Progressive postnatal pansynostosis

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

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. The majority of patients have an associated syndrome; Crouzon syndrome being the most common .

Clinical presentation

The late fusion of cranial sutures means that the diagnosis is often delayed and typically presents with signs of increased intracranial pressure with rather normal, albeit smaller than average, head size .

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.

See also