Occipital remodeling and suboccipital decompression in severe craniosynostosis associated with tonsillar herniation.

OBJECTIVE: The goal was to describe a surgical technique allowing occipital vault remodeling and suboccipital decompression in patients affected by multiple-suture synostosis presenting severe occipital flattening and chronic tonsillar herniation (CTH).
METHODS: Four patients (two with Crouzon's syndrome, one with Kleeblattschädel, and one with complex craniosynostosis) presenting multiple-suture synostosis with severe occipital flattening, posterior fingerprint impressions, and CTH were operated on in the prone position. For three patients, occipital vault remodeling and suboccipital decompression without dural opening were performed; for one patient affected by Kleeblattschädel, an upper cervical laminectomy and dural opening were performed. All patients were studied with magnetic resonance imaging pre- and postoperatively.
RESULTS: No complications were observed. In all cases, postoperative magnetic resonance imaging revealed good decompression of the craniocervical junction, with resolution of brain stem displacement. In one case, CTH recurred 15 months after surgery, although in a less severe form.
CONCLUSION: In selected cases of complex or syndromic craniosynostosis with predominant posterior deformity and CTH, this technique was safe and useful in the management of cranial reconstruction, allowing posterior vault remodeling and prophylactic suboccipital decompression. After validation with a larger number of patients, it could prove to be a useful option in all cases of complex craniosynostosis with CTH in which a staged repair of the craniosynostosis is to be considered.