Iatrogenic neurological injury in children with trisomy 21

Children with trisomy 21 are at a greater risk for craniocervical junction instability than the general population. These children frequently require administration of anesthesia due to surgical (including otolaryngological) interventions and are at risk for neurological injury. We reviewed the current literature describing iatrogenic neurological injury in children with trisomy 21 undergoing anesthesia in order to facilitate the development of safety recommendations.

A systematic review of the literature was performed using Medline, Embase, Scopus, and Google Scholar, following the PRISMA statement. All cases of perioperative neurological injury in children with trisomy 21, aged 18 and under were identified. Clinical and radiographic data were extracted for each report. The data were synthesized to develop recommendations regarding perioperative management.

Of 348 articles screened, 16 cases of iatrogenic neurological injury (in children ages 0.7-18 years) were identified. Three injuries occurred during otolaryngological surgeries, nine during sedation for intubation for non-otolaryngological surgery, one during sedation for neuroimaging, one while restraining a child, and two were due to intraoperative head and neck positioning while anesthetized. Preoperative screening was reported in four cases. A diagnosis of atlantoaxial instability (AAI) or atlantooccipital instability (AOI) was made immediately following symptom presentation in three cases but was often delayed by a median (IQR) of 30(11.5-912.5) days. No cases resolved spontaneously, with 2 patients progressing to brain death and 12 requiring surgical stabilization. Of the latter, seven showed improvement, whereas one died 5 months later. No intraoperative precautions during the index procedure were reported in any of the 16 cases.

Iatrogenic neurological injury in children with trisomy 21 are rare but severe and likely under reported. Although the role of preoperative screening remains controversial, all children with trisomy 21 undergoing surgery should be considered at risk for neurological injury due to confirmed or undiagnosed AAI or AOI and should be transferred and positioned with appropriate caution. Children with instability should be referred for neurosurgical attention for preoperative stabilization to mitigate perioperative risk. It is imperative to consider the possibility of neurological injury secondary to medical procedures, as it is clear that neck manipulation of any sort places these children at risk.