Clinical Characteristics and Surgical Decision Making for Infants with Metopic Craniosynostosis in Conjunction with Other Congenital Anomalies

Abstract

Background:Metopic craniosynostosis can occur in isolation or in conjunction with other congenital anomalies. The surgical decision making and outcomes between these 2 groups are analyzed.Methods:A retrospective review of all children evaluated in the craniofacial clinic at Seattle Children’s Hospital for metopic craniosynostosis between 2004 and 2009 was performed. Physical examination and CT scan characteristics were analyzed as were the treatment decisions and surgical outcomes.Results:From 2004 to 2009, 282 patients were evaluated and 100 were determined to have metopic craniosynostosis. Of these, 19 patients were found to have additional congenital anomalies. Review of these patients’ CT scans revealed 13 with classic trigonencephaly, 3 with microcephaly, and 3 with narrow frontal bones, abnormal orbits, and small anterior fossa. Patients (90%) with isolated metopic craniosynostosis underwent cranial vault expansion, whereas only 63% of the complex group did so. The complex metopic group had a longer hospital stay (5 d vs 3.4 d), more intraoperative complications, and required more repeat surgery.Conclusion:Patients with metopic craniosynostosis and additional anomalies require special consideration when deciding upon surgical intervention and should be cared for by a multidisciplinary team to address their additional needs.

Superior Long-term Appearance of Strip Craniectomy Compared with Cranial Vault Reconstruction in Metopic Craniosynostosis

“…Metopic craniosynostosis is treated with surgery in infancy to prevent social and neurodevelopmental sequelae. 1 Differing surgical approaches have been developed with good safety profiles and each with advantages and disadvantages, but none has demonstrated aesthetic, social, or neurodevelopmental superiority. [2][3][4][5][6] Open cranial vault reconstruction (OCVR) remains the historical gold standard treatment.…”

CranioRate TM: An Image-Based, Deep-Phenotyping Analysis Toolset and Online Clinician Interface for Metopic Craniosynostosis

“…This is crucial in examining the significant variation in the methods by which craniofacial surgeons decide whether to operate on a particular patient with craniosynostosis. 31,32 A major limitation of implementing standardized protocols is the lack of objective quantification of severity. The Whitaker classification has been the accepted standard for aesthetic outcomes reporting in craniosynostosis research, [33][34][35][36] but has been shown to have low interrater reliability and cannot predict the need for future interventions.…”

New method for quantification of the relative severity and (a)symmetry of isolated metopic synostosis

“…Clinical examination focuses on the classical clinical features, which include straight, narrowed frontal bones, orbital narrowing, biparietal widening, and temporal constriction. However, these and other features are not pathognomonic for trigonocephaly, but can be present as a normal variation in children with different malformations 5,6 . Therefore, CT scanning can be used to confirm the diagnosis.…”