Dislocation of the Atlas in Mongolism

Tishler, Jack M.; Martel, William

doi:10.1148/84.5.904

Cited by 79 publications

(24 citation statements)

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“…Craniovertebral instability in Down's syndrome patients has been receiv ing increasing interest every since the report by Spitzer et al [2] of atlantooccipital dislocation in 9 of 29 patients investigated with Down's syndrome. Atlantoaxial insta bility in Down's syndrome has been the most described after the initial publication by Tishlcr and Martel [3] in 1965.…”

Section: Introductionmentioning

confidence: 99%

Craniovertebral Abnormalities in Down’s Syndrome

Menezes

Ryken²

1992

Pediatr Neurosurg

111

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The ligamentous laxity associated with atlantoaxial subluxation has been assumed as a normal occurrence in 15–20% of Down’s syndrome patients. The occipitoatlantoaxial instability that accompanies this entity has been poorly recognized. The medical records and radiographic findings of 18 symptomatic patients with Down’s syndrome and cervicomedullary compromise were reviewed (1979–1991). All patients were evaluated prospectively under protocol for abnormalities of the cranial vertebral junction. There were 11 males and 7 females (age 3–42). ‘Fixed’ atlantoaxial luxation was seen in 8 (5 developed precipitous onset of cervical medullary compression). Occipitoatlantoaxial instability was present in 9 and associated rotary luxation in 9. The average predental space was 8 mm in the neutral position in 18 individuals. Two adolescents had previously undergone atlantoaxial dorsal fusion with subsequent progressive basilar invagination due to unrecognized occipitoat-lantal instability. An os odontoideum was seen in 3 patients. Irreducible invagination in 2 was treated with anterior decompression followed by dorsal occipital cervical fixation. The occipital cervical fixation was utilized in 10 individuals. Atlantoaxial dorsal fusion was made in 7 and 2 patients with acute rotary luxation of C1 and C2 were treated with immobilization. Halo immobilization in two individuals following a dorsal occipital cervical fixation produced an anterior fusion at the cranial vertebral complex indicating active vertebral ligamentous pathology. The review focuses on increased incidence of occiput cervical instability in the spectrum of craniovertebral junction abnormalities associated with Down’s syndrome. A large percentage has an odontoid ossicle probably as a result of repeated minor trauma. The results of surgical stabilization have been shown to be excellent.

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Section: Introductionmentioning

confidence: 99%

Craniovertebral Abnormalities in Down’s Syndrome

Menezes

Ryken²

1992

Pediatr Neurosurg

111

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“…In extension, the gap tended to decrease and the odontoid peg was not shown as well as on the neutral film. The size of the odontoid peg in our patients with Down's syndrome was (14) Female (n=39) [2][3][4][5][6][7][8] (1-2) 3 (8) Total (n=67) [2][3][4][5][6][7][8][9] (1-2)* 7 (10)* ¢15 years Male (n=51) 21 (1-8) 1 (2) Female (n=43) 1-8 (0.9) 1 (2) Total (n=94) 1-9 (1-5)* 2 (2)* *Significant difference, p<O05. 1486 Elliott, Morton, and Whitelaw compared with the peg size for the same age range in normals (shown in fig 2).…”

Section: Resultsmentioning

confidence: 99%

Atlantoaxial instability and abnormalities of the odontoid in Down's syndrome.

Elliott

Morton

Whitelaw

1988

Archives of Disease in Childhood

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“…In 1965, evidence of pathological motion at the atlantoaxial segment in these patients was defined by Tishler and Martel. [41] The initial case of symptomatic atlantoaxial subluxation as well as successful reduction and fusion was reported the following year. [9] Since that time, a significant body of literature has developed in which the pathological entities of the CVJ in patients with Down's syndrome are described.…”

Section: Discussionmentioning

confidence: 99%

Instability of the craniovertebral junction and treatment outcomes in patients with Down's syndrome

Taggard¹,

Menezes²,

Ryken³

1999

FOC

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Operative intervention for the treatment of instability at the craniovertebral junction in patients with Down's syndrome has become somewhat controversial because some authors have reported high surgery-related complication rates and suggested that the incidence of neurological abnormality associated with this abnormal motion may be low. In this report, the authors describe the clinical and radiographic findings in 33 patients treated at their institution. Common presenting symptoms included neck pain (14 patients), torticollis (12 patients), and myelopathy manifested as hyperreflexia (21 patients), or varying degrees of quadriparesis (11 patients). Four patients suffered acute neurological insults, two after receiving routine general anesthetics for minor surgical procedures and two other patients following minor falls. Atlantoaxial instability was the most common abnormality documented on radiography (22 patients). Atlantooccipital instability (15 patients) was also frequently observed and was coexistent with the presence of atlantoaxial luxations in 14 patients. A rotary component of the atlantoaxial luxation was present in 13 cases. In 17 patients bony anomalies were present, the most frequent of which was os odontoideum (10 patients). Twenty-four patients underwent operative intervention, and successful fusion was achieved in 23. In six of nine patients with basilar invagination, reduction was achieved with preoperative traction and thus avoided the need for ventral decompressive procedures. There were no cases of postoperative deterioration, and 22 patients made excellent or good recoveries. The results of this series highlight the clinicopathological phenomena of craniovertebral instability in patients with Down's syndrome and suggest that satisfactory outcomes can be achieved with a low rate of surgical morbidity.

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Dislocation of the Atlas in Mongolism

Cited by 79 publications

References 0 publications

Craniovertebral Abnormalities in Down’s Syndrome

Craniovertebral Abnormalities in Down’s Syndrome

Atlantoaxial instability and abnormalities of the odontoid in Down's syndrome.

Instability of the craniovertebral junction and treatment outcomes in patients with Down's syndrome

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