2015 HSC Section 1 Book of Articles

Management of nonsyndromic craniosynostosis Okada and Gosain

FIGURE 3. 6-month-old female with unicoronal synostosis.

syndromic cases, most commonly in Crouzon syn- drome [10]. There is disagreement on whether operative intervention for craniosynostosis prevents mental disability. Renier [11] contended that surgical correc- tion allows maintenance of normal mental develop- ment. On the contrary, Kapp-Simon et al. compared the developmental quotient in children with non- syndromic single suture disease who underwent surgery and those whose parents declined and found that developmental quotient did not correlate with operative intervention [12]. Developmental studies are unfortunately limited as childrenwhohave devel- opmental delay often normalize later, and such studies fail to assess subtle differences. Although developmental studies on infants who undergo surgery show little difference in scores, a study on 16 children with nonsyndromic sagittal craniosynos- tosis showed that half of them had a reading and/or spelling disability [13]. Whether surgery for nonsyn- dromic craniosynostosis has a positive effect on subtle mental ability needs further study. PREOPERATIVE CONSIDERATIONS AND THE USE OF IMAGING With the proper understanding of Virchow’s law and physical examination, clinicians can diagnose

a single suture craniosynostosis based on cranial shape. Whereas CT has been used to confirm the diagnosis of craniosynostosis, its use for preopera- tive assessment of single suture synostosis is con- troversial. Proponents of preoperative CT scanning cite cases of misdiagnosis of craniosynostosis as deformational plagiocephaly and the utility of these studies in preoperative planning [14]. The unnecessary use of ionizing radiation and the risk of cancer and developmental delay, and its associated sedation, is always a concern. Proponents of CT scanning argue that, despite repeated scans, the cumulative radiation is far less than the levels shown to increase carcinogenesis or cognitive delay. In a study of 77 patients by Jaffurs and Denny [15], newly diagnosed patients underwent an average of 1.74 scans per year at 1.39mSv per scan, and 4.11 total scans throughout treatment. Syndromic patients underwent an average of 9.73 total scans throughout treatment. The authors felt that these radiation doses are 100-fold less than published levels shown to cause carcinogenesis, and 40-fold less than levels for cognitive delay. However, harm- ful doses of radiation in infants have not yet been established. Other authors feel that clinical diagnosis alone is sufficient for the diagnosis and treatment of patients

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