Pathophysiology of primary spinal syringomyelia

Heiss, John D.; Snyder, Kendall; Peterson, Matthew M.; Patronas, Nicholas J.; Butman, John A.; Smith, Rachel L.; DeVroom, Hetty L.; Sansur, Charles A.; Eskioglu, Eric; Kammerer, William; Oldfield, Edward H.

doi:10.3171/2012.8.spine111059

Cited by 96 publications

(69 citation statements)

References 64 publications

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“…Cysts usually accompanied a high-grade astrocytoma. Syrinxes, which form after a change in CSF flow [19,20], were observed in one of 11 high-grade cases and in five of seven low-grade cases. This could be due to the fact that low-grade tumors have fewer infiltrates than highgrade tumors, resulting in a greater impact on CSF flow in Fig.…”

Section: Discussionmentioning

confidence: 99%

A retrospective observational study on the treatment outcomes of 26 patients with spinal cord astrocytoma including two cases of malignant transformation

Ryu

Kim

et al. 2016

Eur Spine J

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Purpose To determine the biologic behavior and prognostic factors of spinal cord astrocytoma, we reviewed surgical and clinical outcomes. Due to the rarity of spinal cord astrocytoma, there is a lack of research regarding this type of tumor and malignant transformation. Methods We retrospectively reviewed the data from all patients on whom we performed spinal cord tumor removal between 1983 and 2014. Twenty-six patients were pathologically confirmed to have spinal cord astrocytoma or glioblastoma. Surgical extent and disease progression were confirmed by the surgeon based on operative findings, postoperative MRI, and outpatient department (OPD) follow-up. Results Pain or neurological deficit was the chief complaint for all patients. With MRI studies, there is a tendency for highgrade astrocytomas to show as enhanced and heterogeneous images. Two of the low-grade astrocytomas showed malignant transformation over the course of 4 and 11 months, respectively. The overall survival (OS) for low-grade astrocytoma was 28-480 months (mean 156.38 months); the OS for highgrade astrocytoma was 1-36 months (mean 12.00 months). Conclusions Two of 12 low-grade cases showed malignant transformations at 4 and 11 months, respectively, based on pathological confirmation. With spinal cord astrocytomas, enhanced MRI results appeared similar to those of a malignant lesion. We suggest close observation and image correlation of low-grade astrocytomas, even when pathologically confirmed as low-grade. In this review, we found that histologic grade is the most important prognostic factor, although it is not always concordant with biologic behaviors.

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Section: Discussionmentioning

confidence: 99%

A retrospective observational study on the treatment outcomes of 26 patients with spinal cord astrocytoma including two cases of malignant transformation

Ryu

Kim

et al. 2016

Eur Spine J

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“…More recently, Heiss et al 71,72 and Oldfield et al 112 proposed new insights into the pathophysiology of syringomyelia and its implications for the diagnosis and treatment of CM-I. Obviously, the creation and enlargement of a syrinx may contribute to the transformation of asymptomatic to symptomatic CM-I.…”

Section: 153mentioning

confidence: 99%

Pediatric and adult Chiari malformation Type I surgical series 1965–2013: a review of demographics, operative treatment, and outcomes

Arnautovic

Splavski

Boop

et al. 2015

PED

205

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OBJECT Chiari malformation Type I (CM-I) is a hindbrain disorder associated with elongation of the cerebellar tonsils, which descend below the foramen magnum into the spinal canal. It occurs in children and adults. Clinical symptoms mainly develop from alterations in CSF flow at the foramen magnum and the common subsequent development of syringomyelia. METHODS The authors reviewed English-language reports of pediatric, adult, and combined (adult and pediatric) surgical series of patients with CM-I published from 1965 through August 31, 2013, to investigate the following: 1) geographical distribution of reports; 2) demographics of patients; 3) follow-up lengths; 4) study durations; 5) spectrum and frequency of surgical techniques; 6) outcomes for neurological status, syrinx, and headache; 7) frequency and scope of complications; 8) mortality rates; and 9) differences between pediatric and adult populations. Research and inclusion criteria were defined, and all series that contained at least 4 cases and all publications with sufficient data for analysis were included. RESULTS The authors identified 145 operative series of patients with CM-I, primarily from the United States and Europe, and divided patient ages into 1 of 3 categories: adult (> 18 years of age; 27% of the cases), pediatric (≤ 18 years of age; 30%), or unknown (43%). Most series (76%) were published in the previous 21 years. The median number of patients in the series was 31. The mean duration of the studies was 10 years, and the mean follow-up time was 43 months. The peak ages of presentation in the pediatric studies were 8 years, followed by 9 years, and in the adult series, 41 years, followed by 46 years. The incidence of syringomyelia was 65%. Most of the studies (99%) reported the use of posterior fossa/foramen magnum decompression. In 92%, the dura was opened, and in 65% of these cases, the arachnoid was opened and dissected; tonsillar resection was performed in 27% of these patients. Postoperatively, syringomyelia improved or resolved in 78% of the patients. Most series (80%) reported postoperative neurological outcomes as follows: 75% improved, 17% showed no change, and 9% experienced worsening. Postoperative headaches improved or resolved in 81% of the patients, with a statistical difference in favor of the pediatric series. Postoperative complications were reported for 41% of the series, most commonly with CSF leak, pseudomeningocele, aseptic meningitis, wound infection, meningitis, and neurological deficit, with a mean complication rate of 4.5%. Complications were reported for 37% of pediatric, 20% of adult, and 43% of combined series. Mortality was reported for 11% of the series. No difference in mortality rates was seen between the pediatric and adult series. CONCLUSIONS Before undergoing surgical treatment for CM-I, symptomatic patients and their families should be given clear information about the success of treatment and potential complications. Furthermore, surgeons may benefit from comparing published data with their own. In the future, operative CM-I reports should provide all details of each case for the purpose of comparison.

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“…8,9 We expect that in this setting the patients with a distant focal stenosis within the spinal canal would be at an increased risk for syringomyelia as a result of a pressure differential between the subarachnoid space cisterns and the central canal. While we found no difference in cerebellar tonsil descent between patients with and without a syrinx, this retrospective study was limited in its inability to record and measure tonsil position changes with CSF pulsations.…”

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

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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.

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Pathophysiology of primary spinal syringomyelia

Cited by 96 publications

References 64 publications

A retrospective observational study on the treatment outcomes of 26 patients with spinal cord astrocytoma including two cases of malignant transformation

A retrospective observational study on the treatment outcomes of 26 patients with spinal cord astrocytoma including two cases of malignant transformation

Pediatric and adult Chiari malformation Type I surgical series 1965–2013: a review of demographics, operative treatment, and outcomes

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

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