Adult postrepair myelomeningocoele and tethered cord syndrome: good surgical outcome after abrupt neurological decline.

Adults who have had repair of an open myelomeningocoele at birth are susceptible to a variant of adult onset tethered cord syndrome (ATCS). Precipitous and profound loss of lower extremity motor function occurred in two postrepair adult patients, but was not seen in any of our 12 cases of adult tethered cord with any other aetiologies. Both postrepair ATCS patients made a good recovery after surgical release of the tether. For the patients with other aetiologies, surgery yielded improvement or recovery of urinary continence in 57%, relief from pain in 78% and improved strength in 80%. Evidence of retethering was observed in 25% of the operated patients at intervals ranging from 1 to 9 years postoperatively. We conclude that surgical release of tether can reverse incontinence in ATCS of any aetiology and that in the post-myelomeningocoele repair patient, both dexamethasone and surgical intervention are helpful in reversing acute neurological deterioration.