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Posterior Cranial Vault Distraction Osteogenesis Utilizing a Posterior-Superior Distraction Vector in the Treatment of Mercedes Benz Pattern Craniosynostosis
Fady P. Marji, MD; Lisa M. Block, MD; Erin E. Anstadt, MD; Lucas A. Dvoracek, MD; Abraham A. Williams, BS; Jennifer A. Hall, BS; Joseph E. Losee, MD; Jesse A. Goldstein, MD
University of Pittsburgh Medical Center
2020-02-15
Presenter: Fady P. Marji
Affidavit:
The work represents the original work of the residents involved with guidance and revisions by attending physicians.
Director Name: Vu T. Nguyen NM
Author Category: Other Specialty Resident
Presentation Category: Clinical
Abstract Category: Craniomaxillofacial
Background: Bilateral lambdoid and sagittal synostosis (BLSS), or Mercedes Benz Syndrome, is a rare complex craniosynostosis resulting in an anteriorly displaced cranial vertex. It's ideal surgical correction must result in expansion, skull elongation, and caudal repositioning of the vertex. For this, posterior vault distraction osteogenesis (PVDO) utilizing a posterior-superior distraction vector may be ideal.
Methods: A retrospective review was performed to analyze outcomes of PVDO using a posterior-superior distraction vector from 2016 to 2019. Cranial vertex position was measured as a fraction of the occipitofrontal diameter from rostral to caudal (0-1.0).
Results: Four patients underwent PVDO at mean age 10.61 ± 3.16 months. Linear distraction distance averaged 30.30 ± 0.90 mm with a mean consolidation period of 3.98 ± 0.72 months. Mean corrected change in intra-cranial volume was 236.30 ± 3.71 mL (p = 0.002), at an average rate of 7.81 ± 2.00 mL/mm. Significant mean increases in anterior cranial height (7.83 ± 2.51 mm, p = 0.008), middle cranial height (8.43 ± 4.21 mm, p = 0.03), posterior cranial height (13.15 ± 7.45 mm, p = 0.04), and posterior cranial fossa height (21.99 ± 8.55 mm, p =0.01) were observed. Basofrontal angle did not change significantly (p >0.05). Cranial vertex demonstrated a significant mean posterior movement of 0.18 ± 0.13 (p= 0.03).
Conclusion: PVDO utilizing a posterior-superior distraction vector for management of BLSS effectively increases intracranial volume and height and provides an aesthetic outcome with posterior movement of the cranial vertex.
