Presence Of Syrinx Research Articles

Predictors of syrinx presentation and outcomes in pediatric Chiari malformation type I: a single institution experience of 218 consecutive syrinx patients.

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. 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. 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). 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.

Characterizing syringomyelia and its clinical significance in 140 patients with lipomyelomeningocele.

The aims of this study were to characterize syringomyelia in patients with lipomyelomeningocele (LMMC) and investigate the association between the presence of a syrinx and patient neurological deficits. Patients with LMMC who had been admitted between 2007 and 2020 were included in the study. Syringomyelia on magnetic resonance imaging (MRI) was classified into three groups: true syrinx, early syrinx, and presyrinx. The correlation of clinical deficits (at birth, new onset, and progressive) and type of lipoma with the presence and type of syrinx was studied. Among a total of 140 patients, the median age was 2.5 (IQR 1.3-9) years. Neurological deficits were present at birth in 75 (53.6%) patients, but 67 (47.9%) had new-onset deficits or progression of their birth deficits. The most common type of spinal lipoma was the transitional type (75.7%). Thirty-nine (27.9%) patients had a syrinx (37 with a true syrinx, 2 with an early syrinx), and 25 (17.9%) patients had a presyrinx. There was no significant correlation between the presence of a syrinx and patient neurological deficits. However, patients with a syrinx that was not adjacent to the lipoma (≥ 1 vertebral level away) had a higher incidence of deficits at birth (p = 0.045), whereas those with a syrinx spanning ≥ 5 vertebral levels had a higher incidence of progressive neurological deficits (p = 0.04). Six (75%) of 8 patients in whom serial MRI had shown syrinx progression had clinical worsening. Syrinx location with respect to the spinal lipoma, syrinx length, and progressive syringomyelia may have clinical significance in patients with LMMC.

An Uncommon Case of Post-Traumatic Syringobulbia: A Case Report

Background and aim: Syringobulbia is a rare progressive neurological condition characterised by the presence of syrinx or an elongated fluid-filled cavity in the brainstem with multiple possible underlying aetiologies. However, to date, only a few cases of syringobulbia presenting as a late complication of post-traumatic syringomyelia (PTS) have been reported. Methods: We present a case of a 26-year-old morbidly obese gentleman with T4 AIS C incomplete spinal cord injury following T7 burst fracture 2 years prior requiring spinal instrumentation. He then experienced implant failure 1 year later, necessitating implant removal. Subsequently, he reported worsening back pain and neurology deterioration over his left upper and lower limb. In addition to that, pain and muscle spasms over the left upper limb extending up to the left side of his neck, face, and head warranted a repeat imaging of the brain and spinal cord. Magnetic resonance imaging (MRI) revealed syringobulbia predominantly affecting the left side of the medulla oblongata with extensive syringomyelia originating from the C1 to T12 vertebral level. Moreover, the old T7 fracture site remained malunited. Results: Despite a successful second spinal decompressive surgery and combined rehabilitation, there was no neurological recovery. However, the patient described significant improvement in pain and spasms. Conclusion: PTS complicated with syringobulbia, although rare, is one of the causes of the delayed-onset neurological deterioration in spinal cord injured patients. It might be a diagnostic dilemma as some cases exhibit atypical presentations and mimic the pre-existing neurological deficit. Syrinx formation due to alteration of subarachnoid cerebrospinal fluid (CSF) flow in post-traumatic spinal deformity might benefit from decompressive surgery, avoiding the need for intradural shunt placement, coupled with medication and physical therapy. Even though there was no neurological recovery, symptomatic alleviation of neuropathic pain and spasm improved quality of life.

Early- and long-term surgical outcomes in 109 children with lipomyelomeningocele.

To determine the functional outcomes and risk factors for outcomes following surgery for lipomyelomeningocele (LMMC). Data from 109 children with LMMC who underwent surgery from January 2008 to December 2017 were retrospectively studied to evaluate functional outcomes and possible risk factors for early- (at discharge from hospital or within 1 month of surgery) and long-term outcomes after surgery. There were 53 boys and 56 girls with median age of 36 months (IQR 12-90 months; range, 4 months to 18 years) at surgery. At presentation, neurological function was normal (asymptomatic group) in 28 (25.7%) children while there was neurological dysfunction (symptomatic group) in 81 (74.3%). Near total or radical excision of lipoma was done in 71 (65.1%) children. Six (5.8%) children had deterioration of neurological function in the post-operative period with recovery of function in three of them. There were no statistically significant risk factors for early outcomes. At a mean follow-up of 62.5 months (IQR 35-82 months; range, 12-146 months), 21 (27.6%) of the 76 symptomatic group children (including 59.3% with incomplete bladder dysfunction) showed improvement, 52 (68.4%) remained the same, while 3 (4%) deteriorated. In 31 children (symptomatic group) who developed symptoms after 1 year of age, early surgery after development of symptoms was associated with better chances of recovery (p = 0.0008). In the asymptomatic group, 23 (88.5%) had normal neurological function at mean follow-up of 57.5 months (IQR 30-77 months; range, 12-141 months) and 3 (11.5%) had neurological deterioration. The mean time to late deterioration (re-tethering) from surgery was 51.3 months (IQR 24-75 months; range, 24-84 months). Presence of syrinx on initial MRI (p = 0.008) and partial resection of the lipoma (p = 0.02) were independent risk factors for delayed deterioration. Radical resection of LMMC probably helps in preserving neurological function in > 90% of children at long-term follow-up. Fifty-five percent of children > 2 years of age with incomplete bladder dysfunction regained normal function following surgery. In children who develop symptoms after birth, early surgical intervention can reverse the neurological deficits.

Functional Outcomes and Temporal Profile of Recovery in Patients with Intradural Extramedullary Spinal Cord Tumors with Poor Nurick Grade

Patients with spinal intradural extramedullary (IDEM) tumors usually have a good functional outcome after tumor excision. However, the literature is sparse on the functional outcome in patients with poor Nurick grade (NG 4 and 5). A retrospective review of 81 patients with IDEM tumors presenting with a poor NG was performed to determine postoperative functional outcome and the temporal pattern of recovery. The following risk factors were analyzed: preoperative NG, duration of symptoms, tumor location, peritumoral edema, presence of syrinx, and tumor type. Neurologic function started recovering soon after surgery, with >80% of the patients improving by ≥1 grade at the end of 1 week after surgery. Of the 66 patients available for follow-up of >6 months after surgery, 63 (95.5%) improved to NG 0-2 and 51 (77.2%) became asymptomatic (NG 0 or 1). Three patients had a poor functional outcome on follow-up of >31 months; 2 had improved from NG 5 to NG 4, whereas 1 patient continued to be in NG 4. Factors associated with a poor outcome were an upper thoracic location (P= 0.025) and presence of a syrinx (P= 0.004). None of the patients had bladder dysfunction at follow-up of >6 months. After excision of spinal IDEM tumors, in patients who present with a poor neurologic function (NG 4 and 5), good functional outcome (NG 0-2) can be expected in >95% of patients. No recovery can be anticipated beyond 1 year after surgery.

Observational outcome in surgery for Chiari malformation patients

Chiari malformation is the commonest anomaly of the craniovertebral junction involving both the skeletal as well as the neural structures. It is congenital anomaly of the hindbrain characterised by downward elongation of the brain stem and cerebellum into the cervical portion of spinal cord. Most common presenting symptoms was pain in the nape of neck with sub-occipital headache and weakness. If not intervened early in these cases they may progress to quadriparesis and respiratory failure. This study includes authors experience of 30 surgical corrections of Chiari malformation performed at civil hospital Ahmedabad from 2017 to 2019. The age and sex of the patient, the presence of syrinx, the type of surgical procedure and the clinical outcome were determined post-operatively and on follow up. Cerebro spinal fluid leak and collection were observed in patient who undergone duroplasty only with no leakage in patient undergone syringo-subarachnoid shunt. Overall, tingling/numbness had best improvement showed improvement in 13 out of 16 patients. Power showed improvement in 20 out of 27 patients and pain showed improvement in 18 patients. Wasting, clawing and cerebellar signs and bony deformity showed no improvement in any of the above procedures. Authors can conclude for Chiari malformation decompression with or without duroplasty with additional procedure with post-operative physiotherapy and analgesia is the suitable treatment.

Posterior spinal cord indentation: imaging findings and clinical outcome

To identify the imaging findings associated with worse clinical outcome in posterior spinal cord indentation. A retrospective search for cases of dorsal cord indentation on magnetic resonance imaging (MRI) from April 2009 to March 2016 was undertaken. Imaging follow-up and clinical data were recorded. Two neuroradiologists blinded to the clinical data assessed the imaging findings. Differences and association of imaging and clinical findings were assessed via t-test and Fisher's exact and chi-squared tests for continuous and categorical data. Inter-rater agreement was calculated. Seventy-three patients were included, 65 were clinically stable, or the finding was incidental and eight had clinical worsening or required surgery. There was a significant difference in the percentage of cord diameter decrease between the two clinical groups (p=0.013, reader 1; p=0.027 reader 2). The clinical course was significantly associated with subjective cord indentation depth assessment (p=0.03 reader 1) and presence of syrinx (p=0.017 reader 2) on original MRI and worsening on imaging follow-up (p=0.03). The interrater agreement was moderate for syrinx identification (k=0.58). There was only fair agreement for the scalpel sign classification and in the final diagnosis (k=0.35 and 0.28). The degree of cord indentation, the presence of syrinx, and worsening of imaging findings on follow-up are associated with worse clinical course and can be useful in guiding management and directing subspecialty referrals.

Intrathecal Morphine in the Presence of a Syrinx in Pediatric Spinal Deformity Surgery.

Intrathecal morphine (IM) is a popular adjunct for pain relief during pediatric spinal deformity surgery. There is no literature regarding its usefulness and safety in the presence of a spinal cord syrinx for patients undergoing spinal instrumentation. Anesthesiologists have previously been reluctant to use IM in the presence of any syrinx. We retrospectively reviewed all patients with a preoperatively diagnosed spinal cord syrinx undergoing spinal deformity surgery who received IM and did not receive IM (non-IM). We recorded location of the syrinx, surgical time, length of stay, unexpected pediatric intensive care unit (PICU) admission, IM related complications (neurological, respiratory depression, or pruritus, nausea/vomiting), and reason for no IM administration. Patients with a syrinx and myelodysplasia (8), tethered spinal cord (4), paraplegia (1), holocord (1), neuroblastoma (1), and spinal cord glioma (1) were not given IM. Other reasons included a failed attempt (1), expectedly short surgical time (1), and anesthesiologist declined (2). There were 42 patients who met the inclusion criteria. Twenty-two patients received IM, while 20 patients did not. Patients receiving IM had 4 cervical, 5 cervicothoracic, 12 thoracic syrinxes, and 1 holocord syrinx. The non-IM group had 8 cervicothoracic, 6 thoracic, 4 holocord syrinxes, and 2 had unclassified locations. There were no neurological complications in the IM group, and 1 patient experienced respiratory depression following a shorter than expected surgery and was observed overnight in the PICU. One patient in the non-IM group with a holocord syrinx had temporary lower extremity weakness postoperatively that completely resolved and 4 patients were unexpectedly admitted to the PICU. Pruritus and nausea/vomiting was mild and similar in both groups. Our study demonstrates that with careful preoperative evaluation, most patients with a spinal cord syrinx can safely be given IM. Certain patients, such as those with a spinal holocord syrinx may have anatomic reasons to avoid IM, but those who are deemed appropriate for IM can receive it safely. Level III-therapeutic study; retrospective comparative study.

Systematic review and meta-analysis of imaging characteristics in Chiari I malformation: does anything really matter?

Previous studies have attempted to evaluate the utility of preoperative magnetic resonance imaging (MRI) parameters in predicting outcomes in Chiari I malformation. We performed a systematic review and meta-analysis to determine what preoperative imaging features (if any) predict (1) presence of preoperative symptoms or associated findings, (2) need for surgical decompression, or (3) improvement after surgical decompression. All publications through June 2018 on PubMed, Embase, and Cochrane Library databases were searched using the keywords "Chiari I malformation" AND "decompression" OR "imaging." One thousand two hundred ten publications were identified, and 20 were included for our systematic review; nine were included in the meta-analysis. Tonsil position, clivus gradient, and scoliotic curve of > 20° were all associated with the presence of preoperative syrinx. Degree of scoliotic curve was associated with length of syrinx. Pre-operative findings of central syrinx morphology, shorter syrinx, and scoliotic curve < 20° were associated with post-operative stability/improvement. Post-operative symptomatic improvement was associated with preoperative pB-C2 line ≥ 3 mm, absence of scoliosis, and presence of syrinx. By meta-analysis, there was no significant difference in post-operative improvement between patients with and without syrinx (OR = 0.89; 95% CI 0.58-1.37). Meta-analysis showed no significant difference in post-operative improvement between patients with and without basilar invagination (OR = 1.31; 95% CI 0.72-2.36). Multiple studies have attempted to identify preoperative imaging parameters to predict post-operative improvement, but no consistently reliable criteria have been defined. This review and meta-analysis highlight the importance of considering each patient's clinical history and physical exam within the context of associated radiographic abnormalities.

Stratification of Grade of Spinal Cord Non-Ependymal Gliomas by Magnetic Resonance Imaging

The purpose of this study was to demonstrate the usefulness of conventional magnetic resonance imaging (MRI) in stratification of spinal cord non-ependymal gliomas. Forty-one patients (mean age, 29.9 ± 16.8 years; 27 men) with first-diagnosed spinal cord non-ependymal gliomas underwent conventional MRI including T1-weighted, T2-weighted (T2W) and contrast-enhanced T1-weighted imaging sequences. The tumors were removed by surgery and processed by histology 1 week after MRI. Patients were divided into low-grade and high-grade groups according to histological findings. The MRI features of gliomas including signal-to-noise-ratio (SNR), margins, heterogeneity, edema, enhancement, tumor size, longitudinal location, and presence of syrinx were measured and compared between low-grade and high-grade groups using the χ2 test and the Mann-Whitney U test. The SNR of low-grade gliomas on T2W images was significantly higher than that of high-grade gliomas (165.0 ± 108.7 vs. 98.6 ± 44.4; P= 0.016 by the Mann-Whitney U test). The margins of low-grade gliomas were found to be clearer compared to that of high-grade gliomas (54.5% vs. 5.3%; P=0.001). Receiver-operating-characteristic analysis revealed that the area under the curve of 1/SNR, margin and the combination of 1/SNR and margin was 0.722 (95% confidence interval [CI], 0.564-0.880), 0.736 (95% CI, 0.578-0.894), and 0.845 (95% CI, 0.717-0.973), respectively. No significant differences were found in the heterogeneity, edema, enhancement level,enhancement SNR, size, longitudinal location, cyst, hemorrhage, and intramedullary location between low-grade and high-grade non-ependymal gliomas (all P > 0.05). The characteristics of signal intensity and margin on T2W imaging are valuable in stratification of spinal cord non-ependymal gliomas.

An Interesting Case of Thoracic Dermal Sinus

11 years old girl presented with history of on and off discharge from her upper back since childhood. Examination revealed presence of dermal sinus on her back (Figure 1). Neurological examination was normal. MRI revealed presence of track going upwards to the cervical region with presence of syrinx and diastematomyelia.

The association between Chiari malformation Type I, spinal syrinx, and scoliosis.

OBJECT Chiari malformation Type I (CM-I) is often found in patients with scoliosis. Most previous reports of CM-I and scoliosis have focused on patients with CM-I and a spinal syrinx. The relationship between CM-I and scoliosis in the absence of a syrinx has never been defined clearly. The authors sought to determine if there is an independent association between CM-I and scoliosis when controlling for syrinx status. METHODS The medical records of 14,118 consecutive patients aged ≤ 18 years who underwent brain or cervical spine MRI at a single institution in an 11-year span were reviewed to identify patients with CM-I, scoliosis, and/or syrinx. The relationship between CM-I and scoliosis was analyzed by using multivariate regression analysis and controlling for age, sex, CM-I status, and syrinx status. RESULTS In this cohort, 509 patients had CM-I, 1740 patients had scoliosis, and 243 patients had a spinal syrinx. The presence of CM-I, the presence of syrinx, older age, and female sex were each significantly associated with scoliosis in the univariate analysis. In the multivariate regression analysis, older age (OR 1.02 [95% CI 1.01-1.03]; p < 0.0001), female sex (OR 1.71 [95% CI 1.54-1.90]; p < 0.0001), and syrinx (OR 9.08 [95% CI 6.82-12.10]; p < 0.0001) were each independently associated with scoliosis. CM-I was not independently associated with scoliosis when controlling for these other variables (OR 0.99 [95% CI 0.79-1.29]; p = 0.9). CONCLUSIONS A syrinx was independently associated with scoliosis in a large pediatric population undergoing MRI. CM-I was not independently associated with scoliosis when controlling for age, sex, and syrinx status. Because CM-I is not independently associated with scoliosis, scoliosis should not necessarily be considered a symptom of low cerebellar tonsil position in patients without a syrinx.

Correlation of Functional Outcome and Natural History With Clinicoradiological Factors in Surgically Managed Pediatric Chiari I Malformation

Chiari I malformation occurs because of an underdeveloped posterior fossa with reduced volume that cannot accommodate the normally developed hindbrain. To study the clinical presentation and surgical outcome of pediatric Chiari I malformation and to correlate outcome with demographic and clinical factors and radiological changes in the syrinx, spinal cord, and preoperative intracranial, posterior fossa, and foramen magnum dimensions. This retrospective study spanning 9 years included 20 symptomatic patients who underwent foramen magnum decompression, shrinkage of tonsils, and duraplasty. Improvement at follow-up was assessed with a modified Asgari scoring system. Mean differences in the dimensions of the syrinx and cord, foramen magnum morphometry, and intracranial and posterior fossa for 2 groups (with or without improvement) were analyzed with the independent-sample Student t test. Correlation of outcome in relation to change in radiological factors and influence of variables such as age, type and duration of symptoms, and presence of syrinx were evaluated with the Pearson χ² test. Sixty percent of patients showed functional improvement at follow-up. Of various demographic and radiological factors assessed, there was significant difference in mean values of change in cord diameter for the entire cohort (P = .05) and for the subgroup with preoperative syringes (P = .03). There was no correlation between change in any of these factors and functional improvement (χ² range, 0-4.673; P > .05). More than half the pediatric patients with Chiari I malformation improve after surgery. The age at presentation, duration and type of symptoms, cranial and foramen magnum morphometry, and syrinx-related changes have no bearing on outcome at short-term follow-up. The spinal cord diameter differs significantly in patients with and without functional improvement.

Neuroimaging of Tuberculous Myelitis: Analysis of Ten Cases and Review of Literature

We retrospectively reviewed the clinical and neuroimaging features of 10 patients with tuberculous myelitis. The most common presenting symptoms were fever (70%) and paraplegia (60%). Bladder and bowel symptoms were present in 90% patients. On MRI, the involvement of the cervical/thoracic segment of the spinal cord was most commonly seen (90%). The most consistent finding was hyperintense signals on T2-weighted MRI. T1-weighted images showed isointense (n= 5) and hypointense (n= 4) signals in the spinal cord lesions. Post-contrast enhancement was present in 6 patients, epidural enhancement in 4 patients, and cord swelling in 2 patients. We reviewed more than 250 published cases with the diagnosis of tuberculous myelitis and radiculomyelitis with special attention to MRI findings. It is predominantly a disease of the thoracic spinal cord. Most spinal cord lesions appear as hyperintense on T2 and iso- or hypointense on T1-weighted images. MRI findings in patients with spinal cord tuberculosis have both diagnostic and prognostic significance. Cord atrophy or cavitation and the presence of syrinx on MRI may be associated with poor outcome.

Prevalence and clinical significance of superficial abdominal reflex abnormalities in idiopathic scoliosis

To determine prevalence and significance of abnormal superficial abdominal reflexes (SARs) in idiopathic scoliosis. Study of 73 patients with presumed idiopathic scoliosis referred for magnetic resonance imaging (MRI), either as a routine pre-operative assessment (n=42) or because of abnormal symptoms or neurological signs (n=31). All patients were examined prior to magnetic resonance imaging (MRI), and the presence of abnormal SARs was noted. All patients then underwent MRI of the whole spine from the foramen magnum to the sacrum. The presence of Chiari 1 malformation and syrinx was recorded. The study group consisted of 11 males and 62 females with a mean age at time of MRI of 18 years (range 5-51 years) and a mean Cobb angle of 48 degrees (range 10-104 degrees). Abnormality of the SARs was recorded in eight cases (prevalence 11%). An abnormal MRI study was recorded in nine cases (12.3%), all patients having a syrinx and four having in addition, a Chiari 1 malformation. Of the patients with abnormal SARs, only 2 (25%) had an abnormal MRI study; 1 had unilateral absence of the reflexes whereas the other had complete absence of SARs. Of patients referred for MRI as a routine pre-operative assessment, 5 (11.6%) had an abnormal MRI study. In patients with idiopathic scoliosis, abnormality of the SARs was recorded in 11% of cases. Unilateral absence was present in one case only and was associated with the presence of syrinx. Other patterns of abnormality were not a useful indicator of underlying cord abnormality.