Head and Neck Imaging

Clinical and Imaging Analysis of Familial Chiari Malformation*

Author:QI Wei, DENG Xiao-feng, YANG Chen-long, et al.

affiliation:Department of Neurosurgery, Beijing Tiantan Hospital Affiliated to Capital Medical University, Beijing 100050, P.R. China

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Abstract

Objective To analyze the clinical and imaging characteristics of familial Chiari malformations. Methods Clinical and imaging studies of 3 families with 6 Chiari malformation patients were retrospectively studied, 4 of which underwent posterior fossa decompression and the other 2 patients were not surgically treated. Results Patients of the first family were twin brothers, both with Chiari malformation type 1.5 (CM 1.5) and syrinx. The elder brother had scoliosis and the younger brother had hydrocephalus. Patients of the second family are mother and son, both with Chiari malformation type I (CMI) and syrinx. Besides, the mother had scoliosis and the son had hydrocephalus. Patients of the third family are non-twin sisters, and the elder sister had CM I and syrinx, but the younger sister had CM 1.5 without syrinx. The 4 patients who underwent posterior fossa decompression had good outcome. Conclusions Familial Chiari malformations may occur in many kinds of relationships, such as twins, mother and son, and non-twin sisters, and clinical and imaging manifestations maybe various.

【Keyword】Chiari Malformation; Syrinx; Familial; Posterior Fossa Decompression; MRI

【Chart number】R445.2;R744

【Document Identification Number】A

【DOI】10.3969/j.issn.1672-5131.2015.12.011