Progressive postnatal pansynostosis
Updates to Article Attributes
Body
was changed:
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 1.
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 shapesize 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
- obstructive hydrocephalus
- intracranial masses
See also
-<p><strong>Progressive postnatal pansynostosis</strong> (<strong>PPP</strong>) is a rare form of craniosynostosis characterized by late fusion of all cranial sutures.</p><h4>Epidemiology</h4><p>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; <a href="/articles/crouzon-syndrome">Crouzon syndrome</a> being the most common <sup>1</sup>.</p><h4>Clinical presentation</h4><p>The late fusion of cranial sutures means that the diagnosis is often delayed and typically presents with signs of <a title="Raised intracranial pressure" href="/articles/raised-intracranial-pressure">increased intracranial pressure</a> with rather normal albeit smaller than average, head shape <sup>1,2</sup>.</p><h4>Radiographic features</h4><p>CT is the imaging modality of choice. It shows:</p><ul>-<li>signs of increased intracranial pressure with <a title="Effacement" href="/articles/efface">effacement</a> of the ventricles, basal cisterns and other CSF spaces</li>-<li>bone window shows <a title="Copper beaten skull" href="/articles/copper-beaten-skull">copper beaten skull</a>, characteristic endocortical scalloping</li>- +<p><strong>Progressive postnatal pansynostosis</strong> (<strong>PPP</strong>) is a rare form of craniosynostosis characterized by the late fusion of all cranial sutures.</p><h4>Epidemiology</h4><p>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; <a href="/articles/crouzon-syndrome">Crouzon syndrome</a> being the most common <sup>1</sup>.</p><h4>Clinical presentation</h4><p>The late fusion of cranial sutures means that the diagnosis is often delayed and typically presents with signs of <a href="/articles/raised-intracranial-pressure">increased intracranial pressure</a> with rather normal, albeit smaller than average, head size <sup>1,2</sup>.</p><h4>Radiographic features</h4><p>CT is the imaging modality of choice. It shows:</p><ul>
- +<li>signs of increased intracranial pressure with <a href="/articles/efface">effacement</a> of the ventricles, basal cisterns and other CSF spaces</li>
- +<li>bone window shows <a href="/articles/copper-beaten-skull">copper beaten skull</a>, characteristic endocortical scalloping</li>
-<li><a title="Obstructive hydrocephalus" href="/articles/obstructive-hydrocephalus">obstructive hydrocephalus</a></li>- +<li><a href="/articles/obstructive-hydrocephalus">obstructive hydrocephalus</a></li>
Unable to process the form. Check for errors and try again.