Asymmetric Multisutural Craniosynostosis: an Algorithm of Early Intervention to Prevent Evolving Deformity

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Asymmetric multisutural craniosynostosis (AMC) is characterized by fusion of a midline suture combined with unilateral fusion of at least 1 nonmidline suture. Due to its rarity, complexity, and high rate of reoperation, the purpose of this study is to evaluate outcomes of our staged approach to AMC.


Patients treated for craniosynostosis between January 2004 and December 2013 were identified retrospectively. Only patients with AMC and a minimum follow-up of 2 years were included. The 3-staged algorithm includes: extended strip craniectomy of fused sutures; postoperative helmet molding; and fronto-orbital advancement versus a touch-up procedure. Morphologic, aesthetic, and functional outcomes were evaluated.


Nine patients (6.8% of cases) were treated for AMC (mean follow-up: 4.1 years). Sixty-seven percent of patients (6 of 9) demonstrated signs of elevated intracranial pressure. Patients were divided into 2 groups: “Group A” included patients treated according to the staged algorithm (n = 5); “Group B” included those treated by traditional techniques (n = 4). Group A underwent their first calvarial vault procedure earlier than those from Group B (2.7 vs. 13.2 months; P < 0.02). Postoperatively, no Group A patients had developmental delay, signs of elevated intracranial pressure, or reoperation. Three of 5 patients (60%) were rated Whitaker Classification II and the others (40%) rated Whitaker Classification III.


Asymmetric multisutural synostosis results in a complex and evolving deformity involving the entire craniofacial complex. However, when asymmetric multisutural synostosis is approached in stages with early intervening helmet therapy, acceptable functional and appearance-related outcomes can be obtained with minimal complications.


Level of Evidence: III

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