Tethered Cord Syndrome in Low Motor Level Children with Myelomeningocele

Abstract

The clinical presentation of tethered spinal cord and the results of tethered cord release were examined in a group of 30 low motor level (L3 and below) children with a history of myelomeningocele without concomitant CNS complications. Changes in orthopedic and/or neurologic status formed the basis of consideration for tethered cord release. Clinically, these patients presented with a new onset or recently progressing scoliosis, spasticity with or without contractures, decrease in motor function and low back pain at the site of closure. One or more of these findings was present in all cases and led to the suspicion of tethered spinal cord. The diagnosis of tethered cord was confirmed in all cases by MRI or CT myeolography. In order to isolate tethering as the etiology for the patients’ clinical deterioration, patients with concomitant CNS complications, e.g. shunt dysfunction or hydromyelia were excluded from the study. Twenty-nine such patients, of an initial 59, who would have otherwise been considered, were excluded on the basis of this criteria of concomitant CNS complications. The results of release 1 year after the procedure were as follows: regarding scoliosis, in 75% of cases the curve either remained stable or decreased by more than 10°, with 25% experiencing curve progression of >10°. The most recent follow-up in this group revealed that 11.8% experienced a decrease in curvature of >10°; 47.1% remained stable, and 41.2% ultimately progressed 10°. In the group with spasticity, 43.8% improved; 56.3% remained stable, and none worsened. Most (78.6%) of the children who had experienced a decline in motor function improved postopera-tively, and all those with back pain experienced complete resolution. In conclusion, tethered cord release in symptomatic low lumbar and sacral level children with myelomeningocele appears to be of benefit, especially with respect to stabilization of scoliosis in selected patients, back pain at the site of closure, and prior decline in motor function. Results in the cases with spasticity were more equivocal.

Spinal Lipomas in Children

“…It has been proposed that the pathophysiology of tethering originates from the stretching effect on the spinal cord [18, 39, 40]. The resulting decreased spinal cord blood flow, and impairment of mitochondrial oxidative metabolism, have been proposed as mechanisms leading to neuronal dysfunction [40].…”

Tethered Cord Syndrome: An Updated Review

“…In children, some series have reported success rates of 100% for pain relief[ 4, 50]. In the adult population, where pain tends to be a more prominent component of TCS, improvement in pain status is seen in over 75% of surgically untethered patients[ 25,51,52,53].…”

“…In both pediatric and adult surgical series, stabilization or improvement in neurological function is reported to be achieved in roughly 80–90% of patients[ 25, 39, 41, 51, 52, 54, 55]. Significant improvement in motor function is achieved in most large series in the 25–80% range[ 17, 25, 39, 41, 44,50,51,52,53,54,55,56,57]. There does not appear to be a difference in motor improvement rates between pediatric and adult series.…”

“…As with neurological function, a large proportion of patients obtain stabilization of curve progression or improvement with surgical untethering, with long-term stabilization/improvement rates of 43–63%[ 50, 61, 62]. Nearly all patients with curves greater than 40°[ 61, 62], or thoracic-level myelomeningoceles[ 63], fail to stabilize with untethering and ultimately require fusion.…”

Fetal surgery for spina bifida: Past, present, future

“…While surgical release can limit further damage in some patients, the functional decline may be irreversible in others. [21,22] Therapeutic interventions aimed at preventing these complications could significantly impact the quality of life of children with MMC. In utero intervention may hold the key for reversing the hindbrain herniation, limiting the need for ventriculoperitoneal shunting due to hydrocephalus, and preventing late loss of function due to tethering.…”