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Fibrous dysplasia is a benign bone disease characterized by the replacement of normal bone tissue with fibrous tissue, resulting in irregular bone structure. Cases of craniofacial fibrous dysplasia in children associated with Chiari type I malformation and syringomyelia are extremely rare. This case illustrates the complex clinical manifestations of craniofacial fibrous dysplasia along with Chiari type I malformation and syringomyelia, in which surgical intervention significantly improved the prognosis, and follow-up revealed near-complete resolution of the syringomyelia. It offers valuable insights for managing similar cases in the future.

Abstract BACKGROUND Occipital-cervical fusion (OCF) and ventral decompression (VD) may be used in the treatment of pediatric Chiari-1 malformation (CM-1) with syringomyelia (SM) as adjuncts to posterior fossa decompression (PFD) for complex craniovertebral junction pathology. OBJECTIVE To examine factors influencing the use of OCF and OCF/VD in a multicenter cohort of pediatric CM-1 and SM subjects treated with PFD. METHODS The Park-Reeves Syringomyelia Research Consortium registry was used to examine 637 subjects with cerebellar tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and at least 1 yr of follow-up after their index PFD. Comparisons were made between subjects who received PFD alone and those with PFD + OCF or PFD + OCF/VD. RESULTS All 637 patients underwent PFD, 505 (79.2%) with and 132 (20.8%) without duraplasty. A total of 12 subjects went on to have OCF at some point in their management (PFD + OCF), whereas 4 had OCF and VD (PFD + OCF/VD). Of those with complete data, a history of platybasia (3/10, P = .011), Klippel-Feil (2/10, P = .015), and basilar invagination (3/12, P < .001) were increased within the OCF group, whereas only basilar invagination (1/4, P < .001) was increased in the OCF/VD group. Clivo-axial angle (CXA) was significantly lower for both OCF (128.8 ± 15.3°, P = .008) and OCF/VD (115.0 ± 11.6°, P = .025) groups when compared to PFD-only group (145.3 ± 12.7°). pB-C2 did not differ among groups. CONCLUSION Although PFD alone is adequate for treating the vast majority of CM-1/SM patients, OCF or OCF/VD may be occasionally utilized. Cranial base and spine pathologies and CXA may provide insight into the need for OCF and/or OCF/VD. Graphical Abstract Graphical Abstract

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.

Background Syringomyelia is a rare disease with diverse etiologies, and the syrinx is typically confined to certain segments of the spinal cord. Case of syringomyelia affecting the whole cord due to tethered cord is extremely rare, and the underlying pathophysiological mechanisms remain poorly understood. Case presentation We described an 18-year-old male patient who presented with progressive weakness in both lower extremities and bladder dysfunction over the past four years. Magnetic resonance imaging (MRI) of the entire spine revealed a tethered spinal cord with a large syrinx extending from C1 to L5. Common causes of syrinx such as Chiari malformation, intramedullary tumors and spinal cord injury were systematically ruled out, leading to a strong suspicion that the tethered cord was the primary etiology of the extensive syringomyelia. After undergoing un-tethering surgery, the patient experienced significant symptomatic improvement, and the subsequent follow-up MRI examinations demonstrated a remarkable reduction and eventual resolution of the large syrinx. Conclusions Although rare, tethered cord syndrome can serve as the sole etiology for extensive syringomyelia. For such patients, performing un-tethering surgery can lead to complete resolution of the syrinx and achieve a satisfactory clinical outcome.

Posttraumatic spinal cord tethering and syringomyelia frequently lead to progressive neurological loss. Although several studies demonstrated favourable outcome following spinal cord detethering with/without shunting, additional research is required as no clear consensus exists over the ideal treatment strategy and knowledge about prognostic demographic determinants is currently limited. In this investigation, we retrospectively investigated 67 patients (56 men, 11 women) who were surgically treated and followed for symptomatic spinal cord tethering and syringomyelia from 2012 to 2022 at our center. Age (B-coefficient 0.396) and severity of trauma to the spinal cord (B-coefficient − 0.462) have been identified as independent predictors for the rate of development of symptomatic spinal cord tethering and syringomyelia (p < 0.001). Following untethering surgery including expansion duraplasty with/without shunting, 65.9% of patients demonstrated an improvement of neurological loss (p < 0.001) whereas 50.0% of patients displayed amelioration of spasticity and/or neuropathic pain (p < 0.001). Conclusively, active screening for symptomatic spinal cord tethering and syringomyelia, particularly in younger patients with severe spinal trauma, is crucial as surgical untethering with/without shunting is able to achieve favourable clinical outcomes. This knowledge may enable clinicians to tailor treatment strategies in spinal cord injury patients suffering from progressive neurological loss towards a more optimal and personalized patient care.

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.

Purpose Chiari I malformation (CM-I) in pediatric patients can impose substantial neurologic and functional impairment. Additionally, the presence of syrinx is often a harbinger of clinical compromise, but little attention has been devoted to identifying features associated with syrinx development and the clinical impact of syrinx resolution. Therefore, this study aims to identify clinical and radiographic variables associated with preoperative syrinx presence and postoperative syrinx reduction in pediatric patients with CM-I and determine the relationship between postoperative syrinx reduction and clinical symptom improvement. Methods The authors performed a retrospective analysis of 435 consecutive pediatric patients who underwent surgical treatment of CM-I from 2001 to 2021 at a single tertiary pediatric medical center. All patients underwent pre- and postoperative MRI, and clinical and radiographic variables were recorded and subject to inferential analysis. Results Syrinx at presentation was independently associated with symptoms of spinal cord dysfunction at presentation (OR 2.17 (95% CI 1.05–4.48); p  = 0.036), scoliosis (OR 5.33 (2.34–10.86); p  = 0.001), and greater pB-C2 (posterior basion to C2 distance) measurement length (OR 1.14 (95% CI 1.01–1.30); p  = 0.040). Syrinx at presentation was inversely associated with tussive headaches at presentation (OR 0.27 (95% CI 0.16–0.47); p  = 0.001) and cranial nerve deficits at presentation (OR 0.49 (95% CI 0.26–0.92); p  = 0.025). Postoperatively, patients with radiographic evidence of syrinx improvement had greater rates of symptom improvement (93.1% vs 82.1%; p  = 0.049), better CCOS scores (15.4 vs 14.2; p  = 0.001), and decreased rates of readmission (6.0% vs 25.0%, p  = 0.002) and reoperation (0.5% vs 35.7%; p  = 0.001). The difference in syrinx resolution was similar but not statistically significant (10.3% vs 16.7%; p  = 0.251). AO joint anomaly (OR 0.20, 95% CI 0.04–0.95; p  = 0.026) and foramen magnum diameter (OR 1.12, 95% CI 1.00–1.25; p  = 0.049) were the only independent predictors of syrinx improvement, and surgical technique was the only predictor for syrinx resolution (OR 2.44, 95% CI 1.08–5.50; p  = 0.031). Patients that underwent tonsil reduction surgery whose syrinx improved had a wider foramen magnum diameter than those whose did not improve (34.3 vs 31.7; p  = 0.028). Conclusions Radiographic syrinx improvement is associated with greater rates of symptom improvement and less readmissions and reoperations for CM-I. AO joint anomalies and narrower foramen magnums were independent risk factors for the lack of syrinx improvement. These novel insights will help guide preoperative patient counseling, pre- and intraoperative surgical decision-making, and postoperative clinical prognostication in the treatment of pediatric CM-I.

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.

BackgroundNeuropathic arthropathy (NA) of the shoulder is a chronic progressive process characterized by joint destruction in the presence of a neurosensory deficit. Syringomyelia, a spinal cord disease, is the leading cause of NA in the upper extremity. ObjectiveWe present a systematic review of NA with syringomyelia cases alongside a case report of an adult with NA of the shoulder that occurs a few 4 years after a revelation and surgical management of a Chiari malformation with syringomyelia.MethodsA systematic review was conducted following PRISMA guidelines. A PubMed, Scopus, Isiknowledge, and manual search through references of relevant publications were used to identify all published case reports of NA. Data were collected from each case report on patient characteristics.ResultsThe systematic review identified 56 publications and 85 patients (including ours): nearly the same number of males (n = 41) and females (n = 44). The mean age was 50,69. Presentations included reduction of mobility (n = 66), swelling (n = 61) and sensory disorder (n = 63). The pain was absent in 41 cases. In the majority of reported cases 56 (65.1%), syringomyelia was revealed by neuropathic arthropathy, and eleven patients (12.9%) had a history of syringomyelia. Treatment was categorized into non-operative management (37[43.5%]), operative management (27[31.7%]). Following-up was non-reported in 31 (36%) cases. Improvement was reported more with patients who underwent a surgical approach than medical one 28.5% versus 8.1%.ConclusionPhysicians need to be more aware of this destructive joint disease, rare, and often misdiagnosed. Also, it is imperative to integrate clinical, pathological, and imaging findings for accurate diagnosis and for delivering appropriate therapy.

PurposeTo evaluate the effectiveness of brace treatment in patients with Chiari malformation type 1 (CM-1) or syringomyelia associated scoliosis without neurosurgical intervention.MethodsThis was a retrospective case–control study. 34 CM-1 or syringomyelia (CMS) patients who received brace treatment without neurosurgical intervention were recruited. Another 68 matched patients with idiopathic scoliosis who received bracing served as the control group. The matching criteria included gender, age (± 1 years), Risser sign (± 1 grade), initial curve magnitude (± 5°), curve patterns and follow-up time (± 6 months). Patients who encountered curve progression and scoliosis surgery were compared between different groups.ResultsUntil the last visit, 16 (47%) patients in CMS group and 18 (26%) patients in IS group occurred curve progression; 9 (26%) patients and 15 (22%) patients underwent scoliosis surgery, respectively. Compared to idiopathic scoliosis, patients with CMS-associated scoliosis had a significantly higher rate of curve progression (P = 0.038). However, no significant difference was observed between two groups regarding to the rate of surgery (P = 0.867). Patients with combined CM-1 and syringomyelia had a higher rate of surgery than patients with isolated CM-1 or syringomyelia (P = 0.049). The double major curve pattern was identified as the risk factor for curve progression.ConclusionBrace treatment is effective for CMS-associated scoliosis without neurosurgical intervention. Compared to idiopathic scoliosis, brace can provide similar prevention for scoliosis surgery in CMS patients, but slight or moderate curve progression may occur. Specifically, patients with combined CM-1 and syringomyelia should be followed closely with a higher expectation of curve progression.