TETHERED CORD IN PATIENTS AFFECTED BY ANORECTAL MALFORMATION: A SURVEY FROM THE ARM-NET CONSORTIUM

Purpose: Variation exists in the management of tethered cord (TC) in patients with anorectal malformations (ARM). The goal of this study was to determine the degree of consensus in the current management of TC in a large cohort of European paediatric centres. Methods: A survey was sent in February 2017 to all paediatric surgeons members of the ARM-Net Consortium. Survey questions pertained to definition of TC, prevalence, screening, complementary studies and management. Results: Twenty-three surgeons (79,3% of eligible surgeons) from 10 different countries completed the survey. Overall prevalence of TC among ARM patients was below 15% in 47,6% of centers. Ninety-five percent agree on screening all patients for TC regardless type of ARM. Most of them start screening their patients at birth (76,2%). Responses varied substantially in tethered cord definition and diagnostic tools. The majority (50%) prefers ultrasound imaging. In 76,2% of centers there is a paediatric radiologist specialized in ARM. Discrepancy exists in complementary test: 80% urodynamic studies, 14,3% 30 manometry and 38,1% somatosensory-evoked potentials. Ninety percent refer the patient to a paediatric neurosurgeon. Prophylactic untethering is performed in only two centers (9,5%), while 90,5% do not. Conclusions: Association between TC and ARM is well established. We support TC screening in all patients with ARM and conservative management of TC. A paediatric radiologist specialized in ARM and paediatric neurosurgeon must be part of the multidisciplinary team. There is discrepancy in definition of TC, screening tools and complementary test. More accurate consensus on these topics is needed to standardize patient care.