Progressive postnatal pansynostosis (PPP) is a rare form of craniosynostosis characterized by the late fusion of all cranial sutures.
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Epidemiology
This type of craniosynostosis occurs insidiously after birth and presents later in life unlike other types of craniosynostosis which occur during the prenatal period. Most patients have an associated syndrome; Crouzon syndrome is the most common 1.
Clinical presentation
The late fusion of cranial sutures means that the diagnosis is often delayed and typically presents signs of increased intracranial pressure with relatively normal, albeit smaller than average, head size 1,2.
Radiographic features
CT is the imaging modality of choice. It shows:
signs of increased intracranial pressure with effacement of the ventricles, basal cisterns, and other CSF spaces
bone window shows copper beaten skull, characteristic endocortical scalloping
signs of venous hypertension like widened occipital mastoid emissary foramina and prominent subgaleal veins
3D volume rendered bone window shows fusion of all major cranial sutures (pansynostosis)
Differential diagnosis
Includes other causes of raised intracranial pressure which may cause the copper beaten skull appearance.
other types of craniosynostosis
intracranial masses
Hypophosphatasia: pansynostosis occurs in patients with hypophosphatasia associated with other bony features of hypophosphatasia.
See also
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