Progressive postnatal pansynostosis
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Title
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Progressive postnatal pancraniosynostosis (PPP)
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Progressive postnatal pansynostosis (PPP(PPP) is a rare form of craniosynostosis characterized by 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 common1.
Clinical presentation
The late fusion of cranial sutures makemeans that the diagnosis is often delayed and typically presents with signs of increased intra-cranialintracranial pressure with rather normal albeit smaller than average, head shape1,2.
Radiographic features
CT is the imaging modality of choice. It shows;:
- signs of increased
intra-cranialintracranial 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 Diagnosisdiagnosis
Includes other causes of raised intracranial pressure which causesmay cause the copper beaten skull appearance.
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Otherother types of craniosynostosis -
Obstructiveobstructive hydrocephalus -
Intracranialintracranial masses
See also
-<p><strong>Progressive postnatal pansynostosis</strong> (PPP) 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 title="Crouzon syndrome" 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 make the diagnosis delayed and typically with signs of increased intra-cranial pressure with rather normal albeit smaller than average, head shape<sup>1,2</sup>.</p><h4>Radiographic features</h4><p><strong>CT</strong> is the imaging modality of choice. It shows;</p><ul>-<li>signs of increased intra-cranial pressure with effacement of the ventricles, basal cisterns and other CSF spaces.</li>-<li>bone window shows copper beaten skull, characteristic endocortical scalloping,</li>-<li>signs of venous hypertension like widened occipital mastoid emissary foramina and prominent subgaleal veins.</li>-<li>3D volume rendered bone window shows fusion of all major cranial sutures (pansynostosis).</li>-</ul><h4>Differential Diagnosis</h4><p>Includes other causes of raised intracranial pressure which causes copper beaten skull appearance.</p><ul>-<li>Other types of <a title="Craniosynostosis" href="/articles/craniosynostosis">craniosynostosis</a>- +<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>
- +<li>signs of venous hypertension like widened occipital mastoid emissary foramina and prominent subgaleal veins</li>
- +<li>3D volume rendered bone window shows fusion of all major cranial sutures (pansynostosis)</li>
- +</ul><h4>Differential diagnosis</h4><p>Includes other causes of raised intracranial pressure which may cause the copper beaten skull appearance.</p><ul>
- +<li>other types of <a href="/articles/craniosynostosis">craniosynostosis</a>
-<li>Obstructive hydrocephalus</li>-<li>Intracranial masses</li>-</ul><h4>See also</h4><p><a title="Craniosynostosis" href="/articles/craniosynostosis">Craniosynostosis</a></p><p><!--[if gte mso 9]><xml>- +<li><a title="Obstructive hydrocephalus" href="/articles/obstructive-hydrocephalus">obstructive hydrocephalus</a></li>
- +<li>intracranial masses</li>
- +</ul><h4>See also</h4><ul><li><a href="/articles/craniosynostosis">craniosynostosis</a></li></ul><p><!--[if gte mso 9]><xml>
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