The Cause of Syringomyelia and its Surgical Treatment

Wj, Gardner; Angel, Jessie

doi:10.3949/ccjm.25.1.4

Cited by 84 publications

(36 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Gardner and colleagues [6][7][8] postulated that delayed and incomplete embryonic opening of the outlet of the fourth ventricle, leading to retained communication of the cen-tral canal with the fourth ventricle, is the etiology of syringomyelia associated with Chiari malformation. This theory, based on a hydrodynamic mechanism, states that, with continuing partial obstruction of the outflow of CSF from the fourth ventricle through the central canal, each arterial pulse transmits a "water-hammer" effect into the syrinx causing development and progression of cord cavitation.…”

Section: Discussionmentioning

confidence: 99%

Surgical management of syringomyelia-Chiari complex

Ergün¹,

Akdemir²,

Gezici³

et al. 2000

European Spine Journal

View full text Add to dashboard Cite

IntroductionMagnetic resonance imaging (MRI) has facilitated the diagnosis of syringomyelia in recent years, providing sharp delineation of syrinxes and accurate identification of malformations at the cranio-cervical junction. What is the mechanism of origin and maintenance of syringomyelia? Syringomyelia cannot be ascribed to a single pathophysiological mechanism, and its etiology and natural history are quite variable. It occurs in relation to intramedullary tumors, compressive myelopathy, spinal trauma, hydrocephalus and anomalies such as the Chiari malformation. Syringomyelia, is most frequently associated with Chiari malformation, and several hypotheses have been proposed to explain its pathophysiology in this particular manifestation.One explanation for syrinx formation, which has recently been reported by Oldfield et al. [18], requires no Abstract Great variety exists in the indications and techniques recommended for the surgical treatment of syringomyelia-Chiari complex. More recently, magnetic resonance (MR) imaging has increased the frequency of diagnosis of this pathology and offered a unique opportunity to visualize cavities inside the spinal cord as well as their relationship to the cranio-cervical junction. This report presents 18 consecutive adult symptomatic syringomyelia patients with Chiari malformation who underwent foramen magnum decompression and syringosubarachnoid shunting. The principal indication for the surgery was significant progressive neurological deterioration. All patients underwent preoperative and postoperative MRI scans and were studied clinically and radiologically to assess the changes in the syrinx and their neurological picture after surgical intervention. All patients have been followed up for at least 36 months. No operative mortality was encountered; 88.9% of the patients showed improvement of neurological deficits together with radiological improvement and 11.1% of them revealed collapse of the syrinx cavity but no change in neurological status. None of the patients showed further deterioration of neurological function. The experience obtained from this study demonstrates that foramen magnum decompression to free the cerebro-spinal fluid (CSF) pathways combined with a syringosubarachnoid shunt performed at the same operation succeeds in effectively decompressing the syrinx cavity, and follow-up MR images reveal that this collapse is maintained. In view of these facts, we strongly recommend this technique, which seems to be the most rational surgical procedure in the treatment of syringomyeliaChiari complex.

show abstract

Section: Discussionmentioning

confidence: 99%

Surgical management of syringomyelia-Chiari complex

Ergün¹,

Akdemir²,

Gezici³

et al. 2000

European Spine Journal

View full text Add to dashboard Cite

show abstract

“…An early explanation, the water-hammer theory, proposed that increased pulse pressure from fourth ventricle or foramen magnum outlet flow directs CSF into the central canal. 4 Tubbs et al hypothesized that posterior odontoid inclination results in posterior inclination of the brainstem at the level of the obex, opening up the foramen cecum and diverting greater egress of CSF flow into the central canal. 15 Their findings that the obex-McRae line length correlated with degree of inclination, coupled with an overall shorter distance in patients with CM-I, often below the foramen magnum, support this hypothesis.…”

Section: What Is the Origin Of Syringomyelia In Cm-i?mentioning

confidence: 99%

Evaluating the relationship of the pB–C2 line to clinical outcomes in a 15-year single-center cohort of pediatric Chiari I malformation

Ladner¹,

Dewan²,

Day

et al. 2015

PED

View full text Add to dashboard Cite

OBJECT The clinical significance of radiological measurements of the craniocervical junction in pediatric Chiari I malformation (CM-I) is yet to be fully established across the field. The authors examined their institutional experience with the pB–C2 line (drawn perpendicular to a line drawn between the basion and the posterior aspect of the C-2 vertebral body, at the most posterior extent of the odontoid process at the dural interface). The pB–C2 line is a measure of ventral canal encroachment, and its relationship with symptomatology and syringomyelia in pediatric CM-I was assessed. METHODS The authors performed a retrospective review of 119 patients at the Monroe Carell Jr. Children's Hospital at Vanderbilt University who underwent posterior fossa decompression with duraplasty, 78 of whom had imaging for review. A neuroradiologist retrospectively evaluated preoperative and postoperative MRI examinations performed in these 78 patients, measuring the pB–C2 line length and documenting syringomyelia. The pB–C2 line length was divided into Grade 0 (< 3 mm) and Grade I (≥ 3 mm). Statistical analysis was performed using the t-test for continuous variables and Fisher's exact test analysis for categorical variables. Multivariate logistic and linear regression analyses were performed to assess the relationship between pB–C2 line grade and clinical variables found significant on univariate analysis, controlling for age and sex. RESULTS The mean patient age was 8.5 years, and the mean follow-up duration was 2.4 years. The mean pB–C2 line length was 3.5 mm (SD 2 mm), ranging from 0 to 10 mm. Overall, 65.4% of patients had a Grade I pB–C2 line. Patients with Grade I pB–C2 lines were 51% more likely to have a syrinx than those with Grade 0 pB–C2 lines (RR 1.513 [95% CI 1.024–2.90], p = 0.021) and, when present, had greater syrinx reduction (3.6 mm vs 0.2 mm, p = 0.002). Although there was no preoperative difference in headache incidence, postoperatively patients with Grade I pB–C2 lines were 69% more likely to have headache reduction than those with Grade 0 pB–C2 lines (RR 1.686 [95% CI 1.035–2.747], p = 0.009). After controlling for age and sex, pB–C2 line grade remained an independent correlate of headache improvement and syrinx reduction. CONCLUSIONS Ventral canal encroachment may explain the symptomatology of select patients with CM-I. The clinical findings presented suggest that patients with Grade I pB–C lines2, with increased ventral canal obstruction, may experience a higher likelihood of syrinx reduction and headache resolution from decompressive surgery with duraplasty than those with Grade 0 pB–C2 lines.

show abstract

“…8,9 Earlier suggestions of Gardner 10 and Williams 11 were replaced by proposals from Oldfield. 12 The majority of this work has been based on the syringomyelia associated with hindbrain hernias but more recent work by Stoodley 13 has used specifically the model of post-traumatic syringomyelia.…”

Section: Discussionmentioning

confidence: 99%