Resolution of type I Chiari malformation and associated syringomyelia following intrathecal chemotherapy: case report

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  • 1 Section of Neurosurgery,
  • 2 Department of Diagnostic Radiology, and
  • 3 Department of Pediatrics, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan;
  • 4 Division of Neurosurgery, Department of Surgery, National Taiwan University Hospital Hsin-Chu Branch, Hsinchu City; and
  • 5 Department of Cell Biology and Anatomy and
  • 6 Institute of Basic Medical Sciences, College of Medicine, National Cheng Kung University, Tainan, Taiwan
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Type I Chiari malformation (CM-I) consists of downward herniation of the cerebellar tonsils below the foramen magnum and often requires surgical decompression if symptomatic. Spontaneous resolution of CM-I is rare. We present a case of resolved CM-I without surgery in a 6-year-old boy with B-cell lymphoma who was diagnosed with CM-I during lymphoma staging. Cerebrospinal fluid cytology and brain MRI revealed negative CNS involvement but showed CM-I with tonsillar ectopia 19 mm below the foramen magnum. The patient underwent induction chemotherapy including 5 doses of intrathecal chemotherapy. Follow-up MRI demonstrated marked regression of CM-I to less than 6 mm in 3 months, and complete resolution of CM-I was observed in 2 years. To the best of our knowledge, this is the first case of resolved CM-I and syringomyelia following chemotherapy. In this case report, the authors summarize all of the clinical characteristics, the radiological appearance, and the potential causes of resolution based on a review of the literature and propose the mechanisms through which intrathecal chemotherapy contributed to the CM-I and syringomyelia resolution in the present case.

ABBREVIATIONS ara-C = cytarabine; CM-I = type I Chiari malformation; FM = foramen magnum; LP = lumbar puncture; MTX = methotrexate; TIT = triple intrathecal chemotherapy with hydrocortisone, MTX, and ara-C.

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Contributor Notes

Correspondence Jung-Shun Lee: College of Medicine, National Cheng Kung University, Tainan, Taiwan. nslee1218@gmail.com.

INCLUDE WHEN CITING Published online December 4, 2020; DOI: 10.3171/2020.7.PEDS20411.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

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