Revision Chiari surgery in adults: surgical evaluation algorithm and outcomes in a continuity-of-care practice.

OBJECTIVE: Patients with a prior surgical history for Chiari malformation (CM) as a child or an adult who presents with a recurrence or persistence of symptoms pose a challenging clinical scenario. The authors analyzed preoperative presentations, surgical histories, and outcomes of adult CM revision cases to create an algorithm for the assessment of revision surgery in adult CM patients and contrast this with the care of pediatric CM patients within the context of an integrated continuity-of-care CM practice seeing pediatric and adult patients. METHODS: The authors retrospectively reviewed the records of patients with CM0, -1, and -1.5 who were 21 years of age or older, had a prior history of CM surgery as either children or adults, and underwent revision surgery as adults at the authors' institution from December 2009 to February 2020. The cohort was divided into three groups depending on the type of revision surgery performed: posterior fossa decompression with or without duraplasty (PFD/D group), occipitocervical fusion without ventral decompression (OCF group), and ventral decompression in the form of endoscopic endonasal odontoidectomy (VD group). RESULTS: A total of 50 adult revision cases were divided into 30 PFD/D cases, 13 OCF cases, and 7 VD cases. Forty cases had previously undergone one CM surgery, and 10 had undergone two. Preoperatively, the PFD/D group had significantly fewer cases of diagnosed craniocervical instability (CCI), Ehlers-Danlos syndrome, and dysautonomia than the OCF and VD groups. A retroflexed odontoid was significantly more common in the VD group than in the PFD/D and OCF groups. Postoperatively, rates of improvement were 60.0% with PFD/D, 84.6% with OCF, and 85.7% with VD, but differences in these rates were not statistically significant. CONCLUSIONS: In this challenging group of CM patients, possible etiologies warranting additional surgery included residual dorsal compression, persistent syrinx, CCI, and ventral brainstem compression from a significant retroflexed odontoid. Outcomes after revision were often favorable, but careful evaluation, patient selection, and communication with patients are needed in deciding to reoperate. The suggested algorithm can aid decision-making.

Surgical modeling of Chiari-like malformation in rats: Insights from canine morphology.

BACKGROUND: Chiari-like malformation in dogs and Chiari malformation type 1 in humans are conditions characterized by a relatively small caudal cranial fossa, leading to cerebellar herniation. This study aimed to develop a rat model of Chiari-like malformation using surgical techniques based on morphological characteristics observed in dogs. METHODS: Endocranial magnetic resonance images of both normal dogs and dogs diagnosed with Chiari-like malformation were retrospectively analyzed. Measurements of the caudal cranial fossa volume, rostral and medial fossa volume, and volume index were taken. The differences in caudal cranial fossa volume and volume index between normal dogs and those diagnosed with Chiari-like malformation were then utilized to create a rat model of Chiari-like malformation through surgical intervention. The measurements were conducted on both the rat Chiari-like malformation models and normal rats, with each measurement taken twice and the mean values calculated. RESULTS: Significant differences were found between normal dogs and dogs diagnosed with Chiari-like malformation in terms of the volume of the caudal cranial fossa (27.62% reduction) and the volume index (23.36% reduction) (p<0.05). These differences were used to develop a rat model, which also showed significant reductions in both caudal cranial fossa volume (29.52%) and volume index (28.30%) compared to normal rats (p<0.05). The condition in the rat model was confirmed through magnetic resonance imaging, which revealed cerebellar herniation into the foramen magnum. CONCLUSIONS: The study successfully established a rat model of Chiari-like malformation that accurately reproduces the morphological features observed in dogs. This model potentially serves as a valuable tool for investigating the pathological mechanisms and potential therapeutic approaches for Chiari-like malformation in veterinary medicine.

Vidian Canal in Chiari Type I Malformation: A Computed Tomography Study.

OBJECTIVE: To evaluate the position and dimension of the Vidian canal (VC) in Chiari type I malformation (CIM). MATERIALS AND METHODS: Radiologic views of 49 CIM (mean age: 23.58±15.62 y, sex: 23 males/26 females) and 51 healthy subjects (mean age: 42.50±20.12 y, sex: 21 males/30 females) were included in this computed tomography study. RESULTS: In comparison with controls, the VC angle and the distance of the round foramen to VC were greater in CIM, but VC length and the distances of the superior wall of the bony sphenoidal sinus, midsagittal plane, and vomerine crest to VC were smaller in CIM. Relative to the sphenoid bone, the position of VC in CIM was determined as type 1 (59.2%) >type 2 (28.6%) >type 3 (12.2%), whereas in controls as type 1 (54.9%) >type 3 (25.5%) >type 2 (19.6%). Relative to the medial pterygoid plate, the position of VC in CIM was determined as type A (63.3%) >type B (20.4%) >type C (16.3%), while in controls as type B (43.1%) >type A (40.2%) >type C (16.7%). CONCLUSION: VC size and position correlated with CIM. Compared with controls, CIM patients had more partially protruded VC into the bony sphenoidal sinus and more medially located VC according to the medial pterygoid plate.

Chiari I Deformity: Beyond 5 mm below the Foramen Magnum.

Although originally described as a consecutive series of pathologic changes, Chiari syndrome represents a spectrum of disease divided into two subsets: development deformities of the paraxial mesoderm manifesting after birth (types 0-1.5) and true congenital malformations due to failure of neural tube closure present in utero (types 2-5). Heterogeneity among patients with a Chiari deformity and incomplete understanding of its pathophysiologic characteristics have led to inconsistency in radiologic reporting and difficulty in defining appropriate management strategies tailored to an individual patient's condition. The radiologist is tasked with going beyond the criteria for cerebellar tonsillar herniation to define an individual patient's disease state, determine candidacy for surgery, and assist in selecting the proper surgical approach. In addition, the radiologist must be able to identify conditions that result in cerebellar tonsillar herniation that are not related to Chiari deformity to avoid inappropriate surgery. Last, the radiologist must be able to interpret postoperative imaging examinations to assess for adequacy of treatment and complications. The authors summarize recent literature regarding the pathophysiologic basis of Chiari 1 and related deformities and detail the ideal morphologic and physiologic imaging assessment, focusing on Chiari 1 and related deformities (Chiari 0, 0.5, and 1.5). Also discussed are surgical techniques and "pearls" of postsurgical imaging, including complications that must be recognized. This review provides clarity to a commonly encountered but less understood condition to optimize outcomes for patients with Chiari 1 and related deformities. ©RSNA, 2024 Supplemental material is available for this article. See the invited commentary by Huisman in this issue.

The Surgical Strategy Cerebrospinal Fluid Decompression Facilitates Outcomes of Adults with Chiari Malformation Type I: An Observational, Real-World, Single-Center Study of 528 Patients.

OBJECTIVE: We designed this study to introduce the surgical strategy cerebrospinal fluid (CSF) decompression in treatment of Chiari malformation type I and compared CSF decompression with other surgical strategies to provide a solid basis for patient counseling. METHODS: The study enrolled 528 consecutive patients with CMI who underwent surgical interventions from 2012 to 2022. The surgical strategy for these patients was bony and dural decompression, anatomical reduction of herniated tonsils, or CSF decompression. Short-term results were determined after 3 months; long-term outcomes were evaluated at last follow-up (at least 18 months). RESULTS: CSF decompression was independently associated with better long- or short-term primary outcomes than anatomical reduction of herniated tonsils or bony and dural decompression (P < 0.001). Compared with short-term, the long-term outcomes were better in patients who underwent CSF decompression (P = 0.035), but were worse in patients with bony and dural decompression (P = 0.03). Specific surgical techniques cannot affect the long- and short-term outcomes of patients with Chiari malformation type I. CSF decompression provided better long-term syringomyelia improvement than short-term (181/218, 83% vs. 169/218, 77.5%; P < 0.001). CONCLUSIONS: CSF decompression, but not a specific surgical technique or operative method, was associated with favorable neurological outcomes in ADULT patients with Chiari malformation type I. The surgical technique and operative method should be selected according to the characteristics of each patient and the intraoperative condition to normalize CSF circulation at the craniovertebral junction area. The intraoperative target, smooth CSF flow out from the fourth ventricle and in to the bilateral Luschka foramina, could be observed.

Transient Decrease in Cerebrospinal Fluid Motion Is Related to Cough-Associated Headache in Chiari I Malformation.

BACKGROUND: Short-lasting cough-associated headache (CAH) in patients with Chiari I malformation (CMI) is believed to be due to transient worsening of cerebrospinal flow (CSF) obstruction at the foramen magnum. We assessed changes in CSF flow in response to coughing in CMI patients with CAH and compared with those without CAH and healthy participants (HPs) using real-time magnetic resonance imaging. METHODS: Seventeen CMI patients (12 with CAH, 5 without CAH) and 6 HPs were prospectively assessed using real-time pencil-beam imaging magnetic resonance sequence. A 64-mm length pencil-beam imaging cylinder was placed at the craniocervical junction. CSF stroke volume (SVCSF) was assessed during resting, postcoughing, and relaxation phases via a 90-second scan. SVCSF was measured at 6 levels at 5-mm intervals between 10 and 35 mm below the foramen magnum. During each phase, SVCSF was compared between CMI with and without CAH and HPs and corrected for multiple comparisons. RESULTS: At multiple consecutive levels, postcoughing SVCSF was significantly lower in CMI with CAH compared with both CMI without CAH and HP (P < 0.05). No differences in postcoughing SVCSF were seen between CMI without CAH and HP. At rest or relaxation phase, no differences in SVCSF were seen between patients with and without CAH but minimal differences were seen between CMI with CAH and HP. CONCLUSIONS: A decrease in CSF flow after coughing in CMI patients with CAH supports the notion that CAH is caused by transient worsening of CSF flow obstruction at the foramen magnum.

Intracranial pulse pressure amplitude as an indicator of intracranial compliance: observations in symptomatic children with Chiari malformation type I.

OBJECTIVE: Reduced intracranial compliance (ICC) may be an important factor in the pathophysiology of Chiari malformation type I (CM-I). However, direct measurement of ICC is controversial because of its invasiveness, particularly in children. Instead, ICC may be estimated from continuous measurements of intracranial pressure (ICP), where the metric mean wave amplitude (MWA) has been found to be more useful as a surrogate marker of ICC than mean ICP. This observational study investigated the distribution of MWA and mean ICP in symptomatic children with CM-I, as well as their association with clinical and radiological findings. METHODS: From a consecutive series of children treated for CM-I at a single institution between 2006 and 2023, the authors analyzed ICP scores in those who underwent an overnight preoperative ICP recording in which MWA was calculated. Clinical and radiological data were retrieved from the patient records. RESULTS: Thirty-seven children (mean age 12.4 ± 3.6 years) with symptomatic CM-I were identified. From the overnight ICP measurements, the average MWA was 5.2 ± 1.3 mm Hg: 56% of children had an abnormal MWA (> 5 mm Hg) and 33% had a borderline MWA (4-5 mm Hg). In contrast, the average mean ICP was 9.7 ± 4.1 mm Hg: 8% of children had an abnormal mean ICP (> 15 mm Hg) and 41% had a borderline mean ICP (10-15 mm Hg). Thus, more children were found to have an abnormal MWA than an abnormal mean ICP (p < 0.001). MWA was significantly higher in the subgroup of children with medullary compression in the foramen magnum, as seen on MRI, than in those without (5.6 ± 1.0 mm Hg vs 4.7 ± 1.4 mm Hg, p = 0.03), whereas a similar difference was not observed for mean ICP (9.9 ± 4.6 mm Hg vs 9.7 ± 3.7 mm Hg, p = 0.889). CONCLUSIONS: In this cohort of symptomatic children with CM-I, MWA was more frequently abnormal than mean ICP, with a clinically significant discrepancy in half of the patients. Moreover, MWA was significantly higher in patients with medullary compression. Based on these findings, the authors' interpretation is that in children with CM-I, the ICC may be reduced, as indicated by increased MWA, even though the mean ICP is within normal thresholds.

Predicting the presence of 4th ventricular outlet obstruction in Chiari I Malformation.

INTRODUCTION: A subset of children with Chiari 1 malformation (CM-1) have a 4th ventricle arachnoid veil-a thin membrane covering the outlet of the 4th ventricle. Studies suggest that failure to disrupt this veil during posterior fossa decompression can reduce the likelihood of syringomyelia resolution. However, there is no reliable method for predicting the presence of the veil without direct surgical exploration. This study aims to evaluate the association between pre-operative symptoms, radiographic measurements, and the arachnoid veil. METHODS: A retrospective review of an institutional database of children evaluated for CM-I was conducted. For patients treated with surgery, operative notes were reviewed to determine if an arachnoid veil was present. Logistic regression was used to test for relationship of clinical variables and radiographic measurements with the presence of an arachnoid veil. RESULTS: Out of 997 children with CM-1, 226 surgical patients were included in the analysis after excluding those with inadequate documentation. An arachnoid veil was found in 23 patients (10.2%). Larger syrinx, spinal canal, and thecal sac diameters were significantly associated with the presence of a veil, with odds ratios of 1.23 (95% CI 1.2-1.48; p = 0.03), 1.27 (95% CI 1.02-1.59; p = 0.03), and 1.35 (95% CI 1.03-1.77; p = 0.03), respectively. No significant associations were found with any signs or symptoms. CONCLUSIONS: Arachnoid veil was present in 10% of cases. Radiographic measurements indicating larger syrinx size were the only variables found to be significantly associated with an arachnoid veil. Exploration of the 4th ventricular outlet is recommended for CM-I decompression in the setting of expansile syringomyelia.

Pediatric cervical spine instability: evolving concepts.

The pediatric cervical spine is structurally and biomechanically unique in comparison to adults. Guidelines to assess for cervical spine instability and standard of care treatments in the pediatric population have yet to be delineated. This is due to the rarity of the condition and the lack of multicenter data published on the topic. Our review explores the biomechanics of the pediatric cervical spine and highlights evolving concepts/research over the last several decades, with special attention to the Down syndrome and complex Chiari malformation cohorts.

Perioperative Segmentation of the Posterior Fossa and the Keel of Goodrich in Surgical Outcomes of Chiari Malformations.

BACKGROUND: Chiari I malformation (CM-I) is defined as the extension of brain tissue into the spinal cord. This study aimed to refine the methodology for the acquisition of 3-dimensional measurements of the posterior fossa and introduce occipital keel size as a new marker and its impact in patients with CM. METHODS: In this retrospective study, all patients who underwent Chiari decompression surgery at Montefiore Medical Center from April 2012 to April 2022 were included. Perioperative clinical information was obtained in addition to maximal keel thickness (KT), foramen magnum area, and preoperative and postoperative posterior fossa volumes for each patient and age-matched controls. Volumetric measurements were obtained using artificial intelligence-based semiautomated segmentation. RESULTS: A total of 107 patients with CM including 37 males, and 70 females were studied with a mean age of 26.56 ± 17.31 compared with 103 controls without CM. The comparison between the CM and the general population groups demonstrated a significantly increased keel size in Chiari patients. Keel size had a significant relationship with dysphagia, paresthesia, and intraoperative blood loss, while posterior volume change had a significant relationship with sex and early symptomatic improvement. The Foramen magnum area was related to tonsillar descent and more prominent in patients with spina bifida. CONCLUSIONS: The Keel of Goodrich is a new anatomical factor that should be taken into consideration when evaluating preoperative symptoms, and intraoperative complications in patients with CM-I. Volumetric analyses demonstrated that posterior fossa volume change had a significant impact on early symptom improvement in patients with Chiari, as did the choice of operative approach. The routine use of semiautomated segmentation of the posterior fossa may help stratify Chiari patients in the future and should be implemented in routine clinical care.

Are Chiari Malformation and Basilar Invagination Associated with Jugular Foramen Stenosis?

OBJECTIVE: We compared the diameter of the jugular foramen (JF) between normal individuals and those with a diagnosis of craniovertebral junction abnormalities, such as Chiari malformation type I (CMI) and/or basilar invagination (BI). METHODS: This cross-sectional analytical study measured the diameter of the right and left JF on magnetic resonance imaging scans of patients divided into 4 groups: 68 with combined CMI and BI (CMI+BI), 42 with isolated BI, 45 with isolated CMI, and 102 control cases. The t test determined sex differences, and univariate analysis of variance with Tukey's post hoc test assessed JF differences between groups. RESULTS: Considering all combinations of laterality and sex, the CMI+BI group had a JF narrower than their respective control groups. The JF was bilaterally narrower in the BI group than in the control group only in the women. Regardless of laterality, the CMI group had a wider JF than did the CMI+BI group in men and did not differ from that of the control group. In the female patients with CMI, the right JF did not differ from that of the controls and was wider than that in the CMI+BI group. Also, the left JF was narrower than that in the control group and did not differ from that in the CMI+BI group. CONCLUSIONS: JF narrowing is associated with CMI and BI when combined in the same patient. When CMI or BI are detected separately, they do not imply evident changes in JF morphometry. It is expected that these findings could bring empiric substrate to support theories that claim a possible role of intracranial pressure disturbances as a triggering factor for CMI.

Predictors of syrinx presentation and outcomes in pediatric Chiari malformation type I: a single institution experience of 218 consecutive syrinx patients.

PURPOSE: Chiari I malformation (CM-I) in pediatric patients can impose substantial neurologic and functional impairment. Additionally, the presence of syrinx is often a harbinger of clinical compromise, but little attention has been devoted to identifying features associated with syrinx development and the clinical impact of syrinx resolution. Therefore, this study aims to identify clinical and radiographic variables associated with preoperative syrinx presence and postoperative syrinx reduction in pediatric patients with CM-I and determine the relationship between postoperative syrinx reduction and clinical symptom improvement. METHODS: The authors performed a retrospective analysis of 435 consecutive pediatric patients who underwent surgical treatment of CM-I from 2001 to 2021 at a single tertiary pediatric medical center. All patients underwent pre- and postoperative MRI, and clinical and radiographic variables were recorded and subject to inferential analysis. RESULTS: Syrinx at presentation was independently associated with symptoms of spinal cord dysfunction at presentation (OR 2.17 (95% CI 1.05-4.48); p = 0.036), scoliosis (OR 5.33 (2.34-10.86); p = 0.001), and greater pB-C2 (posterior basion to C2 distance) measurement length (OR 1.14 (95% CI 1.01-1.30); p = 0.040). Syrinx at presentation was inversely associated with tussive headaches at presentation (OR 0.27 (95% CI 0.16-0.47); p = 0.001) and cranial nerve deficits at presentation (OR 0.49 (95% CI 0.26-0.92); p = 0.025). Postoperatively, patients with radiographic evidence of syrinx improvement had greater rates of symptom improvement (93.1% vs 82.1%; p = 0.049), better CCOS scores (15.4 vs 14.2; p = 0.001), and decreased rates of readmission (6.0% vs 25.0%, p = 0.002) and reoperation (0.5% vs 35.7%; p = 0.001). The difference in syrinx resolution was similar but not statistically significant (10.3% vs 16.7%; p = 0.251). AO joint anomaly (OR 0.20, 95% CI 0.04-0.95; p = 0.026) and foramen magnum diameter (OR 1.12, 95% CI 1.00-1.25; p = 0.049) were the only independent predictors of syrinx improvement, and surgical technique was the only predictor for syrinx resolution (OR 2.44, 95% CI 1.08-5.50; p = 0.031). Patients that underwent tonsil reduction surgery whose syrinx improved had a wider foramen magnum diameter than those whose did not improve (34.3 vs 31.7; p = 0.028). CONCLUSIONS: Radiographic syrinx improvement is associated with greater rates of symptom improvement and less readmissions and reoperations for CM-I. AO joint anomalies and narrower foramen magnums were independent risk factors for the lack of syrinx improvement. These novel insights will help guide preoperative patient counseling, pre- and intraoperative surgical decision-making, and postoperative clinical prognostication in the treatment of pediatric CM-I.

An autologous duraplasty in situ technique in the treatment of Chiari malformation Type I: a prospective study.

OBJECTIVE: Our study aims to prospectively compare an autologous duraplasty in situ technique (IS group) with the synthetic dural graft duraplasty (SDG group) to clarify the effectiveness and superiority of the former in the treatment of patients with Chiari malformation type 1 (CM-I). METHOD: 29 patients with CM-I were randomly assigned to either IS or SDG group. In both groups, a dissection from the occipital bone was performed. All procedures were performed by the same surgeon. The two duraplasty methods were compared in terms of surgical factors and complications. Data analysis was done for the baseline material, the neurological outcome and MRI-documented syrinx size at the 6 month follow-up. RESULT: 29 patients were enrolled in this study, 14 in the IS group and 15 in the SDG group. The results showed no significant difference in operation time (P = 0.916), amount of bleeding (P = 0.120), operation complications, hospitalization time (P = 0.854) and prognosis between the two groups. The hospitalization cost of IS group was 15,125 yuan less than that of SDG group (P < 0.05). CONCLUSION: The autogenous duraplasty in situ technique is a novel, simple, effective and economical surgical management for patients with CM-I.

Revisiting the Pathophysiology of Intracranial Hemorrhage in Fetuses with Chiari II Malformation: Novel Imaging Biomarkers of Disease Severity?

BACKGROUND AND PURPOSE: Intracranial hemorrhage (ICH) has emerged as a notable concern in Chiari II malformation (CM II), yet its origins and clinical implications remain elusive. This study aims to validate the in utero prevalence of ICH in CM II and investigate contributing factors, and visualize the findings in a network format. MATERIALS AND METHODS: A single-center retrospective review of fetal MRI scans obtained in fetuses with CM II (presenting January 2007 to December 2022) was performed for ICH utilizing EPI-T2* blood-sensitive sequence. Fetuses with aqueduct stenosis (AS) were included as a control group. The incidence of ICH and corresponding gestational ages were compared between CM II and AS cases, and morphometric measurements (inner/outer CSF spaces, posterior fossa, venous structure) were compared among the 4 1:1 age-matched groups: CM II+ICH, CM II-ICH, AS+ICH, and AS-ICH. Additionally, a co-occurrence network was constructed to visualize associations between phenotypic features in ICH cases. RESULTS: A total of 101 fetuses with CM II and 90 controls with AS at a median gestational age of 24.4 weeks and 22.8 weeks (P = .138) were included. Prevalence of ICH in fetuses with CM II was higher compared with the AS cases (28.7% versus 18.9%, P = .023), accompanied by congested veins (deep vein congestion mainly in young fetuses, and cortical veins may also be affected in older fetuses). ICH was notably correlated with specific anatomic features, essentially characterized by reduced outer CSF spaces and clivus-supraocciput angle. The co-occurrence network analysis reveals complex connections including bony defects, small posterior fossa dimensions, vermis ectopia, reduced CSF spaces, as well as venous congestion and venous sinus stenosis as pivotal components within the network. CONCLUSIONS: The high prevalence of ICH-detected by fetal MRI-among fetuses with CM emphasizes the pathophysiologic importance of venous congestion, ICH, and vasogenic edema. As indicators of disease severity, these features may serve as helpful additional imaging biomarkers for the identification of potential candidates for fetal surgery.

A comparative analysis of neuroendoscopic foramen magnum decompression versus traditional open surgery for Chiari Malformation Type I.

PURPOSE: Chiari Malformation Type I (CM1) is characterized by the downward displacement of the cerebellar tonsils below the foramen magnum. The standard surgical treatment for CM1 is foramen magnum decompression and atlas laminectomy (FMD-AL). However, there is a growing interest in exploring minimally invasive techniques, such as neuroendoscopically assisted FMD-AL, to optimize surgical outcomes. The aim is to present the results of the less invasive neuroendoscopic-assisted system application as an alternative to decompression surgery in patients with CM-1 with/without syringomyelia. PATIENTS AND METHODS: A retrospective analysis was conducted on 76 patients with CMI who underwent either neuroendoscopic-assisted FMD-AL (n = 23) or open surgery (n = 53). Preoperative and postoperative assessments were performed, including pain levels, functional assessment, outcome and serum creatinine kinase (CK) levels. Surgical parameters and radiological imaging were also evaluated and compared. RESULTS: Both surgical groups showed improvements in pain levels and increase in postoperative CK levels. There were no statistically significant differences between the groups in terms of postoperative JOA scores, VAS scores, CCOS, or syrinx resolution. However, the neuroendoscopic group had significantly lower CK levels, shorter hospital stays, less blood loss, and shorter operation times compared to the open surgery group, indicating reduced muscle damage and potential benefits of the neuroendoscopic assisted approach. CONCLUSION: Both neuroendoscopy and open surgery groups can effectively alleviate symptoms and improve outcomes in patients with CM1. The neuroendoscopic assisted technique offers the advantage of reduced muscle damage and shorter hospital stays. The choice of surgical technique should be based on individual patient characteristics and preferences. LEVEL OF EVIDENCE: 3 (Retrospective case-control study) according to using the Oxford Centre for Evidence-Based Medicine (CEBM) Table.

Volumetric segmentation in the context of posterior fossa-related pathologies: a systematic review.

BACKGROUND: Segmentation tools continue to advance, evolving from manual contouring to deep learning. Researchers have utilized segmentation to study a myriad of posterior fossa-related conditions, such as Chiari malformation, trigeminal neuralgia, post-operative pediatric cerebellar mutism syndrome, and Crouzon syndrome. Herein, we present a summary of the current literature on segmentation of the posterior fossa. The review highlights the various segmentation techniques, and their respective strengths and weaknesses, employed along with objectives and outcomes of the various studies reported in the literature. METHODS: A literature search was conducted in PubMed, Embase, Cochrane, and Web of Science up to November 2023 for articles on segmentation techniques of posterior fossa. The two senior authors searched through databases based on the keywords of the article separately and then enrolled joint articles that met the inclusion and exclusion criteria. RESULTS: The initial search identified 2205 articles. After applying inclusion and exclusion criteria, 77 articles were selected for full-text review after screening of titles/abstracts. 52 articles were ultimately included in the review. Segmentation techniques included manual, semi-automated, and fully automated (atlas-based, convolutional neural networks). The most common pathology investigated was Chiari malformation. CONCLUSIONS: Various forms of segmentation techniques have been used to assess posterior fossa volumes/pathologies and each has its advantages and disadvantages. We discuss these nuances and summarize the current state of literature in the context of posterior fossa-associated pathologies.

The role of occipital condyle and atlas anomalies on occipital cervical fusion outcomes in Chiari malformation type I with syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Congenital anomalies of the atlanto-occipital articulation may be present in patients with Chiari malformation type I (CM-I). However, it is unclear how these anomalies affect the biomechanical stability of the craniovertebral junction (CVJ) and whether they are associated with an increased incidence of occipitocervical fusion (OCF) following posterior fossa decompression (PFD). The objective of this study was to determine the prevalence of condylar hypoplasia and atlas anomalies in children with CM-I and syringomyelia. The authors also investigated the predictive contribution of these anomalies to the occurrence of OCF following PFD (PFD+OCF). METHODS: The authors analyzed the prevalence of condylar hypoplasia and atlas arch anomalies for patients in the Park-Reeves Syringomyelia Research Consortium database who underwent PFD+OCF. Condylar hypoplasia was defined by an atlanto-occipital joint axis angle (AOJAA) ≥ 130°. Atlas assimilation and arch anomalies were identified on presurgical radiographic imaging. This PFD+OCF cohort was compared with a control cohort of patients who underwent PFD alone. The control group was matched to the PFD+OCF cohort according to age, sex, and duration of symptoms at a 2:1 ratio. RESULTS: Clinical features and radiographic atlanto-occipital joint parameters were compared between 19 patients in the PFD+OCF cohort and 38 patients in the PFD-only cohort. Demographic data were not significantly different between cohorts (p > 0.05). The mean AOJAA was significantly higher in the PFD+OCF group than in the PFD group (144° ± 12° vs 127° ± 6°, p < 0.0001). In the PFD+OCF group, atlas assimilation and atlas arch anomalies were identified in 10 (53%) and 5 (26%) patients, respectively. These anomalies were absent (n = 0) in the PFD group (p < 0.001). Multivariate regression analysis identified the following 3 CVJ radiographic variables that were predictive of OCF occurrence after PFD: AOJAA ≥ 130° (p = 0.01), clivoaxial angle < 125° (p = 0.02), and occipital condyle-C2 sagittal vertical alignment (C-C2SVA) ≥ 5 mm (p = 0.01). A predictive model based on these 3 factors accurately predicted OCF following PFD (C-statistic 0.95). CONCLUSIONS: The authors' results indicate that the occipital condyle-atlas joint complex might affect the biomechanical integrity of the CVJ in children with CM-I and syringomyelia. They describe the role of the AOJAA metric as an independent predictive factor for occurrence of OCF following PFD. Preoperative identification of these skeletal abnormalities may be used to guide surgical planning and treatment of patients with complex CM-I and coexistent osseous pathology.