Progressive postnatal pansynostosis

Last revised by Dalia Ibrahim on 24 Feb 2023

Progressive postnatal pansynostosis (PPP) is a rare form of craniosynostosis characterized by the late fusion of all cranial sutures.

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.

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.

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)

Includes other causes of raised intracranial pressure which may cause the copper beaten skull appearance.

Hypophosphatasia: pansynostosis occurs in patients with hypophosphatasia associated with other bony features of hypophosphatasia.


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