The 4S of spinal astrocytoma: specific location, syrinx, spasticity and score on Modified Mccormick Scale (MMS) predict long term outcomes in patients undergoing surgical resection of intramedullary spinal astrocytomas.

OBJECTIVES: The aim of this study was to explore the factors that could predict long term clinical outcomes in SA. METHODS: A retrospective study was conducted wherein SA patients undergoing surgical resection with a minimum follow up of 12 months were included in this study. Modified Mccormick Scale (MMS) was utilized to record the neurological status of the patients both preoperatively and at last follow up. Outcomes were assessed as: long term neurological status, that is final MMS grade and neurological deterioration, defined as increase in MMS score as compared to preoperative MMS score. Survival analysis was performed using the kaplan meier curves. RESULTS: 71 patients were included in this study with mean age of 33.07years. At a mean follow up of 57 months, preoperative MMS was the single independent predictor for moderate-severe neurological deficit (MMS III to V) on multivariate analysis (OR: 30.2, p < 0.001) and had an outstanding AUC of 0.91. Six patients had neurological deterioration at long term follow up. Absence of spasticity (p = 0.028), thoracic-thoracolumbar tumors (p = 0.006), low MMS score (p = 0.01) and hypointense T1 weighted MRI (p = 0.009) were significant predictors of long term neurological deterioration. The median overall survival was 48 months and was significantly higher in low grade tumors (p < 0.001). CONCLUSION: The study highlights the efficacy of clinical features as a predictor of long term functional outcomes in SA patients. Role of spasticity as a prognostic factor was explored for the first time in this study.

Acute Respiratory Failure Requiring Invasive Ventilation in Adults With Congenital Syringomyelia/Arnold-Chiari Malformations: A Systematic Review.

Arnold-Chiari malformations (ACM) and congenital syringomyelia/syrinx are rare neurological phenomenons that can present as acute respiratory failure and contribute to multiple extubation failures despite surgical intervention. A systematic review was conducted to scrutinize the current literature, screening 65 papers and including 12 papers (13 patients). Sixty-one percent of patients had type 1 ACM and 70% had a congenital syringomyelia. Neurosurgical intervention occurred in seven patients, five patients had at least one extubation failure which was due to apnea or reoccurrence of respiratory failure, and eight patients needed tracheostomies. The neurosurgical intervention aims to improve patient symptoms, but our data and current literature suggest that patients with these pathologies still undergo long ventilation weans and are not liberated from the ventilator due to ongoing respiratory failure.

Case report: CSF hypotension secondary to a free syringo-subarachnoid-peritoneal shunt.

INTRODUCTION: Syringomyelia is present in 40% of pediatric patients with Chiari malformation. Typically treated with posterior fossa decompression, some cases require further intervention such as syrinx shunting. CASE REPORT: We report a 16-year-old female with Chiari type 1 malformation and syringomyelia who underwent posterior fossa decompression and subsequent free syringo-subarachnoid-peritoneal shunting. The patient developed symptoms of CSF overdrainage, and imaging indicated CSF hypotension. A distal catheter ligation temporarily improved symptoms, but eventually, a programmable ventricular shunt was necessary due to shunt dependence. CONCLUSION: This case highlights the rare complication of CSF overdrainage from syrinx shunting and the importance of shunt selection considerations.

Chiari malformation presenting with subarachnoid hemorrhage: a case report.

Chiari malformations (CM) are often diagnosed in childhood and younger adults, with an incidence of only 0.77% in adult populations. Patients with CM may develop syringomyelia and increased intracranial pressure (ICP) due to cerebrospinal fluid (CSF) obstruction and altered fluid dynamics at the cervicomedullary junction. We describe the case of a 65-year-old female presenting with an angionegative subarachnoid hemorrhage (SAH) with concomitant new diagnosis of CM type I with syringomyelia. After ruling out any aneurysm or vascular malformations, she underwent a suboccipital craniectomy for a Chiari decompression with a C1 laminectomy. There were no complications with the surgery and her symptoms improved. This case report highlights the unusual presentation of a CM.

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.

Longitudinal assessment of syringomyelia in Pomeranians.

Introduction: Chiari-like malformation (CM) and syringomyelia (SM) are disorders that, in dogs, affect mainly small and toy breeds, including the Pomeranian. These disorders are linked to a great number of (owner-reported) clinical signs (ORCS) suggestive of pain. Aging was associated with an increased risk of having SM in several studies. However, there are only a few longitudinal studies that assess the presence and severity of CM/SM over time in CKCS dogs and progression of SM was linked to progression of clinical signs. The aim of this study was to investigate ORCS, CM/SM classification, and quantitative syrinx parameters in relation to progression of time (age) within individual Pomeranians. Materials and methods: Pomeranians with or without ORCS and with or without diagnoses of CM/SM were included that had undergone two (or more) MRI studies of the craniocervicothoracic region between January 2020 and June 2023. Classification of CM/SM and quantitative syrinx measurements were performed. Absolute values as well as ratios for syrinx height, width, and cross-sectional area were included for analysis. Results: A total of 19 Pomeranians were included in the study, of which 11 were male (58%) and 8 were female (42%). The median age at the time of MRI1 was 26 months (range 7-44 months). The median scan interval was 26 months (range 11-49 months). Eleven dogs (58%) were presented with ORCS at the time of MRI1, whereas the other 8 dogs (42%) had no ORCS at that time. At the time of MRI2, there were 17/19 dogs (89%) with ORCS and 2/19 dogs without ORCS (11%). Dogs were significantly more likely to have ORCS at MRI2 than MRI1 (p = 0. 0411). There was no significant difference between CM/SM classification at the time of MRI1 and MRI2. Significant differences were found between MRI1 and MRI2 for syrinx height (based on transverse images) (absolute value and ratio P = 0.0059), syrinx width (absolute value P = 0.1055, ratio P = 0.0039), and syrinx cross sectional area (absolute value P = 0.0195, ratio P = 0.0217). Discussion: There are differences in the presence or absence of ORCS as well as quantitative syrinx measurements in Pomeranians at different ages. This finding supports that longitudinal changes occur in the SM status of Pomeranians.

Longitudinal Pulse-Synchronous Bouncing During Catheter Angiography-A Phenomenon Specific to Spinal Hemangioblastomas.

BACKGROUND: Spinal hemangioblastomas are often evaluated with catheter angiography for both workup and treatment planning. We report a unique longitudinal pulse-synchronous bouncing phenomenon observed during their angiographic evaluation and consider the association of pulse-synchronous bouncing with syringomyelia, another pathologic feature associated with hemangioblastomas. METHODS: Preoperative spinal angiograms and associated magnetic resonance imagings (MRIs) obtained over a 16-year period at a single institution were retrospectively evaluated. Magnetic resonance imaging (MRI) parameters included lesion and syrinx location and size. Angiograms were evaluated for bouncing phenomena. Student's t-test and Chi square test compared characteristics between groups. Linear regression analyses evaluated maximum amplitude of dynamic motion and any associated syrinx. RESULTS: Nineteen hemangioblastoma patients had preoperative angiograms available for review. Eight exhibited bouncing behavior. Between the dynamic and nondynamic cohorts, there was no difference in presence or volume of syrinxes. Lesions in the dynamic cohort trended towards a cervical location (75% vs. 36.3%, P = 0.10). No significant correlation was found between bouncing amplitude and syrinx size (R2 = 0.023). Dural contact may be related to this dynamic behavior since other high-flow lesions like AVMs do not demonstrate this phenomenon, and AVMs are pial-based and more likely to contact stationary dura. Here, there were fewer lesions abutting the thecal sac in the dynamic cohort (50% vs. 81.8%, P = 0.14). CONCLUSIONS: Though no significant relationship was established between this bouncing behavior and syrinx formation, noted trends included a greater range of motion for cervical lesions and limited motion in tumors abutting the thecal sac.

Does long segment subarachnoido-subarachnoid shunt prevent posttraumatic and postinfectious spinal arachnoid cyst recurrence?: technical note and case series.

Syringomyelia associated with extensive spinal adhesive arachnoiditis (SAA) can be defined as a rare but progressive disease with potentially devastating clinical consequences. Diagnosis can be challenging due to the absence of specific clinical findings, confusion with other pathologies, and late imaging modalities. The treatment strategy for syringomyelia associated with extensive SAA should include direct drainage of the syringomyelia, and dissection of the adhesive arachnoid with expansive duraplasty. Hence, several approaches have been reported for arachnoid dissection and decompression of the subarachnoid space. The high risk of recurrence after the operation is one of the most challenging situations. First two cases were operated previously, and the cyst walls were removed and anastomosed to the intact subarachnoid space at the upper and lower ends by each other. However, in both cases, the cyst recurred after a short time and they were anastomosed again in our clinic. A subarachnoid-subarachnoid shunt was placed with a multi-hole silicone tube extending to the proximal and distal intact subarachnoid spaces and passing through the cyst removal area. In our third case, the shunt system we described was applied directly, and satisfactory results were obtained in the clinical follow-up of the patient. It has been demonstrated that subarachnoido-subarachnoid shunt is a very satisfactory treatment option for this type of pathology, which is difficult to follow and treat.

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.

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.

Duraplasty using a combination of a pedicled dural flap and collagen matrix in posterior fossa decompression for pediatric Chiari malformation type 1 with syrinx.

PURPOSE: In posterior fossa decompression for pediatric Chiari malformation type 1 (CM-1), duraplasty methods using various dural substitutes have been reported to improve surgical outcomes and minimize postoperative complications. To obtain sufficient posterior fossa decompression without cerebrospinal fluid-related complications, we developed a novel duraplasty technique using a combination of a pedicled dural flap and collagen matrix. The objective of this study was to describe the operative nuances of duraplasty using a combination of a pedicled dural flap and collagen matrix in posterior fossa decompression for pediatric CM-1. METHODS: We reviewed the clinical and radiographic records of 11 consecutive pediatric patients who underwent posterior fossa decompression with duraplasty using a combination of a pedicled dural flap and collagen matrix followed by expansile cranioplasty for CM-1. The largest area of the syrinx and the size of the posterior fossa were calculated. RESULTS: The maximum syrinx area was reduced by a mean of 68.5% ± 27.3% from preoperatively to postoperatively. Four patients (36.4%) had near-complete syrinx resolution (> 90%, grade III reduction), five (45.5%) had 50% to 90% reduction (grade II), and two (18.2%) had < 50% reduction (grade I). The posterior fossa area in the midsagittal section increased by 8.9% from preoperatively to postoperatively. There were no postoperative complications, including cerebrospinal fluid leakage, pseudomeningocele formation, or infection. CONCLUSION: Duraplasty using a combination of a pedicled dural flap and collagen matrix in posterior fossa decompression is a promising safe and effective surgical technique for pediatric CM-1 with syrinx.

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.

Natural history of Chiari I malformation with syrinx and dilatation of the central canal in the pediatric population: the CHEO experience.

PURPOSE: Given that syrinx is often considered an indication of surgery in children with Chiari I malformation (CM1), understanding of the natural history of these patients is very challenging. In this study, we investigate the natural history of children with CM1 that have syrinx and/or prominence of the central canal on presentation. METHODS: All pediatric Chiari I patients who had syrinx and/or prominence of the central canal who underwent MR imaging of the head and spine from 2007 to 2020 were reviewed. Patients were divided into 3 groups (early surgery, delayed surgery, and conservative management). We focused on those patients who did not initially undergo surgery and had at least 1 year of clinical follow-up. We assessed if there were any radiological features that would correlate with need for delayed surgical intervention. RESULTS: Thirty-seven patients met the inclusion criteria. Twenty-one patients were female and 16 were male. The mean age at presentation was 8.7 (5.8 SD). Fourteen (38%) patients had early surgical intervention, with a mean of 2.5 months after initial presentation, 8 (16%) had delayed surgery due to new or progressive neurological symptoms and 46% of patients did not require intervention during follow-up. The length of tonsillar herniation and the position of the obex were associated with the need of surgery in patients who were initially treated conservatively. CONCLUSION: In pediatric patients with CM1 with syringomyelia and prominence of the central canal, conservative treatment is initially appropriate when symptoms are absent or mild. Close follow-up of patients with CM1 and dilatation of the central canal who have an obex position below the foramen magnum and greater tonsillar herniation is suggested, as these patients show a trend towards clinical deterioration over time and may require earlier surgical intervention.

Homology and the evolution of vocal folds in the novel avian voice box.

The origin of novel traits, those that are not direct modifications of a pre-existing ancestral structure, remains a fundamental problem in evolutionary biology. For example, little is known about the evolutionary and developmental origins of the novel avian vocal organ, the syrinx. Located at the tracheobronchial junction, the syrinx is responsible for avian vocalization, but it is unclear whether avian vocal folds are homologous to the laryngeal vocal folds in other tetrapods or convergently evolved. Here, we identify a core developmental program involved in avian vocal fold formation and infer the morphology of the syrinx of the ancestor of modern birds. We find that this ancestral syrinx had paired sound sources induced by a conserved developmental pathway and show that shifts in these signals correlate with syringeal diversification. We show that, despite being derived from different developmental tissues, vocal folds in the syrinx and larynx have similar tissue composition and are established through a strikingly similar developmental program, indicating that co-option of an ancestral developmental program facilitated the origin of vocal folds in the avian syrinx.

Ostrich (Struthio camelus) syrinx morphology and vocal repertoire across postnatal ontogeny and sex: Implications for understanding vocal evolution in birds.

Vocal production in birds has been the target of considerable research that mostly has focused on phylogenetically well-nested songbirds. Anatomical descriptions and recordings of many non-songbirds have often only focused on a single ontogenetic stage or sex. While basic morphology of the vocal organ (syrinx) of ostrich (Palaeognathae, Struthio camelus) has been known since the 1800s, descriptions of its vocal repertoire and syrinx anatomy since then have been incomplete or inconsistent. New toolkits now enable detailed qualitative description of internal anatomy and meristic data and allow it to be compared to vocal production. Here we describe the anatomy of the syrinx in Struthio camelus for three post-hatching ontogenetic stages and both an adult male and female utilizing dissection and contrast enhanced X-ray computed tomography (diceCT). We find changes in ring geometry and spacing through ontogeny as well as lateral labia thickness. We document a small unpaired, midline, cartilaginous structure, a "pessuliform process" at the tracheobronchial juncture present throughout ontogeny and in both males and females. Investigation of the vocal repertoire of ostriches across ontogeny using a new dataset of 77 recordings led to identification of four vocalizations not previously reported in the literature, including the simultaneous production of a hiss and tonal. We find syrinx morphology largely consistent across ontogeny and in male and female adults. Both are capable of producing long duration tonal calls, but these may be more frequent in male birds. Closed-mouth boom calls remain unique to males. A detailed understanding of diversity in parts of early diverging clades is pivotal in attempting to estimate features of the ancestral syrinx in birds and how avian vocalization evolved.

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.

Chiari Type 1 Malformation and Syringomyelia in Children: Classification and Treatment Options.

Chiari type 1 malformation (CIM) is defined as tonsillar ectopia of >5 mm, while syringomyelia (SM) is defined as a cerebrospinal fluid (CSF)-filled cavity larger than 3 mm dissecting the spinal cord. Over the last decades, our understanding of these pathologies has grown; however, many controversies still exist almost in every aspect of CIM and SM, including etiology, indication for treatment, timing of treatment, surgical technique, follow-up regime, and outcome. This chapter provides a comprehensive overview on different aspects of CIM and SM and on the still existing controversies, based on the evidence presently available. Future directions for clinical research concerning CIM and SM treatment and outcome are elaborated and discussed as well.

Single-level ossified ligamentum flavum causing a holocord syrinx: illustrative case.

BACKGROUND: Syringomyelia is a neurological disorder that is caused by abnormal cerebrospinal fluid flow or circulation. It is an incidental finding in most cases, predominantly presenting with sensory symptoms of insensitivity to pain and temperature. Spinal ossified ligamentum flavum (OLF) leading to syringomyelia is one of the rare causes. The authors report an unusual case of syringomyelia due to a thoracic OLF. OBSERVATIONS: A 54-year-old female presented with backache, difficulty walking, spasticity in the bilateral lower limbs, tingling sensation in the bilateral lower limbs, and paraparesis for 5 years. Her radiological investigations were suggestive of an OLF causing a syrinx. She underwent laminectomy, and her syrinx resolved on subsequent follow-up. LESSONS: A syrinx due to a single-level OLF is rare, and this uncommon cause should be kept in mind while formulating treatment plans.

Three-Dimensional Volumetric Magnetic Resonance Imaging Analysis of Syringomyelia Evolution After Posterior Fossa Decompression for Chiari Malformation Type1.

OBJECTIVE: This study aims to introduce a method of three-dimensional (3D) volume magnetic resonance imaging (MRI) measurements to evaluate the evolution of syringomyelia (SM) in adults with Chiari malformation type 1 (CM-1) after posterior fossa decompression (PFD), to provide reproducible and accurate measurements. METHODS: Radiologic and clinical data were obtained for 28 patients. MRI 3D syrinx volumes were evaluated by 2 observers using Philips Healthcare Tumor Tracking tool. RESULTS: The mean SM preoperative volume was 9.1 ± 15.3 cm3 versus 4.6 ± 8.7 cm3 postoperatively, corresponding to a volume variation reduced by -50% ± 33.4%. A total of 21 patients (75%) showed >25% reduction in SM volume postoperatively and 25 patients (89.2%) showed good to excellent postoperative clinical outcomes. Very high interobserver and intraobserver agreement was noted for 3D volumetric analysis with Cohen weighted κ coefficients (0.899-0.991). A negative correlation was found between the number of involved metameres and the postoperative SM volume reduction (Pearson correlation coefficient = 0.378; P = 0.047). Patients with better clinical outcomes had fewer syrinx septae (F = 8.830; P = 0.001). Similarly, better syrinx volume reduction was associated with fewer septae (F = 1.554; P = 0.233). A positive relationship was observed between clinical outcomes and postoperative syrinx volume reduction (F = 1.554; P= 0.233), with data similar to the reported literature. CONCLUSIONS: This study shows that 3D volume measurement of the syrinx cavity on MRI is a reproducible and accurate method to assess the postoperative evolution of SM in CM-1 after PFD. Most patients (75%) showed a reduction of SM volume, with a mean volume decrease of 50%. The reliability is yet to be validated in further larger studies.

Spontaneous improvement in syringomyelia in a patient with Chiari 1 malformation: illustrative case.

BACKGROUND: "Chiari malformation" refers to a spectrum of hindbrain abnormalities characterized by impaired cerebrospinal fluid circulation through the foramen magnum. Syringomyelia is frequently found in patients with Chiari malformation type 1. Although many theories have suggested how cerebrospinal fluid enters and makes the cystic cavity in the spinal cord, the pathogenesis of syringomyelia remains controversial. This report documents a case with spontaneous resolution of syringomyelia followed up by 3-year serial magnetic resonance imaging (MRI). These kinds of cases support a more conservative approach. OBSERVATIONS: A 59-year-old female presented to the authors' clinic in June 2019 with a history of Chiari malformation type 1. This symptomatic patient has been followed up with serial MRI. When the last MRI was performed in August 2022, compared with previous imaging, resolution of the syringomyelia was recognized. LESSONS: Because the natural evolution of mildly symptomatic/asymptomatic patients with syringes is unclear, these patients pose a treatment dilemma. Although surgical intervention is a widely accepted therapeutic method, a more conservative approach can be considered in cases with spontaneous resolution. Especially for patients without progressive symptoms, the surgical approach should not be considered as the first step. In view of relapses, follow-up with periodic neurological examinations and radiological imaging is preferrable.