Orbital rhinocerebral mucormycosis associated with diabetic ketoacidosis: report of survival of a 10-year-old boy.

Cerebral mucormycosis is a rare fungal infection that occurs mostly in association with diabetic ketoacidosis. This central nervous system infection is characterized by a rapid decline in clinical status, and has been recognized as a uniformly fatal event if aggressive therapy is not instituted. We report a diabetic child who presented with blurred vision, chemosis, and pain in the left periorbital region noted for about 1 week during an episode of ketocidosis. Neurologic examinations revealed that there was a decreased range of motion in the upward and lateral gaze, along with incomplete pupillary dilatation and papilledema of the left eye. Imaging studies demonstrated left-side orbital cellulitis, paranasal sinusitis, and a large lobulated abscess in the left frontal lobe. Two surgical procedures, including functional endoscopic sinus surgery for sinus debridement and a subsequent open craniotomy for abscess resection were performed. Pathologic specimens obtained from the abscess wall revealed necrotic inflammation and wide, nonseptate hyphae with right angle branching, which are typical characteristics of the family Mucoraceae. Postoperatively, the patient was treated with 1.5 g of amphotericin B over a 7-week period, and aggressive diabetic control for 2 months. Through the combination of medical and surgical treatment the child made an uneventful recovery.