Immediate postoperative resolution of syrinx post-C1/C2 fixation in an operated case of foramen magnum decompression for Chiari malformation: Is Goel's procedure a rescue surgery or a gold standard?

Chiari malformation (CM) is a common neurological disorder with foramen magnum decompression (FMD) as a commonly accepted treatment. The authors present a case of CM-1 wherein there was no radiological instability preoperatively and FMD was done as a treatment, after which the patient improved transiently only to deteriorate further. Atlantoaxial fixation was done as a second-stage procedure, after which the patient improved clinically and radiologically. The knowledge of this case and surgical entity should be borne in mind before the formulation of a treatment plan. It is important that the solution is to identify and treat the underlying pathology rather than to decompress and directly manipulate the tonsils.

Treatment of Syringomyelia Characterized by Focal Dilatation of the Central Canal Using Mesenchymal Stem Cells and Neural Stem Cells.

BACKGROUND: Syringomyelia is a progressive chronic disease that leads to nerve pain, sensory dissociation, and dyskinesia. Symptoms often do not improve after surgery. Stem cells have been widely explored for the treatment of nervous system diseases due to their immunoregulatory and neural replacement abilities. METHODS: In this study, we used a rat model of syringomyelia characterized by focal dilatation of the central canal to explore an effective transplantation scheme and evaluate the effect of mesenchymal stem cells and induced neural stem cells for the treatment of syringomyelia. RESULTS: The results showed that cell transplantation could not only promote syrinx shrinkage but also stimulate the proliferation of ependymal cells, and the effect of this result was related to the transplantation location. These reactions appeared only when the cells were transplanted into the cavity. Additionally, we discovered that cell transplantation transformed activated microglia into the M2 phenotype. IGF1-expressing M2 microglia may play a significant role in the repair of nerve pain. CONCLUSION: Cell transplantation can promote cavity shrinkage and regulate the local inflammatory environment. Moreover, the proliferation of ependymal cells may indicate the activation of endogenous stem cells, which is important for the regeneration and repair of spinal cord injury.

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.

Familial Chiari malformation: a systematic review and illustrative cases.

OBJECTIVE: 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.

MRI Insights in Chiari Malformation Type 1 and Variations With Hydrosyringomyelia.

Chiari malformation (CM) type 1 is a complex neurological disorder characterized by the displacement of the cerebellar tonsils into the upper spinal canal. Hydrosyringomyelia (HSM), which frequently coexists with this condition, presents diagnostic and treatment problems due to its broad spectrum of symptoms. There are various forms of CMs, with CM type 1 (CM1) being the most common type. Magnetic resonance imaging (MRI) is the best imaging technique to properly identify and diagnose CM1 and HSM. Important imaging findings include downward displacement of the cerebellar tonsils across the foramen magnum, the appearance of the syrinx in the spinal cord, and the alteration of the flow dynamics of the cerebrospinal fluid. This study was conducted at Datta Meghe Medical College, Nagpur, and Government Medical College & Super Speciality Hospital, Nagpur, India. It focuses on the diagnostic use of MRI in CM1 and its variations associated with HSM. Individuals who are asymptomatic may not need any treatment; however, those who are symptomatic or have HSM may require surgical decompression and restoration of the flow. We discuss the findings of MRI of six cases of CM1 and its variants with HSM and search for possible underlying causes. We conclude that magnetic resonance imaging is an imaging modality for the identification and evaluation of CM1 in cases of HSM.

Evaluation and Treatment of Patients with Small Posterior Cranial Fossa and Chiari Malformation, Types 0 and 1.

The diagnosis of Chiari I malformation is straightforward in patients with typical signs and symptoms of Chiari I malformation and magnetic resonance imaging (MRI) confirming ≥5 mm of cerebellar tonsillar ectopia, with or without a syrinx. However, in many cases, Chiari I malformation is discovered incidentally on MRI to evaluate global headache, cervical radiculopathy, or other conditions. In those cases, the clinician must consider if cerebellar tonsillar ectopia is related to the presenting symptoms. Surgical decompression of the cerebellar tonsils and foramen magnum in patients with symptomatic Chiari I malformation effectively relieves suboccipital headache, reduces syrinx distension, and arrests syringomyelia progression. Neurosurgeons must avoid operative treatments decompressing incidental tonsillar ectopia, not causing symptoms. Such procedures unnecessarily place patients at risk of operative complications and tissue injuries related to surgical exploration. This chapter reviews the typical signs and symptoms of Chiari I malformation and its variant, Chiari 0 malformation, which has <5 mm of cerebellar tonsillar ectopia and is often associated with syringomyelia. Chiari I and Chiari 0 malformations are associated with incomplete occipital bone development, reduced volume and height of the posterior fossa, tonsillar ectopia, and compression of the neural elements and cerebrospinal fluid (CSF) pathways at the foramen magnum. Linear, angular, cross-sectional area, and volume measurements of the posterior fossa, craniocervical junction, and upper cervical spine identify morphometric abnormalities in Chiari I and Chiari 0 malformation patients. Chiari 0 patients respond like Chiari I patients to foramen magnum decompression and should not be excluded from surgical treatment because their tonsillar ectopia is <5 mm. The authors recommend the adoption of diagnostic criteria for Chiari 0 malformation without syringomyelia. This chapter provides updated information and guidance to the physicians managing Chiari I and Chiari 0 malformation patients and neuroscientists interested in Chiari malformations.

Successful treatment of unilateral facial nerve palsy in a pediatric patient with syringobulbia and Chiari malformation type I: illustrative case.

BACKGROUND: Unlike syringomyelia, syringobulbia is not commonly observed in pediatric patients with Chiari malformation type I (CMI). Previous series have reported the incidence of syringobulbia as between 3% and 4% in these patients. Presentation is typically chronic, with the slow onset of neurological symptoms and cranial nerve (CN) palsies resulting from lower brainstem involvement. The authors report the first case of a pediatric patient with simultaneous CMI, syringobulbia, and unilateral CN VII palsy. OBSERVATIONS: A 7-year-old male presented with right facial weakness in addition to headaches, ataxia, urinary incontinence, and falls. Magnetic resonance imaging revealed CMI with a syrinx of the cervicothoracic spine and syringobulbia. Posterior fossa decompression with duraplasty was performed without complications, and the patient was discharged home on postoperation day 5. At the 3-week follow-up, the patient's neurological deficits had largely subsided. At the 3-month follow-up, his CN VII palsy and syringobulbia had completely resolved. LESSONS: Pediatric CMI patients with syringomyelia are at risk for developing syringobulbia and brainstem deficits, including unilateral facial palsy. However, craniocervical decompression can prove successful in treating such deficits.

Neuroendoscopy-Assisted Minimal Invasive Management of Chiari 1 Malformation.

Objective The aim this study is to present the results of the minimal invasive neuroendoscopic-assisted system application as an alternative to traditional surgery in patients with Chiari malformation type 1 (CM type 1) with/without syringomyelia. Design, Setting, and Participants In the study, data of 22 symptomatic patients were prospectively collected. Before and after the operation, patient characteristics, computed tomography, magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) flow dynamics MRI, and outcome scales scores were recorded. Foramen magnum decompression and C1 total laminectomy were performed. The fibrous band at the craniocervical junction was opened and a durotomy was performed. In patients with a syrinx, the pre- and postoperative axial and sagittal lengths of the syrinx were measured and compared. Results The mean age of the patients was 32 ± 5 years. There were eight male patients. Ten patients had syrinx. The mean visual analog scale (VAS) score before and after surgery was 8 ± 1.06 and 2.18 ± 1.13, respectively. When evaluated according to the Chicago Chiari Outcome Scale, there was improvement in 20 patients, while there was no change in 2 patients. Syrinx resolved completely in 3 of 10 (13.6%) patients with syringomyelia, and the syrinx volume decreased in 3 patients (13.6%). In 4 of 10 (18.1%) patients, there was no significant change in the syrinx volume. The average operation time was 105 minutes (80-150 minutes). The average blood loss was 40 mL (20-110 mL). Conclusion Although the study was limited due to the small number of patients with a short follow-up, endoscopic decompression was a safe and effective technique for surgery in CM type 1 patients.

Surgical Management of Chiari 1.5 in Children: A Truly Different Disease?

Background: In patients with Chiari 1.5 malformation (CM1.5), a more aggressive disease course and an increased association with craniovertebral junction (CVJ) anomalies has been suggested. The best management of this subgroup of patients is not clearly defined, also due to the lack of specific series elucidating this anomaly's peculiar characteristics. Methods: We evaluated a series of 33 patients (25 females, 8 males; mean age at surgery: 13 years) fulfilling the criteria for Chiari 1.5 diagnosis who underwent posterior fossa decompression and duraplasty (PFDD) between 2006 and 2021. Results: Headache was present in all children, five presented central apnea, five had dysphagia, and three had rhinolalia. Syringomyelia was present in 19 (58%) children. Twenty patients (61%) showed various CVJ anomalies, but only one child presented instability requiring arthrodesis. The mean tonsil displacement below the foramen magnum was 19.9 mm (range: 12-30), without significant correlation with the severity of symptoms. Syringomyelia recurred or was unchanged in three patients, and one needed C1-C2 fixation. The headache disappeared in 28 children (84%). Arachnoid opening and tonsil coagulation or resection was necessary for 19 children (58%). Conclusions: In our pediatric CM series, the need for tonsil resection or coagulation was higher in CM1.5 children due to a more severe crowding.

Volumetric assessment of Posterior cranial fossa in a West African population.

BACKGROUND AND OBJECTIVES: Posterior fossa pathologies can have potentially devastating outcomes. The volumetric capacity of this fossa, known to have ethnoregional variations, can thus be critical in determining outcomes and intervention measures and approaches to pathologies involving this region. This study aimed to evaluate the normal posterior fossa volumes within the West-African subpopulation. METHODS: This was a descriptive study of all patients presenting for a cranial imaging study at the study location within a two-year period using a 1.5T MRI of this cranial region. Obtained data included the transverse and anteroposterior diameters, and the height of this fossa and the obtained data was analyzed. P values < 0.05 was statistically significant. RESULTS: A total of 315 patients were recruited (165 males and 150 females). The average posterior fossa transverse diameter, anteroposterior diameter and height were 108.19 mm, 71.58 mm and 35.53 mm respectively for males, and 105.7 mm, 66.48 mm, and 34.24 mm for females respectively. The average posterior fossa volume for males (292.36 cm3) was significantly higher than for females (252.90 cm3) (p= 0.0038). The highest average posterior fossa volume was between 16-30 years for males and above 75 years for females. CONCLUSION: Posterior cranial fossa volumes for the West African population is significantly higher than those obtained for other regions. In addition to being beneficial in some posterior fossa space occupying lesions, this larger volume can explain the relative rarity and sexual preferences of some posterior fossa congenital abnormalities like Chiari-1 malformation amongst the West African population.

Craniovertebral and spinal adhesive arachnoiditis: a late complication of ruptured vertebral and posterior inferior cerebellar arteries aneurysms.

BACKGROUND: Adhesive arachnoiditis is a rare yet serious complication that may occur following subarachnoid hemorrhage (SAH). In this circumstance, it is mainly due to ruptured vertebral artery (VA) or posterior inferior artery (PICA) aneurysms. It disrupts cerebrospinal fluid (CSF) flow leading to complications such as spinal arachnoiditis, syringomyelia, trapped 4th ventricle, or a combination of these conditions. Evidence for effective treatment strategies is currently limited. We aimed to review the epidemiology, clinical characteristics, treatment, complications, outcomes, and prognosis of cranio-vertebral junction and spinal adhesive arachnoiditis resulting from ruptured VA and PICA aneurysms. METHODS: This study involved a comprehensive literature review and complemented by our own case. We focused on adult cases of arachnoiditis, syringomyelia, and trapped 4th ventricle with SAH caused by ruptured VA or PICA aneurysms, excluding cases unrelated to these aneurysms and those with insufficient data. RESULTS: The study included 22 patients, with a mean age of 52.4 years. Symptoms commonly manifest within the first year after SAH and timely diagnosis requires a high index of suspicion. Treatment approaches included lysis of adhesions and various shunt procedures. Most patients showed improvement post-treatment, though symptom recurrence is significant. CONCLUSION: Adhesive arachnoiditis is a critical complication following SAH, most commonly from ruptured VA and PICA aneurysms. Early detection and individualized treatment based on the type of arachnoiditis and CSF flow impact are crucial for effective management. This study underscores the need for tailored treatment strategies and further research in this field.

A complicated Chiari type 1 malformation and holocord syrinx as a likely cause for heel pain.

BACKGROUND: Chiari malformations are a rare group of rhomboencephalic abnormalities involving the brain, craniocervical junction and spine. They may manifest in a variety of clinical presentations which relate to the variable involvement of the cerebellum, brainstem, lower cranial nerves, spinal cord and altered CSF flow dynamics. METHOD: We report an unusual case of incidental diagnosis of a type I Chiari malformation with secondary cystic cerebellar tonsillar encephalomalacia and holocord syrinx following investigation of a 5YO girl presenting with heel swelling related to progressive neuropathic osteoarthropathy of the posterior calcaneal body and apophysis. RESULT: The child was treated with decompressive suboccipital craniectomy and C1 laminectomy and tonsillar resection. Cerebellar tonsillar gliosis and cystic degeneration were confirmed on histopathology. Referral for ongoing engagement with occupational and physical therapy. CONCLUSION: Most type I Chiari malformations in the paediatric population are incidental and asymptomatic. Neurological symptoms are typically mild and relate to altered CSF flow dynamics; however, we present a complex case of type I Chiari malformation with an unusual constellation of associated complications.

Multicenter comparison of Chiari malformation type I presentation in children versus adults.

OBJECTIVE: Treatment for Chiari malformation type I (CM-I) often includes surgical intervention in both pediatric and adult patients. The authors sought to investigate fundamental differences between these populations by analyzing data from pediatric and adult patients who required CM-I decompression. METHODS: To better understand the presentation and surgical outcomes of both groups of patients, retrospective data from 170 adults and 153 pediatric patients (2000-2019) at six institutions were analyzed. RESULTS: The adult CM-I patient population requiring surgical intervention had a greater proportion of female patients than the pediatric population (p < 0.0001). Radiographic findings at initial clinical presentation showed a significantly greater incidence of syringomyelia (p < 0.0001) and scoliosis (p < 0.0001) in pediatric patients compared with adult patients with CM-I. However, presenting signs and symptoms such as headaches (p < 0.0001), ocular findings (p = 0.0147), and bulbar symptoms (p = 0.0057) were more common in the adult group. After suboccipital decompression procedures, 94.4% of pediatric patients reported symptomatic relief compared with 75% of adults with CM-I (p < 0.0001). CONCLUSIONS: Here, the authors present the first retrospective evaluation comparing adult and pediatric patients who underwent CM-I decompression. Their analysis reveals that pediatric and adult patients significantly differ in terms of demographics, radiographic findings, presentation of symptoms, surgical indications, and outcomes. These findings may indicate different clinical conditions or a distinct progression of the natural history of this complex disease process within each population, which will require prospective studies to better elucidate.

Syringo-Subarachnoid Shunt with Tube Versus T-Tube via the Dorsal Root Entry Zone Approach for Eccentric Syringomyelia.

OBJECTIVE: This study compared the clinical therapeutic efficacy of syringo-subarachnoid shunt placement with direct tube and T-tube via the dorsal root entry zone (DREZ) approach for treatment of eccentric syringomyelia. METHODS: A retrospective study was performed of 41 patients with idiopathic or secondary eccentric syringomyelia from November 2011 to December 2022. Syringo-subarachnoid shunt placement with direct tube or T-tube via the DREZ approach was performed. The modified Japanese Orthopaedic Association low back pain scale was used to investigate the severity of clinical symptoms. Magnetic resonance imaging was used to investigate therapeutic efficacy（reduction of the cavity volume by >10% was considered an improvement and 50% was considered a significant improvement). RESULTS: Incision length of the spinal cortex in the direct tube group was shorter than in the T-tube group (3.10 ± 0.28 cm vs. 5.03 ± 0.19 cm), with a significant difference between the 2 groups (t = -52.56, P < 0.001). Modified Japanese Orthopaedic Association score 3 months postoperatively was significantly better than the preoperative score in both the direct tube group（t = 40.954, P < 0.001） and the T-tube group（t = 24.769, P < 0.001）. Statistical comparison revealed there was no difference in imaging improvement between the direct tube group and T-tube group 3 months (χ2 = 0.20, P = 0.655) and 12 months (χ2 = 0.21, P = 0.647) postoperatively. CONCLUSIONS: Syringo-subarachnoid shunt placement with direct tube via the DREZ approach for treatment of eccentric syringomyelia is safer than with T-tube via the DREZ approach due to smaller incision length and less of a space-occupying effect with same therapeutic efficacy.

Role of Preoperative Albumin Quotient in Surgical Planning for Posttraumatic Syringomyelia: A Comparative Cohort Study.

OBJECTIVE: Surgical procedures for patients with posttraumatic syringomyelia (PTS) remain controversial. Until now, there have been no effective quantitative evaluation methods to assist in selecting appropriate surgical plans before surgery. METHODS: We consecutively enrolled PTS patients (arachnoid lysis group, n = 42; shunting group, n = 14) from 2003 to 2023. Additionally, 19 intrathecal anesthesia patients were included in the control group. All patients with PTS underwent physical and neurological examinations and spinal magnetic resonance imaging preoperatively, 3-12 months postoperatively and during the last follow-up. Preoperative lumbar puncture was performed and blood-spinal cord barrier disruption was detected by quotient of albumin (Qalb, cerebrospinal fluid/serum). RESULTS: The ages (p = 0.324) and sex (p = 0.065) of the PTS and control groups did not differ significantly. There were also no significant differences in age (p = 0.216), routine blood data and prognosis (p = 0.399) between the arachnoid lysis and shunting groups. But the QAlb level of PTS patients was significantly higher than that of the control group (p < 0.001), and the shunting group had a significantly higher QAlb (p < 0.001) than the arachnoid lysis group. A high preoperative QAlb (odds ratio, 1.091; 95% confidence interval, 1.004-1.187; p = 0.041) was identified as the predictive factor for the shunting procedure, with the receiver operating characteristic curve showing 100% specificity and 80.95% sensitivity for patients with a QAlb > 12.67. CONCLUSION: Preoperative QAlb is a significant predictive factor for the types of surgery. For PTS patients with a QAlb > 12.67, shunting represents the final recourse, necessitating the exploration and development of novel treatments for these patients.

Investigating post-traumatic syringomyelia and local fluid osmoregulation via a rat model.

BACKGROUND: Syringomyelia (SM) is characterized by the development of fluid-filled cavities, referred to as syrinxes, within the spinal cord tissue. The molecular etiology of SM post-spinal cord injury (SCI) is not well understood and only invasive surgical based treatments are available to treat SM clinically. This study builds upon our previous omics studies and in vitro cellular investigations to further understand local fluid osmoregulation in post-traumatic SM (PTSM) to highlight important pathways for future molecular interventions. METHODS: A rat PTSM model consisting of a laminectomy at the C7 to T1 level followed by a parenchymal injection of 2 µL quisqualic acid (QA) and an injection of 5 µL kaolin in the subarachnoid space was utilized 6 weeks after initial surgery, parenchymal fluid and cerebrospinal fluid (CSF) were collected, and the osmolality of fluids were analyzed. Immunohistochemistry (IHC), metabolomics analysis using LC-MS, and mass spectrometry-based imaging (MSI) were performed on injured and laminectomy-only control spinal cords. RESULTS: We demonstrated that the osmolality of the local parenchymal fluid encompassing syrinxes was higher compared to control spinal cords after laminectomy, indicating a local osmotic imbalance due to SM injury. Moreover, we also found that parenchymal fluid is more hypertonic than CSF, indicating establishment of a local osmotic gradient in the PTSM injured spinal cord (syrinx site) forcing fluid into the spinal cord parenchyma to form and/or expand syrinxes. IHC results demonstrated upregulation of betaine, ions, water channels/transporters, and enzymes (BGT1, AQP1, AQP4, CHDH) at the syrinx site as compared to caudal and rostral sites to the injury, implying extensive local osmoregulation activities at the syrinx site. Further, metabolomics analysis corroborated alterations in osmolality at the syrinx site by upregulation of small molecule osmolytes including betaine, carnitine, glycerophosphocholine, arginine, creatine, guanidinoacetate, and spermidine. CONCLUSIONS: In summary, PTSM results in local osmotic disturbance that propagates at 6 weeks following initial injury. This coincides with and may contribute to syrinx formation/expansion.

Direction-selective Resistance to Cerebrospinal Fluid Flow as the Cause of Syringomyelia.

The pathophysiology of syringomyelia remains poorly understood. Two prevailing challenges stand out: the need for a comprehensive understanding of its diverse types and the yet-to-be-explained mechanism of cerebrospinal fluid (CSF) retention in the syrinx despite its higher pressure than that in the adjacent subarachnoid space. Expanding on our previous proposal that direction-selective resistance to subarachnoid CSF flow drives syringomyelia genesis, this study uses a computer model to explore this mechanism further. We developed a computer simulation model to study spinal CSF dynamics, employing a lumped parameter approach with multiple compartments. This model replicated the to-and-fro movement of CSF in the spinal subarachnoid space and within an intraspinal channel. Subsequently, a direction-selective resistance-opposing only the caudal subarachnoid CSF flow-was introduced at a specific location within the subarachnoid space. Following the introduction of the direction-selective resistance, a consistent pressure increase was observed in the intraspinal channel downstream of the resistance. Importantly, this increase in pressure accumulated with every cycle of to-and-fro CSF flow. The accumulation results from the pressure drop across the resistance, and its effect on the spinal cord matrix creates a pumping action in the intraspinal channel. Our findings elucidate the mechanisms underlying our hypothesis that a direction-selective resistance to subarachnoid CSF flow causes syringomyelia. This comprehensively explains the various types of syringomyelia and resolves the puzzle of CSF retention in the syrinx despite a pressure gradient.

Management of Persistent Syringomyelia in Patients Operated for Chiari Malformation Type 1.

BACKGROUND: The treatment of persistent syringomyelia associated with Chiari malformation type 1 (CM1) is unclear. This study aims to evaluate the clinical and radiologic outcomes of syringo-subarachnoid shunt (SSS) as a treatment for persistent syringomyelia following posterior fossa decompression (PFD) for CM1. METHODS: Forty-nine cases treated for CM1 associated syringomyelia at a single center were analyzed, 17 of them undergoing reoperation due to persistent syrinx formation. The patients' demographic data, neurologic presentations, and radiologic results were analyzed, including cerebellar herniation, posterior fossa volume, the level at which the syrinx started and finished, the size and diameter of the syrinx. RESULTS: Seventeen patients underwent SSS placement, with 1 minor surgical complication (a cerebrospinal fluid leak) occurring and requiring revision. No morbidity or mortality was observed. Among these 17 reoperated patients, partial or complete resorption of the syrinx was observed in all cases. The results suggest that if the syrinx diameter is >10 mm at its thickest point, extends for more than 10 vertebrae, and starts from the upper cervical region and extends to the upper thoracic region, the syrinx may not regress after the first surgery and potentially predicting the need for a second operation before PFD. CONCLUSIONS: SSS placement for persistent syrinx following PFD for CM1 is a safe and effective surgical treatment method. These criteria may also help predict the need for a second surgery and the overall disease outcome for both the surgeon and patient.

A long-term follow-up study of adults with Chiari malformation type I combined with syringomyelia.

Background: There is a considerable amount of controversy regarding the treatment and prognosis of adult patients with Chiari malformation type I (CM-I) at home and abroad; furthermore, no large-sample, long-term, follow-up studies have examined CM-I patients with syringomyelia (SM) comparing posterior fossa decompression with resection of tonsils (PFDRT) vs. posterior fossa decompression with duraplasty (PFDD). Objective: This study retrospectively analyzed the factors affecting the treatment and long-term prognosis of adults with CM-I combined with SM. Methods: We retrospectively analyzed data from 158 adult CM-I patients combined with SM who underwent PFDRT or PFDD, including 68 patients in group PFDRT and 90 patients in group PFDD. We examined the clinical manifestations, imaging features, and follow-up data of patients. Clinical outcomes were assessed using the Chicago Chiari Outcomes Scale (CCOS), and radiographic outcomes were indicated by the syrinx remission rate. Multivariate logistic regression analysis and multiple linear regression analysis were used to explore the relevant factors affecting the long-term prognosis of patients. Results: This study showed that compared with preoperative patients in the PFDRT group and PFDD group, the sensory impairment, cough-related headache, and movement disorder were significantly improved (p < 0.01); meanwhile, the diameter of the syrinx and the volume of the syrinx decreased significantly (p < 0.001). Additionally, the study found that there were significant differences in the syrinx remission rate (p = 0.032) and the clinical cure rates (p = 0.003) between the two groups. Multivariate logistic regression analysis showed that age (p = 0.021), cerebellar-related symptoms (p = 0.044), preoperative cisterna magna volume (p = 0.043), and peak systolic velocity (p = 0.036) were independent factors for clinical outcomes. Multiple linear regression analysis showed that different surgical procedures were positively correlated with the syrinx remission rate (p = 0.014), while preoperative syrinx diameter (p = 0.018) and age (p = 0.002) were negatively correlated with the syrinx remission rate. Conclusion: In conclusion, this study suggested that, in a long-term follow-up, although both surgical procedures are effective in treating patients with CM-I and SM, PFDRT is better than PFDD; age and cerebellar-related signs independently affect the patient's prognosis. Additionally, an effective prognosis evaluation index can be developed for patients, which is based on imaging characteristics, such as preoperative cisterna magna volume, preoperative syrinx diameter, and preoperative cerebrospinal fluid (CSF) hydrodynamic parameters to guide clinical work.

Decoding Chiari Malformation and Syringomyelia: From Epidemiology and Genetics to Advanced Diagnosis and Management Strategies.

Chiari Malformation and Syringomyelia are neurosurgical entities that have been the subject of extensive research and clinical interest. Globally prevalent, these disorders vary demographically and have witnessed evolving temporal trends. Chiari Malformation impacts the normal cerebrospinal fluid flow, consequently affecting overall health. Key observations from canine studies offer pivotal insights into the pathogenesis of Syringomyelia and its extrapolation to human manifestations. Genetics plays a pivotal role; contemporary knowledge identifies specific genes, illuminating avenues for future exploration. Clinically, these disorders present distinct phenotypes. Diagnostically, while traditional methods have stood the test of time, innovative neurophysiological techniques are revolutionizing early detection and management. Neuroradiology, a cornerstone in diagnosis, follows defined criteria. Advanced imaging techniques are amplifying diagnostic precision. In therapeutic realms, surgery remains primary. For Chiari 1 Malformation, surgical outcomes vary based on the presence of Syringomyelia. Isolated Syringomyelia demands a unique surgical approach, the effectiveness of which is continually being optimized. Post-operative long-term prognosis and quality of life measures are crucial in assessing intervention success. In conclusion, this review amalgamates existing knowledge, paving the way for future research and enhanced clinical strategies in the management of Chiari Malformation and Syringomyelia.