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Acquired Chiari type I malformation: a late and misunderstood supratentorial over-drainage complication

Alberto D'Amico1, Giuseppe Roberto Giammalva2, Giulia Melinda Furlanis3

  • 1Department of Neurosciences DNS, Academic Neurosurgery, University of Padua, Padua, 35128, Italy. alberto.damico@unipd.it.

Child'S Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery
|December 1, 2022
PubMed

Insights

Acquired Chiari I malformation can develop after supratentorial shunting due to skull thickening and reduced posterior cranial fossa volume. Standard Chiari decompression effectively relieved symptoms in a case study, highlighting the need for careful patient selection for shunting.

Area of Science:

  • Neurosurgery
  • Pediatric Neurology
  • Radiology

Background:

  • Acquired Chiari I malformation is a rare complication of supratentorial shunting in children.
  • It results from abnormal cranial vault thickening, reducing posterior cranial fossa (PCF) volume, particularly in patients with a small pre-existing PCF.
  • Management aims to expand PCF volume through decompression or remodeling.

Observation:

  • A 16-year-old girl developed symptomatic acquired Chiari I malformation years after surgery for a sellar-suprasellar glioneuronal tumor and ventriculo-peritoneal shunting.
  • Retrospective analysis of MRI and CT scans revealed progressive calvarial thickening, especially of the occipital bone.
  • This thickening led to reduced PCF volume and the development of acquired Chiari I malformation.

Findings:

  • Standard Chiari decompression with C0-C1 craniectomy and duroplasty was successfully performed.
  • The procedure effectively restored PCF volume and alleviated the patient's symptoms.
  • Radiological assessment confirmed the effectiveness of the decompression in addressing the malformation.

Implications:

  • Acquired Chiari I malformation following supratentorial shunting requires vigilant clinical and radiological monitoring to prevent over-drainage.
  • Pre-operative assessment of PCF volume is crucial for selecting pediatric patients at higher risk for this complication.
  • Early identification and appropriate surgical intervention are key to managing this challenging condition.
Abstract

Keywords:
CSF shuntChiari malformationOver-drainageSupratentorial shuntVentriculo-peritoneal shunt

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