Progressive postnatal pansynostosis
Citation, DOI and case data
Young girl with Crouzon syndrome presented with headache and vomiting. Physical exam revealed a normal head shape. On fundoscopy, bilateral grade I papilledema. Serial measurement of head circumference showed a decline in percentile head size starting from 38 months old.
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An unenhanced brain CT was performed. Topogram showed copper beaten skull. Axial CT soft tissue cuts revealed signs of increased intracranial pressure with effacement of the ventricles, basal cisterns and other CSF spaces.
Bone window series showed characteristic endocortical scalloping, widened left mastoid emissary foramina and prominent subgaleal veins, consistent with venous hypertension. 3D volume rendered bone window showed fusion of all major cranial sutures (pansynostosis).
This type of craniosynostosis occurs insidiously after birth and presents later in life, unlike other types of craniosynostosis which occur during the prenatal period.
The late fusion of cranial sutures means that the diagnosis is delayed and typically presents with signs of an increased intracranial pressure with a rather normal, albeit smaller than average, head size.