目的：分析婴儿Chiari畸形Ⅱ型的临床资料，加深儿科医师的认识。方法回顾性分析1例确诊为Chiari畸形Ⅱ型患儿的临床、实验室、影像学资料，并复习相关文献。结果患儿，男，2个月，因颈部肿物2个月入院。颅脑MRI示小脑扁桃体部分疝入枕骨大孔。予手术治疗后一直间断发热，不能竖头、独坐，前囟门进行性增大，CT诊断脑积水。结论 Chi-ari畸形Ⅱ型会导致神经功能异常，预后差，病死率高；早期诊断，积极手术，可改善预后。%Objective To analyze the clinical data of an infant with Chiari II malformation so as to deepen understanding of this disease in pediatrics. Methods The clinical, laboratory and imaging data of an infant with Chiari II malformation were retrospectively analyzed and a literature review was performed. Results A 2-month-old male infant was admitted to our hospital due to a neck tumor for two months. Brain magnetic resonance imaging (MRI) showed herniation of the cerebellar tonsils into the foramen magnum. The infant was finally diagnosed with Chiari II malformation based on the result of MRI and the presence of myelomeningocele. After repair of spinal cord and meninges and decompression of pillow macroporous by surgery, the infant presented with intermittent fever and progressively enlarged anterior fontanel, with culture-positive cerebrospinal fluid for bacte-ria and hydrocephalus diagnosed by CT, and intracranial infection was considered. Ventriculoperitoneal shunt was not performed due to uncontrolled intracranial infection after treatment, and motor retardation was found during the follow-up. Conclusions Chiari II malformation may results in nervous system dysfunction with poor diagnosis and high case fatality rate. Early diagnosis and operation may be helpful to the improvement of prognosis.
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