Cystic degeneration of the cerebellar tonsils in pediatric patients with Chiari Type I malformation: Clinical article

Charles B. Stevenson, James L. Leach, Anita Gupta, Kerry R. Crone

Research output: Contribution to journalArticlepeer-review

8 Scopus citations

Abstract

Object. The operative indications and treatment algorithms for pediatric patients with Chiari Type I malformation (CM-I) vary widely. When an intradural approach and duraplasty are thought necessary at the time of surgery, neurosurgeons may elect to fulgurate or resect a portion of the cerebellar tonsils. Histological analyses of cerebellar tonsils resected during decompression in pediatric patients with CM-I have revealed multiple abnormal findings including extensive ischemic and degenerative changes. The authors describe an interesting phenomenon of cystic degeneration in the distal ends of the cerebellar tonsils in children undergoing operative treatment of CM-I. Methods. The authors reviewed the clinical database of 440 pediatric patients who underwent surgical decompression for CM-I performed by a single surgeon. The clinical course, preoperative MR imaging and intraoperative ultrasound characteristics, and histological findings in 3 children found to have tonsillar cystic degeneration were analyzed and detailed. Results. Cystic changes were subtle but uniformly evident on preoperative MR imaging and were more readily apparent on intraoperative ultrasonography. In each patient, the tonsillar cyst was resected. Histological examination revealed areas of cystic degenerative change characterized by distortion of the normal cerebellar architecture with absent Purkinje and internal granular cell layers. All children experienced improvement in their symptoms, without complication, postoperatively. Conclusions. Cystic degeneration of the tonsils in pediatric patients with CM-I is an uncommon pathological process most likely resulting from long-standing and excessive compression. Based on their experience, the authors advocate expeditious surgical treatment, including intradural exploration and capacious duraplasty, for patients in whom there is evidence of this phenomenon on preoperative imaging.

Original languageEnglish (US)
Pages (from-to)557-563
Number of pages7
JournalJournal of Neurosurgery: Pediatrics
Volume4
Issue number6
DOIs
StatePublished - Dec 2009
Externally publishedYes

Keywords

  • Cerebellar tonsils
  • Chiari I malformation
  • Cyst
  • Ischemia

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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