Tethered cord in patients affected by anorectal malformations: a survey from the ARM-Net Consortium
María Fanjul1, I Samuk2, P Bagolan3
1Department of Pediatric Surgery, Gregorio Marañón University General Hospital, Calle del Dr. Esquerdo, 46, 28007, Madrid, Spain. maria.fanjul@salud.madrid.org.
Insights
This study found consensus on screening all patients with anorectal malformation (ARM) for spinal cord tethering (TC) at birth. However, significant discrepancies exist in TC definition, diagnostic tools, and complementary tests among European pediatric centers.
Area of Science:
- Pediatric Surgery
- Neurology
- Urology
Background:
- Spinal cord tethering (TC) is a potential complication in patients with anorectal malformation (ARM).
- Management of TC in ARM patients lacks standardized protocols across European pediatric centers.
Purpose of the Study:
- To assess the consensus on spinal cord tethering (TC) management in patients with anorectal malformation (ARM) across European pediatric centers.
- To identify variations in diagnostic approaches and treatment strategies for TC in ARM.
Main Methods:
- A survey was distributed to pediatric surgeons within the ARM-Net Consortium.
- Data collected included TC prevalence, screening practices, diagnostic tools, and treatment preferences.
Main Results:
- High agreement (96%) exists for screening all ARM patients for TC, with 79% starting at birth.
- Significant variability observed in TC definition, diagnostic tools (ultrasound, MRI), and complementary tests (urodynamic studies, somatosensory-evoked potentials).
- Prophylactic untethering is rarely performed (8% of centers).
Conclusions:
- Consensus supports universal TC screening in ARM patients, with a preference for conservative management.
- Discrepancies in TC definition, screening, and diagnostic methods necessitate the development of standardized protocols.
- Standardized protocols are crucial to reduce variability in TC management for ARM patients.
Purpose:
The goal of this study was to determine the degree of consensus in the management of spinal cord tethering (TC) in patients with anorectal malformation (ARM) in a large cohort of European pediatric centers.
Methods:
A survey was sent to pediatric surgeons (one per center) members of the ARM-Net Consortium.
Results:
Twenty-four (86%) from ten different countries completed the survey. Overall prevalence of TC was: 21% unknown, 46% below 15, and 29% between 15 and 30%. Ninety-six agreed on screening all patients for TC regardless the type of ARM and 79% start screening at birth. Responses varied in TC definition and diagnostic tools. Fifty percent of respondents prefer ultrasound (US), 21% indicate either US or magnetic resonance (MRI) based on a pre-defined risk of presenting TC, and 21% perform both. Discrepancy exists in complementary test: 82% carry out urodynamic studies (UDS) and only 37% perform somatosensory-evoked potentials (SSEP). Prophylactic untethering is performed in only two centers (8%).
Conclusions:
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.
