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  • Updated 01.19.2021
  • Released 08.08.1994
  • Expires For CME 01.19.2024

Tethered spinal cord

Introduction

Overview

Although historically thought of as a childhood disorder, data demonstrate that tethered cord syndrome can present even in adults. Furthermore, the diagnosis and treatment are relatively straightforward in most patients, and permanent disability may result if tethered cord is not detected and corrected early. The authors describe the etiology, pathogenesis, presentation, diagnosis, and treatment of tethered cord syndrome in both adults and children.

Key points

• Although usually considered a childhood disorder, tethered cord syndrome can present in adulthood.

• Prior neurosurgical, urological, or orthopedic surgeries may increase the risk of tethering if around the spine, or have been performed due to symptoms that were not appreciated as due to tethered cord.

• Failure to timely diagnose and treat tethered cord syndrome results in worse long-term outcome.

• Neurologists are often the medical providers that make the diagnosis.

• Tethered cord syndrome describes a constellation of symptoms secondary to tethering of the spinal cord.

• Occult tethered cord syndrome is increasingly being recognized, presenting with either pain or urological symptoms in adults or children, respectively.

• Radiological diagnosis includes filum terminale thickness greater than 2 mm or conus medullaris position below L1-L2 on MRI.

Historical note and terminology

The first clinical description of the tethered cord syndrome appears to be in an 1857 report by A Johnson of a young child with worsening symptoms that improved after surgery; a lesion consistent with a lipoma was found, and the spinal cord was freed from its dural attachments. In 1875, Virchow introduced the term “spina bifida occulta.” Then, in 1891, a patient underwent untethering surgery by WL Jones for lower extremity weakness, atrophy, and deformities; division of a “dense adventitious fibrous band” was done, and the patient showed improvement 6 months after surgery. In 1916, the first description that symptoms could be worsened by activity was reported by WG Spiller (101). Recognition that early treatment might result in improved outcome was noted as early as 1918 by WM Brickner. Subsequent articles have reported tethered cord syndrome in many clinical situations, both in children as well as adults. The widespread recognition of the tethered cord syndrome is relatively recent, particularly as it relates to cases of myelomeningocele and lipo(myelo)meningoceles. The first recognition that multiple pathophysiological entities could give rise to this common clinical presentation was in the 1950s, and that was when terms such as “filum terminale syndrome” and “cord-traction syndrome” began to be used (28). The first major series was that of Hoffman, Hendrick, and Humphreys in 1976 (41), in which the term “tethered spinal cord” originated. Our current understanding of this syndrome has advanced to the point where it is now considered a lesion complex with heterogeneous causes (44; 02; 60; Yamada and Won, 2007; 120). Operative care of this lesion complex has advanced over the last few decades. Finally, our understanding of the pathophysiology of this condition was advanced by Yamada and colleagues who, using an experimental model of spinal cord tethering, found evidence of cellular ischemia (120; 25). An animal model was reported for tethered cord syndrome, based on chronic slow traction, which will hopefully yield new information on the pathogenesis of this syndrome, as well as help delineate when and if patients should undergo surgical intervention (43).

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