![]() On MR images, the malformation is characterized by a constellation of morphological features (Table 1). Usually, Chiari II malformation is clinically diagnosed with the help of MR imaging. ![]() The mortality in this symptomatic group is 15 to 35 %. Approximately one third of the patients with Chiari II malformation develop signs and symptoms of brainstem compression. The decreased inductive pressure on the surrounding mesenchyme results in an abnormally small posterior fossa. McLone and Knepper hypothesized that leakage of cerebrospinal fluid through the spinal anomaly reduces the distension of the embryonic ventricular system. Chiari II malformation is almost uniquely associated with open spinal dysraphism. It is characterized by a small posterior fossa and downward displacement of the cerebellum and brainstem through an enlarged foramen magnum (hindbrain herniation). Chiari II malformation is a complex developmental malformation of the central nervous system.
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