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.

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.

[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).

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.

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.

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.

A Rare Case Report of Eight Syndrome Secondary to Syringomyelia Associated with Type I Chiari Malformation.

Eight syndrome is defined as the combination of a unilateral conjugate gaze palsy and ipsilateral seventh cranial nerve palsy. It may occur as a result of demyelinating, vascular, infectious, or compressive lesions of the brainstem localized to the caudal pontine tegmentum. A 43-year-old woman was admitted to our clinic with complaints of headache, inability to look to the left, and weakness on the left side of her face. The complaints had begun abruptly about a month before her admission. Suboccipital decompression surgery for type I Chiari malformation had been performed 10 years earlier. Neuro-ophthalmological examination revealed left-sided horizontal gaze palsy and anisocoria. Cranial and cervical magnetic resonance images revealed cerebellar tonsillar herniation and syringomyelia, the latter of which was considered to be the cause of eight syndrome. No interventions were performed, and periodic follow-up was advised on neurosurgical consultation. Left gaze palsy and facial palsy recovered almost completely in three months, while the anisocoria persisted. Syringomyelia should be considered among the causes of horizontal gaze palsy plus ipsilateral seventh nerve palsy, termed as eight syndrome. Clinical suspicion and appropriate radiological examination can aid in the diagnosis.

Resolution of a Focal Pruritic Rash Following Neurosurgical Decompression in an Adolescent With Chiari I and Syringomyelia.

BACKGROUND: Chiari I malformation (Chiari I) is defined by the downward displacement of one or both cerebellar tonsils below the foramen magnum of the skull with crowding altering cerebrospinal fluid flow. It can be associated with the development of a fluid-filled cavity within the spinal cord, syringomyelia. Neurological deficits or symptoms can occur at the level of anatomic involvement of syringomyelia. CASE PRESENTATION: A young man presented to dermatology clinic for evaluation of a pruritic rash. Recognizing a unique left "cape-like" distribution of neuropathic itch leading to prurigo nodularis, he was referred for further evaluation by neurology in the local emergency department. After additional history and neurological exam, a magnetic resonance imaging confirmed Chiari I with an associated syringobulbia and a syrinx extending to T10/11 of the spinal cord. Anteriorly the syrinx extended into the left parenchyma of his spinal cord involving the dorsal horn, a lesion explaining his neuropathic itch. The sensation of itch and rash resolved after posterior fossa craniectomy and C1 laminectomy with duraplasty. CONCLUSION: Neuropathic itch, in addition to pain, can be a symptom of Chiari I with syringomyelia. Focal pruritus without an obvious cutaneous trigger should prompt providers to consider a central neurological pathology. While many patients with Chiari I are asymptomatic, the presence of neurological deficits and syringomyelia, are indications for neurosurgical evaluation.

Syringomyelia secondary to thoracic spinal dural arteriovenous fistula presenting as paraparesis.

Spinal dural arteriovenous fistula (SDAVF) is also known as a type 1 spinal arteriovenous malformation, representing the most frequent vascular malformation of the spine. A high suspicion index is often required for the initial diagnosis of SDAVF because of subtle magnetic resonance imaging signs. We present the case of a patient with SDAVF associated with syringomyelia of the thoracic spinal cord and hypothesize that a fistula might induce intramedullary fluid accumulation due to venous hypertension, which leads to syrinx formation.

Cerebrospinal Fluid Flow and Brain Motion in Chiari I Malformation: Past, Present, and Future.

Cranio-spinal volume and pressure changes associated with the cardiac-cycle and respiration are altered in Chiari I malformation (CMI) due to obstruction of cerebrospinal fluid (CSF) flow at the foramen magnum. With the introduction of motion-sensitive MRI sequences, it was envisioned that these could provide noninvasive information about volume-pressure dynamics at the cranio-cervical junction in CMI hitherto available only through invasive pressure measurements. Since the early 1990s, multiple studies have assessed CSF flow and brain motion in CMI. However, differences in design and varied approaches in the presentation of results and conclusions makes it difficult to fully comprehend the role of MR imaging of CSF flow and brain motion in CMI. In this review, a cohesive summary of the current status of MRI assessment of CSF flow and brain motion in CMI is presented. Simplified versions of the results and conclusions of previous studies are presented by dividing the studies in distinct topics: 1) comparing CSF flow and brain motion between healthy subjects (HS) and CMI patients (before and after surgery), 2) comparing CSF flow and brain motion to CMI severity and symptoms, and 3) comparing CSF flow and brain motion in CMI with and without syringomyelia. Finally, we will discuss our vision of the future directions of MR imaging in CMI patients. EVIDENCE LEVEL: 2. TECHNICAL EFFICACY: 5.

Natural History of Chiari 1 Deformity.

PURPOSE: Chiari type 1 deformity (Ch1) is associated with bony deformity of the skull base and herniation of cerebellar tonsils more than 5 mm below the foramen magnum. Magnetic resonance imaging (MRI) is used for diagnosis and surgery is advised for symptomatic children. We present a case series using MRI including cerebrospinal fluid (CSF) flow, in children with Ch1 to demonstrate a variety of outcomes, both surgical and spontaneous: spontaneous resolution, spontaneous worsening, postsurgical improvement, and postsurgical deterioration. CASE: A 2-week-old female newborn underwent brain MRI demonstrating an ectopic neurohypophysis, under opercularization suggesting brain immaturity and a normal craniocervical junction (CCJ). Follow-up (F/U) MRI at 6 years of age showed interval spontaneous development of Ch1 with decreased CSF spaces at CCJ. CASE: A 6-year-old girl referred for imaging with short stature and growth hormone deficiency demonstrated incidental findings of Ch1 without syringomyelia. There was 15-mm protrusion of pointed cerebellar tonsils through the foramen magnum and a reduced CSF space at the craniocervical junction. No surgery was performed, and F/U MRI at the age of 7 years demonstrated spontaneous resolution of the tonsillar ectopia (cerebellar tonsils now 3 mm right and 6 mm left) and expansion of the CSF spaces at CCJ. CASE: A 7-year-old boy with headaches and staring spells underwent an MRI demonstrating 6-mm protrusion of pointed cerebellar tonsils and CSF space reduction at CCJ. No surgery was performed, and F/U imaging at the age of 9 years demonstrated spontaneous improvement in cerebellar tonsillar position and increased bidirectional CSF flow at CCJ. CASE: A 17-month-old boy underwent brain MRI for unsteady gait and poor vestibular response, which showed Ch1 and narrow CSF spaces at the foramen magnum and with reduced CSF flow. At the age of 3 years, after posterior fossa decompression, F/U MRI showed postsurgical improvement of the position of the cerebellar tonsils and increased CSF space at CCJ. CASE: A 4-month-old male infant with a history of 34-week prematurity, prior germinal matrix hemorrhage, and neonatal subdural hemorrhage was referred for MRI of the cervical and thoracic spine for evaluation of developmental delay and hypotonia with torticollis. Magnetic resonance imaging of the spine demonstrated mild protrusion of inferiorly pointed cerebellar tonsils up to the foramen magnum, with visible CSF and without retroflexion of the dens. Follow-up MRI scans demonstrated progressive worsening of the Chiari 1 deformity, even after multiple surgeries. CONCLUSIONS: It is important to be aware of a variety of different outcomes with Ch1, including spontaneous resolution, spontaneous worsening, improvement with surgery, and even deterioration after surgery. More research is required to determine objective criteria for predicting outcome, which include both anatomic measures and physiologic measures of CSF flow, so that better surgical decisions can be made and for evaluating patients who have undergone surgery.