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We report a child with osteopetrosis, oxycephaly and Chiari type I malformation who presented with raised intracranial pressure. During cranial expansion surgery the patient developed sudden cardiac arrest which we believe was probably related to the Chiari malformation. The case highlights a previously unrecognized association between osteopetrosis, craniosynostosis and a persistently open fontanelle at age 4 years. In addition it supports the existing literature in emphasizing the need for careful preoperative work up, choice of approach and operative technique in children with complex craniosynostosis and Chiari malformation.
Jamjoom et al. (Thu,) studied this question.