Lee E J, Lee M Y, Hung Y C, Wang L C
Department of Surgery, National Cheng Kung University Medical Center, Tainan, Taiwan.
J Formos Med Assoc. 1998 Oct;97(10):720-3.
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.
脑毛霉菌病是一种罕见的真菌感染，主要发生于糖尿病酮症酸中毒患者。这种中枢神经系统感染的特点是临床状况迅速恶化，如果不采取积极治疗，一直被认为是致死性疾病。我们报告1例糖尿病患儿，在酮症酸中毒发作期间，出现视力模糊、球结膜水肿和左侧眶周疼痛1周左右。神经系统检查发现向上和向外凝视的活动范围减小，左侧瞳孔扩张不全及视乳头水肿。影像学检查显示左侧眼眶蜂窝织炎、鼻窦炎和左侧额叶大的分叶状脓肿。进行了两次外科手术，包括功能性鼻内镜鼻窦手术以清除鼻窦病灶，随后进行开颅手术切除脓肿。从脓肿壁获取的病理标本显示坏死性炎症以及宽的、无隔膜的呈直角分支的菌丝，这是毛霉科的典型特征。术后，患者在7周内接受了1.5 g两性霉素B治疗，并积极控制糖尿病2个月。通过药物和手术治疗相结合，患儿顺利康复。
