Detection and Management of Tethered Cord in Anorectal Malformation: A Survey of Pediatric Neurosurgeons in the United States

Abstract

Introduction: The reported prevalence of tethered spinal cord in patients with anorectal malformations (ARM) ranges from 9% to 64%. Practice patterns surrounding the diagnosis and management of tethered cord are suspected to vary, with consideration to type of spine imaging, adjunct imaging modalities, what patients are offered surgical intervention, and how patients are followed after detethering. We sought to determine what consensus, if any, exists among pediatric neurosurgeons in the United States in terms of diagnosis and management of tethered cord and specifically, patients with tethered cord and ARM. Methods: A survey was sent to members of the American Society of Pediatric Neurosurgeons (ASPN). Members of the ASPN received an email with a link to an anonymous REDCap survey that asked about their experience with detethering procedures, indications for surgery, diagnostic tools used, and follow-up protocols. Results: The survey was completed by 93 of the 192 ASPN members (48%). When respondents were asked about the total number of all simple filum detetherings they performed annually, 61% (N=57) indicated they perform less than 10 for all tethered cord patients (TC). Ninety-three percent (N=87) of neurosurgeons performed these procedures in patients with simple filum tethered cord and ARM patients (TC + ARM) specifically. When asked about prophylactic detethering in those with a confirmed diagnosis of low lying conus and with a filum fatty terminale, 59.1% (N=55) indicated they would offer this to TC + ARM patients regardless of their age. Regarding pre-operative workup for simple filum detethering, all respondents indicated they would order an MRI in both TC and TC + ARM patients, with a minority also requiring additional testing such as urodynamics, neuro-developmental assessments, and anorectal manometry for both groups. When following patients post-operatively, almost all respondents indicated they would require clinical neurosurgical follow-up with a clinic visit (100% in all simple filum TC patients, 98.9% in fatty filum/low lying conus TC + ARM patients), but there was wide variation in the use of other tools such as urological testing, neurodevelopmental assessment, and anorectal manometry. Discussion/Conclusions: A wide variety of diagnostic criteria and indication for procedural intervention exists for management of tethered cord patients with and without ARM. Further studies are needed to determine outcomes. Prospective protocols need to be developed and evaluated to standardize care for this patient population and determine best practices.