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

BACKGROUND: 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.

METHODS: 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.

RESULTS: 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.

CONCLUSIONS: 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.