History of Management of Chiari Malformations in Children

Understanding of Disease

  • 1891 – first Chiari paper: Dr. Hans Chiari, a pathological anatomy professor at the German University in Prague, described Chiari malformation types I–III in his first paper published in 1891 (60).
  • 1896 – Chiari credits Cleland and Arnold: In 1896, Chiari published a second paper, which expanded on pathophysiology, described a Chiari IV malformation, and gave credit to John Cleland and Julius Arnold, who had previously described the Chiari II malformation in patients with associated spina bifida (61).

Technological Development

  • Improved imaging increased awareness: From the 1950s to the 1980s the frequency of diagnosis of Chiari malformations increased as a result of the introduction of CT and MRI (41).
  • CSF motion imaging: Motion-sensitive MRI, phase-contrast or cine-mode MRI, as well as hydrodynamic modeling of the posterior fossa (e.g., computational fluid dynamics) that allow prediction of physical interactions between anatomical components have led to a better understanding of the pathophysiology and CSF dynamics in this region (42).

Surgical Technique

  • First surgical decompression reported: The first description of a surgical procedure for correction of hindbrain herniation and improvement of cerebellar flow was reported in Dr. Cornelis Joachimus van Houwenige Graftdijk’s doctoral thesis. However, the patient developed a series of complications and died about 3 months later (62, 63).
  • Later reports: In 1938, Penfield and Coburn attempted a posterior fossa exploration that was unsuccessful and suggested that future surgery for hindbrain herniation should include resection of the posterior margin of the foramen magnum and posterior elements of C1 and C2 (52). Also in that year, McConnell and Parker published their results of posterior fossa decompression in five patients with Chiari I malformation (64).
  • First successful surgery reported: The first successful Chiari decompression series was published in 1950 by Gardner and Goodall and led to the widespread adoption of this procedure (53).