In the past 30 years, a better understanding of the pathophysiology and management of craniosynostosis has developed. Currently, surgery is usually for cranial deformity correction for infants with fusion of 1-2 sutures that results in a misshapen head. For infants with microcephaly (ie, secondary craniosynostosis), surgery usually is not required.
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In patients with microcephaly, investigate the cause of microcephaly.
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Measure head circumference longitudinally and monitor development. Ensure normal brain growth in patients with primary craniosynostosis.
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Carefully monitor signs and symptoms of elevated intracranial pressure.
Examine the fundi and alert parents to report persistent vomiting or lethargy promptly.
Should elevated intracranial pressure be suspected, an emergent neurosurgical consult would be appropriate.
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To preserve visual function in patients with elevated intracranial pressure, an emergent ophthalmological consult would be appropriate.
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Address the appearance of the skull abnormality.
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Diagram of a neonate's skull demonstrating the location of the sutures.
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Frontal view showing a fused and ridged metopic suture on 3-dimensional CT.
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CT image demonstrating features of secondary craniosynostosis with cerebral atrophy. Cerebral atrophy is not present in primary craniosynostosis.
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Positional molding. Note the anterior displacement of the right occiput and of the right frontal region on the same side, which differentiate positional molding from posterior plagiocephaly craniosynostosis.
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Posterior view of 3-dimensional cranial CT demonstrating early fusion of the lambdoid suture.
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Isolated fusion of the metopic suture. Note that the remaining sutures are open.
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Trigonocephaly. Note the triangular shape of the head.
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Infant with primary craniosynostosis. The specific deformity of the skull is Kleeblattschadel or cloverleaf skull.
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Skull deformities associated with single suture synostosis.
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Positional plagiocephaly. Note anterior position of the ear on the side with occipital flattening.
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Sagittal synostosis and the associated scaphocephaly seen on skull radiograph and 3-dimensional craniofacial CT scan.
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Sagittal synostosis before and after cranial vault surgery and the associate improvement of scaphocephaly.
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Unilateral coronal deformity with retrusion of the orbit and harlequin eye deformity. Note the ipsilateral deviation of the nasal radix and the contralateral deviation of the nasal tip.
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Fusion of coronal sutures bilaterally. Note the increase transverse dimension and retrusion of the orbital rim.
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Intraoperative view of bilateral coronal synostosis. Note the decreased anterior-posterior dimension, increased transverse width and retruded orbital rim.
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Pre- and postoperative photos of metopic synostosis. Note the prominent forehead keel corrected after surgical repair.