2015 HSC Section 1 Book of Articles

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

FIGURE 3.

6-month-old female with unicoronal synostosis.

Management of nonsyndromic craniosynostosis

Okada and Gosain

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