New-Onset Craniosynostosis After Posterior Vault Distraction Osteogenesis

Abstract

The aims of this study were to document the incidence of new-onset craniosynostosis (NOC) after posterior vault distraction osteogenesis (PVDO), to determine risk factors for the development of NOC, and to deduce the cranial ramifications of NOC. An institutional review board-approved retrospective review of all patients who underwent PVDO at the Children's Hospital of Philadelphia was performed. Demographics, perioperative data, as well as preoperative and postoperative three-dimensional computed tomographic scans were analyzed. Suture patency preoperatively and postoperatively was recorded.Thirty patients underwent PVDO for suspected increased intracranial pressure and/or severe turribrachicephaly from 2008 to 2013. Twenty-four patients had syndromic diagnoses. The average age at the time of PVDO was 2.03 years. Distraction distances ranged from 19 to 40 mm, with an average of 28.7 mm. Among the 19 patients who had patent lambdoid sutures before PVDO, new-onset lambdoid fusion was seen in 17 patients after PVDO (89.5%), whereas the suture remained open in 2 patients (10.5%). New-onset lambdoid fusion was not significantly associated with age at distraction (P = 0.28), sex (P = 0.47), length of distraction (P = 0.93), or diagnosis (P = 0.61). Similarly, new-onset sagittal synostosis was not associated with age at distraction (P = 0.06), sex (P = 0.64), length of distraction (P = 0.83), or diagnosis (P = 0.25). None of the patients who developed NOC had characteristic head shape changes such as mastoid bulges or scaphocephaly. New-onset lambdoid and sagittal synostoses occur frequently after PVDO. Although the diagnosis of NOC is obvious radiographically, the clinical importance of the diagnosis morphometrically, neurodevelopmentally, and in cranial growth has yet to be fully investigated.

Degree of Sagittal Suture Fusion, Cephalic Index, and Head Shape in Nonsyndromic Sagittal Craniosynostosis

“…These clinical observations could substantiate findings from preclinical studies describing regional-specific dural signaling implicated in suture location-specific craniosynostosis 6. Clinically, cranial volume expansion and biomechanical forces secondary to PVDO may lead to inhibition of dural signals, resulting in suture closure 10. These observations are substantiated by research in the basic sciences, which have shown dura matter from animals with fused sutures can induce suture fusion at region-specific areas, likely through FGF2 (fibroblast growth factor 2) and TGFβ (transforming growth factor beta) 6,11.…”

“…These region-specific findings are interesting to consider in the context of high rates of new onset craniosynostosis following posterior vault distraction osteogenesis (PVDO). 10 In this study's cohort, nearly 90% of patients undergoing PVDO demonstrated new onset lambdoid fusion. These clinical observations could substantiate findings from preclinical studies describing regionalspecific dural signaling implicated in suture location-specific craniosynostosis.…”

“…These region-specific findings are interesting to consider in the context of high rates of new onset craniosynostosis following posterior vault distraction osteogenesis (PVDO) 10. In this study’s cohort, nearly 90% of patients undergoing PVDO demonstrated new onset lambdoid fusion.…”

An Algorithm for Managing Syndromic Craniosynostosis Using Posterior Vault Distraction Osteogenesis

“…We have also noted newonset craniosynostosis following posterior vault distraction osteogenesis, the clinical significance of which is unclear. 18 Our consistent use of early posterior vault distraction osteogenesis represents an emphasis on early functional expansion. Parallel bodies of evidence underscore the high prevalence of elevated intracranial pressure, and neurocognitive differences, in patients with craniosynostosis.…”

Treatment Type Is Associated with Population Hand Preferences in Patients with Unilateral Coronal Synostosis

“…Several recent reports suggest that this type of manipulation can alter normal sutural patency and induce premature fusion of previously uninvolved sutures. [35][36][37][38][39][40][41] This can affect subsequent cranial growth and may increase the risk of latent intracranial hypertension.…”