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.

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UNASSIGNED:

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.

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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.

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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.

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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.

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A syringomyelia olyan neu­rológiai állapot, amelyben a gerincvelő­ben egy folyadékkal teli, hosszanti üreg ke­let­ke­zik. Általában a nyaki régióban fordul elő, és Chiari-malformációval, fertőzésekkel, trau­mával és a gerincvelő daganataival hoz­ható összefüggésbe. Mindazonáltal, a nya­ki porckorongbetegséggel társuló sy­rin­go­myelia (syringomyelia with cervical disc dis­ease, SCD) nagyon ritka, és eddig csak né­hány esetről számoltak be. Ez az esetismer­tetés 13 SCD-s eset klinikai és radiológiai le­leteit mutatja be, hogy leírja az SCD tulaj­don­ságait, és feltárja a syringomyelia és a nyaki porckorongbetegség közötti kapcsolat természetét.

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UNASSIGNED:

Tizenhárom, MRI-leletek alap­ján SCD diagnózist kapott esetről szá­molunk be; az esetek között van olyan, ami­kor a sy­rin­go­myelia és a nyaki porc­ko­rong­betegség együttesen fordult elő, van olyan, amikor a nyaki porckorongsérvhez vagy a nyaki porckorong-degenerációhoz társuló nyaki kyphosis miatt másodlagosan alakult ki a cervicalis subarachnoidealis tér szűkülete, valamint olyan, amikor a nyaki porckorongsérv vagy a szegmentális kyphosis és a syrinx ugyanazon a szinten belül helyezkedik el. Az MRI-leletek alapján osztályoztuk a syrinxet, és meghatároztuk, hogy a nyaki porckorongsérv vagy a helyi kyphosis a syrinx proximális vagy distalis végén található.

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Minden betegnél egyszin­tű porckorongsérv vagy kyphosis volt je­len. A leg­gyakoribb szint a C5–6 (n = 6), majd a C6–7 (n = 4) és a C4–5 (n = 3) volt. Nyolc betegnél az SCD distalis típusú volt (a sy­rinx proximális végén elhelyezkedő porc­ko­rong­betegség), míg ötnél a proximális változat for­dult elő (a syrinx distalis végén elhelyezkedő nyaki porckorongbetegség). A syrinx átlagos hossza két csigolyaszegmens volt. Öt esetben végeztek műtétet, és mindegyiküknél megfigyelhető volt a syrinx bizonyos fokú feloldódása.
Megbeszélés – A syringomyelia fő oka a ce­­reb­ro­spinalis folyadék (CSF) járatai­nak elzá­ródása; a teljes elzáródás dis­ta­­lis syrinx, míg a részleges elzáródás proxi­mális vagy distalis syrinx kialakulását okoz­hatja. A liquor­közlekedés helyre­ál­lí­tása a syringomyelia bizonyos fokú meg­szűnését eredményezheti. A nyaki porckorongbetegség és a nyaki sy­rin­go­mye­lia között ok-okozati összefüggés állhat fenn, de ennek igazolása további vizsgálatokat igényel.

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Az SCD a syringomyelia enyhe formája, aminek a tünetei elsősorban porckorongsérv vagy helyi kyphosis miatt jelentkeznek. A nyaki porckorongbetegség műtéti kezelése elegendő, és a syringomyelia bizonyos fokú megszűnését eredményezi.

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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.

UNASSIGNED: Currarino syndrome (CS) is a rare congenital syndrome characterized by a triad of anorectal malformation, sacral deformity, and presacral mass. In about 50% of cases, it is caused by HLXB9 gene mutation in chromosome 7q36. A 13-month-male child presented with presacral discharging sinus with a history of surgery for anorectal malformation and perineal fistula at the time of birth. On detailed investigation, the child revealed to have anal atresia, hemisacrum, and presacral mass. Histopathology of presacral mass showed features of immature teratoma. The presacral mass in CS is mostly an anterior myelomeningocele or presacral teratoma. The development of immature teratoma in presacral mass is very rare. The histopathological identification of immature component of teratoma in the presacral mass of CS is important for risk stratification and further management. Suspicion of CS should be raised in any child presenting with partial phenotype of the triad.

UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.

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.

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.

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.

OBJECTIVE: There is still controversy about whether it is necessary to perform prophylactic neurosurgical decompression for severe scoliosis (SS) with syringomyelia (SM) to reduce the risk of neurological complications during subsequent spinal correction. This study aimed to explore the safety and effectiveness of using traction-assisted single-stage spinal correction as a treatment for patients who had SS with SM (SS-SM).
METHODS: The patients who had SS-SM without previous neurosurgical intervention and who underwent traction-assisted single-stage posterior spinal correction at a single center were included, and the initial, posttraction, and postoperative clinical data were reviewed. Based on preoperative MRI, the included patients were divided into two categories: those with versus those without Chiari malformation type I (CM-I-related SM [CS] vs idiopathic SM [IS]), and those with a moderate syrinx (MS) versus those with a large syrinx (LS). Different groups\' traction and operation contributions were calculated for comparisons (CS vs IS, MS vs LS).
RESULTS: A total of 28 patients were included. The initial mean major scoliosis was 101.0° with a mean flexibility of 21.4%. After the operation, the mean total correction rate for scoliosis was 63.9%. The mean traction and operation contributions were 61.5% and 38.5%, respectively. Most of the patients (75%) underwent spinal corrections without 3-column osteotomies, and only 1 patient reported postoperative regional numbness without motor deficits. No differences were found in the mean total correction rates, traction, and operation contributions when comparing CS versus IS and MS versus LS with the comparable initial clinical data (p > 0.05). More than 50% of the total corrections were achieved by preoperative traction in all groups.
CONCLUSIONS: Traction-assisted single-stage spinal correction can safely and effectively correct SS-SM without prophylactic neurosurgical decompression under strict patient selection. Additionally, traction can achieve more than half of the final spinal correction, even for patients with varying sizes of SMs.