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To review the causes of cerebral edema in diabetic ketoacidosis (CEDKA), including pathophysiology, risk factors, and proposed mechanisms, to review the diagnosis, treatment, and prognosis of CEDKA and the treatment of diabetic ketoacidosis as it pertains to prevention of cerebral edema.A MEDLINE search using OVID was done through 2006 using the search terms cerebral edema and diabetic ketoacidosis.There were 191 citations identified, of which 150 were used. An additional 42 references listed in publications thus identified were also reviewed, and two book chapters were used.The citations were reviewed by the author. All citations identified were used except 25 in foreign languages and 16 that were duplicates or had inappropriate titles and/or subject matter. Of the 194 references, there were 21 preclinical and 40 clinical studies, 35 reviews, 15 editorials, 43 case reports, 29 letters, three abstracts, six commentaries, and two book chapters.The data are summarized in discussion.The causes and mechanisms of CEDKA are unknown. CEDKA may be due as much to individual biological variance as to severity of underlying metabolic derangement of the child's state and/or treatment risk factors. Treatment recommendations for CEDKA and diabetic ketoacidosis are made taking into consideration possible mechanisms and risk factors but are intended as general guidelines only in view of the absence of conclusive evidence.