Clinical Significance of Terminal Syringomyelia in Association with Pediatric Tethered Cord Syndrome

Beaumont, Andrew; Muszynski, Cheryl A.; Kaufman, Bruce A.

doi:10.1159/000098834

Cited by 22 publications

(13 citation statements)

References 37 publications

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“…The traditional diagnosis of TCS has required radiographic evidence of a short, thick or fatty FT and a lowlying CMD with its tip positioned at or below the lower endplate of L 2 [23,48,59,65,74,78,82]. The syndrome is associated with an increased incidence of terminal thoracic syringomyelia [7,12,22]. …”

Section: Discussionmentioning

confidence: 99%

Association of Chiari malformation type I and tethered cord syndrome: preliminary results of sectioning filum terminale

et al. 2009

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Objective The pathogenesis of CM-I is incompletely understood. We describe an association of CM-I and TCS that occurs in a subset of patients with normal size of the PCF. Methods The prevalence of TCS was determined in a consecutively accrued cohort of 2987 patients with CM-I and 289 patients with low-lying cerebellar tonsils (LLCT). Findings in 74 children and 244 adults undergoing SFT were reviewed retrospectively. Posterior cranial fossa size and volume were measured using reconstructed 2D computed tomographic scans and MR images. Results were compared to those in 155 age- and sex-matched healthy control individuals and 280 patients with generic CM-I. The relationships of neural and osseus structures at the CCJ and TLJ were investigated morphometrically on MR images. Intraoperative CDU was used to measure anatomical structures and CSF flow in the lumbar theca. Results Tethered cord syndrome was present in 408 patients with CM-I (14%) and 182 patients with LLCT (63%). In 318 patients undergoing SFT, there were no significant differences in the size or volume of the PCF as compared to healthy control individuals. Morphometric measurements demonstrated elongation of the brain stem (mean, 8.3 mm; P < .001), downward displacement of the medulla (mean, 4.6 mm; P < .001), and normal position of the CMD except in very young patients. Compared to patients with generic CM-I, the FM was significantly enlarged (P < .001). The FT was typically thin and taut (mean transverse diameter, 0.8 mm). After SFT, the cut ends of the FT distracted widely (mean, 41.7 mm) and CSF flow in the lumbar theca increased from a mean of 0.7 cm/s to a mean of 3.7 cm/s (P < .001). Symptoms were improved or resolved in 69 children (93%) and 203 adults (83%) and unchanged in 5 children (7%) and 39 adults (16%) and, worse, in 2 adults (1%) over a follow-up period of 6 to 27 months (mean, 16.1 months ± 4.6 SD). Magnetic resonance imaging 1 to 18 months after surgery (mean, 5.7 months ± 3.8 SD) revealed upward migration of the CMD (mean, 5.1 mm, P < .001), ascent of the cerebellar tonsils (mean, 3.8 mm, P < .001), reduction of brain stem length (mean, 3.9 mm, P < .001), and improvement of scoliosis or syringomyelia in some cases. Conclusions Chiari malformation type I/TCS appears to be a unique clinical entity that occurs as a continuum with LLCT/TCS and is distinguished from generic CM-I by enlargement of the FM and the absence of a small PCF. Distinctive features include elongation and downward displacement of the hindbrain, normal position of the CMD, tight FT, and reduced CSF flow in the lumbar theca. There is preliminary evidence that SFT can reverse moderate degrees of tonsillar ectopia and is appropriate treatment for cerebellar ptosis after Chiari surgery in this cohort.

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Section: Discussionmentioning

confidence: 99%

Association of Chiari malformation type I and tethered cord syndrome: preliminary results of sectioning filum terminale

et al. 2009

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“…In terminal syringomyelia associated with tethered cord, as in our case, spinal dysraphism or intramedullary tumor, the cyst may show regression or even disappear after surgical treatment of the underlying pathologies (2,10,12,15). Although there is a risk of recurrence in cases of the syringomyelia treated without shunting, this risk is known to be low after the definite treatment of the underlying pathology, and restoring the normal CSF flow (2,10,12,15). In the present case, the authors preferred only to untether the cord, thus achieving definite treatment of the underlying pathology and expecting a high rate of success in treating the syringomyelia (2,10,12,15), which may avoid the need for syringosubarachnoid shunting and the insertion of foreign materials.…”

Section: Discussionmentioning

confidence: 69%

“…Although the optimum surgical procedure is still controversial, syringosubarachnoid shunt placement seems to be an effective treatment in symptomatic cases (3,4,10). In terminal syringomyelia associated with tethered cord, as in our case, spinal dysraphism or intramedullary tumor, the cyst may show regression or even disappear after surgical treatment of the underlying pathologies (2,10,12,15). Although there is a risk of recurrence in cases of the syringomyelia treated without shunting, this risk is known to be low after the definite treatment of the underlying pathology, and restoring the normal CSF flow (2,10,12,15).…”

Section: Discussionmentioning

confidence: 75%

Terminal syringomyelia mimicing intramedullary tumor: case report

Abuzayed

Dashti

Erdinçler

et al. 2009

Turkish Neurosurgery

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In this report, the authors present a 14-year-old male patient admitted with progressive paraparesis and urinary incontinence that developed in a 3-month period. Thoracolumbar MRI revealed an intramedullary lesion starting from the level of the T10 vertebra and extending to fill the sacral spinal canal. Based on the radiological findings an intramedullary tumor was suspected. Exploratory operation with right L5 hemilaminectomy was done and the intraoperative finding was syringomyelia which was punctured with releasing of the tethered cord. The patient was followed for 3 years with clinical and radiological regression of the pathology and no symptoms and sings have remained. Syringomyelia can mimic intramedullary tumors clinically and radiologically. That is why it is useful to consider exploratory hemilaminectomy to confirm diagnosis.

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“…This would be concordant with previous pediatric TC studies suggesting the absence of correlation between level of termination of the conus medullaris and clinical symptoms, 6,31 or between a postoperative decrease in the size of a syrinx and clinical symptom resolution. 3,18 This study demonstrated that half of the surgically treated children had a conus medullaris below the level of L-2 and this finding had no significant association with height outcome. While TC is classically associated with a low-lying conus medullaris, other studies have noted that TC can occur in the absence of a low-positioned conus medullaris.…”

Section: Discussionmentioning

confidence: 72%

Putative height acceleration following tethered cord release in children

Foster

Lam

Lin

et al. 2014

PED

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Object Tethered cord (TC) is a neurological disorder caused by tissue attachments that limit the normal movement of the spinal cord. A TC can be unmasked by a cutaneous abnormality or manifest clinically in myriad neurological, urological, and orthopedic symptoms. The relationship between TC and height is previously unknown. This study investigates the association between TC release and changes in height profiles in the pediatric population. Methods Fifty-two children undergoing first-time TC release at a single institution were examined retrospectively. Clinical symptoms, radiographic findings, pre- and postoperative height, and height-for-age percentiles were recorded and analyzed. Results Children with TC experienced a statistically significant increase in age-adjusted height percentiles after TC release (p = 0.0028), with a mean increase of 7 percentile points (from 48.1st to 54.9th percentile). When stratified by age, children 5 years or older (5–18 years) demonstrated a mean percentile increase of 10 percentile points (from 46.7th to 56.4th percentile) (p = 0.0001). Among the same age group, this effect scaled significantly with age (p = 0.02, beta coefficient −1.3). There was no significant difference in height-for-age after detethering surgery in children younger than 5 years. There was no significant association between the presence of clinical symptoms or specific radiographic findings and height outcomes after surgery. Overall, 56% of TC-related clinical symptoms improved after detethering (mean follow-up 4.6 months). Among children younger than 5 years, 82% of TC-related clinical symptoms improved after detethering (average follow-up 4.5 months); in children 5–18 years, 47% of symptoms improved after detethering (average follow-up 4.8 months). Conclusions The authors observed a statistically significant gain in height-for-age percentiles in children undergoing surgical release of TC. The authors' data suggest that such gains may be more significant in older children (≥ 5 years) and the increase appears to scale positively with youth in the older cohort. In this study, postoperative height gains did not appear to correlate with the presence of TC-related clinical symptoms or radiographic findings. Further investigation is necessary to elucidate any potential correlation between release of TC and height changes in children postoperatively.

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Clinical Significance of Terminal Syringomyelia in Association with Pediatric Tethered Cord Syndrome

Cited by 22 publications

References 37 publications

Association of Chiari malformation type I and tethered cord syndrome: preliminary results of sectioning filum terminale

Association of Chiari malformation type I and tethered cord syndrome: preliminary results of sectioning filum terminale

Terminal syringomyelia mimicing intramedullary tumor: case report

Putative height acceleration following tethered cord release in children

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