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.

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 (MRI) from 6 months to 1 year after surgery, and measured radiological parameters using preoperative computed tomography and preoperative/follow-up MRI. RESULTS: Thirty-one patients were enrolled. Among all radiological parameters, the volume of the herniated tonsils (Vtonsil) and the difference between the expected volume-increment and Vtonsil (Vincr-Vtonsil) in improved groups were significantly smaller than those in non-improved groups. 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.

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.

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.

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.

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.

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.

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.

Surgical Outcomes in Chiari 1 and Chiari 1.5 Malformation Treated by Posterior Fossa Reconstruction: A Comprehensive Analysis of 110 Pediatric Cases and Literature Review.

Background/Objectives: The management of Chiari malformations (CMs) remains a clinical challenge and a topic of great controversy. Results may vary between children and adults. The purpose of the current single-center study is to critically assess the one-year surgical outcomes of a cohort of 110 children with CM-1 or CM-1.5 who were treated using "posterior fossa reconstruction" (PFR), a surgical technique described in 1994 that has since been used in both adults and children. We also review the literature and discuss the possible causes of the drawbacks and pitfalls in children in whom PFR was ineffective in controlling the disease. Methods: The present cohort was selected from a prospective registry of adults and children with CMs collected since 2006. Patients included in this study were selected from a group of children with CMs who were operated on in our Pediatric Neurosurgical Unit between 1 January 2007 and 31 November 2023. Surgical outcome was defined based on clinical and neuroradiological results as very good, good, or bad. Results: The mean age of our child cohort was 9.9 ± 4.7 years, with 54 girls (49%) and 56 boys (51%). Sixty-six children had CM-1 (60%) while forty-four had CM-1.5 (40%). Following surgery, there was no neurological worsening or death among the children. Most children (70%) had an uneventful recovery and were discharged home on average one week after surgery. However, in 33 children (30%), we recorded at least one postoperative adverse event. Aseptic meningitis syndrome was the most frequent adverse event (n = 25, 22.7%). The final surgical outcome was evaluated one year after PFR by using both clinical and neuroradiological results. The one-year surgical outcome was excellent in 101 children (91.9%), good in 5 (4.5%), and bad in 4 (3.6%). Conclusions: PFR significantly enlarges the volume of the posterior fossa and recreates a CSF environment that generates buoyancy of the cerebellum, with a high percentage of excellent and good clinical results evaluated one year post-surgery.

Prevalence of neurological diseases associated with cervical pain and/or signs of cervical myelopathy in French bulldogs: a retrospective analysis of 105 cases.

Introduction: French bulldogs can be affected by several neurological diseases, with myelopathies representing the most frequent cause of neurological signs. Studies focusing on the epidemiology of cervical diseases in this breed are lacking. This study aims to describe the prevalence of neurological pathologies responsible for cervical pain and/or signs of cervical myelopathy, assuming that intervertebral disc herniation represents the most common disease; a second aim was to evaluate how often different pathologies coexist in this spinal region in French bulldogs. Materials and methods: A retrospective analysis of medical records from the database of a single referral center (AniCura I Portoni Rossi Veterinary Hospital-Zola Predosa, Bologna, Italy) was performed, including French bulldogs presented for neck pain and/or neurological deficits consistent with cervical myelopathy. Clinical and imaging data were reviewed and used as inclusion criteria. Based on the number of MRI-diagnosed diseases, the eligible dogs were divided into three groups. Results: One hundred five French bulldogs met the inclusion criteria. The most commonly diagnosed condition was an intervertebral disc herniation (66.9%), followed by C2 idiopathic hypertrophic ganglioneuritis (15.1%), cervical syringomyelia (11.5%), congenital osseous malformations (1.4%), spinal arachnoid diverticula (1.4%), neoplasms (1.4%), steroid-responsive meningitis-arteritis (0.7%), traumatic vertebral fractures (0.7%), and other (0.7%). For the vast majority of dogs (75/105) a single pathology was diagnosed, with intervertebral disc herniations accounting for 86.7% of cases, involving C3-C4 IVD most commonly. In the remaining 30 dogs, two or three different and concurrent diseases were detected. Among these 30 dogs, intervertebral disc herniations still remained the most diagnosed condition, in combination with C2 idiopathic hypertrophic ganglioneuritis and syringomyelia in 19 and seven cases, respectively. Conclusion: The results of this study highlight that different pathologies can affect the cervical spinal cord in French bulldogs, with intervertebral disc herniations representing the most frequent condition, as previously described in the veterinary literature. In almost a third of cases, different pathologies can coexist at the cervical level. However, for cases in which different pathologies are present at the same time, it is not always possible to clearly establish their clinical significance.

A Case Report of Neuropathic Arthropathy in Chronic Syrinx Raising Suspicion of Malignancy.

Syringomyelia is a prevalent cause of Charcot arthropathy, notably affecting the elbow and less frequently the shoulder. Before attributing neuropathic arthropathy (NA) to a syrinx, careful investigation of various potential causes is vital. We present a unique case of NA affecting the left shoulder, secondary to a longstanding syrinx presenting as an expansile mass on imaging, raising suspicion of malignancy. The patient presented with progressive left arm swelling, limited mobility, and a history of chronic left shoulder pain. Through clinical evaluation and imaging, including X-rays and CT scans, significant bone destruction and a large fluid-filled mass in the left shoulder were observed. Laboratory tests ruled out other potential diagnoses, and a bone biopsy excluded malignancy. This study emphasizes the importance of thorough differential diagnosis and appropriate imaging techniques to distinguish NA from other conditions. The diagnosis of NA relies on a comprehensive assessment involving clinical signs, symptoms, radiological imaging, and additional tests aimed at excluding other potential causes, including soft tissue tumors. Management strategies, including conservative approaches and surgical interventions like neurosurgical decompression and shoulder arthroplasty, are discussed. The study sheds light on the challenges in diagnosing and managing NA associated with syringomyelia and emphasizes the significance of a multidisciplinary approach for optimal outcomes.

Cervical syringomyelia associated with cervical disc disease.

Background and purpose:

Syringomyelia is a neurological condition in which a longitudinal fluid-filled cavity is formed within the spinal cord. It usually occurs in the cervical region and is associated with Chiari malformation, infections, trauma, and tumors of the spinal cord. However, syringomyelia associated with cervical disc disease (SCD) is very rare and only a few cases have been reported so far. This case report presents the clinical and radiological findings of 13 cases of SCD to describe the properties of SCD and explore the nature of the relationship between syringomyelia and cervical disc disease.

SCD was diagnosed in 13 using MRI findings, including coexistence of syringomyelia and cervical disc disease, presence of narrowed cervical subarachnoid space secondary to the cervical disc herniation or cervical local kyphosis associated with cervical disc degeneration or herniation, and the cervical disc herniation or segmental kyphosis and syrinx should be located within the same levels. The MRI findings were used to grade the syrinx and determine whether the cervical disc herniation or local kyphosis was located at the proximal or distal end of the syrinx.

All patients had single-level disc herniation or kyphosis, the most common level being C5–6 (n = 6), followed by C6–7 (n = 4) and C4–5 (n = 3). Eight patients had a distal type (disc disease located in the proximal end of the syrinx) SCD while five had the proximal variety (cervical disc disease located in the distal end of the syrinx). The average length of the syrinx was two vertebral segments. Surgery was performed in five cases and some degree of syrinx resolution was observed in all of them.
Discussion – The main cause of syringomyelia is obstruction of cerebrospinal fluid (CSF) pathways; total obstruction could cause distal syrinx, whereas partial obstruction could cause proximal or distal syrinxes. Restoration of CSF pathways may result in some degree of resolution of syringomyelia. A causal association may exist between cervical disc disease and cervical syringomyelia but needs further exploration.

SCD is a mild form of syringomyelia with symptoms primarily arising due to disc herniation or local kyphosis. The surgical treatment of the cervical disc disease is sufficient and results in a syringomyelia resolution of some degree.

Combined Chiari Malformation Type I and Syringohydromyelia in a Patient With Intractable Headache.

Chiari malformations (CM) are a spectrum of hindbrain abnormalities involving the cerebellum, brainstem, skull base, and cervical cord. The most common is Chiari I malformation, in which the cerebellar tonsils descend through the foramen magnum. As opposed to types II-IV, which are congenital, type I can manifest in late childhood or adulthood with headaches and focal neurological symptoms. It can be caused by genetic variation, conditions that alter the basal skull or increase intracranial pressure, and even injury. Syringohydromyelia (SHM) is a neurological disorder characterized by longitudinal dilation of the central canal of the spinal cord with accumulated cerebrospinal fluid. This case report demonstrates a 35-year-old male with headaches, neck pain, back pain, and paresthesias who was found to have CM type-I malformation and syringohydromyelia.

Surgical Management of Chiari Malformation Type I in the Pediatric Population: A Single-Center Experience.

Background: Chiari malformation type 1 (CM-1) involves the cerebellar tonsils' descent below the foramen magnum. In Chiari malformation type 1.5 (CM-1.5), both the cerebellar tonsils and the brainstem are herniated. Common symptoms include headaches and cervical pain, often associated with conditions like syringomyelia and hydrocephalus. Surgical treatment is not performed in asymptomatic patients, while the presence of syringomyelia represents an indication for surgery. Methods: This study retrospectively examined pediatric patients with CM-1 and CM-1.5 at Giannina Gaslini Hospital from 2006 to 2020, analyzing demographics, radiological findings, surgical interventions, and outcomes. Results: Out of 211 patients who underwent surgery, 83.9% were diagnosed with CM-1 and 16.1% with CM-1.5. Headaches were prevalent (69%) and cerebellar signs were noted in 29% of patients. Syringomyelia and hydrocephalus were present in 28.4% and 8% of cases, respectively. Intraoperative ultrasonography guided interventions, with 59.8% requiring bony and ligamentous decompression, and 27.1% undergoing duraplasty. Conclusions: The surgical treatment of CM-1/CM-1.5 involves posterior cranial fossa decompression. Choosing between bony decompression alone and its combination with duraplasty has always been controversial in the pediatric population. If we consider as surgical endpoint the restoration of cerebrospinal fluid (CSF) flux, intraoperative ultrasound may be a real-time helpful tool in orienting the surgical strategy, yet refinement with quantitative measures is needed.

Upper end of the central canal of the human spinal cord: Quantitative anatomical study and 3D modeling.

The upper end of the central canal of the human spinal cord has been repeatedly implicated in the pathogenesis of various diseases, yet its precise normal position in the medulla oblongata and upper cervical spinal cord remains unclear. The purpose of this study is to describe the anatomy of the upper end of the central canal with quantitative measurements and a three-dimensional (3D) model. Seven formalin-embalmed human brainstems were included, and the central canal was identified in serial axial histological sections using epithelial membrane antigen antibody staining. Measurements included the distances between the central canal (CC) and the anterior medullary fissure (AMF) and the posterior medullary sulcus (PMS). The surface and perimeter of the CC and the spinal cord were calculated, and its anterior-posterior and maximum lateral lengths were measured for 3D modeling. The upper end of the CC was identified in six specimens, extending from the apertura canalis centralis (ACC) to its final position in the cervical cord. Positioned on the midline, it reaches its final location approximately 15 mm below the obex. No specimen showed canal dilatation, focal stenosis, or evidence of syringomyelia. At 21 mm under the ACC in the cervical cord, the median distance from the CC to the AMF was 3.14 (2.54-3.15) mm and from the CC to the PMS was 5.19 (4.52-5.43) mm, with a progressive shift from the posterior limit to the anterior third of the cervical spinal cord. The median area of the CC was consistently less than 0.1 mm2. The upper end of the CC originates at the ACC, in the posterior part of the MO, and reaches its normal position in the anterior third of the cervical spinal cord less than 2 cm below the obex. Establishing the normal position of the upper end of this canal is crucial for understanding its possible involvement in cranio-cervical junction pathologies.