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. 2006 Aug;30(4):284-9.
doi: 10.1007/s00264-005-0070-y. Epub 2006 Mar 7.

Cervical spine abnormalities associated with Down syndrome

Fawzi Elhami Ali  1 , Mahmoud A Al-Bustan, Waleed A Al-Busairi, Fatema A Al-Mulla, Emad Y Esbaita
Affiliations

Cervical spine abnormalities associated with Down syndrome

Fawzi Elhami Ali et al. Int Orthop. 2006 Aug.

Abstract
in English, French

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 > or = 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.

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 une spondylolyse cervicale, avec une co-morbidité entraînant une moelle à risque.

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Figures

Fig. 1
Fig. 1
Drawing of a normal atlantoaxial joint showing the base of the skull, the upper three cervical vertebrae (numbered), and the measurements of the AAOD and PAOD
Fig. 2
Fig. 2
a–b Lateral view of the cervical spine of a 45-year-old man in neutral (a) and flexion (b). The AAOD is 1 mm in neutral but 4 mm in flexion, indicating AAI (arrowheads). There are degenerative changes in the form of mild narrowing of the C5–6 space and osteophytes
Fig. 3
Fig. 3
Lateral view of the cervical spine in neutral of a 15-year-old male showing a normal AAOD of 3 mm. There are congenital anomalies: odontoid hypoplasia (slender arrow), os odontoideum (thick arrow), and ossiculum terminale (arrowhead)
Fig. 4
Fig. 4
Lateral view of the cervical spine in neutral of a 34-year-old man showing a normal AAOD of 3 mm. There are degenerative changes: narrowing of the C3–4, C5–6, and C6–7 spaces (arrowheads) and posterior osteophytes (arrow)
Fig. 5
Fig. 5
Lateral view of the cervical spine in neutral of a 29-year-old man showing an AAOD of 8 mm indicating AAI (long arrow). There is ossiculum terminale (short arrow) and degenerative changes in the form of narrowing at the C2–3 and C3–4 spaces (arrowheads) with anterior and posterior osteophytes
See this image and copyright information in PMC

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References

    1. {'text': '', 'ref_index': 1, 'ids': [{'type': 'DOI', 'value': '10.1016/0270-3092(86)90019-6', 'is_inner': False, 'url': 'https://doi.org/10.1016/0270-3092(86)90019-6'}, {'type': 'PubMed', 'value': '2938541', 'is_inner': True, 'url': 'https://pubmed.ncbi.nlm.nih.gov/2938541/'}]}
    2. Alvarez N, Rubin L (1986) Atlantoaxial instability in adults with Down syndrome: a clinical and radiological survey. Appl Res Ment Retard 7:67–78 - PubMed
    1. {'text': '', 'ref_index': 1, 'ids': [{'type': 'PubMed', 'value': '7596705', 'is_inner': True, 'url': 'https://pubmed.ncbi.nlm.nih.gov/7596705/'}]}
    2. American Academy of Pediatrics, Committee on Sports Medicine and Fitness (1995) Atlantoaxial instability in Down syndrome. Subject review. Pediatrics 96:151–154 - PubMed
    1. {'text': '', 'ref_index': 1, 'ids': [{'type': 'PubMed', 'value': '2934395', 'is_inner': True, 'url': 'https://pubmed.ncbi.nlm.nih.gov/2934395/'}]}
    2. Burke SW, French HG, Roberts JM, Johnston CE 2nd, Whitecloud TS 3rd, Edmunds JO Jr (1985) Chronic atlanto-axial instability in Down syndrome. J Bone Joint Surg Am 67:1356–1360 - PubMed
    1. {'text': '', 'ref_index': 1, 'ids': [{'type': 'PMC', 'value': 'PMC1029517', 'is_inner': False, 'url': 'https://pmc.ncbi.nlm.nih.gov/articles/PMC1029517/'}, {'type': 'PubMed', 'value': '8215544', 'is_inner': True, 'url': 'https://pubmed.ncbi.nlm.nih.gov/8215544/'}]}
    2. Cremers MJ, Ramos L, Bol E, van Gijn J (1993) Radiological assessment of the atlantoaxial distance in Down’s syndrome. Arch Dis Child 69:347–350 - PMC - PubMed
    1. {'text': '', 'ref_index': 1, 'ids': [{'type': 'PubMed', 'value': '2136808', 'is_inner': True, 'url': 'https://pubmed.ncbi.nlm.nih.gov/2136808/'}]}
    2. Crosby ET, Lui A (1990) The adult cervical spine: implications for airway management. Can J Anaesth 37:77–93 - PubMed