Secondary tethered cord syndrome in spinal dysraphism

Ohe, Naoyuki; Futamura, Atsuroh; Kawada, Rieko; Minatsu, Hiroshi; Kohmura, H.; Hayashi, Kazuko; Miwa, Keishi; Sakai, Nobuaki

doi:10.1007/pl00007291

Cited by 35 publications

(19 citation statements)

References 22 publications

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“…Loss of neurological function has also been demonstrated to have a logarithmic association with increasing patient age and is believed to be secondary to increased stretch on the spinal cord with axial growth spurts. 26,27,33,52 Other theories regarding the mechanism of progression of symptoms include decreased perfusion secondary to stretch on the spinal cord, increased mass effect from progressive deposition of fat, and stretching effects on the spinal cord. 51,52,71 Hoffman et al 27 observed that 62.5% of patients were neurologically asymptomatic prior to 6 months of age, while only 29.3% were asymptomatic after 6 months of age.…”

Section: Diagnosis and Presentationmentioning

confidence: 99%

Lipomyelomeningocele: pathology, treatment, and outcomes

Sarris

Tomei²,

Carmel³

et al. 2012

FOC

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Lipomyelomeningocele represents a rare but complex neurological disorder that may present with neurological deterioration secondary to an inherent tethered spinal cord. Radiological testing is beneficial in determining the morphology of the malformation. Specialized testing such as urodynamic studies and neurophysiological testing may be beneficial in assessing for neurological dysfunction secondary to the lipomyelomeningocele. Early surgical intervention may be beneficial in preventing further neurological decline.

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Section: Diagnosis and Presentationmentioning

confidence: 99%

Lipomyelomeningocele: pathology, treatment, and outcomes

Sarris

Tomei²,

Carmel³

et al. 2012

FOC

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“…Surgical series have shown retethering rates approaching 45% in more complex cases of myelomeningocele and lipomyelomeningocele; in cases of thick/fatty filum terminale sectioning, considerably lower rates have been reported, and scattered reports in the literature cite a retethering rate of about 8%-9% on long-term follow-up. 6,7,16 Most retethering is attributed to scar and arachnoid adherence around the neural elements attached to the spinal meninges, developing traction and nerve root dysfunction over time. 15 Maintenance of a pristine subarachnoid space during surgery and robust irrigation prior to dural closure have been espoused as technical nuances thought to minimize the risk of intradural adhesion.…”

Section: Discussionmentioning

confidence: 99%

Fecal incontinence as a predominant symptom in a case of multiply recurrent tethered cord: diagnosis and operative strategies

Behaine

Latif

Greenfield

2015

PED

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T eThered cord syndrome (TCS) may be associated with myelomeningocele, lipomyelomeningocele, and lumbosacral lipoma or fibrolipomatous filum terminale or may be secondary to arachnoiditis or postsurgical scarring following an initial operative approach. Rates of retethering after sectioning a fatty filum terminale are low. 15,16 Retethering may produce neurological symptoms through nerve root traction and ischemia or tension distributed through the spinal cord. 3,4,12 We present a case of simple tethered cord due to a fatty filum terminale with 3 distinct episodes of recurrence, each presenting with fecal incontinence and resolving with operative intervention. At the last procedure, a discrete sacral nerve root was recognized and individual nerve roots were separated from the filum before its eventual complete resection. Symptoms had not returned at the 9-month follow-up. This case represents a unique clinical course and interesting pathophysiology that, to the best of our knowledge, has not been reported. case report History and ExaminationA healthy 4-year-old boy initially presented with severe constipation and abdominal pain concomitant with low-back and leg pain for which he was being managed with bowel regimens without improvement. Magnetic resonance imaging of the spine demonstrated a fatty filum terminale and tethered cord. The patient underwent tethered cord release at another institution, and his symptoms partially improved for 6 months before a recurrence of his initial symptoms. First RecurrenceThe patient's physical therapist noted new mild weakness of the left leg. Magnetic resonance imaging confirmed release of the filum terminale with the lipoma clearly disconnected, and the surgeon declined to reexplore. The child presented to our office at this time for a second opinion. We offered to perform an exploration at the original surgical abbreviatioN TCS = tethered cord syndrome. Recurrent manifestations of tethered spinal cord after an initial operative intervention for a simple fatty filum terminale is fairly uncommon. The authors present the case of an unusual clinical course in which there were 3 distinct episodes of recurrence, each time presenting predominantly as fecal incontinence and resolving with operative intervention. Typical signs of tethering were absent on radiological evaluation, and operative intervention was based on clinical grounds. Intraoperatively, sacral nerve roots to the anal sphincter were found tethered to the filar stump with electrophysiological evidence of regained activity on disentanglement. To the best of the authors' knowledge, a similar clinical course or operative findings have not been reported.

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“…Overall, approximately 75-90% of patients experience improvement or stabilization of symptoms. 1,7,9,12,18 Pain is most amenable to improvement; AlHolou and col leagues 1 recently reported improvement in pain in 75% of patients. 9,10,12,20 Bowel and bladder function, on the other hand, are less likely to improve, with only 13-46% of patients reporting improved function (20% in this se ries).…”

Section: Posttreatment Outcomesmentioning

confidence: 99%

“…1,7,9,12,18 Pain is most amenable to improvement; AlHolou and col leagues 1 recently reported improvement in pain in 75% of patients. 9,10,12,20 Bowel and bladder function, on the other hand, are less likely to improve, with only 13-46% of patients reporting improved function (20% in this se ries). 1,7,17,22 In addition, we suspect that untethering contrib uted to the stabilization of bowel and bladder function in the other patients who presented with these symptoms.…”

Section: Posttreatment Outcomesmentioning

confidence: 99%

“…Measures are therefore necessary to minimize the risk of iatrogenic injury during untethering procedures. 3,8,12,19,20 Previous reports, including a recent one by AlHolou and colleagues, 1 have described the benefits of surgical untethering in this patient population, clearly document ing the benefits of intervention. 7 In this study, we again assess outcomes of untethering in this population.…”

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confidence: 95%

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Electrophysiologically guided untethering of secondary tethered spinal cord syndrome

Pouratian¹,

Elias²,

Phillips

et al. 2010

FOC

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Object Many patients develop neurological symptoms related to spinal cord tethering after perinatal repair of myelomeningocele. This is referred to as secondary tethered cord syndrome (STCS). The authors describe their methodology and evaluate the intraoperative utility and postoperative outcomes of electrophysiologically guided untethering for STCS. In addition, the authors describe the use of electrophysiological guidance to identify an “autonomous placode” in the untethering of the cord in STCS. Methods The authors retrospectively identified 46 untethering procedures in 38 patients who had undergone perinatal myelomeningocele repair and in whom the index surgery was for tethered cord release at the site of the repair. In all cases, both passive (electromyography) and active (detection of compound muscle action potentials) electrophysiological monitoring was used. The proximity to neural elements was determined based on the current used; eliciting compound muscle action potentials with a ≤ 10-mA stimulation was assumed to represent direct neural stimulation. Clinical records were reviewed to evaluate the utility of electrophysiological guidance and patient outcomes. Results The median age at the time of untethering was 9.5 years (range 0.5–54 years). The median follow-up time was 42 months (range 3–172 months). Progressive bowel and bladder dysfunction, diagnosed either clinically or by cystometrogram, and low-back pain were the most common presenting symptoms. Intraoperative findings indicated that the most common causes of tethering were dense scar (76%) and a tethered placode (39%). Electrophysiological monitoring identified functional neural tissue near tethered elements and provided intraoperative guidance in all cases. In 41% of cases (19 cases), the untethering plan was noted to have been significantly influenced by intraoperative neurophysiological findings. Moreover, an autonomous placode was identified in 6 patients who were nonambulatory preoperatively and had presented with increasing pain and spasticity. In electrophysiologically silent areas, more aggressive dissection and untethering were possible. Symptoms of low-back pain, lower-extremity paresthesia, and lower-extremity spasticity were most likely to improve after untethering surgery (91, 88, and 82%, respectively). Sectioning above the electrophysiologically defined autonomous placode resulted in significant improvement in back pain and lower-extremity spasticity in 5 of 6 patients. There was 1 case of immediate postoperative neurological deterioration (fecal incontinence). All patients remained clinically stable or improved on long-term follow-up, except for 6 (16% of patients) who required a total of 7 additional procedures for recurrent symptoms (median time to repeat surgery 36 months). Complications were noted in 8 cases, including infections and CSF leaks. Conclusions Surgical untethering of STCS halts progression and often improves preoperative symptoms. Electrophysiological monitoring, using both a threshold-based interpretation system and continuous electromyography monitoring, provides an efficient, effective, and reliable method for intraoperative guidance, thereby limiting iatrogenic injury and providing a means to identify and untether autonomous placodes. Electrophysiological monitoring also allows for more aggressive dissection and untethering in functionally silent regions, possibly decreasing retethering rates.

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Secondary tethered cord syndrome in spinal dysraphism

Cited by 35 publications

References 22 publications

Lipomyelomeningocele: pathology, treatment, and outcomes

Lipomyelomeningocele: pathology, treatment, and outcomes

Fecal incontinence as a predominant symptom in a case of multiply recurrent tethered cord: diagnosis and operative strategies

Electrophysiologically guided untethering of secondary tethered spinal cord syndrome

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