Symptomatic Atlantoaxial Subluxation in Persons with Down Syndrome

Pueschel, Siegfried M.; Herndon, James H.; Gelch, Melvyn M.; Senft, Karen E.; Scola, Francis H.; Goldberg, Michael J.

doi:10.1097/01241398-198411000-00005

Cited by 90 publications

(37 citation statements)

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“…The 18% prevalence of asymptomatic AAI in this study is comparable to the previously reported ranges of 14 to 20% [7,11,12,15,16,20]. Since the AAOD was greater in flexion than neutral and no patient had AAI in neutral who did not have it in flexion, it may be enough to take a single lateral neck radiograph in flexion to diagnose AAI.…”

Section: Discussionsupporting

confidence: 73%

“…Manifestations include neck discomfort, abnormal gait, change in sphincteric control, upper motor neuron lesion (UMNL), paralysis, and even death. In over 80% of symptomatic patients, this is the end result of chronic gradual instability [15]. In the remaining patients, where it may be preceded by a normal radiograph, it can be precipitated by neck trauma, sports injury, endotracheal intubation, or head and neck surgery [9,13].…”

Section: Introductionmentioning

confidence: 99%

“…The danger of AAI is that it becomes symptomatic in 1-2% of individuals with DS when the displaced odontoid impinges the spinal cord [15]. Manifestations include neck discomfort, abnormal gait, change in sphincteric control, upper motor neuron lesion (UMNL), paralysis, and even death.…”

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: Discussionsupporting

confidence: 73%

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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“…As there are no conservative means of improving or restoring the biomechanical function of the ligament, significant translatory C1/C2 instability is addressed surgically by posterior fusion [8]. The condition in the presented case with isolated rotatory dislocation however, did not indicate biomechanical compromise of the transverse atlantal ligament.…”

Section: Discussionmentioning

confidence: 69%

Spontaneous rotatory atlantoaxial dislocation without neurological compromise in a child with Down syndrome: a case report

et al. 2008

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Spontaneous atlantoaxial dislocation is a rare recognised complication of Down syndrome. In the majority of cases, dislocation takes place in an anteroposterior direction and is often associated with abnormalities of odontoid development or ossification. Rotatory atlantoaxial dislocation is extremely rare in Down syndrome and this is to our knowledge the first reported case in which modern imaging methods have been described; surface shaded reformats derived from a multislice CT scan were of fundamental importance in making the diagnosis.

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“…The incidence of C1-C2 instability in children with Down syndrome ranges from 7.6 to 22% [29,[82][83][84]. The incidence of symptomatic atlantoaxial instability is much less and varies between 0 and 2.6% [84].…”

Section: Down Syndromementioning

confidence: 99%

Pediatric cervical spine instability

Ghanem

Hage

Rachkidi

et al. 2008

Journal of Children's Orthopaedics

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Cervical spine instability in children is rare but not exceptional and may be due to many factors. Although it mostly occurs at the upper cervical spine, all vertebrae from the occiput to T1 may be involved. It may be acute or chronic, occurring secondary to trauma or due to congenital anomaly, skeletal or metabolic dystrophy or rheumatoid arthritis. It can be isolated or associated with other musculoskeletal or visceral anomalies. A thorough knowledge of embryology, anatomy, physiology and physiopathology of the cervical spine in children is essential to avoid pitfalls, recognize normal variants and identify children at risk of developing cervical spine instability and undertake the appropriate treatment.

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Symptomatic Atlantoaxial Subluxation in Persons with Down Syndrome

Cited by 90 publications

References 0 publications

Cervical spine abnormalities associated with Down syndrome

Cervical spine abnormalities associated with Down syndrome

Spontaneous rotatory atlantoaxial dislocation without neurological compromise in a child with Down syndrome: a case report

Pediatric cervical spine instability

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