CLOVERLEAF DEFORMITY (aka Kleeblattschädel)
We can visualize in this case…
- Metopic synostosis, which causes the forehead pinching.
- Bicoronal synostosis causes skull widening.
- This particular pattern of multi-suture closure is consistent with cloverleaf deformity (aka Kleeblattschädel), which occurs when multiple sutures fuse prematurely – unfortunately, the brain actually can grow through the anterior fontanelle.
Craniosynostosis - Multiple Sutures
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Descriptive Terminology
Cloverleaf Deformity (aka Kleeblattschädel)
- Multiple sutures fuse prematurely – unfortunately, the brain actually can grow through the anterior fontanelle, can be a finding of certain genetic syndromes as described below.
FGFR2 gene (Fibroblast Abnormality)
Apert Syndrome
- Multiple suture synostoses w/possible cognitive delays
- Syndactly vs Polydactly
- Possible hearing loss, hyperhidrosis, spine fusion, oro-palatal malformations
- Genetics: Autosomal dominant, FGFR2 gene abnormality, which is important in fibroblast embryonic development of bone.
Crouzon Syndrome
- Multiple suture synostoses w/o cognitive delays
- Possible hearing loss and oro-palatal malformations
- Genetics: Autosomal dominant, FGFR2 gene abnormality, which is important in fibroblast embryonic development of bone.
Pfeiffer Syndrome
- Multiple suture synostoses w/anywhere from no neurologic complications to significant complications.
- Syndactly vs Brachydactly
- Possible Anykylosis (bone fusion at the joints)
- Genetics: Autosomal dominant, FGFR2 gene abnormality, which is important in fibroblast embryonic development of bone.
RAB23 or MEGF8 gene (vesicle transport)
Carpenter syndrome
- Multiple suture synostoses w/possible cognitive delays
- Brachydactly vs Polydactly vs Syndactly
- Cryptorchidism
- Kyphoscoliosis
- Genetics: Autosomal recessive, RAB23 or MEGF8 gene mutations, which is important in vesicle transport.