2015 HSC Section 1 Book of Articles

Maxillofacial surgery

FIGURE 2. 3-month-old female with sagittal synostosis.

Mutations in fibroblast growth factor receptors 1, 2, and 3, TWIST and MSX2 have been implicated in certain syndromic craniosynostoses [7]. The exact pathways of how these mutations cause premature sutural fusion are still unknown.

craniosynostosis, one must be cognizant of these findings in any patient with craniosynostosis. The standard for measuring ICP is direct intradural or intraventricular monitoring for a 24-h period to record fluctuations during activity, sleep, and the elevations caused by airway obstruction. Measuring ICP via lumbar puncture is less invasive; however, the results vary by positioning and provide measure- ment at only a single point in time, making it less reliable. Bulging fontanelles only offer a qualitative assessment of intracranial hypertension. Papille- dema has a sensitivity of only 22% in detecting elevated ICP in children under 8 years old [9]. Copper beating seen on either computed tomo- graphy (CT) or plain radiographic studies is a late finding of intracranial hypertension, caused by remodeling of the inner table due to adjacent gyri. Hydrocephalus is a rare finding in nonsyndromic craniosynostosis. In a large series of 1727 cases of craniosynostosis, hydrocephalus was found in only 0.3% of nonsyndromic patients and 12.1% in

INTRACRANIAL PRESSURE, HYDROCEPHALUS AND COGNITIVE DEFICITS

Intracranial hypertension results from a mismatch between a constricted cranial vault and the growth of the underlying brain. This condition is an indication for early operative treatment for craniosynostosis, as delayed treatment has irrevers- ible consequences for vision and cognitive develop- ment. Marchac and Renier [8] found a 42% incidence of intracranial hypertension in multisuture synos- tosis and 13% in single suture synostosis. Although changes associated with elevated intracranial pressure (ICP) are more common in syndromic

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