BACKGROUND: 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.
METHODS: 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.
CONCLUSIONS: This case highlights the rare complication of CSF overdrainage from syrinx shunting and the importance of shunt selection considerations.

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.

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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.

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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.

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.
METHODS: 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.
CONCLUSIONS: 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.

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.

BACKGROUND: Central neuropathic pain after foramen magnum decompression (FMD) for Chiari malformation type 1 (CM-1) with syringomyelia can be residual and refractory. Here we present a case of refractory central neuropathic pain after FMD in a CM-1 patient with syringomyelia who achieved improvements in pain following spinal cord stimulation (SCS) using fast-acting sub-perception therapy (FAST™).
METHODS: A 76-year-old woman presented with a history of several years of bilateral upper extremity and chest-back pain. CM-1 and syringomyelia were diagnosed. The pain proved drug resistant, so FMD was performed for pain relief. After FMD, magnetic resonance imaging showed shrinkage of the syrinx. Pain was relieved, but bilateral finger, upper arm and thoracic back pain flared-up 10 months later. Due to pharmacotherapy resistance, SCS was planned for the purpose of improving pain. A percutaneous trial of SCS showed no improvement of pain with conventional SCS alone or in combination with Contour™, but the combination of FAST™ and Contour™ did improve pain. Three years after FMD, percutaneous leads and an implantable pulse generator were implanted. The program was set to FAST™ and Contour™. After implantation, pain as assessed using the McGill Pain Questionnaire and visual analog scale was relieved even after reducing dosages of analgesic. No adverse events were encountered.
CONCLUSIONS: Percutaneously implanted SCS using FAST™ may be effective for refractory pain after FMD for CM-1 with syringomyelia.