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Delayed Presentation of Unicoronal Craniosynostosis-Masked by Ipsilateral Deformational Plagiocephaly
Ian Chow, MD; Joseph E. Losee, MD, FACS, FAAP
University of Pittsburgh Medical Center
2019-02-15
Presenter: Ian Chow
Affidavit:
I certify that the material proposed for presentation in this abstract has not been published in any scientific journal or previously presented at a major meeting. The program director is responsible for making a statement within the confines of the box below specific to how much of the work on this project represents the original work of the resident. All authors/submitters of each abstract should discuss this with their respective program director for accurate submission of information as well as the program director's approval for inclusion of his/her electronic signature.
Director Name: Vu Nguyen, MD
Author Category: Resident Plastic Surgery
Presentation Category: Clinical
Abstract Category: Craniomaxillofacial
Introduction: The prompt diagnosis and management of patients with craniosynostosis is of the utmost importance. Changes associated with each of these syndromes can directly oppose each other and could result in a head shape that is mistaken purely for deformational plagiocephaly.
Case: The authors present two cases where patients presented with concerns for head shape abnormality in a delayed fashion and were ultimately found to have unilateral craniosynostosis.
The first patient presented to the Plastic Surgery Cleft and Craniofacial clinic at 18 months of age after an MRI and CT for developmental delay demonstrated a right-sided unicoronal craniosynostosis. The patient had no papilledema, headaches, or other clinical or radiologic signs of elevated ICP and because his craniofacial asymmetry was minimal, surgery was deferred with routine follow-up.
The second patient presented at 5.8 years on the referral of Ophthalmology due to the presence of optic nerve pallor on fundoscopic examination in association with an abnormal head shape. His examination was notable for right-sided deformational plagiocephaly with anterior displacement of the right cranium, but he was brachy-turricephalic so a CT scan and MRI were performed which were consistent with a right-sided unicoronal craniosynostosis with evidence of long-standing intracranial hypertension. Given these findings, the patient underwent cranial vault remodeling and fronto-orbital advancement.
Discussion/Conclusion: Both patients presented in this case series were born prematurely and with multiple comorbidities requiring significant medical care from a multi-disciplinary care team. The severity of each patient's deformational plagiocephaly masked their unicoronal craniosynostosis delaying prompt diagnosis and appropriate treatment.
