Spinal Adhesive Arachnoidopathy, the Disorder More Than Simply Adhesive Arachnoiditis: A Comprehensive Systematic Review of 510 Cases.

BACKGROUND: Spinal adhesive arachnoidopathy (SAA) is a chronic pathology associated with persistent inflammatory responses in the arachnoid. Adhesive arachnoiditis (AA) is one of the major forms of SAA, with accompanying secondary complications. Therefore, we aimed to systematically review both clinical and animal model studies related to SAA to gain a deeper understanding of this unique pathology. METHODS: A literature search was conducted in PubMed, EMBASE, and Cochrane Library databases to retrieve relevant publications up to October 2022. Clinical manifestations, etiologies, imaging modalities, treatments, and prognosis in patients with SAA were collected. Data from animal experiments related to SAA were also extracted. RESULTS: A total of 176 studies, including 147 clinical and 29 animal model studies, with a total of 510 patients were enrolled in this study. Pain (37.5%), abnormal nerve sensations (39.58%), and abnormal motor function (78.75%) were the top three common symptoms of SAA. Major etiologies included trauma (22.7%), infection (17.73%), surgery (15.37%), and hemorrhage (13.48%). MRI was widely used to confirm the diagnosis. AA could be involved in cervical (96/606, 15.84%), thoracic (297/606, 49.01%), lumbar (174/606, 28.71%), and sacrococcygeal (39/606, 6.44%) vertebral segments. Patients with AA in cervical segments had a higher post-surgery recovery rate (p = 0.016) compared to that of other segments. The common pathological diagnoses of SAA were AA (80.82%), AA combined with arachnoid cyst (12.79%), arachnoid calcification/scars (3.43%), and arachnoid web/fibrosis (2.97%). Patients with AA were more likely to develop syringomyelia, compared with patients with other forms of SAA (p < 0.001). Animal studies mainly focused on new AA therapeutic agents (n = 14), the pathomechanism of AA (n = 14), and the development of new MRI sequences for improved diagnosis (n = 1). CONCLUSIONS: The pathological consequences of SAA are more complex than AA and manifest in different forms, such as AA combined with arachnoid cyst, arachnoid calcification/scars, and arachnoid web/fibrosis. In many instances, AA was associated with secondary syringomyelia. Unspecific clinical manifestations of SAA may easily lead to misdiagnosis and missed diagnosis. Although SAA may result from multiple etiologies, including spinal trauma, meningitis, spinal surgery, and hemorrhage, the pathogenesis and treatment of SAA have still not been standardized.

Chiari Formation or Malformation? Trends in the Pathophysiology and Surgical Treatment of an Ever-Elusive Entity.

Background: Chiari malformation type 1 (CM1) remains a complex neurosurgical condition with ongoing debate regarding its optimal management. Methods: This narrative review examines key controversies surrounding the pathophysiology, surgical indications, and treatment strategies for CM1. Results: We highlight the challenges posed by the wide spectrum of CM variants and the evolving understanding of its association with syringomyelia, basilar invagination, and craniovertebral instability. Emerging surgical techniques, including minimally invasive approaches and the use of new technologies such as endoscopes and exoscopes, are evaluated for their potential to improve outcomes. Recent consensus guidelines are also discussed. Conclusions: The need for individualized treatment plans for CM1 is emphasized, with special focus put on the connection between novel pathophysiological insights, technological advancements and opportunities for a more nuanced surgical management. Further research is necessary to establish solid foundations for more individualized treatments.

Pediatric Spinal Vascular Abnormalities: Overview, Diagnosis, and Management.

Hemangioblastomas are true benign vascular neoplasms arising from pluripotent mesenchymal stem cells that give rise to vascular endothelial cells and are most commonly found in the cerebellum, spinal cord, brainstem, and retina. These tumors may be isolated sporadic lesions or may be associated with hereditary genetic factors in the case of von Hippel-Lindau (VHL) syndrome. Spinal cord haemangioblastomas constitute 1.1% to 2.4% of all central nervous system tumors105, with the majority being single tumors that present in the fourth decade of life 106. In the pediatric population, sporadic spinal cord hemangioblastomas are exceedingly rare. The prevalence of spinal cord hemangioblastomas in children is increased among those with VHL syndrome. The thoracic cord is the most common site for spinal cord hemangioblastomas, followed by the cervical cord. Although these tumors are benign, they cause disabling symptoms due to spinal cord compression, syringomyelia, or hemorrhage from the tumor itself or from aneurysms that form on tumor-feeding arteries or intra-tumoral vessels.

Paragangliomas and syringomyelia in Tetralogy of Fallot-A case report and literature review.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries, paragangliomas, and syringomyelia are uncommon diseases. Furthermore, in the absence of any genetic link and with less than five reported adult patients surviving unrepaired rare form of Tetralogy of Fallot, our case shows noteworthiness. The possibility of definitive treatment of these conditions is rendered unsafe due to this persistent defect. Thus, management and ongoing survival of this patient remains complex and challenging.

Cerebrospinal fluid flow in small-breed dogs with idiopathic epilepsy observed using time-spatial labeling inversion pulse images: a preliminary study.

Cerebrospinal fluid (CSF) circulation diseases, such as hydrocephalus and syringomyelia, are common in small-breed dogs. In human patients with CSF circulation diseases, time-spatial labeling inversion pulse (time-SLIP) sequence performed to evaluate CSF flow before and after treatment allows visualization of the restoration of CSF movement. However, studies evaluating CSF flow using the time-SLIP method in small-breed dogs are limited. Therefore, the present study aimed to evaluate intracranial CSF flow on time-SLIP images in small-breed dogs with idiopathic epilepsy, as an alternative model to healthy dogs. Time-SLIP images were obtained at two sites: 1) the mesencephalic aqueduct (MA) area (third ventricle, MA, and brain-base subarachnoid space [SAS]) and 2) the craniocervical junction area (fourth ventricle, brainstem, and cervical spinal cord SAS) to allow subsequent evaluation of the rostral and caudal CSF flow using subjective and objective methods. In total, six dogs were included. Caudal flow at the MA and brain-base SAS and rostral flow in the brainstem SAS were subjectively and objectively observed in all and 5/6 dogs, respectively. Objective evaluation revealed that a significantly smaller movement of the CSF, assessed as the absence of CSF flow by subjective evaluation, could be detected in some areas. In small-breed dogs, the MA, brain-base, and brainstem SAS would be appropriate areas for evaluating CSF movement, either in the rostral or caudal flows on time-SLIP images. In areas where CSF movement cannot detected by subjective methods, an objective evaluation should be conducted.

Epidemiological aspects of syringomyelia in a 19-year old cohort of spinal cord injury patients.

OBJECTIVE: To determine the prevalence, clinical and radiological risk factors, and surgical management of post-traumatic syringomyelia (PTS) in a 19-year cohort study of Spinal Cord Injury (SCI) patients treated at a SCI rehabilitation center. METHODS: Retrospective study of SCI patients in whom PTS was radiologically confirmed between January 2000 and December 2018. Protocols for assessing signs and symptoms of PTS were applied prior to PTS diagnosis and treatment and later at neurosurgical and rehabilitation reviews. The variables analyzed were prevalence, demographic data, trauma event, clinical and radiological risk factors, location and size of the syrinx, and effectiveness of the surgical procedures. RESULTS: Over the 19-year period, review of 920 SCI patients revealed 85 patients who met the clinical and neuroradiological criteria for the diagnosis of PTS and who were prospectively followed. Road traffic accidents were the leading cause of injury (n = 58; 68.2%), syringomyelia was most commonly observed in the thoracic spine (n = 56; 65.9%), and upper extremity paresis was the most common indication for surgical treatment (n = 27; 45%). Surgical treatment was indicated in 48 patients and the operative procedures included 29 syringopleural shunts (60.4%), 17 adhesiolysis (35.4%), and two syringosubarachnoid shunts (4.1%). The prevalence of PTS was 9% and was higher in patients with ASIA impairment scale grade A injuries. Most patients with PTS (63/85, 74.1%) were treated surgically at the time of injury. There was a significant reduction both in the extent (p = 0.05) and largest area (p = 0.001) of the syrinx after surgical treatment. Reoperation rates were 47% and 37.9% for adhesiolysis and syringopleural shunting, respectively. CONCLUSION: Follow-up and routine clinical examination of SCI patients is critical for the diagnosis of PTS in patients with late neurological deterioration. Surgical treatment has a positive impact in reducing the size of the syrinx as seen on postoperative MRI.

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.

A case of sleep-disordered breathing presenting with increased intracranial pressure in a child with type 1 Chiari malformation.

Sleep-disordered breathing (SDB), with both central and obstructive sleep apneas, has been reported in association with Chiari malformation type 1 (CM1). CM1 is a congenital or acquired herniation of the cerebellar tonsils through the foramen magnum. In this case, a five-year-old girl with a history of CM1 and syringomyelia experienced worsening intracranial pressure (ICP) secondary to SDB. This case highlights the importance of early recognition of sleep related respiratory disorders in patients with Chiari malformation and its association with increased intracranial pressure.

Preoperative Predictors of Foramen Magnum Decompression with Dural Splitting for Amelioration of Syringomyelia Associated with Chiari Type 1 Malformation.

OBJECTIVE: Among several procedures for syringomyelia associated with Chiari type 1 malformation (CM-1), foramen magnum decompression (FMD) with dural splitting is one of the treatment choices with low complication rates. However, some meta-analyses have suggested that FMD with dural splitting may be inferior to FMD with duraplasty based on clinical outcomes; therefore, a predictor of a good surgical outcome with dural splitting is essential. This study aimed to clarify the preoperative parameters for good outcomes in patients with FMD with dural splitting, including the volumetric perspective. METHODS: We reviewed the cases of patients diagnosed with CM-1 and syringomyelia who underwent FMD with dural splitting at our institution. We included patients who underwent magnetic resonance imaging from 6 months to 1 year after surgery and measured radiological parameters using preoperative computed tomography and preoperative/follow-up magnetic resonance imaging. RESULTS: Thirty-one patients were enrolled. Among all radiological parameters, the volume of the herniated tonsils (Vtonsil) in improved group was significantly smaller, and the difference between the expected volume increment and Vtonsil (Vincr-Vtonsil) in improved group was significantly larger than those in non-improved group. To predict the improvement of the syrinx, Vincr-Vtonsil of 0.77 mL was the optimal cutoff value and yielded 100% sensitivity and 48.0% specificity. CONCLUSIONS: Vtonsil and Vincr-Vtonsil, which are novel predictors based on the volumetric perspective, might be the optimal predictors for improvement of the syrinx associated with CM-1 by 1 year after surgery.

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.

Clinical analysis of atlanto-occipital decompression in the management of chiari malformation with multi-segmental syringomyelia.

Background: Chiari malformation type I with syringomyelia (CM-I-S) is a very common disease in neurosurgery. There are also various surgical methods. But it is controversial. There are few reports about children, especially the treatment of multi-segmental beaded syringomyelia. The purpose of this study was to explore the clinical effects of atlanto-occipital decompression (AOD) in the management of Chiari malformation type I (CM-I) with multi-segmental beaded syringomyelia (MSBS) in pediatric patients. Methods: This retrospective study were pediatric patients with CM-I combined with MSBS who were treated in our hospital from January 2015 to December 2023. The patients who received the AOD treatment were screened according to inclusion and exclusion criteria. Outcomes were assessed by comparison of pre- and postoperative clinical, Chicago Chiari Outcome Scale (CCOS), the diameter and volume of the syringomyelia, morphological parameters of posterior cranial fossa (cervical spinal cord angle of medulla oblongata, CSC-MO) and complications in the enrolled children. Results: This study ultimately included 21 eligible pediatric patients with CM-I and MSBS. All the patients successfully completed the operation, which consists of atlanto-occipital decompression, partial resection of the posterior arch of the atlas, electrocoagulation of the cerebellar tonsil, pseudomembrane resection of the central canal orifice (latch) of the spinal cord, and artificial dura mater expansion repair. No death, no relapses, no serious neurological dysfunction and other complications. At the last follow-up, the clinical symptoms of all patients basically disappeared. The results of magnetic resonance imaging (MRI) showed that the average preoperative cerebellar tonsillar hernia was 12.4 ± 4.6 mm, and the postoperative were all above the foramen magnum. The average preoperative syringomyelia volume was 11.7 ± 3.7 cm2, and the syringomyelia disappeared or significantly shrunk after operation, with the volume unable to be accurately measured. The median preoperative CSC-MO was 132.5°, and the median postoperative CSC-MO was 150°, with a significant difference. The median preoperative pain and non-pain score of CCOS was 4 and 3 respectively. The symptoms disappeared after operation, and the score was 4. Only 5 children had cerebrospinal fluid leakage. Conclusion: The AOD is safe and effective in CM-I with MSBS in pediatric patients. Pseudomembrane resection of the central canal orifice (latch) of the spinal cord is crucial for the treatment of syringomyelia.

Syringomyelia-induced wrist Charcot neuroarthropathy.

We present a rare case of wrist Charcot neuroarthropathy secondary to post-tuberculosis syringomyelia.Level of evidence: V.

Comprehensive comparative study of Chiari-like malformation in veterinary and human medicine.

This review aims to enrich our understanding of Chiari-like malformation (CLM) by combining human and veterinary insights, and providing a detailed cross-species overview. CLM is a developmental abnormality characterised by caudal displacement of the hindbrain into the foramen magnum due to an entire brain parenchymal shift caused by insufficient skull volume. This malformation leads to a progressive obstruction at the craniocervical junction, which disrupts the normal cerebrospinal fluid flow, leading to secondary syringomyelia. The clinical signs of CLM and syringomyelia include phantom scratching, head tilt, head tremor, ataxia, tetraparesis, pain, muscle atrophy, and scoliosis or torticollis. Magnetic resonance imaging remains the gold standard for diagnosing CLM, since it allows the visualisation of abnormal findings such as the caudal cerebellar herniation, caudal cerebellar compression from occipital dysplasia, and attenuated cerebrospinal fluid cisternae. Although various medical and surgical interventions, including foramen magnum decompression, can provide temporary symptomatic/clinical sign relief, current literature shows a lack of sustained long-term efficacy. Therefore, additional research is needed to evaluate the long-term effects of existing treatment strategies and to compare different techniques utilised in conjunction with foramen magnum decompression.

Optimizing Therapeutic Strategies for Syringomyelia Associated with Tethered Cord Syndrome: A Comprehensive Review.

This review highlights the need for therapeutic guidelines for syringomyelia associated with tethered cord syndrome (TCS) caused by spinal dysraphism (SD). A comprehensive literature review was conducted, selecting twelve articles to analyze common therapeutic strategies. Surgical cord untethering alone has recently become a preferred treatment, with 45 ± 21.1% of patients experiencing remission or improvement, 47 ± 20.4% unchanged and asymptomatic, and 4 ± 8% worsened. Untethering with direct surgical drainage for the syrinx had better outcomes than untethering alone (78% vs. 45%, p = 0.05). Terminal syringostomy was beneficial for syrinxes extending to the filum terminale but not for asymptomatic small syrinxes with a syrinx index < 0.4. Syrinx shunting was recommended for symptomatic large syrinxes (>2 cm in length and syrinx index > 0.5). Various shunt procedures for syrinxes are still advocated, mainly for refractory syringomyelia in Chiari malformation, posttraumatic cases, SD, or other causes. Personalized surgical methods that address the root cause of syringomyelia, particularly those improving cerebrospinal fluid flow, offer promising results with minimized complications. Ongoing studies are required to enhance management strategies for syringomyelia associated with TCS, optimize patient outcomes, and reduce the risk of recurrent symptoms.

Cauda equina syndrome due to posttraumatic syringomyelia in conus medullaris - A case report.

Background: Most posttraumatic syringomyelias occur in the cervical or thoracic spinal cord, where they contribute to myelopathic deficits. Here, a 40-year-old patient presented with the left leg monoparesis due to syringomyelia involving the conus medullaris 10 years after an L2 vertebral "crush" fracture. Case Description: Ten years following an L2 vertebral "crush" fracture, a 40-year-old male presented with the new onset of left lower leg paresis. The magnetic resonance imaging showed a T12-L1 syrinx associated with accompanying high-intensity areas above the syrinx located between the T11 and T12 levels. One month after placing a syringosubarachnoid (SS) shunt, both the syrinx and high-intensity area rapidly disappeared, and the left distal motor weakness resolved. Conclusion: Ten years following an L2 "crush" fracture, a 40-year-old male presented with the new onset of a cauda equina syndrome secondary to a posttraumatic T12-L1 syringomyelia causing expansion of the conus medullaris.

Charcot Neuroarthropathy of the Shoulder Caused by Cervical Spondylotic Myelopathy: A Case Report and Literature Review.

Charcot neuroarthropathy (CN) is a chronic degenerative disorder of bones and joints, mostly associated with diabetes mellitus and human immunodeficiency virus. CN of the upper limb is rare, with only 58 case reports identified on PubMed with the majority of cases being closely associated with syringomyelia. Very rarely, cervical spondylotic myelopathy (CSM) is associated with CN of the upper limb; with very few literature reporting this association. This case report presents a rare case of Charcot arthropathy of the shoulder caused by CSM. A 57-year-old female presented to the emergency department following trauma to the right shoulder. On clinical examination, there was evidence of tenderness, extensive swelling, and bruising with a lack of range of motion along with numbness in the right arm and legs. Through radiographic and laboratory investigations, a diagnosis of CN secondary to CSM was made. A reverse total shoulder arthroplasty was performed however, this was complicated at two weeks with an atraumatic glenoid fracture and dislocation. First-stage revision surgery was then performed to allow fracture healing pending second-stage revision surgery. This report provides insight into the very rare possibility of the association of CN of the shoulder with CSM. A review of the literature suggests reverse shoulder arthroplasty is the gold standard for cases of severe bone and soft tissue damage. When undergoing investigations for Charcot neuroarthropathy, physicians must undertake a full detailed history along with a detailed neurological examination and imaging of the cervical spine to not miss the association with CSM.

C1-C2 Fixation for Chiari with Syringomyelia Associated with Atlantoaxial Instability, Atlantooccipital Assimilation and an Anomalous Vertebral Artery Course.

Chiari malformations, characterized by the herniation of cerebellar tonsils through the foramen magnum, are complex neurosurgical conditions that pose significant diagnostic and therapeutic challenges.1 Traditional interventions, such as foramen magnum decompression, have been the mainstay of treatment but are not universally effective, especially in cases with underlying atlantoaxial instability. However, recent studies highlight the importance of atlantoaxial instability in their pathogenesis, shifting the therapeutic focus towards atlantoaxial fixation.2 Research shows that atlantoaxial fixation can effectively address the underlying instability, leading to better symptomatic relief and neurological outcomes compared to traditional decompression methods.3,4-17 The findings from these studies consistently demonstrate that atlantoaxial instability is a critical factor in the development of Chiari malformations. Atlantoaxial fixation not only resolves the mechanical instability but also leads to significant symptomatic relief and improved neurological outcomes. Comparative analysis indicates superior efficacy of atlantoaxial fixation over traditional decompression techniques, especially in patients with concurrent basilar invagination and syringomyelia. This surgical Video 1 illustrates a case of a 20-year-old male patient with tonsillar herniation, syringomyelia, atlantooccipital assimilation, atlantoaxial instability, basilar invagination and dorsolumbar scoliosis, who was successfully managed with cervical fixation using a bilateral C1 lateral mass and C2 transpedicular screw construct. This surgery was able to achieve clinical and radiologic improvement without the need for a foramen magnum decompression. The patient consented to the procedure and to the publication of his images.

Syringosubarachnoid shunting with augmented reality navigation through a keyhole laminectomy of the upper thoracic spine: illustrative case.

BACKGROUND: A syringosubarachnoid (SS) shunt combined with keyhole hemilaminectomy is a beneficial procedure that can reduce the size of the skin incision and the risk of complications. However, ingenuity is needed to confirm the position of the syrinx during surgery. The authors present a case in which they treated syringomyelia in the upper thoracic spine using augmented reality (AR) to confirm syrinx formation, bone resection, and skin incision. OBSERVATIONS: Microscope-based AR was an appropriate and practical choice in this case. By placing the reference array at the Mayfield clamp, it was possible to use AR from the point of skin incision. Under AR navigation, an SS shunt tube can be placed in the short syrinx. LESSONS: AR navigation enables pinpoint SS shunt tube insertion with minimal skin incision and bone resection. It is particularly useful for upper thoracic and small syrinx lesions. https://thejns.org/doi/10.3171/CASE24130.