Complex Craniosynostosis

• DOI: 10.1097/prs.0b013e3182268ca6
• Publication Date: 2011-06-16T21:09:49Z
• Authors: Marcin Czerwinski; John C. Kolar; Jeffrey A. Fearon
• Keywords: Synostosis; Craniosynostoses

Background: Complex craniosynostoses (i.e., multisutural, nonsyndromic) are rare and present unique treatment challenges. The authors sought to assess long-term outcomes, including postsurgical growth development, develop evidence-based algorithms. Methods: A retrospective review of all patients identified as having multiple sutural synostosis excluding bicoronal FGFR- TWIST-associated synostoses was conducted. Data were summarized using descriptive statistics. Results: Over an 18-year period, 858 underwent craniosynostosis correction, 31 (3.6 percent) satisfied inclusion criteria. Average number affected sutures 2.9 (lambdoid, 36 percent; sagittal, coronal, 18 metopic, 15 percent), 1.7 procedures performed per patient (mean follow-up, 3.5 years). hospital stay 2.3 days, 21 percent required blood transfusions, there no major complications. For patterns isolated one side the anterior sagittal suture (anterior or posterior skull halves), 93 corrected with a single procedure. When pattern crossed both halves, 80 two (p < 0.001). Forty developed acquired Chiari deformations; these, 60 decompression. incidence deformations increased from 7 70 lambdoid involvement 0.002). Anthropometric data revealed postoperative impairment. Gross developmental delays noted in 20 (mild, 16 moderate severe, 4 percent). Conclusions: associated higher (especially involvement), require operative procedures, may have more than synostoses. recommend surgical paradigms based on involvement, compensatory overcorrection, routine magnetic resonance imaging monitoring for deformations. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.