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

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Abstract

Purpose

Acquired Chiari I malformation is an uncommon but possible late complication of supratentorial shunting in children. This condition can be caused by an abnormal thickening of the cranial vault and consequent reduction of the posterior cranial fossa (PCF) volume especially in children with already a small PCF volume. The management of Acquired Chiari I malformation is very challenging, and several options have been proposed for this condition. These are aimed to expand the PCF volume both through decompression and PCF remodeling in order to relieve symptoms of acquired Chiari I malformation. A cranial vault remodeling or a standard Chiari decompression is two proposed techniques aimed to expand the PCF volume thus relieving symptoms .

Methods

We describe the case of a 16-year-old girl undergone surgical removal of sellar-suprasellar glioneuronal tumor and ventriculo-peritoneal shunting, who developed an acquired symptomatic Chiari type I malformation some years after ventricular-peritoneal shunting. For this condition, she underwent successful standard Chiari decompression with C0–C1 craniectomy and duroplasty.

Results

We retrospectively analyzed MRI and CT scan performed during follow-up, in order to evaluate the volume of the posterior cranial fossa and to measure the variation of skull thickness at different periods. MRI and CT scan analysis showed a progressive thickening of the calvaria, in particular of the occipital bone, leading to a progressive reduction of PCF volume with the establishment of acquired Chiari type I malformation. In this case, standard C0–C1 Chiari decompression was effective in restoring PCF volume and relieving symptoms.

Conclusion

Acquired Chiari I malformation due to chronic overhunting could be a severe and late complication in patient undergone supratentorial shunting. These patients require careful clinical and radiological follow-up to avoid over-drainage. According to our analysis, a careful selection of pediatric patients for supratentorial shunting should be made according to pre-operative PCF volume in order to foresee higher odds of possible late complications from over-drainage.

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Authors and Affiliations

  1. Department of Neurosciences DNS, Academic Neurosurgery, University of Padua, Padua, 35128, Italy

    Alberto D’Amico, Giulia Melinda Furlanis, Valentina Baro & Luca Denaro

  2. Unit of Neurosurgery, Department of Biomedicine, Neuroscience and Advanced Diagnostics, University of Palermo, Post Graduate Residency Program in Neurosurgery, Palermo, 90100, Italy

    Giuseppe Roberto Giammalva & Rosario Maugeri

  3. Otolaryngology Department, Aulss2 Marca Trevigiana, Treviso, 31100, Italy

    Enzo Emanuelli

Authors
  1. Alberto D’Amico
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  2. Giuseppe Roberto Giammalva
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  3. Giulia Melinda Furlanis
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  4. Enzo Emanuelli
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  5. Rosario Maugeri
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  6. Valentina Baro
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  7. Luca Denaro
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Contributions

Alberto D’Amico and Giuseppe Roberto Giammalva contributed to manuscript conceptualization and data curation; Alberto D’Amico contributed to original draft preparation, software analysis, investigation, and figures preparation; Giuseppe Roberto Giammalva contributed to methodology, manuscript review, and editing; Giulia Melinda Furlanis, Enzo Emanuelli, Rosario Maugeri, and Valentina Baro contributed to visualization and review of manuscript; Luca Denaro contributed to manuscript validation and project supervision. All authors have reviewed and agreed to the final version of the manuscript.

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Correspondence to Alberto D’Amico.

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D’Amico, A., Giammalva, G.R., Furlanis, G.M. et al. Acquired Chiari type I malformation: a late and misunderstood supratentorial over-drainage complication. Childs Nerv Syst 39, 343–351 (2023). https://doi.org/10.1007/s00381-022-05775-2

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