Management of Coincident Metopic and Unilateral Lambdoid Craniosynostosis, a Rare Entity

Abstract

Introduction: Multi-suture craniosynostosis is rare and comprises only 3% to 7% of all craniosynostosis cases. Depending on the sutures involved, there are multiple operative approaches for this disease. In this report, we describe the staged surgical management of a patient who presented with metopic and unilateral lambdoid synostosis with initial posterior cranial vault remodeling followed by fronto-orbital advancement (FOA). Methods: At our institution, case reports detailing a single patient are not considered human research by the Institutional Review Board and official approval was not sought. Written consent for inclusion of patient information and images was obtained from the patient’s legally authorized representative. Results: A male patient was treated for metopic and right unilateral lambdoid synostosis. Physical examination was notable for trigonocephaly, right sided posterior plagiocephaly, right mastoid bulging, and contralateral parietal bossing. Treatment of his synostotic deformities was delayed and treated in stages. This was achieved with initial posterior vault expansion, to treat his lambdoid synostosis, via a modification to the classic switch cranioplasty at 5 months of age. The patient subsequently underwent fronto-orbital advancement for correction of metopic synostosis at 10 months of age. The posterior cranial vault remodeling procedure permitted sufficient cranial vault expansion to delay fronto-orbital advancement until 10 months of age. Of note, excellent bone fill and union were observed of the posterior bone grafts at the time of scalp flap elevation during this second procedure. His most recent follow up appointment at 11 months status post FOA demonstrated continued deformational correction as well as good bone fill and wound healing. Conclusions: Multi-suture craniosynostosis is rare with only a single other report in the literature discussing concurrent metopic and unilateral lambdoid synostosis. A multi-stage approach is the most commonly employed technique and must be tailored to each patients’ unique presentation.