Atlanto-occipital and atlanto-axial dislocations with spinal cord compression in Down's syndrome: a case report and review of the literature

Hungerford, G D; Akkaraju, V.; Rawe, Stephen E.; Young, G F

doi:10.1259/0007-1285-54-645-758

Cited by 56 publications

(17 citation statements)

References 12 publications

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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%

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%

Craniovertebral Abnormalities in Down’s Syndrome

Menezes

Ryken²

1992

Pediatr Neurosurg

112

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“…Together with several other authors, McRae believed that os odontoideum had embryological origin, but required the ongoing stresses of life to trigger instability and produce symptoms after childhood. [8,10,13,17,18,20,27,41,44,50,55,66] It is important to review embryological development and the blood supply to the axis vertebra. [15,37,49] The caudal-most occipital sclerotome or the proatlas divides into a ventral rostral segment and a corresponding dorsal caudal portion.…”

Section: Origins Of Os Odontoideummentioning

confidence: 99%

Pathogenesis, dynamics, and management of os odontoideum

Menezes¹

1999

FOC

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Os odontoideum is an uncommon craniovertebral junction (CVJ) abnormality that exists as a separate ossicle apart from a hypoplastic dens. It usually moves with the clivus or the anterior arch of C-1 (dystopic) or rarely with the dens (orthotopic). Its genesis and natural history have been debated, and its proper treatment remains uncertain. Two hundred and sixty patients, with symptomatic os odontoideum, were evaluated by the author over a 20-year period; the author performed surgery in 134 of these patients. In a prospective study the author evaluated the early childhood history of trauma, the dynamic studies of motion, and the effects of traction by using pleuridirectional tomography, computerized tomography (CT), CT myelography and magnetic resonance (MR) imaging. Operative findings were documented. Early childhood trauma to the CVJ was recorded in 74 patients, in 30 of whom normal odontoid processes were documented at initial examination prior to the patient reaching age 4 years. Acute neurological deterioration following trauma occurred in 63 of 134 patients. Symptoms were insidious in 71 of 134 patients. In six patients, who presented with acute neurological deterioration after trauma and in whom an abnormal spinal cord signal in the cervicomedullary junction was demonstrated on MR imaging, normal CVJ motion dynamics were shown. Motion dynamics varied and were unique to each patient. Irreducible ventral CVJ disease causing cervicomedullary compromise occurred in 28 patients in whom a transpharyngeal ventral decompressive procedure was necessitated. During the transoral operation, the transverse portion of the cruciate ligament was found to be located anterior to the axis body. All patients required dorsal CVJ arthrodesis, which, in 46, was limited to the C1-2 segment. Instability at the C1-2 joints was always multidirectional, as demonstrated on preoperative neuroimaging studies as well as at operation. Sixteen patients presented after completed primary C1-2 dorsal fusion and with worsening deficits. They improved when the range of the fusion was extended to the occiput or if the ventrally located lesion was excised. Os odontoideum is associated with early childhood trauma and is an acquired phenomenon. The presence of abnormal motion dynamics necessitates surgical intervention as do associated neurological deficits. Asymptomatic patients in whom os odontoideum is incidentally discovered and in whom no abnormal motion dynamics are demonstrated should be followed closely.

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“…In many of these cases, as in our patient, there was no discernible precipitating cause. The vast majority of Downs' patients presenting with anteroposterior atlantoaxial dislocation have an associated neurological deficit due to cord compression [5]. Underlying congenital anomalies of C1 and C2 are common; in one series 12 out of 21 had congenital abnormalities such as ossiculum terminale, os odontoideum or hypoplasia of the odontoid process [6], predisposing the odontoid process to slip beneath the transverse ligament.…”

Section: Discussionmentioning

confidence: 99%

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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Atlanto-occipital and atlanto-axial dislocations with spinal cord compression in Down's syndrome: a case report and review of the literature

Cited by 56 publications

References 12 publications

Craniovertebral Abnormalities in Down’s Syndrome

Craniovertebral Abnormalities in Down’s Syndrome

Pathogenesis, dynamics, and management of os odontoideum

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

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