Atlantoaxial Instability in down Syndrome-Guidelines for Screening and Detection

M, Roy; Baxter, Mike; A, Roy

doi:10.1177/014107689008300708

Cited by 43 publications

(25 citation statements)

References 15 publications

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“…It has not been firmly established that asymptomatic AAI is a precursor to the symptomatic condition. The correlation between radiological and neurological abnormalities was found to be poor [19]. Even the reliability of radiography in diagnosing AAI has been called into question [20].…”

Section: Discussionmentioning

confidence: 99%

“…1). The upper limit of a normal AAOD is 4 mm in subjects who are younger than 15 years and 3 mm in older subjects [3,10,11,19]. The anterior space between the atlas and the axis normally opens in flexion and closes in extension, which is why the AAOD is greater when the neck is radiographed in flexion than it is in neutral or extension positions [16].…”

Section: Introductionmentioning

confidence: 99%

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Cervical spine abnormalities associated with Down syndrome

Ali

Al-Bustan

Al-Busairi

et al. 2006

International Orthopaedics (SICO

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Atlantoaxial instability (AAI) affects 10-20% of individuals with Down syndrome (DS). The condition is mostly asymptomatic and diagnosed on radiography by an enlarged anterior atlanto-odontoid distance. Symptomatic AAI, which affects 1-2% of individuals with DS, manifests with spinal cord compression. Cervical spondylosis, which is common in DS, also has the potential for cord damage but it has received less attention because paediatric populations were mostly studied. Forty-four Kuwaiti subjects with DS, whose ages were ≥15 years, were evaluated clinically and radiographically. Lateral neck radiographs were taken in the neutral and flexion positions. Asymptomatic AAI was diagnosed in eight subjects (18%) and congenital anomalies of C1-2 were found in five (12%). Five patients had AAI in flexion only while three patients had it in both views. Three patients with AAI had odontoid anomalies contributing to the condition. When assessing AAI, the posterior atlanto-odontoid distance has to be considered because it indicates the space available for the cord. Cervical spondylosis was noted in 16 (36%) subjects. Degenerative changes increased with age, occurred earlier than in the normal population, and affected mostly the lower cervical levels. Half the patients with AAI had cervical spondylosis, a comorbidity that puts the cord at increased risk.Résumé L'instabilité de la charnière occipito atloïdienne affecte 10 à 20% des sujets présentant un Down syndrome (trisomie 21). Cette instabilité est souvent asymptomatique et seulement diagnostiquée sur les radiographies qui montrent un élargissement de la distance antérieure atloïdo-odontoide. L'asymptologie de l'instabilité occipito atloïdienne affecte 1 à 2% des sujets présentant un Down syndrome avec des signes manifestes de compression médullaire. La spondylose cervicale qui est habituelle dans le Down syndrome peut potentialiser des lésions de la moelle. 44 sujets Koweitis présentant un Down syndrome dont l'âge était supérieur à 15 ans ont été évalués cliniquement et radiographiquement. Des radiographies de la colonne cervicale de profil ont été effectuées en position neutre et en position de flexion. Une instabilité occipito atloïdienne a été diagnostiquée chez 8 sujets (18%) avec des anomalies congénitales de C1 C2, chez 5 sujets (12%). 5 patients présentaient une instabilité uniquement en flexion et 3 patients présentaient une telle instabilité sur tous les clichés. 3 patients avec une instabilité occipito atloïdienne présentaient des anomalies de l'odontoide aggravant encore les conditions locales. Lorsqu'il existe une instabilité occipito atloïdienne, la distance C1 odontoide doit être évaluée car elle indique l'espace possible pour la moelle. Une spondylolyse cervicale a été relevée chez 16 patients (36% des sujets). Avec l'âge, les lésions dégénératives peuvent évoluer, survenant plus tôt que dans la population normale et peuvent léser les différents niveaux de la colonne cervicale. La moitié des patients avec une instabilité occipito atloïdienne ont un...

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Cervical spine abnormalities associated with Down syndrome

Ali

Al-Bustan

Al-Busairi

et al. 2006

International Orthopaedics (SICO

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“…Current evidence does not support performing routine screening radiographs for assessment of potential atlantoaxial instability in asymptomatic children. [55][56][57][58][59][60][61][62][63][64] Parents should be advised that participation in some sports, including contact sports such as football and soccer and gymnastics (usually at older ages), places children at increased risk of spinal cord injury 65 and that trampoline use should be avoided by all children with or without Down syndrome younger than 6 years and by older children unless under direct professional supervision. 66,67 Special Olympics has specific screening requirements for participation in some sports.…”

Section: The Asymptomatic Childmentioning

confidence: 99%

Health Supervision for Children With Down Syndrome

2011

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These guidelines are designed to assist the pediatrician in caring for the child in whom a diagnosis of Down syndrome has been confirmed by chromosome analysis. Although a pediatrician's initial contact with the child is usually during infancy, occasionally the pregnant woman who has been given a prenatal diagnosis of Down syndrome will be referred for review of the condition and the genetic counseling provided. Therefore, this report offers guidance for this situation as well.

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“…Previous publications have identified soft tissue changes including decreased paraxial muscle tone and increased ligamentous laxity in patients with Down syndrome [4, 7, 8, 11]. More recently, abnormally flattened articulations between the occipital condyles and atlas lateral masses have also been identified, increasing the degrees of movement at the craniocervical junction [7, 17-20].…”

Section: Discussionmentioning

confidence: 99%

Dynamic MRI in the Evaluation of Atlantoaxial Stability in Pediatric Down Syndrome Patients

Melamed

Krieger

2019

Pediatr Neurosurg

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Background/Aims: Down syndrome is the most common inherited disorder. Some patients develop atlantoaxial instability. Existing screening guidelines were developed prior to availability of MRI. We present predictors for deficit using dynamic MRI of the craniocervical junction. Methods: A retrospective review of Down syndrome patients from 2001 to 2015 was carried out. Patients were considered symptomatic if they had clinical deficits or signal change on MRI. Measurements were taken at the atlantoaxial junction and structural abnormalities noted. Analysis was performed with SPSS. Results: A total of 36 patients were included. Patients averaged 93 months of age with a follow-up of 57 months. No asymptomatic patients developed myelopathy during follow-up. During dynamic imaging, symptomatic patients had greater changes in space available for the cord (SAC) (5.2 vs. 2.7 mm; p < 0.001) and atlantodental interval (ADI) (2.8 vs. 1.3 mm; p = 0.04). These patients were also more likely to have a bony anomaly (50 vs. 13%; p = 0.03). Conclusion: This study characterizes the range of motion seen on dynamic MRI and provides parameters that can be used to distinguish patients at risk for neurologic injury. Changes greater than 3 mm in ADI or 5 mm in SAC during dynamic MRI or any bony abnormality warrants further investigation. Patients without these features may be able to avoid an unnecessary intervention.

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Atlantoaxial Instability in down Syndrome-Guidelines for Screening and Detection

Cited by 43 publications

References 15 publications

Cervical spine abnormalities associated with Down syndrome

Cervical spine abnormalities associated with Down syndrome

Health Supervision for Children With Down Syndrome

Dynamic MRI in the Evaluation of Atlantoaxial Stability in Pediatric Down Syndrome Patients

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