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Incidental Diagnosis Of Craniosynostosis At The ER: Case Series Of 331 Patients.
Monica Manrique, MD, Esperanza Mantilla-Rivas, MD, Justin Bryant, MD, Robert Keating, MD, Marius G. Linguraru, Albert K. Oh, MD, Gary F. Rogers, MD.
Children's National Hospital, Washington, DC, USA.

PURPOSE: Craniosynostosis (CS) is usually diagnosed during early childhood for the presence of skull deformities, asymmetry of the face, or increased intracranial pressure. However, some patients present a diagnostic challenge due to delayed CS, since the clinical presentation can be either mild or absent. Although the clinical consequences of this are not certain, the emergency department (ED) might present a particularly well-suited alternative for the diagnosis of these individuals. In this light, the purpose of this paper is to document the prevalence of incidentally-identified CS in a sample of patients coming from the ED from 1 to 5 years of age, at our institution.
METHODS: A total of 331 computerized tomography (CT) scans from children between 1 to 5 years old (mean age 2.4 1.3 years) were retrospectively reviewed. These images were taken at the ED from June 2005 to October 2016. Reasons for the imaging included: trauma, seizures and headache, among others. Patients with a past medical history of shunt placement and CS associated syndromes were excluded. CS was diagnosed via review of CT scans by either three plastic and reconstructive surgeons or one neurosurgeon. Suture fusion, radiology report, the reason for CT scan and past medical history were recorded as covariates. Cranial shape analysis was performed on the patients founded with premature suture fusion.
RESULTS: A total of eleven patients were found to have CS. Of these, 18.1% were reported by the radiologist. All the fused sutures were found to be sagittal. Indications for CT in patients who were found to have CS were: Trauma (63.6%), followed by seizures (18.1%), headaches (9.1%) an dog bite (9.1%). Most of the patients had no significant past medical history. Cranial shape analysis was performed in 5 of these patients. The cranial shape in all patients was grossly normal, but the shape analysis found that 4 had subtle phenotypic changes along the spectrum of scaphocephaly. The remaining case was deemed normal by shape analysis.
CONCLUSION: Craniosynostosis without the characteristic phenotypic changes was present in 3.3% of our cohort; evidencing CS can be under-recognized in the ED. In this study, we highlight the relevance of performing an extensive evaluation the head CT scans on patients younger than 5 years of age. Radiologist must be aware of the possible presence of CS, regardless of the chief complaint for ED consultations. Even though clinical significance of this finding is unclear, these patients may require careful clinical monitoring for elevated intracranial pressure and neurocognitive manifestations. Subtle phenotypic can help identify these patients, but this requires sophisticated shape analysis algorithms that are not widely available. We have adapted our shape analysis algorithms for use with three-dimensional photogrammetry and this may provide a radiation-free method of detection in future.


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