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Distinctive tomographic abnormalities of the craniocervical region in a patient with osteogenesis imperfecta type IV B.

Kaissi AA, Klaushofer K, Grill F - Clinics (Sao Paulo) (2010)

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Osteogenesis imperfecta is a clinically and genetically heterogeneous group of heritable disorders of connective tissue characterized by reduced bone mass (osteopenia) with associated bone fragility... Basilar impression involves the upward (vertical) migration of the odontoid process into the foramen magnum with a depression in the cranium... The combination of platybasia and basilar impression can lead to severe distortion of the spinal cord and the anterior brain stem. – The specific structures that can be involved include the upper cervical cord, medulla, pons, mid-brain, cerebellum, as well as the vertebrobasilar system... Lateral skull radiographs showed significant frontal and occipital bossing, multiple wormian bones in the lambdoid suture associated with ill-defined skull base deformity and total loss of teeth (Figure 1)... Anteroposterior skull radiograph showed excessive aeration of the frontal sinuses, osteopenia and total loss of teeth because of dentinogenesis imperfecta (Figure 2)... Strikingly, 3D sagittal CT scan showed assimilation of the entire anterior arch of the atlas with the foramen magnum... The odontoid process was greater than 7.8 mm above Chamberlain's line* (a) associated with thinned and dysplastic clivus... Note the Wachenheim-Clivus line** (b) in our patient the line does not intersect and is not tangential to the odontoid process (this confirms the existence of marked craniocervical abnormalities)... Severe platybasia associated with significant distortion of the anatomical relations of the posterior fossa... In general, OI type I is a purely qualitative collagen defect, whereas OI of type II, III, and IV are qualitative and quantitative alterations in collagen synthesis. – Levin et al. have proposed the addition of subtypes A and B to the modified Sillence classification system based on the absence or presence of dentinogenesis imperfecta, respectively... He believes that basilar impression occurs with the highest frequency in patients with OI type IVB (patients with mild or moderate risk of fractures and normal scleras, but with dentinogenesis imperfecta)... Most cases of basilar impression in patients with OI and or other forms of osteochondrodysplasias are probably due to the congenital bone malformation of the posterior fossa (primary basilar impression/invagination). – MacKenzie et al. reported the sudden death of a patient with Marfan syndrome that was attributable to a primary type of basilar invagination... Secondary basilar impression, as seen in OI, results from abnormally soft bone that, in effect, slowly melts over the upright cervical spine, which progressively protrudes through the foramen magnum. ,,,, Odontoid abnormalities have a reputation for being overlooked, resulting in a situation involving future instability and a potential risk for neurologic injury.

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Anteroposterior skull radiograph showed excessive aeration of the frontal sinuses, osteopenia and total loss of teeth because of dentinogenesis imperfecta.
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f2-cln_65p647: Anteroposterior skull radiograph showed excessive aeration of the frontal sinuses, osteopenia and total loss of teeth because of dentinogenesis imperfecta.

Mentions: Lateral skull radiographs showed significant frontal and occipital bossing, multiple wormian bones in the lambdoid suture associated with ill-defined skull base deformity and total loss of teeth (Figure 1). Anteroposterior skull radiograph showed excessive aeration of the frontal sinuses, osteopenia and total loss of teeth because of dentinogenesis imperfecta (Figure 2).


Distinctive tomographic abnormalities of the craniocervical region in a patient with osteogenesis imperfecta type IV B.

Kaissi AA, Klaushofer K, Grill F - Clinics (Sao Paulo) (2010)

Anteroposterior skull radiograph showed excessive aeration of the frontal sinuses, osteopenia and total loss of teeth because of dentinogenesis imperfecta.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2898544&req=5

f2-cln_65p647: Anteroposterior skull radiograph showed excessive aeration of the frontal sinuses, osteopenia and total loss of teeth because of dentinogenesis imperfecta.
Mentions: Lateral skull radiographs showed significant frontal and occipital bossing, multiple wormian bones in the lambdoid suture associated with ill-defined skull base deformity and total loss of teeth (Figure 1). Anteroposterior skull radiograph showed excessive aeration of the frontal sinuses, osteopenia and total loss of teeth because of dentinogenesis imperfecta (Figure 2).

View Article: PubMed Central - PubMed

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Osteogenesis imperfecta is a clinically and genetically heterogeneous group of heritable disorders of connective tissue characterized by reduced bone mass (osteopenia) with associated bone fragility... Basilar impression involves the upward (vertical) migration of the odontoid process into the foramen magnum with a depression in the cranium... The combination of platybasia and basilar impression can lead to severe distortion of the spinal cord and the anterior brain stem. – The specific structures that can be involved include the upper cervical cord, medulla, pons, mid-brain, cerebellum, as well as the vertebrobasilar system... Lateral skull radiographs showed significant frontal and occipital bossing, multiple wormian bones in the lambdoid suture associated with ill-defined skull base deformity and total loss of teeth (Figure 1)... Anteroposterior skull radiograph showed excessive aeration of the frontal sinuses, osteopenia and total loss of teeth because of dentinogenesis imperfecta (Figure 2)... Strikingly, 3D sagittal CT scan showed assimilation of the entire anterior arch of the atlas with the foramen magnum... The odontoid process was greater than 7.8 mm above Chamberlain's line* (a) associated with thinned and dysplastic clivus... Note the Wachenheim-Clivus line** (b) in our patient the line does not intersect and is not tangential to the odontoid process (this confirms the existence of marked craniocervical abnormalities)... Severe platybasia associated with significant distortion of the anatomical relations of the posterior fossa... In general, OI type I is a purely qualitative collagen defect, whereas OI of type II, III, and IV are qualitative and quantitative alterations in collagen synthesis. – Levin et al. have proposed the addition of subtypes A and B to the modified Sillence classification system based on the absence or presence of dentinogenesis imperfecta, respectively... He believes that basilar impression occurs with the highest frequency in patients with OI type IVB (patients with mild or moderate risk of fractures and normal scleras, but with dentinogenesis imperfecta)... Most cases of basilar impression in patients with OI and or other forms of osteochondrodysplasias are probably due to the congenital bone malformation of the posterior fossa (primary basilar impression/invagination). – MacKenzie et al. reported the sudden death of a patient with Marfan syndrome that was attributable to a primary type of basilar invagination... Secondary basilar impression, as seen in OI, results from abnormally soft bone that, in effect, slowly melts over the upright cervical spine, which progressively protrudes through the foramen magnum. ,,,, Odontoid abnormalities have a reputation for being overlooked, resulting in a situation involving future instability and a potential risk for neurologic injury.

Show MeSH
Related in: MedlinePlus