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Onset and Resolution of Chiari Malformations and Hydrocephalus in Syndromic Craniosynostosis following Posterior Vault Distraction

Lin, Lawrence O. B.S.M.E.; Zhang, Rosaline S. B.A.; Hoppe, Ian C. M.D.; Paliga, J. Thomas M.D.; Swanson, Jordan W. M.D.; Bartlett, Scott P. M.D.; Taylor, Jesse A. M.D.

Plastic and Reconstructive Surgery: October 2019 - Volume 144 - Issue 4 - p 932-940
doi: 10.1097/PRS.0000000000006041
Pediatric/Craniofacial: Original Articles

Background: Patients with syndromic craniosynostosis have an increased incidence of progressive hydrocephalus and Chiari malformations, with few data on the relative benefit of various surgical interventions. The authors compare the incidence and resolution of Chiari malformations and hydrocephalus between patients undergoing posterior vault distraction osteogenesis (PVDO) and patients undergoing conventional cranial vault remodeling.

Methods: Patients with syndromic craniosynostosis who underwent cranial vault surgery from 2004 to 2016 at a single academic hospital, with adequate radiographic assessments, were reviewed. Demographics, interventions, the presence of a Chiari malformation on radiographic studies and hydrocephalus requiring shunt placement were recorded. Mann-Whitney U and Fisher’s exact tests were used as appropriate.

Results: Forty-nine patients underwent PVDO, and 23 patients underwent cranial vault remodeling during the study period. Median age at surgery (p = 0.880), sex (p = 0.123), and types of syndrome (p = 0.583) were well matched. Patients who underwent PVDO had a decreased incidence of developing Chiari malformations postoperatively compared with the cranial vault remodeling cohort (2.0 percent versus 17.4 percent; p = 0.033). Not surprisingly, no significant difference was found between the groups with regard to the incidence of postoperative hydrocephalus requiring shunt placement (PVDO, 4.1 percent; cranial vault remodeling, 4.3 percent; p = 0.999).

Conclusions: As expected, PVDO did not significantly affect intracranial hydrodynamics to the extent that hydrocephalus shunting rates were different for patients with syndromic craniosynostosis. However, PVDO was associated with a reduced risk of developing a Chiari malformation; however, prospective evaluation is needed to determine causality.


Philadelphia, Pa.

From the Division of Plastic and Reconstructive Surgery, Children’s Hospital of Philadelphia.

Received for publication August 29, 2018; accepted February 28, 2019.

Disclosure:None of the authors has a financial interest in any of the products or devices mentioned in this article.

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Jesse A. Taylor, M.D., Department of Surgery, Children’s Hospital of Philadelphia, University of Pennsylvania, Colket Translational Research Building, 9th Floor, Philadelphia, Pa. 19104,

Copyright © 2019 by the American Society of Plastic Surgeons