Tethered cord in patients affected by anorectal malformations: a survey from the ARM-Net Consortium

Fanjul, M; Samuk, Inbal; Bagolan, Pietro; Leva, Ernesto; Sloots, Cornelius E. J.; Giné, C; Aminoff, Dalia; Midrio, Paola

doi:10.1007/s00383-017-4105-4

Cited by 15 publications

(12 citation statements)

References 27 publications

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“…Our results showed that patients with pre-operative bowel dysfunction, did not experienced a functional improvement after detethering. Despite the relatively small number of patients in this series, these data support the idea that the anorectal symptoms only, in both patient groups, are not sufficient to justify the neurosurgical indication [10,12]. Urodynamics (UDS) has been commonly suggested in children with ARM-associated SD, either in the preoperative evaluation or in the postoperative course, after untethering surgery [13].…”

Section: Discussionsupporting

confidence: 56%

Impact of the associated anorectal malformation on the outcome of spinal dysraphism after untethering surgery

et al. 2018

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Purpose To analyze the outcome after untethering surgery in patients with spinal dysraphism (SD), with or without associated anorectal malformation (ARM). Methods Patients operated on for SD, with (Group A) or without (Group B) associated ARM (1999ARM ( -2015, were included. The post-operative outcome was analyzed in the two groups in terms of improving of clinical symptoms (neuro-motor deficits, bladder dysfunction, bowel dysfunction) and of instrumental examinations (urodynamics, bladder ultrasound, neurophysiology). Fisher's exact test and χ 2 test were used as appropriate; p < 0.05 was considered statistically significant. Main results Ten patients in Group A and 24 in Group B were consecutively treated. One patient was lost at follow up. Six patients (25%) in Group B underwent prophylactic surgery. The analysis of the pre-operative symptoms in the two groups showed that a significantly higher number of patients in group A needed bowel management and presented with neuro-motor deficits, compared to group B (p = 0.0035 and p = 0.04, respectively). Group A showed a significant post-operative neuromotor improvement as compared to group B (p = 0.002). Conclusions Based on our results, untethering seems to be effective in neuro-motor symptoms in selected patients with ARM. In ARM patients, untethering surgery does not seem to benefit intestinal and urinary symptoms. The presence of the associated ARM does not seem to impact the medium-term outcome of patients operated for SD.

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Section: Discussionsupporting

confidence: 56%

Impact of the associated anorectal malformation on the outcome of spinal dysraphism after untethering surgery

et al. 2018

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“…Caudal regression (represented by sacral ratio) and tethered cord are associated with anorectal malformation and specifically with cloacal anomalies as is a poorly developed sacrum [9]. Tethered cord is present in 30% of the patients with cloaca [10]. In our population it was present in 30% in the TUM group and also 30% in the complete separation group.…”

Section: Discussionmentioning

confidence: 55%

Factors predicting the need for vaginal replacement at the time of primary reconstruction of a cloacal malformation

Vilanova-Sánchez

Halleran

Reck

et al. 2020

Journal of Pediatric Surgery

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“…Their findings similarly showed lack of consensus in definition of TC, screening tools, and complementary tests [9]. This consortium came to the agreement that screening for TC in all ARM patients should occur at birth, all patients with TC + ARM should have urodynamics performed after diagnosis of TC, observational follow-up should be favored over prophylactic surgery, and a multidisciplinary approach with a pediatric radiologist and specialized neurosurgeon dedicated to patients with ARM should be advocated for [9]. Our study also sought to determine if consensus in any of these components of care for this patient population existed in practice, by surveying pediatric neurosurgeons specifically.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2022

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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.

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Tethered cord in patients affected by anorectal malformations: a survey from the ARM-Net Consortium

Abstract: Survey results support TC screening in all patients with ARM and conservative management of TC. There is discrepancy in the definition of TC, screening tools, and complementary test. Protocols should be developed to avoid such variability in management.

Cited by 15 publications

References 27 publications

Impact of the associated anorectal malformation on the outcome of spinal dysraphism after untethering surgery

Impact of the associated anorectal malformation on the outcome of spinal dysraphism after untethering surgery

Factors predicting the need for vaginal replacement at the time of primary reconstruction of a cloacal malformation

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

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