Posterior atlantooccipital subluxation in Down syndrome.

el-Khoury, G Y; Clark, Charles R.; Dietz, Frederick R.; Harre, R G; Tozzi, James E.; Kathol, Mary H.

doi:10.1148/radiology.159.2.2938213

Cited by 29 publications

(16 citation statements)

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“…Occipitoatlantoaxial instability has been reported infre quently [33][34][35]. The natural history and contribution of this dislocation in Down's syndrome is unknown.…”

Section: Discussionmentioning

confidence: 99%

“…We reported our earlier series in 1980 with Down's syndrome and occipitoatlan toaxial instability responding to traction, dorsal occipito cervical fusion and halo cast immobilization [ 19], In 1986. El'Khoury et al [33] described 3 patients with posterior occipital translation, 2 of whom showed no atlantoaxial instability, and 1 had a spontaneously reducible rotary lux ation of Cl. Rosenbaum et al [36] described two cases of asymptomatic atlantoaxial instability in Down's syn drome.…”

Section: Discussionmentioning

confidence: 99%

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Craniovertebral Abnormalities in Down’s Syndrome

Menezes

Ryken²

1992

Pediatr Neurosurg

112

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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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“…Occipitoatlantoaxial instability has been reported infre quently [33][34][35]. The natural history and contribution of this dislocation in Down's syndrome is unknown.…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Craniovertebral Abnormalities in Down’s Syndrome

Menezes

Ryken²

1992

Pediatr Neurosurg

112

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“…Atlanto-occipital subluxation was first reported in association with Down syndrome in 1981 18 and, in contrast with that seen in trauma, is almost always posterior. Other groups of patients have been described by El-Khoury, 19 Brooke, 5 Tredwell, 7 Stein 4 and most recently by Parfenchuck. 6 In 1991, Stein presented five new patients and reviewed nine others previously presented in the literature.…”

Section: Discussionmentioning

confidence: 93%

Atlanto-Occipital and Atlanto-Axial Instability in Children with Down Syndrome

Kattan

McDonald

1996

Ann Saudi Med

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“…[1,20,27,31,32] Atlantooccipital instability and rotary luxation of the atlas are well described in patients with Down's syndrome and should not be overlooked. [11,27,33,37,39,44] Parfenchuck, et al, [30] have suggested that the incidence of posterior atlantooccipital hypermobility in Down's syndrome is 8.5%. Bony anomalies such as os odontoideum and hypoplasia of various craniovertebral elements, including the atlantal ring and condyles, likely increase the risk for instability at the CVJ.…”

Section: Discussionmentioning

confidence: 99%

“…[11,17,24,30,42,43] Clinical correlations were not made in the first description of antlantooccipital luxation by Spitzer, et al [40] Neurological abnormalities have been found in up to 66% of patients with antlantooccipital pitoatlantal instability. [30] In a prospective review of 64 patients with antlantooccipital instability, 61% of the patients were shown to have a posterior occipital subluxation greater than 4 mm and 21% to have a predental space of greater than 5 mm.…”

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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Posterior atlantooccipital subluxation in Down syndrome.

Cited by 29 publications

References 0 publications

Craniovertebral Abnormalities in Down’s Syndrome

Craniovertebral Abnormalities in Down’s Syndrome

Atlanto-Occipital and Atlanto-Axial Instability in Children with Down Syndrome

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

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