Adult tethered cord syndrome in patients with postrepair myelomeningocele: an evidence-based outcome study

Abstract

Object: As patients with myelomeningocele age, tethered cord syndrome (TCS) in adults with postrepair myelomeningocele has become more common. The authors have structured an evidence-based review of the literature for the purpose of addressing the following questions. (1) How is the diagnosis made? (2) What is the natural history that occurs in adults with postrepair myelomeningocele and TCS? (3) What are the criteria for operative intervention? (4) What is the functional outcome for patients with postrepair myelomeningocele?

Methods: A computerized search of the National Library of Medicine of the English-language literature published from 1966 to 2003 was performed. Articles pertaining to the clinical aspects and management of postrepair myelomeningocele in adults, TCS in adults resulting from closed defects, and pediatric patients with postrepair myelomeningocele were reviewed. No Level 1 or 2 data were located; however, the search yielded Level 3 and 4 evidence in the literature in which the clinical syndrome, underlying pathology, and the imaging and electrophysiological evaluation for TCS in adults are discussed.

Conclusions: Analysis of the available data indicates the following. (1) A lower lesion level predisposes patients to symptomatic tethering; moreover, orthopedic and urological deterioration will occur in the majority of these patients. (2) Tethered cord release should be considered for adult patients with postrepair myelomeningocele when clinical symptoms, imaging studies, urodynamics, and somatosensory evoked potentials are consistent with TCS. (3) Prompt, aggressive untethering surgery within 5 years of symptom onset, along with long-term follow up to check for delayed retethering, is recommended. The overall outcome for patients with postrepair myelomeningocele may not be as good as the outcome for adults with closed dysraphism.