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PURPOSE: Posterior cranial vault distraction has recently been popularized as a first intervention technique in the management of syndromic and multi-suture craniosynostosis. The purpose of this study was to present our experience with posterior cranial vault distraction in the management of these complex patients and to perform a 3D photographic and CT-based morphometric analysis examining pre- and post-expansion changes in intracranial volume, vertical height, frontal-orbital and mid-sagittal plane contour to determine the effectiveness of this technique.
METHODS: 11 patients (4M, 7F: age 4 months to 63 months) with syndromic multi-suture craniosynostosis (2 Apert, 2 Pfeiffer, 1 7p-deletion, 1 Muenke, 2 combined sagittal/lambdoid, 3 unknown) seen at the Hospital for Sick Children underwent posterior craniotomy with application of KLS-Martin distractors for correction of elevated intracranial pressure (n=6) and/or correction of turribrachycephaly (n=9). Distraction (0.5 mm bid) commenced on the first post-operative day and the consolidation period was 8 to 10 weeks. Follow-up ranged from 6 to 36 months. Intracranial volumes and sagittal ray analysis pre- and post-distraction were determined with CT images imported to a work-station and analyzed with GE Healthcare AW VolumeShare2 software. Fronto-orbital remodeling was assessed using 3D photographs (3dMD).
RESULTS: All patients tolerated the distraction protocol well and no mortalities or significant morbidities were encountered. Posterior cranial vault distraction was successful in achieving stable normocephaly in 8 patients. 6 patients underwent subsequent planned fronto-orbital advancement and 3 patients are awaiting a planned fronto-facial advancement. Elevated ICP was resolved in 5/6 patients. Two patients required VP shunt for persistent CSF leak. Resolution of Chiari I malformation occurred in 2/2 patients. Transfusion rate was 70%. Complications included 1 technical failure of the distractor requiring redo surgery, 2 skin breakdowns overlying the distractor, 1 infection post ACVR/FOA and simultaneous distractor removal and 2 CSF leaks. Mean intracranial volume post-distraction increase was 34% (range 24-42%). Sagittal ray analysis demonstrated mean reduction on vertical height of the cranium of 7.7% (range 5.0 to 10.4%) based on a standard cranial index of vertical height over transverse width.
CONCLUSIONS: Posterior cranial vault distraction provides a controlled expansion of the brachycephalic cranium with correction of turribrachycephaly and resolution of intracranial hypertension in select cases. Disadvantages of a second procedure to remove the distractors and cost of hardware may be outweighed by the advantages of shorter procedure time, less blood loss, no need to remove bone over the confluence of the venous sinuses and spontaneous remodeling of the forehead. The future application of posterior cranial vault distraction in the management of complex syndromic and multi-suture craniosynostosis patients will be discussed.