The odontoid process in Morquio-Brailsford's disease. The effects of occipitocervical fusion

Stevens, J. M.; Kendall, B. E.; Crockard, H A; Ransford, A. O.

doi:10.1302/0301-620x.73b5.1910048

Cited by 131 publications

(89 citation statements)

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“…The blood supply to the ossicle is then supplied via the proximal arterial arcade, and this could account for hypertrophy of the anterior atlantal arch, which shares the same blood supply. [25,64] There is now increasing evidence that os odontoideum is more frequently associated with trauma and upper respiratory infections and, hence, is also found in conditions such as the Down's syndrome, Morquio's syndrome, and spondyloepiphyseal dysplasia [4,5,16,23,31,50,52,53,[58][59][60] because these children are more prone to trauma in this region.…”

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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Section: Origins Of Os Odontoideummentioning

confidence: 99%

Pathogenesis, dynamics, and management of os odontoideum

Menezes¹

1999

FOC

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“…Servikal düzeyde spinal kanal anteroposterior çapının 14 mm'nin altında olmasına spinal stenoz denir (1,8). Spinal kord kompresyonu; üst servikal, servikotorasik ve torakolomber düzeylerde olabilir (1,5,7,(9)(10)(11)(12).…”

Section: Discussionunclassified

Spinal involvement in mucopolysaccharidosis type IVa (Morquio syndrome): The importance of MRG in diagnosis and follow-up

Aydın¹,

Eraslan²,

Canda³

et al. 2017

Ege Tıp Dergisi

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ÖzAmaç: Mukopolisakkaridoz (MPS) tip IVa (Morquio Sendromu) otozomal resesif geçişli N-asetilgalaktozamin-6-sulfataz eksikliği sonucu görülen multisistemik tutulum gösteren bir hastalıktır. İskelet sisteminin etkilenmesi temel bulgudur ve odontoid hipoplaziye bağlı servikal miyelopati ve atlantoaksiyal subluksasyona bağlı ağır nörolojik komplikasyon riski yüksektir. Şiddetli kifoskolyoz, platispondili, vertebra korpuslarında yuvarlaklaşma da sıklıkla izlenebilen diğer iskelet deformiteleridir. MPS IVa tanısıyla izlenen 12 hastanın klinik bulguları ve spinal manyetik rezonans görüntüleme (MRG) bulguları değerlendirildi. Gereç ve Yöntem: MPS tip IVa tanısıyla izlenen 12 hastanın demografik özellikleri, sistem tutulumları ve spinal MRG bulguları kaydedildi.Bulgular: Çalışmaya alınan hastaların yaş ortalaması 13.5 (4-33) iken, ortalama tanı yaşı 8 (3-32) idi. 12 hastadan 5'i erkek, 7'si kadındı. Sistemik muayenede korneal bulutlanma (11 olgu) işitme kaybı (5 olgu), hepatomegali (4 olgu), adenoid hipertrofi (opere edilmiş 4 olgu), kardiyak tutulum (9 olgu), tüm hastalarda kemik incelemede disostozis multipleks bulguları saptandı. Spinal MRG'de tüm hastalarda vertebra korpus yükseklik kaybı ve vertebra plana bulgusu, intervertebral disk mesafelerinde genişleme, farklı seviyelerde disk protrüzyonları, foramen magnumda daralma tespit edildi. Dört hastada foramen magnum düzeyinde kranioservikal bileşkede myelopatik değişiklikler görüldü.Sonuç: Hastalar özellikle klinik ve radyolojik bulguları ile tedavi seçenekleri ve dekompresif cerrahi gerekliliği açısından değerlendirilmelidir. Hastaların multidisipliner izlemi, komplikasyonların önlenmesinde ve erken tedavide önem taşımaktadır.

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“…ment of the craniovertebral junction is an important feature of the disorder, marked by dysplasia of the odontoid process and laxity of the ligamentous structures [2,3] . Neurological manifestations result from compression of the brainstem and upper cervical spinal cord from atlantoaxial instability and cervicomedulllary region stenosis [1] .…”

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confidence: 99%