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Non-syndromic unilambdoid craniosynostosis

ORPHA:620113· ICD-10 Q75.0

Definition

A form of non-syndromic unisutural craniosynostosis characterized by premature fusion of one of the two lambdoid sutures, resulting in ipsilateral occipitoparietal flattening, contralateral parietal and frontal bossing, occipitomastoid bulge, and posterior-inferior displacement of the ipsilateral ear. The skull appears trapezoid from above, while the face is typically C-shaped with ipsilateral convexity.

Prevalence
<1 / 1 000 000
Age of onset
Neonatal