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UBC Theses and Dissertations

Determining the accuracy of screening for surgically actionable craniosynostosis through the assessment of caregiver provided photographs Cheong, Alexander Ryan


Introduction: Assessment of head shape is a common reason for referrals to pediatric neurosurgery clinics. To streamline patient assessments, we investigated whether craniofacial practitioners can accurately screen for surgically actionable craniosynostosis by using standardized screening photographs provided by parents. Methods: Parents of children referred to the neurosurgery clinic at BC Children’s Hospital for assessment of a suspected abnormal head shape prospectively submitted a series of standardized photographs which were blindly evaluated by a panel of 7 independent raters, made up of 3 neurosurgeons, 2 plastic surgeons, a pediatrician, and an occupational therapist, both with experience running a head shape clinic. Each rater indicated their likely diagnosis (presence or absence of potentially surgically actionable craniosynostosis) and their level of confidence in that diagnosis. All children were subsequently evaluated in person by an experienced pediatric neurosurgeon with or without the additional consultation of a plastic surgeon. The diagnosis made at the time of in-person evaluation was used as the gold standard. Results: Of 74 children evaluated, 24 (32.4%) had craniosynostosis diagnosed during an in-person assessment. Fleiss-kappa, a measure of assessing agreement among raters, amongst all 7 raters was 0.76 (0.69, 0.82), indicating substantial agreement related to the diagnosis of craniosynostosis based on photographic assessment. Individual raters' diagnostic performance based on photographic evaluation (sensitivity range 0.86-1.0; specificity range 0.77-0.90) compared favourably with the performance of the "majority rule" photographic diagnosis of all 7 raters combined [sensitivity=1.0 (0.88, 1.0); specificity=0.88 (0.76, 0.95)], or the 3 neurosurgeons [sensitivity=1.0 (0.85, 1.0); specificity=0.88 (0.76, 0.95)]. Conclusion: Based on the evaluation of standardized photographs, experienced craniofacial practitioners were able to accurately identify infants with craniosynostosis who may require surgical intervention as well as those whose head shape abnormality would not require surgery. Individual trained raters appear to perform as well as group consensus. Photographic pre-screening may be useful for discriminating between craniosynostosis and plagiocephaly, the two most common etiologies of an abnormal head shape. Implementation of this tool may help reduce wait times and prevent unnecessary travel for patient families, especially those that live in remote communities.

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