hemivertebrae
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Hemivertebra is a type of vertebral anomaly and results from a lack of formation of one half of a vertebral body. It is a common cause of congenital scoliosis.
Epidemiology
The estimated incidence is at ~0.3 per 1000 live births .
Pathology
It falls under the spectrum of segmentation anomalies and can involve one or multiple levels.
A hemivertebra acts as a wedge within the vertebral column, resulting in curvature away from the side on which it is present.
Associations
Recognized associations are many and include:
- Aicardi syndrome
- cleidocranial dysostosis
- gastroschisis
- Gorlin syndrome
- fetal pyelectasis
- Jarcho-Levin syndrome
- OEIS complex
- VACTERL association
- mucopolysaccharidosis
Classification
A hemivertebra can be classified according to many types which include:
Attachment
- fully segmental (free)
- not attached to either vertebra above or below
- most concerning
- semi segmental: half segment is fused with vertebra above or below with no intervening intervertebral disc
- non-segmental
- not separated from (i.e. connected to) either level above or below
- causes less of a concern
- incarcerated
- joined by pedicles to levels above and below
- causes less of a concern
Orientation
- dorsal hemivertebra: classically results in kyphosis
- lateral hemivertebra: classically results in scoliosis
- ventral hemivertebra (extremely rare): results in lordosis
Radiographic features
Antenatal ultrasound
A hemivertebra may be seen as an asymmetrical vertebral body on sagittal or coronal scanning, while on axial scanning, a focal defect may be seen on either side of the vertebral column .
Plain radiograph / CT
Usually directly outlines the bony anomaly and is often seen as a wedge-shaped vertebral body.
Treatment and prognosis
The prognosis can be variable dependent on the type of segmentation anomaly, from being progressive to a non-progressive deformity. The strongest negative impact is when a hemivertebra occurs at the lumbosacral level .
Differential diagnosis
Consider
Siehe auch:
und weiter:
- angeborene Wirbelanomalien
- Kleidokraniale Dysplasie
- Skoliose
- Gorlin-Goltz-Syndrom
- VACTERL-Assoziation
- Aicardi-Goutières-Syndrom
- skelettale Anomalien des Thorax
- OEIS complex
- Goldenhar-Gorlin-Syndrom
- Jarcho-Levin-Syndrom
- vertebrale Segmentationsanomalien
- Robinow-Syndrom
- spondylothoracic dysotosis
- hemivertebra with congenital scoliosis
- kostovertebrale Dysostose
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