Tethered spinal cord

Stephen L Nelson Jr MD PhD (Dr. Nelson of Tulane University School of Medicine has no relevant financial relationships to disclose.)
Michael V Johnston MD, editor. (Dr. Johnston of Johns Hopkins University School of Medicine and Chief Medical Officer at Kennedy Krieger Institute has no relevant financial relationships to disclose.)
Originally released August 8, 1994; last updated March 3, 2016; expires March 3, 2019

This article includes discussion of tethered spinal cord and tethered cord syndrome. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

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 is relatively straightforward in most patients, and permanent disability may result if tethered cord is not detected and corrected early. The author describes the etiology, pathogenesis, presentation, diagnosis, and treatment of tethered cord syndrome in both adults and children.

Key points

 

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

 

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

 

• Although more commonly a childhood disorder, it can also occur in previously asymptomatic adults with undiagnosed spinal dysraphism.

 

• Early recognition and treatment may be important for improved outcome, especially in children.

 

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

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 (Spiller 1916). 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 (Garceau 1953). The first major series was that of Hoffman, Hendrick, and Humphreys in 1976 (Hoffman et al 1976), 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 (Humphreys 1986; Agarwalla et al 2007; Lew and Kothbauer 2007; Yamada and Won, 2007; Yamada et al 2007). 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 (Yamada et al 2007; Filippidis et al 2010). 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 (Huang et al 2015).

The content you are trying to view is available only to logged in, current MedLink Neurology subscribers.

If you are a subscriber, please log in.

If you are a former subscriber or have registered before, please log in first and then click select a Service Plan or contact Subscriber Services. Site license users, click the Site License Acces link on the Homepage at an authorized computer.

If you have never registered before, click Learn More about MedLink Neurology  or view available Service Plans.