Successful spinal cord stimulation using fast-acting sub-perception therapy for postoperative neuropathic pain of syringomyelia with Chiari malformation type 1: a case report and literature review.

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™). CASE PRESENTATION: 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. CONCLUSION: Percutaneously implanted SCS using FAST™ may be effective for refractory pain after FMD for CM-1 with syringomyelia.

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.

Delayed spinal arachnoiditis with syringomyelia following aneurysmal subarachnoid haemorrhage: a case report with patient experience.

BACKGROUND AND IMPORTANCE: Syringomyelia, or the formation of fluid-filled cysts within the spinal cord, associated with delayed spinal arachnoiditis is an uncommon complication of aneurysmal subarachnoid haemorrhage. To date, about 18 cases have been reported in medical literature, with just two reported in patients under the age of 35 years. CLINICAL PRESENTATION: A 27-year-old female patient complained of sudden, severe headaches in the occipital region, nuchal rigidity, and drowsiness when she presented at our institution. A head computed tomography scan revealed intraventricular bleeding in the lateral and fourth ventricles with more extensive haemorrhaging in the frontal horns. A left posterior inferior cerebellar artery (PICA) aneurysm was confirmed via digital subtraction angiogram, and endovascular embolization was done. Two years later, the patient reported intense pain in the lower back along with symptoms suggestive of spinal cord compression. Spinal magnetic resonance imaging (MRI) showed spinal adhesions from C1 to L4, syringomyelia with some vasogenic oedema extending from T3 to T9 level, and a cyst in the lumbar region. Consequently, a right hemilaminectomy was performed along with microsurgical release of arachnoid adhesions and placement of a subdural drain. Radiological and symptomatic improvements were observed. Since then, the patient's clinical condition has remained stable during the past three years of follow-up visits. CONCLUSIONS: Literature on optimal treatment modalities and patient prognosis is scarce and debated. The time for symptom improvement depends on the level and extent of spinal cord involvement. Rehabilitation may be required for most patients, as complete symptomatic recovery may not be attainable.

Predicting the presence of 4th ventricular outlet obstruction in Chiari I Malformation.

INTRODUCTION: A subset of children with Chiari 1 malformation (CM-1) have a 4th ventricle arachnoid veil-a thin membrane covering the outlet of the 4th ventricle. Studies suggest that failure to disrupt this veil during posterior fossa decompression can reduce the likelihood of syringomyelia resolution. However, there is no reliable method for predicting the presence of the veil without direct surgical exploration. This study aims to evaluate the association between pre-operative symptoms, radiographic measurements, and the arachnoid veil. METHODS: A retrospective review of an institutional database of children evaluated for CM-I was conducted. For patients treated with surgery, operative notes were reviewed to determine if an arachnoid veil was present. Logistic regression was used to test for relationship of clinical variables and radiographic measurements with the presence of an arachnoid veil. RESULTS: Out of 997 children with CM-1, 226 surgical patients were included in the analysis after excluding those with inadequate documentation. An arachnoid veil was found in 23 patients (10.2%). Larger syrinx, spinal canal, and thecal sac diameters were significantly associated with the presence of a veil, with odds ratios of 1.23 (95% CI 1.2-1.48; p = 0.03), 1.27 (95% CI 1.02-1.59; p = 0.03), and 1.35 (95% CI 1.03-1.77; p = 0.03), respectively. No significant associations were found with any signs or symptoms. CONCLUSIONS: Arachnoid veil was present in 10% of cases. Radiographic measurements indicating larger syrinx size were the only variables found to be significantly associated with an arachnoid veil. Exploration of the 4th ventricular outlet is recommended for CM-I decompression in the setting of expansile syringomyelia.

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.

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.

Clinical predictors of syringomyelia in Cavalier King Charles Spaniels with chiari-like malformation based on owners' observations.

BACKGROUND: Syringomyelia (SM) is a prevalent inherited developmental condition in Cavalier King Charles Spaniels (CKCSs) with Chiari-like malformation (CM), accompanied by a variety of clinical manifestations, including signs of neuropathic pain. Magnetic resonance imaging (MRI) is the gold standard in SM diagnosis. However, it is desirable to establish clinical predictors that can identify CKCSs with a large clinical syrinx that needs treatment, as some owners cannot afford or lack access to MRI. The aims of the study were to investigate owner-reported clinical signs of SM and clinical predictors of a large clinical syrinx, using predictive values of significant signs, individually and in combinations. Eighty-nine CKCSs participated in this retrospective study. Based on MRI diagnosis, dogs were distributed into three groups: CM without syrinx or with a maximum transverse width < 2 mm (n = 13), CM with small syrinx 2.00-3.99 mm (n = 26) and CM with large syrinx ≥4 mm (n = 50). A structured investigator-owner interview using a standardized questionnaire was used to collect data regarding clinical signs of CM and SM. The statistical tests Pearson's chi-square, Fisher's Exact and Spearman's rank order were used to assess the difference in owner-reported signs between groups. For signs with significant differences, positive and negative predictive values (PPV and NPV) were calculated. RESULTS: Following clinical signs were reported significantly more frequent in dogs with a large syrinx: phantom scratching, bilateral scratching of the neck or shoulder, aversion when that area is touched, or exacerbation of clinical signs when the dog is emotionally aroused. Each individual sign had a high PPV, indicative of a large clinical syrinx. The PPV increased further when the signs phantom scratching, aversion to touch to the head, neck or shoulder, and a preferred head posture during sleep were present in combination. CONCLUSIONS: Specific clinical signs can be used individually and in combination as clinical predictors of a large clinical syrinx in CKCSs with CM and SM. General practitioners can utilize this information to identify CKCSs with a large syrinx to initiate necessary treatment. This is particularly useful in cases where access to or affordability of an MRI diagnosis is limited.

Spinal cord schistosomiasis in a 6-year-old child with complete recovery after spine surgery and medical treatment: case report and discussion.

Spinal cord schistosomiasis is a rare and severe form of schistosomiasis. The prognosis is largely conditioned by early diagnosis and treatment. The authors present a case of spinal cord schistosomiasis complicated by spinal cord compression syndrome. This is the case of a 6-year-old patient who presented with febrile gastroenteritis followed by complete paralysis of both lower limbs of sudden onset following a brief stay in a village setting with notion of multiple baths at a stream. Spinal cord MRI revealed an enlarged spinal cord spanning D10 to D12 with heterogeneous contrast enhancement and a syrinx cavity above the lesion. Biological workup revealed an inflammatory syndrome. Treatment consisted of decompressive laminectomy with biopsy of the lesion and a syringo-subarachnoid shunt. Pathological analysis revealed fragments of central nervous system tissues with an infiltrate composed of lymphocytes, plasmocytes, and macrophages producing granulomatous foci lined with areas of necrosis in addition to a large contingent of polynuclear eosinophils, agglutinating around or covering in some places elongated ovoid structures, with relatively thick eosinophilic shells and presenting a terminal spur. Adjuvant treatment consisted of praziquantel and corticotherapy for 1 month. The evolution showed marked improvement in the neurological deficits. She now walks unassisted and has good sphincter control. Spinal cord schistosomiasis is rare in our context; its diagnosis is difficult. The treatment is both medical and surgical.

Management of Persistent Syringomyelia in Patients Operated for Chiari Malformation Type 1.

BACKGROUND: The treatment of persistent syringomyelia associated with Chiari malformation type 1 (CM1) is unclear. This study aims to evaluate the clinical and radiologic outcomes of syringo-subarachnoid shunt (SSS) as a treatment for persistent syringomyelia following posterior fossa decompression (PFD) for CM1. METHODS: Forty-nine cases treated for CM1 associated syringomyelia at a single center were analyzed, 17 of them undergoing reoperation due to persistent syrinx formation. The patients' demographic data, neurologic presentations, and radiologic results were analyzed, including cerebellar herniation, posterior fossa volume, the level at which the syrinx started and finished, the size and diameter of the syrinx. RESULTS: Seventeen patients underwent SSS placement, with 1 minor surgical complication (a cerebrospinal fluid leak) occurring and requiring revision. No morbidity or mortality was observed. Among these 17 reoperated patients, partial or complete resorption of the syrinx was observed in all cases. The results suggest that if the syrinx diameter is >10 mm at its thickest point, extends for more than 10 vertebrae, and starts from the upper cervical region and extends to the upper thoracic region, the syrinx may not regress after the first surgery and potentially predicting the need for a second operation before PFD. CONCLUSIONS: SSS placement for persistent syrinx following PFD for CM1 is a safe and effective surgical treatment method. These criteria may also help predict the need for a second surgery and the overall disease outcome for both the surgeon and patient.

Non-sedated fast spine magnetic resonance imaging in pediatric patients.

BACKGROUND: Traditional spine magnetic resonance imaging (MRI) protocols require sedation in young children and uncooperative patients. There is an increased interest in non-sedated pediatric MRI protocols to reduce risks associated with anesthetic agents and improve MRI access. OBJECTIVE: To evaluate the image quality of pediatric non-sedated fast spine MRI. MATERIALS AND METHODS: We retrospectively reviewed 69 pediatric non-sedated fast spine MRI exams performed in 57 patients. Two blinded readers provided image quality ratings for the evaluation of bones, cranio-cervical junction, cerebrospinal fluid (CSF) spaces, spinal cord, soft tissues, ligaments, and overall diagnostic quality on a 1-5 scale, and determined whether there was evidence of syringomyelia, abnormal conus medullaris position, or filum terminale abnormality. RESULTS: Mean patient age was 7.2 years (age range ≤ 1-17). Indications included syringomyelia (n=25), spinal dysraphism (n=4), combination of both syringomyelia and spinal dysraphism (n=8), and other miscellaneous indications (n=32). The inter-observer agreement ranged between moderate and very good for each variable (Cohen's weighted kappa] range=0.45-0.69). The highest image quality ratings were given to CSF spaces (mean image quality=3.5/5 ± 0.8) and cranio-cervical junction evaluations (3.5/5 ± 0.9). Overall diagnostic quality was worst in the <5 years group (P=0.006). Readers independently identified a cervical spinal cord syrinx in 6 cases, and 1 mm spinal cord central canal dilation in one case. Readers agreed on the position of the conus medullaris in 92% of cases (23/25 cases). CONCLUSION: Non-sedated pediatric spine MRI can be an effective diagnostic test to evaluate for spine pathology, especially syringomyelia, Chiari malformation, and conus medullaris anatomy.

[A Surgical Case of Currarino Syndrome with Syringomyelia].

Currarino syndrome is characterized by a triad of anorectal malformations, sacral defects, and presacral masses. Although it is not extremely rare, this report presents a surgical case of Currarino syndrome with syringomyelia and discusses related literature. The patient is a girl, aged 2 years and 2 months, who presented with marked constipation, chronic cystitis, and lower limb weakness. After examining the patient through magnetic resonance imaging, we diagnosed her with rectal compression due to meningocele and syringomyelia. The base of the meningocele was detached, and the spinal cord was untethered. One week after surgery, her lower limb weakness and constipation improved. Following up on symptoms and performing imaging is essential to determine a treatment plan for Currarino syndrome. (Received 28 February, 2023; Accepted 22 March, 2023; Published 1 August, 2023).

Spontaneous Unilateral Chiari I Secondary to Acquired Tonsillar Hypertrophy/Pseudomass With Syringomyelia in a Juvenile With Progressive Scoliosis.

Chiari type 1 malformation (CM-1) is a structural defect of the central nervous system in which part of the cerebellar tonsils descend below the level of the foramen magnum, sometimes with associated syringomyelia. Although Chiari malformations were traditionally believed to be congenital, several cases of acquired CM-1 with syringomyelia have been reported. Usually associated with repeat lumbar puncture, increased intracranial pressure, and craniocephalic disproportion, CM-1 in the absence of an underlying etiology is rare. We report a rare case of spontaneous idiopathic tonsillar hypertrophy causing unilateral CM-1 with syringomyelia associated with progressive scoliosis in a juvenile with a previously normal neonatal MRI brain and no known underlying pathology. A 9-year-old boy was found to have scoliosis at a routine well-child visit with progression indicated on radiographs 4 months later. Whole spine MRI was performed and showed a new CM-1 with globular, mass-like configuration of the descended right tonsil with otherwise normal tonsillar characteristics. Surgical decompression via suboccipital craniectomy and C1 laminectomy with duraplasty was performed with improvement illustrated on repeat MRI 3 months postoperatively. This rare case emphasizes the importance of routine MRI spine early in select patients with idiopathic scoliosis and illustrates the favorable outcomes noted after decompressive craniectomy.

Juvenile scoliosis as the first manifestation of syringomyelia associated with Chiari malformation type I.

BACKGROUND: Scoliosis is a spinal deformity that usually manifests as a structural curve determining a variable deformity of the trunk. According to some published series, 4 to 26% of scoliosis initially classified as idiopathic show neurological alterations when studied with nuclear magnetic resonance, such as syringomyelia and Chiari malformation, among the most frequent. Chiari malformations are characterized by descending herniation of the cerebellar tonsils, brainstem, and IV ventricle into the spinal canal. CASE REPORT: We present the case of a patient whose first symptomatic manifestation was early-onset scoliosis. The overlapping of some physical examination signs, such as postural lateralization and scoliosis, reinforced the active suspicion of neuroaxis alterations. CONCLUSIONS: Early childhood-onset scoliosis should raise a high degree of suspicion for association with neuro-spinal diseases. Although the frequency of Chiari malformation is low, its early approach could reduce the progression of associated comorbidities. An early detection could change the prognosis of the disease.

INTRODUCCIÓN: La escoliosis es una deformidad de la columna que usualmente se manifiesta como una curva estructural que determina una deformidad variable del tronco. Las malformaciones de Chiari se caracterizan por la herniación descendente de las amígdalas cerebelosas, tronco encefálico y IV ventrículo hacia el canal espinal. Según algunas series publicadas, del 4 al 26% de las escoliosis inicialmente catalogadas como idiopáticas muestran alteraciones neurológicas al ser estudiadas con resonancia magnética nuclear, como la siringomielia y malformación de Chiari, dentro de las más frecuentes. CASO CLÍNICO: Se presenta el caso de un paciente cuya primera manifestación sintomática fue escoliosis de comienzo temprano. El solapamiento de algunos signos del examen físico, como la lateralización de la postura y la escoliosis, reforzaron la sospecha activa de alteraciones del neuroeje. CONCLUSIONES: La escoliosis de inicio temprano en la infancia debe despertar un alto grado de sospecha de asociación con enfermedades neuro-espinales. Dentro de este contexto, aunque malformación Chiari es de baja frecuencia, su abordaje precoz permitiría disminuir la progresión de comorbilidades asociadas. La detección temprana podría cambiar el pronóstico de la enfermedad.

Acute presentation of Chiari malformation type I in adulthood with arm swelling, a case report and literature review.

INTRODUCTION: Chiari malformation type I (CM-I) with cervicothoracic syringomyelia can progress slowly this condition which is very common in clinical practice, particularly in children. CASE PRESENTATION: Patients typically present with chronic complaints, including headache, dizziness, and numbness, although are few reports in the literature describing pediatric patients who developed acute neurological deficits caused by CM-I. Here, we report an unusual presentation of this condition; the patient presented with sudden onset arm swelling with no precipitating factors that could explain the diagnosis. DISCUSSION: This is an illustrated case report and literature review. The patient's condition improved post-operatively; in terms of arm and hand swelling which resolved, but he still complained of persistent numbness on a follow-up visit.

Neuronavigated endoscopic aqueductoplasty with panventricular stent plus septostomy for isolated fourth ventricle in complex hydrocephalus and syringomyelia associated with myelomeningocele: how I do it.

BACKGROUND: Isolated fourth ventricle (IFV) is a challenging entity to manage. In recent years, endoscopic treatment for aqueductoplasty has been on the rise. However, in patients with complex hydrocephalus and distorted ventricular system, its implementation can be complex. METHODS: We present a 3-year-old patient with myelomeningocele and postnatal hydrocephalus treated by ventriculoperitoneal shunt. In follow-up, a progressive IFV and isolated lateral ventricle with symptoms of the posterior fossa developed. An endoscopic aqueductoplasty (EA) with panventricular stent plus septostomy guided with neuronavigation was decided due to the complexity of the ventricular system. CONCLUSION: In IFV associated with complex hydrocephalus with distortion of the ventricular system, navigation can be of great help for planning and as a guide for performing EA.