Should Children With Down Syndrome Be Screened for Atlantoaxial Instability?

Symptomatic Atlantoaxial Instability in an Adolescent with Trisomy 21 (Down’s syndrome)

“…While symptomatic AAI is considered rare in people with T21, it is rarer to have documented radiologic evidence of a normal lateral spinal cord radiograph followed by the development of symptomatic AAI, particularly AAI progressing to serious complications (AAP, 1995, Pueschel, 1998, Davidson, 2008). 2-4 This case also emphasizes that AAI is not static and a normal radiologic exam cannot be used to predict future development of AAI.…”

Atlantoaxial subluxation and Down syndrome: A cross-sectional analysis

“…While recommendations for screening have gone in and out of favor over the last few decades, it is more likely that a DS patient will be screened for AAS when compared to a patient without DS, particularly when undergoing preparticipation evaluations for physical activity. [ 10 11 12 ] While recommendations supporting general screening for risk of future development of AAS in DS patients have been declining, it remains common for DS patients to be screened for asymptomatic AAS before engaging in new activities that may include forces to the head and neck such as gymnastics or contact sports. [ 13 ]…”

Repeat Pediatric Trisomy 21 Radiographic Exam: Does Atlantoaxial Instability Develop Over Time?

“…Proponents include some anesthesiologists and surgeons, who point out that the AAP recommendations are meant for normal follow-up and do not account for loss of protective tone under anesthesia nor necessity for at-risk neck positioning for procedures, as well as the devastating clinical severity of the rare injury events which can include quadriplegia. 25 Limitations of the study include the limited time interval average of 4.3 years. However, we included a wide range of ages, and age at final radiograph between stable and unstable groups were not different, thus the data does not indicate that AAI develops linearly with age in patients without os odontoideum and thus we did not recommend an radiographic interval time.…”

“…Opponents of routine surveillance include some pediatricians who point to the low rate of symptomatic AAI, studies that demonstrate safety in contact sports, and findings that the rare neurological injury in an awake patient is generally preceded by a duration of symptoms. Proponents include some anesthesiologists and surgeons, who point out that the AAP recommendations are meant for normal follow-up and do not account for loss of protective tone under anesthesia nor necessity for at-risk neck positioning for procedures, as well as the devastating clinical severity of the rare injury events which can include quadriplegia 25…”