Idiopathic syringomyelia represents a group of patients with a syrinx without an underlying pathological cause. Patients may present with back pain, leg pain and/or neurological dysfunction. We aim to examine the clinico-radiological outcomes of paediatric patients presenting with idiopathic syringomyelia managed with a therapeutic lumbar puncture. All paediatric patients with idiopathic syringomyelia who underwent a lumbar puncture over a 10-year time period were identified and included. Pre- and post-procedural clinico-radiological features were obtained from electronic patient records. Procedural details, including opening pressure and drainage volume, were obtained from electronic operative records. 31 patients underwent a therapeutic lumbar puncture. Median age at time of first lumbar puncture was 12 years (range 2-17). 7/31 (23%) patients had significant long-term improvement or resolution in their symptoms following a single lumbar puncture. 13/31 (42%) patients had temporary improvement of their symptoms and underwent further lumbar punctures. 3/31 (10%) patients had resolution of their symptoms after more than one lumbar puncture. 8/31 (26%) patients did not experience any improvement in their symptoms. Symptomatic improvement was more likely in those with a high opening pressure of equal to or greater than 24cmH2O (OR 13 (1.3 - 126.3) P = 0.02). Median length of follow up was 3 yrs (range 6 months - 9 years). Complications occurred in 9/31 (29%) patients, with 8 of these experiencing a low pressure headache, and 1 patient experiencing temporary back pain following the lumbar puncture. Lumbar puncture may be a safe and effective treatment for symptomatic idiopathic syringomyelia in the paediatric population. It appears to be more effective in those with a higher opening pressure.

Background: Identifying the etiological factors of syringomyelia, which can cause progressive neurological deficits in the spinal cord, is critically important for both diagnosis and treatment. This study aimed to assess the cranial morphometric features of patients with idiopathic syringomyelia by conducting comparative analyses with individuals diagnosed with Chiari Type I, Chiari Type I accompanied by syringomyelia, and healthy controls, in order to elucidate the potential structural contributors to the pathogenesis of idiopathic syringomyelia. Methods: In this retrospective and comparative study, a total of 172 patients diagnosed with Chiari Type I and/or syringomyelia between 2016 and 2024, along with 156 radiologically normal individuals, were included. The participants were categorized into four groups: healthy controls, Chiari Type I, Chiari Type I with syringomyelia, and idiopathic syringomyelia (defined as syringomyelia without an identifiable cause). Midline sagittal T1-weighted MR images were used to obtain quantitative measurements of the posterior fossa, cerebellum, intracranial area, and foramen magnum. All measurements were stratified and statistically analyzed by sex. Results: In cases with idiopathic syringomyelia, both the posterior fossa area and the cerebellum/posterior fossa ratio differed significantly from those of healthy controls. In male patients, the foramen magnum diameter was significantly larger in the Chiari + syringomyelia group compared with the idiopathic group. A significant correlation was found between the degree of tonsillar descent and selected morphometric parameters in female subjects, whereas no such correlation was observed in males. Both Chiari groups exhibited significantly smaller posterior fossa dimensions compared with the healthy and idiopathic groups, indicating greater neural crowding. Additionally, in Chiari Type I patients, increasing degrees of tonsillar descent were associated with a decreased incidence of syringomyelia. Conclusions: Anatomical variations such as a reduced posterior fossa area or altered foramen magnum diameter may contribute to the pathogenesis of idiopathic syringomyelia. Cranial morphometric analysis appears to offer diagnostic value in these cases. Further prospective, multicenter studies incorporating advanced neuroimaging modalities, particularly those assessing cerebrospinal fluid dynamics, are warranted to better understand the mechanisms underlying syringomyelia of unknown etiology.

Syringomyelia is a rare condition characterized by the formation of a fluid-filled cyst within the spinal cord, leading to myelopathy. In addition, the pathological enlargement of the central canal is referred to as hydromyelia or cleft-like syrinx. We present a case of idiopathic syringomyelia and hematomyelia in a 50-year-old female patient with a 5-year follow-up on her disease progression. Magnetic resonance imaging (MRI) images revealed low-signal intensity on T1 and high-signal intensity on T2, with elevated hemorrhagic signal intensity on T1 and low peripheral signal intensity on T2. A fluid-filled lesion measuring 12 × 36 mm was observed between the C7 and Th3 vertebrae, with separation from some of the detailed components. No contrast enhancement was noted following IV contrast administration. Based on the MRI findings, a diagnosis consistent with giant hemorrhagic syringomyelia was established. Subsequently, a neurosurgical intervention was performed, resulting in a reduction in the size of the syringomyelia and a moderate improvement in the patient's symptom profile.

Idiopathic syringomyelia (IS) associated with occult arachnoid pathology is a relatively rare condition characterized by a subtle onset, atypical clinical manifestations, and significant diagnostic and therapeutic challenges. This study aims to evaluate the radiographic and clinicopathological features of IS to improve surgical management and patient outcomes. In this study, clinical and radiologic data were retrospectively extracted from a single-center syringomyelia database (N=1,039) spanning December 2020 to March 2025. Among these, 15 patients diagnosed with IS underwent preoperative magnetic resonance imaging and myelography to identify the responsible spinal segments precisely. Comprehensive perioperative assessments and clinical outcomes were collected. During surgery, the subarachnoid space (SAS) was thoroughly explored, with complete removal of thickened and adherent arachnoid tissue to restore normal cerebrospinal fluid (CSF) circulation. Additionally, clinical data, pathological features, and surgical outcomes of IS were compared to those of posttraumatic delayed syringomyelia (PTDS) to evaluate potential differences. In this series, all patients underwent preoperative myelography, revealing varying degrees of SAS obstruction. For IS cases that received precise and comprehensive arachnoid lysis, overall postoperative outcomes were favorable. Intraoperative pathology confirmed that all IS cases were characterized by noninfectious, nonacute inflammation. The preoperative maximal syrinx/cord ratio averaged 0.70±0.07 (range, 0.54-0.88), while the syrinx resolution rate varied from 12.2% to 100%, with a mean improvement of 29.6%. Patients with PTDS exhibited a relatively higher incidence of hypesthesia and a greater syrinx tension index. However, no significant differences were observed between IS and PTDS in terms of syrinx length, deviation, or location. Notably, the IS group demonstrated significantly better postoperative syrinx resolution and improvement in syringomyelia-related symptoms compared to the PTDS group. While both IS and PTDS share a common underlying mechanism of arachnoid adhesions, they differ significantly in pathological features, treatment approaches, and clinical outcomes. In cases of IS, thorough spinal arachnoid lysis at the affected segment could restore normal spinal cord pulsation and CSF circulation, leading to effective syrinx resolution and a favorable long-term prognosis.

Idiopathic syringomyelia (IS) is defined by the presence of a spinal syrinx without identifiable primary pathology. The natural history, clinical implications, and radiological management of IS in the paediatric patient remain poorly understood, with existing literature providing limited long-term data. This study aims to evaluate the long-term clinical and radiological outcomes of paediatric patients with IS, focusing on understanding predictive factors of clinical or radiological progression, and the correlation between clinical and radiological evolution. This retrospective study included 28 paediatric patients diagnosed with IS, all with a minimum of three years of radiological follow-up. Cases of secondary syrinx caused by upstream pathology were excluded. Clinical and imaging data were analysed to assess the radiological change of the syrinx and change in symptoms. Correlations between radiological and clinical features were explored. The median radiological and clinical follow-up durations were 6.84 years and 7.66 years, respectively. A reduction in syrinx size (≥ 1 mm in width or anterior-posterior diameter) was observed in 53.6% of patients, with no significant association with gender, age at diagnosis, initial syrinx size, or scoliosis. Back pain occurred in 25% of patients; other modes of presentation include urological disturbance and neurological deficits. All patients remained clinically asymptomatic, stable or improved during follow-up. Radiological changes did not correlate with clinical outcomes. No predictors were found for radiological or clinical outcomes. IS in paediatric patients follow a predominantly benign course, with radiological changes showing little clinical relevance. Repeated interval imaging appears unnecessary in stable cases, and management should prioritise clinical symptoms. This study provides the largest long-term dataset to date, supporting a conservative approach to IS management.