patients with a syrinx (12% vs 19%, p = 0.0015). Syrinx resolution was associated with an increase in patients’ subarachnoid space after surgery compared with those patients without resolution (23% vs 10%, p = 0.0323).

CONCLUSIONS Syrinx development in CM-I patients is correlated with the degree to which the subarachnoid CSF spaces are diminished at the cranial outlet. Successful syrinx reduction is associated with the degree to which the subarachnoid spaces are increased following surgery.

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

Acta Neurochirurgica (2021) 163:219–225

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.

Method Arachnoid samples of 162 consecutive foramen magnum decompressions from 2006 to 2016 were studied. Arachnoid thickness and degrees of fibrosis and cellularity were determined with the examiner blinded for clinical data. Based on 145 first time decompressions, a histological classification for arachnoid features was developed.

Results The arachnoid was thicker in secondary compared with primary decompressions (176.1 ± 158.2 μm vs. 35.9 ± 43.5 μm; p = 0.0026) and in adults compared to children (37.3 ± 45.3 μm vs. 21.8 ± 7.7 μm; p = 0.0007). In primary decompressions, arachnoid thickness, degrees of fibrosis, and cellularity followed a normal distribution with all features shifted significantly to higher grades in secondary decompressions. The histological classification correlatedwith the preoperative severity of gait ataxia, motor weakness, and sensory deficits, whereas it had no predictive power for postoperative short- or long-term results. By comparison, the intraoperative evaluation of arachnoid changes accounting for relationships between arachnoid and surrounding tissues showed higher correlations with preoperative symptoms and had significant predictive power for postoperative short- and long-term results.

Conclusions Histological changes of the arachnoid correlate with preoperative symptoms. Relationships between arachnoid and surrounding tissues show even higher correlations with predictive power for short- and long-term outcomes. These findings suggest a pathophysiological role for the arachnoid in Chiari I malformation.

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Syringomyelia Resolution Following Chiari Surgery: A Novel Scale for Communication and Research

Neurosurgery 88( 1) 2021: 131–139

The pathophysiological connection between Chiari malformation and syringomyelia is accepted. Debate remains, however, how can we best define changes in syringomyelia following surgery.

OBJECTIVE: To introduce a grading system focusing on syrinx reduction based on routinely and reproducible radiological information, and provide a suggestion of the application of this scale for prediction of patient’s prognoses.

METHODS: Data from 48 patients with Chiari malformation and syringomyelia were compiled. We calculated syrinx cross-sectional area by approximating an ellipse in the largest axial plane. We compared the percentage of reduction or enlargement following surgery. The percentage change was grouped into four grades: Grade 0 = Increasing size, grade I ≤ 50% reduction, grade II = 50% to 90% reduction, grade III ≥ 90% reduction.

RESULTS: A total of 89.6% of patients had syrinx improvement after surgery. A total of 5 patients were grade 0, 14 were grade I, 20 patients were grade II, and 9 patients met criteria for grade III. The mean postoperative syrinx area was 24.1 mm2 (0-169 mm2) with a mean syrinx reduction of 62.7%.

CONCLUSION: Radiological improvement of syringomyelia can be mathematically defined and standardized to assist in communication in outcome-based trials. Radiological resolution is expected most patients.

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Tonsillectomy with modified reconstruction of the cisterna magna with and without craniectomy for the treatment of adult Chiari malformation type I with syringomyelia

Acta Neurochirurgica (2020) 162:1585–1595

In light of the controversies regarding the surgical treatment of adult Chiari malformation type I (CM-I) with syringomyelia, a retrospective study was conducted to evaluate the safety and efficacy of tonsillectomy followed by modified reconstruction of the cisterna magna with or without craniectomy.

Methods Between 2008 and 2017, 78 adult CM-I patients (36 males and 42 females, mean age 40.6 years old) with syringomyelia were treated with posterior fossa decompression (PFD) with tonsillectomy and modified reconstruction of the cisterna magna. Patients were divided into two study groups: group A (n = 40) underwent cranioplasty with replacement of the bone flap; group B (n=38) underwent suboccipital craniectomy. Neurological outcomes were evaluated by traditional physician assessment (improved, unchanged, and worsened) and the Chicago Chiari Outcome Scale (CCOS). Syringomyelia outcomes were assessed radiologically.

Results The procedure was successfully performed in all patients, and restoration of normal cerebrospinal fluid (CSF) flow was confirmed by intraoperative ultrasonography. The median postoperative follow-up was 20.3 months (range 18– 60 months). Clinical improvement was evident in 66 (84.6%) patients, with no significant differences between the two groups (85.0% vs. 84.2%, P = 0.897). According to the CCOS, 36 patients (90.0%) in group A were labeled as “good” outcome, compared with that of 34 (86.8%) in group B (P = 0.734). Improvement of syringomyelia was also comparable between the groups, which was observed in 35 (87.5%) vs. 33 (86.8%) patients (P = 0.887). The postoperative overall (7.5% vs. 23.7%, P = 0.048) and CSF-related (2.5% vs. 18.4%, P = 0.027) complication rates were significantly lower in group A than group B.

Conclusions Tonsillectomy with modified reconstruction of the cisterna magna without craniectomy seems to be a safe and effective surgical option to treat adult CM-I patients with syringomyelia, though future well-powered prospective randomized studies are warranted to validate these findings.

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Chiari malformation type I and basilar invagination originating from atlantoaxial instability

Acta Neurochirurgica (2020) 162:1553–1563

Recently, a novel hypothesis has been proposed concerning the origin of craniovertebral junction (CVJ) abnormalities. Commonly found in patients with these entities, atlantoaxial instability has been suspected to cause both Chiari malformation type I and basilar invagination, which renders the tried and tested surgical decompression strategy ineffective. In turn, C1-2 fusion is proposed as a single solution for all CVJ abnormalities, and a revised definition of atlantoaxial instability sees patients both with and without radiographic evidence of instability undergo fusion, instead relying on the intraoperative assessment of the atlantoaxial joints to confirm instability.

Methods The authors conducted a comprehensive narrative review of literature and evidence covering this recently emerged hypothesis. The proposed pathomechanisms are discussed and contextualized with published literature.

Conclusion The existing evidence is evaluated for supporting or opposing sole posterior C1-2 fusion in patients with CVJ abnormalities and compared with reported outcomes for conventional surgical strategies such as posterior fossa decompression, occipitocervical fusion, and anterior decompression. At present, there is insufficient evidence supporting the hypothesis of atlantoaxial instability being the common progenitor for CVJ abnormalities. Abolishing tried and tested surgical procedures in favor of a single universal approach would thus be unwarranted.

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Chiari malformation and syringomyelia

J Neurosurg Spine 31:619–628, 2019

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.

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Origin of Syrinx Fluid in Syringomyelia: A Physiological Study

Neurosurgery 84:457–468, 2019

The origin of syrinx fluid is controversial.

OBJECTIVE: To elucidate themechanisms of syringomyelia associated with cerebrospinal fluid pathway obstruction and with intramedullary tumors, contrast transport from the spinal subarachnoid space (SAS) to syrinx was evaluated in syringomyelia patients.

METHODS: We prospectively studied patients with syringomyelia: 22 with Chiari I malformation and 16 with SAS obstruction-related syringomyelia before and 1 wk after surgery, and 9 with tumor-related syringomyelia before surgery only. Computed tomographymyelography quantified dye transport into the syrinx before and 0.5, 2, 4, 6, 8, 10, and 22 h after contrast injection by measuring contrast density in Hounsfield units (HU).

RESULTS: Before surgery, more contrast passed into the syrinx in Chiari I malformation related syringomyelia and spinal obstruction-related syringomyelia than in tumor-related syringomyelia, as measured by (1) maximum syrinx HU, (2) area under the syrinx concentration-time curve (HU AUC), (3) ratio of syrinx HU to subarachnoid cerebrospinal fluid (CSF; SAS) HU, and (4) AUC syrinx/AUC SAS. More contrast (AUC) accumulated in the syrinx and subarachnoid space before than after surgery.

CONCLUSION: Transparenchymal bulk flow of CSF from the subarachnoid space to syrinx occurs in Chiari I malformation-related syringomyelia and spinal obstruction-related syringomyelia. Before surgery, more subarachnoid contrast entered syringes associated with CSF pathway obstruction than with tumor, consistent with syrinx fluid originating from the subarachnoid space in Chiari I malformation and spinal obstruction-related syringomyelia and not from the subarachnoid space in tumor-related syringomyelia. Decompressive surgery opened subarachnoid CSF pathways and reduced contrast entry into syringes associated with CSF pathway obstruction.

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

Neurosurgery 81:217–229, 2017

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.

METHODS: Among 1519 patientswith spinal space occupying lesions, 130 patients demonstrated intradural arachnoid cysts. Neuroradiological and surgical features were reviewed and clinical data analyzed.

RESULTS: Twenty-one patients presented arachnoid cysts as a result of an inflammatory leptomeningeal reaction related to meningitis, subarachnoid hemorrhage, intrathecal injections, intradural surgery, or trauma, ie, secondary cysts. For the remaining 109 patients, no such history could be elucidated, ie, primary cysts. Forty-six percent of primary and 86% of secondary cysts were associated with syringomyelia. Patients presented after an average history of 53±88 months. Therewere 122 thoracic and 7 lumbar cysts plus 1 cervical cyst. Fifty-nine patients with primary and 15 patients with secondary cysts underwent laminotomies with complete or partial cyst resection and duraplasty. Mean follow-up was 57 ± 52 months. In the first postoperative year, profound improvements for primary cysts were noted, in contrast to marginal changes for secondary cysts. Progression-free survival for 10 years following surgery was determined as 83% for primary compared to 15% for secondary cysts. Despite differences in clinical presentation, progression-free survival was almost identical for patients with or without syringomyelia.

CONCLUSIONS: Complete or partial resection leads to favorable short- and long-term results for primary arachnoid cysts. For secondary cysts, surgery can only provide clinical stabilization for a limited time due to the often extensive arachnoiditis.

 

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Two distinct populations of Chiari I malformation based on presence or absence of posterior fossa crowdedness on magnetic resonance imaging

J Neurosurg 126:1934–1940, 2017

A subset of patients with Chiari I malformation demonstrate patent subarachnoid spaces around the cerebellum, indicating that reduced posterior fossa volume alone does not account for tonsillar descent. The authors distinguish two subsets of Chiari I malformation patients based on the degree of “posterior fossa crowdedness” on MRI.

METHODS Two of the coauthors independently reviewed the preoperative MR images of 49 patients with Chiari I malformation and categorized the posterior fossa as “spacious” or “crowded.” Volumetric analysis of posterior fossa structures was then performed using open-source DICOM software. The preoperative clinical and imaging features of the two groups were compared.

RESULTS The posterior fossae of 25 patients were classified as spacious and 20 as crowded by both readers; 4 were incongruent. The volumes of the posterior fossa compartment, posterior fossa tissue, and hindbrain (posterior fossa tissue including herniated tonsils) were statistically similar between the patients with spacious and crowed subtypes (p = 0.33, p = 0.17, p = 0.20, respectively). However, patients in the spacious and crowded subtypes demonstrated significant differences in the ratios of posterior fossa tissue to compartment volumes as well as hindbrain to compartment volumes (p = 0.001 and p = 0.0004, respectively). The average age at surgery was 29.2 ± 19.3 years (mean ± SD) and 21.9 ± 14.9 years for spacious and crowded subtypes, respectively (p = 0.08). Syringomyelia was more prevalent in the crowded subtype (50% vs 28%, p = 0.11).

CONCLUSIONS The authors’ study identifies two subtypes of Chiari I malformation, crowded and spacious, that can be distinguished by MRI appearance without volumetric analysis. Earlier age at surgery and presence of syringomyelia are more common in the crowded subtype. The presence of the spacious subtype suggests that crowdedness alone cannot explain the pathogenesis of Chiari I malformation in many patients, supporting the need for further investigation.

 

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Minimally invasive subpial tonsillectomy for Chiari I decompression

minimally-invasive-subpial-tonsillectomy-for-chiari-i-decompression

Acta Neurochir (2016) 158:1807–1811

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.

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Cerebellar and hindbrain motion in Chiari malformation with and without syringomyelia

Cerebellar and hindbrain motion in Chiari malformation with and without syringomyelia

J Neurosurg Spine 24:546–555, 2016

The pathogenesis of syringomyelia associated with Chiari malformation type I (CM-I) is unclear. Theories of pathogenesis suggest the cerebellar tonsils may obstruct CSF flow or alter pressure gradients, or their motion might act as a piston to increase CSF pressure in the spinal subarachnoid space. This study was performed to measure cerebellar tonsillar and hindbrain motion in CM-I and assess the potential contributions to syrinx formation.

Methods Sixty-four CM-I patients and 25 controls were retrospectively selected from a clinical database, and all subjects had undergone cardiac-gated cine balanced fast-field echo MRI. There were a total of 36 preoperative CM-I scans, which consisted of 15 patients with and 21 patients without syringomyelia. Nineteen patients underwent paired pre- and postoperative imaging. Anteroposterior (AP) and superoinferior (SI) movements of the tip of the cerebellar tonsils, obex, fastigium of the fourth ventricle, pontomedullary junction, and cervicomedullary junction were measured. The distance between the fastigium and tip of the tonsils was used to calculate tonsillar tissue strain.

Results CM-I patients had significantly greater cerebellar tonsillar motion in both the AP and SI directions than controls (AP +0.34 mm [+136%], p < 0.001; SI +0.49 mm [+163%], p < 0.001). This motion decreased after posterior fossa decompression (AP -0.20 mm [-33%], p = 0.001; SI -0.29 mm [-36%]; p < 0.001), but remained elevated above control levels (AP +56%, p = 0.021; SI +67%, p = 0.015). Similar trends were seen for all other tracked landmarks. There were no significant differences in the magnitude or timing of motion throughout the hindbrain between CM-I patients with and without syringomyelia. Increased tonsillar tissue strain correlated with Valsalva headaches (p = 0.03).

Conclusions Cerebellar tonsillar motion may be a potential marker of CM-I and may have use in tailoring surgical procedures. The lack of association with syringomyelia suggests that tonsillar motion alone is not the driver of syrinx formation. Tonsillar tissue strain may play a part in the pathophysiology of Valsalva headaches.

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Perioperative monitoring of pulsatile and static intracranial pressure in patients with Chiari malformation type 1

Natural history of Chiari malformation Type I following decision for conservative treatment

Acta Neurochir (2016) 158:341–347

Patients with Chiari malformation type 1 (CMI) often present with elevated pulsatile and static intracranial pressure (ICP). The preferred treatment of CMI, foramen magnum decompression (FMD), is assumed to normalise ICP and craniospinal pressure dissociation. In order to further explore the mechanisms behind FMD, the present study investigated whether or not pulsatile and static ICP normalises immediately after FMD.

Method The study included CMI patients undergoing FMD with perioperative ICP monitoring as a part of clinical management. The pulsatile and static ICP scores were retrieved from the department’s ICP database, and the clinical and radiological data from the patient records.

Results Eleven patients were included in the study. During the first 3 days following FMD, mixed model analysis revealed no significant time-dependent differences of preoperatively elevated either pulsatile (mean wave amplitude,MWA; p=0.85) and/or static (mean ICP, p = 0.90) ICP. Percentage of mean ICP >15 mmHg increased during days 2 and 3 after FMD. Two patients from the present series had to receive ventriculoperitoneal shunt after FMD in the early postoperative period.

Conclusions The present observations suggest that anatomical restoration of cerebrospinal fluid pathways by FMD does not lead to immediate normalisation of preoperatively altered pulsatile and static ICP in patients withCMI. This finding may explain persistent symptoms during the early period after FMD.

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Is atlantoaxial instability the cause of Chiari malformation? Outcome analysis of 65 patients treated by atlantoaxial fixation

Is atlantoaxial instability the cause of Chiari malformation?

J Neurosurg Spine 22:116–127, 2015

Understanding that atlantoaxial instability is the cause of Chiari malformation (CM), the author treated 65 patients using atlantoaxial stabilization. The results are analyzed.

Methods Cases of CM treated using atlantoaxial fixation during the period from January 2010 to November 2013 were reviewed and analyzed. Surgery was aimed at segmental arthrodesis.

Results The author treated 65 patients with CM in the defined study period. Fifty-five patients had associated syringomyelia. Forty-six patients had associated basilar invagination. Thirty-seven patients had both basilar invagination and syringomyelia. Three patients had been treated earlier using foramen magnum decompression and duraplasty. According to the extent of their functional capabilities, patients were divided into 5 clinical grades. On the basis of the type of facetal alignment and atlantoaxial instability, the patients were divided into 3 groups. Type I dislocation (17 patients) was anterior atlantoaxial instability wherein the facet of the atlas was dislocated anterior to the facet of the axis. Type II dislocation (31 patients) was posterior atlantoaxial instability wherein the facet of the atlas was dislocated posterior to the facet of the axis. Type III dislocation (17 patients) was the absence of demonstrable facetal malalignment and was labeled as “central” atlantoaxial dislocation. In 18 patients, dynamic images showed vertical, mobile and at-least partially reducible atlantoaxial dislocation. All patients were treated with atlantoaxial plate and screw fixation using techniques described in 1994 and 2004. Foramen magnum decompression or syrinx manipulation was not performed in any patient. Occipital bone and subaxial spinal elements were not included in the fixation construct. One patient died, and death occurred in the immediate postoperative phase and was related to a vertebral artery injury incurred during the operation. One patient had persistent symptoms. In the rest of the patients there was gratifying clinical improvement. More remarkably, in 7 patients, the symptoms of lower cranial nerve paresis improved. No patient worsened in their neurological function after surgery. Reductions in the size of the syrinx and regression of the CM were observed in 6 of 11 cases in which postoperative MRI was possible. During the follow-up period, there was no delayed worsening of neurological function or symptoms in any patient. Sixty-three patients improved after surgery, and the improvement was sustained during the average follow-up period of 18 months.

Conclusions On the basis of outcomes in this study, it appears that the pathogenesis of CM with or without associated basilar invagination and/or syringomyelia is primarily related to atlantoaxial instability. The data suggest that the surgical treatment in these cases should be directed toward atlantoaxial stabilization and segmental arthrodesis. Except in cases in which there is assimilation of the atlas, inclusion of the occipital bone is neither indicated nor provides optimum stability. Foramen magnum decompression is not necessary and may be counter-effective in the long run.

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Syrinx resolution is correlated with the upward shifting of cerebellar tonsil following posterior fossa decompression in pediatric patients with Chiari malformation type I

Sirinx resolution

Eur Spine J (2015) 24:155–161

Chiari malformation type I (CMI) is characterized by deformed hindbrain. This study aimed to quantitatively evaluate the alterations in position of hindbrain after Posterior fossa decompression (PFD), and to identify the factors associated with syrinx resolution in pediatric patients with CMI.

Methods Eighty-seven patients, aged from 5 to 18 years, who underwent PFD for CMI between September 2006 and September 2012 were retrospectively reviewed. On midsagittal MR images, the position of medulla oblongata and cerebellum was quantitatively evaluated preoperatively and at follow-up. The maximal syrinx/cord (S/C) ratio and syrinx length were also measured. Significant improvement of syrinx was defined as a more than 20 % decrease in maximal S/C ratio or length on follow-up MRI.

Results Neurological deficits were found in 51 of the 87 patients preoperatively and 37 (72.4 %) of them obtained improvement of their symptoms at the last visit. Overall, upward shifting of the tip of cerebellar tonsil was observed in 66 (75.9 %) patients at the last follow-up. Moreover, the mean longitudinal distance of the tip of cerebellar tonsil changed from 16.47 ± 5.00 to 13.89 ± 4.38 mm (P<0.001) at final follow-up. Significant syrinx resolution was noticed in 79 (90.8 %) cases. Pointed cerebellar tonsils were found in 85 (97.7 %) of our patients preoperatively and 78 (91.8 %) of them acquired round cerebellar tonsils after PFD. The improvement of maximal S/C ratio was significantly correlated with upward shifting of the tip of cerebellar tonsil (P = 0.023).

Conclusions Following PFD for CMI, position and morphology of the cerebellar tonsil could revert to normal in most of the pediatric patients, and the upward shifting of the tip of cerebellar tonsil is significantly correlated with syrinx improvement. From this study, PFD without shunting may be effective for syrinx secondary to CMI in pediatric population.

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Increased body mass index in CM-I and syringomyelia

Increased body mass index in CM-I and syringomyelia

J Neurosurg 119:1058–1067, 2013

In this paper the authors describe an association between increased body mass index (BMI) and Chiari malformation Type I (CM-I) in adults, as well as its relationship to the development of syringomyelia.

Methods. In the period between January 2004 and December 2011, the senior author reviewed the data for all CM-I patients with or without syringomyelia and neurological deficit. Analyzed factors included clinical status (headaches and neurological signs), radiological characteristics of syringomyelia (diameter and vertical extent of syrinx), BMI, and relationship of age to BMI, syrinx diameter, and vertical extent of syrinx.

Results. Sixty consecutive adults had CM-I, 26 of whom also had syringomyelia. The mean BMI among all patients was 30.35 ± 7.65, which is Class I obesity (WHO), and was similar among patients with or without syringomyelia. Extension of the vertical syrinx was greater in overweight patients (p = 0.027) than in those with a normal body weight. Evidence of de novo syrinx formation was found in 2 patients who gained an average BMI of 10.8 points. After repeated decompression and no change in holocord syrinx width or vertical extent, a reduction in the syrinx was seen after BMI decreased 11.7 points in one individual. No correlation was found between patient age and BMI, age and vertical extension of the syrinx, and age and diameter of the syrinx.

Conclusions. An association between increased BMI and CM-I in adults was recognized. Gaining weight may influence the de novo creation of a syrinx in adults who previously had minimally symptomatic or asymptomatic CM-I, and reducing weight can improve a syrinx after unsuccessful surgical decompression. Therefore, a reduction in body weight should be recommended for all overweight and obese patients with CM-I.

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