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Start free with EleplanNon-syndromic unicoronal craniosynostosis
ORPHA:620102Morphological anomaly
Also called Isolated frontal plagiocephaly · Isolated unicoronal craniosynostosis · Non-syndromic anterior synostotic plagiocephaly · Non-syndromic frontoparietal craniosynostosis · Non-syndromic hemicoronal craniosynostosis · Non-syndromic unilateral coronal synostosis
What it is
A form of non-syndromic unisutural craniosynostosis characterized by the premature fusion of one of the two coronal sutures of the newborn, leading to plagiocephaly with flattening of the ipsilateral side of the forehead and a compensatory bulging of the contralateral side. Ocular anomalies (strabismus, hypermetropia, astigmatism) due to orbital dystopia, and ocular torticollis, are commonly associated with in unicoronal craniosynostosis.
Key facts
- Prevalence
- 1-9 / 1 000 000
- Age of onset
- Neonatal
- Inheritance
- Autosomal recessive
- Classified as
- Morphological anomaly
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Common30–79%
9These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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