Congenital odontoid aplasia and posterior circulation stroke in childhood

Phillips, Peter C.B.; Lorentsen, Kevin J.; Shropshire, Lowry C.; Ahn, Hyo Seung

doi:10.1002/ana.410230421

Cited by 32 publications

(16 citation statements)

References 10 publications

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“…The following different congenital cervical anomalies were noted: basilar impression [34], congenital odontoid process defect [35], nonfusion of odontoid with anterior displacement of C1 on C2 [36], ossiculum terminale with subluxation of C1 on C2 [37], congenital odontoid aplasia [38], os odontoid with atlantoaxial dislocation [39], atlantoaxial subluxation with os odontoideum [40], Klippel-Feil syndrome [41, 42]and absent right posterior arch of C1 with superior subluxation [43]. …”

Section: Radiological Investigationsmentioning

confidence: 99%

“…In 2 patients, ventricular shunting [15]and Crutchfield tongs [56]were the only intervention. Six of 10 children with VAD diagnosed as having an underlying congenital cervical anomaly were treated by cervical fusion of the involved cervical vertebra [34, 35, 36, 37, 38, 40]. Experience with endovascular treatment (stent placement and angioplasty for stenosis; coiling of associated aneurysms) for VAD is very limited in children, and clot angioplasty with intra-arterial thrombolysis to recanalize the involved artery was used in only 1 patient [57].…”

Section: Treatmentmentioning

confidence: 99%

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Vertebral Artery Dissection in Children: A Comprehensive Review

et al. 2002

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Vertebral artery dissection (VAD) has been infrequently recognized in children. The authors have reviewed 68 reported cases of VAD in children in the existing literature. An association between routine types of neck movement in sports and the evolution of VAD was recognized in half of the reported cases. Boys outnumbered girls by a ratio of 6.6 to 1, in contrast to adults, for whom the male to female ratio is approximately equal (1.3 to 1). Neck pain, one of the hallmark symptoms of VAD in adults, was infrequently noted in this young population (12%). Most children presented with various combinations of symptoms and signs, including ataxia (53%), headache (38%) and vomiting (34%). Eye signs or symptoms were noted in 72% of patients, and paresis/paralysis of one or more extremity occurred in 54%. Angiography was the method most frequently used to diagnose VAD (63/68; 93%). Magnetic resonance angiography (MRA) revealed pathognomonic signs of VAD in only 3 out of 13 patients evaluated (23%). In this series of 68 patients, 48 reports failed to indicate whether or not a cervical X-ray was performed, but in the 20 patients for whom such information was recorded, half had skeletal abnormalities in the occipital/atlas/axis region. The most common treatments were antiplatelet therapy (n = 15) and anticoagulation with (n = 8) or without (n = 7) supplemental antiplatelet therapy. Asymptomatic recovery occurred in 12 of the 15 patients (80%) who received antiplatelet therapy compared with 4 of the 15 patients (27%) who received anticoagulation therapy with or without antiplatelet therapy. There is a very high incidence of associated cervical anomalies in children with VAD. Further studies are required to determine if noninvasive examinations such as magnetic resonance imaging, ultrasonography, computed tomography angiography and MRA could replace angiography as the modality of choice in establishing the diagnosis of VAD in children. The role of different therapies for children presenting with symptoms related to VAD is unclear.

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Section: Radiological Investigationsmentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

Vertebral Artery Dissection in Children: A Comprehensive Review

et al. 2002

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“…In fact, this seems to be a major factor for posterior circulation stroke at all ages (Hilton-Jones and Warlow 1985). Dissection of the vertebral artery leading to thrombotic occlusion or ischaemia from narrowing of the arterial lumen has been described in children with Klippel-Feil syndrome (Ross et al 1987, Born et al 1988, one with odontoid aplasia Case report (Phillips et al 1988), and one with atlantoaxial subluxation (Kurimoto et al 1988). Patients have been described in whom recurrent trauma to the vertebral arteries has occurred secondary to subluxation and anterior displacement of C1 on C2 (Singer et al 1975, Pascual-Castroveijo et al 1977.…”

mentioning

confidence: 99%

Tethering of the vertebral artery in the congenital arcuate foramen of the atlas vertebra: a possible cause of vertebral artery dissection in children

Cushing¹,

Ramesh

Gardner-Medwin

et al. 2001

Develop Med Child Neuro

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Twelve children with vertebrobasilar artery stroke are reported (seven males, five females; aged 6 months to 15 years). Patient 1 showed an arcuate foramen in the posterior arch of the atlas, an anatomical variant occurring in 3 to 15% of the population. It was hypothesized that the presence of the arcuate foramen might cause tethering of the vertebral artery and lead to its dissection by repetitive trauma. Lateral plain films of the cervical spine in cases of posterior circulation stroke were taken. Eight of 11 patients showed aberrant arcuate foramina. Of the remaining three patients, one had normal cervical spine X‐rays, one had an absent right posterior arch of the atlas following previous surgery for a cervical meningocele, and one patient had incomplete ossification of the vertebrae. Seven of the nine patients with arcuate foramina had vertebral angiograms. In all cases this showed the vertebral artery passing through the arcuate foramen before entering the brain and an appearance consistent with arterial dissection and occlusion at the same site below the foramen. Most documented cases of posterior circulation stroke in children follow trauma, which may be minimal or repetitive, with thrombotic occlusion of the artery at C1‐C2 level. The association with an arcuate foramen and its possible causative role in the genesis of posterior circulation stroke in children has not been previously recognized. There may be a causal association between the presence of an arcuate foramen, tethering of the vertebral artery in the foramen, and dissection from repetitive trauma with movement of the neck.

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“…5 In one study, only 11% of the VBI patients have appropriate dynamic flexion/extension cervical radiographs performed, thus underestimating the association of VBI/PCS with CVJ anomalies. 6 This case was first presented with PCS at the age of 8 years and underlying cause was missed in initial neuroimaging as attendants refused for detailed MRI evaluation with angiogram. It was later diagnosed when PCS reoccurred at the age of 13 years, presented as locked-in syndrome due to pontine infarct.…”

Section: Discussionmentioning

confidence: 99%

Basilar invagination presenting as recurrent posterior circulation stroke with locked-in syndrome in a child: a case report

Kumar

Mehta

Garg

2017

Int J Contemp Pediatr

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Basilar invagination is a type of craniovertebral junction anomaly which occurs when the tip of odontoid process migrates upward into the intracranial space through foramen magnum and most commonly present as progressive neurological deficit implicating the high cervical cord, lower brainstem and cranial nerves. It can rarely present as vertebro-basilar territory insufficiency and occlusion, leading to stroke. Treatment is typically surgical and continues to centre on varying combinations of anterior or posterior decompression with or without traction or fusion. We present a 13-year-old boy with basilar invagination diagnosed on MRI with angiogram, which was missed on initial presentation, leading to recurrent posterior circulation stroke progressed to Locked-in syndrome. This case report emphasis the need of screening for craniovertebral junction anomalies in young patients presenting with posterior circulation stroke.

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Congenital odontoid aplasia and posterior circulation stroke in childhood

Cited by 32 publications

References 10 publications

Vertebral Artery Dissection in Children: A Comprehensive Review

Vertebral Artery Dissection in Children: A Comprehensive Review

Tethering of the vertebral artery in the congenital arcuate foramen of the atlas vertebra: a possible cause of vertebral artery dissection in children

Basilar invagination presenting as recurrent posterior circulation stroke with locked-in syndrome in a child: a case report

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