• The strongest evidence exists for MRI accurately diagnosing tethered spinal cord and studies showed moderate to high sensitivity and specificity (moderate strength of evidence).
• A small number of existing studies indicated benefits of prophylactic surgery for motor function and stability of neurological status over time, but it is also associated with complications such as surgical site infection (low strength of evidence).
• A larger body of evidence evaluated various treatments, primarily surgical detethering, for symptomatic patients. Studies reported improvement of neurological status with surgical detethering (low strength of evidence) but it was also associated with post operative complications such as cerebrospinal fluid leakage (moderate strength of evidence).
• A very small body of evidence exists for revision detethering and spinal column shortening for repeat surgery (low or insufficient strength of evidence for all outcomes).

Objectives. To summarize the evidence regarding diagnosis, prophylactic treatment, symptomatic treatment, and repeat surgery of tethered spinal cord.

Data sources. We searched PubMed®, Embase®, CINAHL, Web of Science, SCOPUS, clinicaltrials.gov, ICTRP, Cochrane Database of Systematic Reviews, PROSPERO, ECRI repository, G-I-N, MagicApp, and ClinicalKey from inception to March 2024; reference-mined reviews; and contacted research authors.

Review methods. The review followed a detailed protocol and was supported by a Technical Expert Panel. Systematic review software (DistillerSR) was utilized for all screening and data extraction tasks. Citation screening was facilitated by machine learning; two independent reviewers each screened full text citations for eligibility; one literature reviewer extracted data and a methodologist checked for accuracy. Risk of bias assessments focused on key sources of bias for diagnostic and intervention studies. We conducted strength of evidence (SoE) and applicability assessments for key outcomes. The protocol for the review has been registered in PROSPERO (CRD42023461296).

Results. Searches identified 6,285 citations; 2,005 were obtained as full text. In total, 103 studies met inclusion criteria, with an additional 355 case series providing additional information. We found the strongest evidence for accuracy of MRI in diagnosing tethered spinal cord. Specifically, studies indicated this modality has medium to high diagnostic sensitivity and specificity (moderate SoE). A small number of existing studies suggested benefits of prophylactic surgery, but it was also associated with complications such as surgical site infection (low SoE). A larger body of evidence evaluated various treatments for symptomatic patients, with the majority focused on surgical detethering. Studies reported improvement of neurological status after surgical detethering (low SoE), but it was also associated with post-operative complications such as cerebrospinal fluid leakage (moderate SoE). A very small body of evidence exists for revision detethering and spinal column shortening for repeat surgery (low or insufficient SoE for all outcomes). Across diagnosis, prophylactic treatment, symptomatic treatment, and repeat surgery there was insufficient evidence for multiple key outcomes (e.g., over- or undertreatment, clinical impact of diagnostic modalities, ambulation or quality of life outcomes) and thus no evidence statements could be derived.

Conclusions. The evidence base for the diagnosis and treatment of tethered spinal cord is limited, with few exceptions (use of MRI or ultrasound for diagnosis, surgical detethering improving neurological status in symptomatic patients, complications associated with open detethering surgery) and would benefit from stronger study designs that include tool evaluations reporting diagnostic performance and treatment studies with concurrent comparator.

This evidence review was funded by the Patient Centered Outcomes Research Institute under Contract No. 75Q80120D00009 from the Agency for Healthcare Research and Quality, U.S. Department of Health and Human Services, by the Patient-Centered Outcomes Research Institute through a memorandum of Agreement Amendment, number 22-642M-22.

Hsieh P, Apaydin E, Briggs RG, et al. Diagnosis and treatment of tethered spinal cord: a systematic review. Pediatrics. 2024 Oct 25:e2024068270. [Epub ahead of print.] doi: 10.1542/peds.2024-068270. PMID: 39449659.

Hsieh P, Apaydin E, Briggs RG, Al-Amodi D, Aleman A, Dubel K, Sardano A, Saint-Val J, Zhang D, Blythe K, Sysawang K, Yagyu S, Motala A, Tolentino D, and Hempel S. Diagnosis and Treatment of Tethered Spinal Cord. Comparative Effectiveness Review No. 274. (Prepared by the Southern California Evidence-based Practice Center under Contract No. 75Q80120D00009.) AHRQ Publication No. 24(25)-EHC031. PCORI® Publication No. 2024-SR-04. Rockville, MD: Agency for Healthcare Research and Quality; October 2024. DOI: https://doi.org/10.23970/AHRQEPCCER274. Posted final reports are located on the Effective Health Care Program search page.