OCCIPITAL REMODELING AND SUBOCCIPITAL DECOMPRESSION IN SEVERE CRANIOSYNOSTOSIS ASSOCIATED WITH TONSILLAR HERNIATION

OEJECTIVE: The goal was to describe a surgical technique allowing occi pital vault remodeling and subocciptal decompression in patients affec ted by multiple-suture synostosis presenting severe occipital flatteni ng and chronic tonsillar herniation (CTH), METHODS: Four patients (two with Crouzon's syndrome, one with Kleeblattschadel, and one with comp lex craniosynostosis) presenting multiple-suture synostosis with sever e occipital flattening, posterior fingerprint impressions, and CBH wer e operated on in the prone position. For three patients, occipital vau lt remodeling and suboccipital decompression without dural opening wer e performed; for one patient affected by Kleeblattschadel, an upper ce rvical laminectomy and dural opening were performed. All patients were studied with magnetic resonance imaging pre-and postoperatively. RESU LTS: No complications were observed. In all cases, postoperative magne tic resonance imaging revealed good decompression of the craniocervica l junction, with resolution of brain stem displacement. In one case, C TH recurred 15 months after surgery, although in a less severe form. C ONCLUSION: 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 poste rior vault remodeling and prophylactic suboccipital decompression, Aft er validation with a larger number of patients, it could prove to he a useful option in all cases of complex craniosynostosis with CTH in wh ich a staged repair of the craniosynostosis is to be considered.