Symptom outcome after craniovertebral decompression for Chiari type 1 malformation without syringomyelia

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ORIGINAL ARTICLE - CSF CIRCULATION

Symptom outcome after craniovertebral decompression for Chiari type 1 malformation without syringomyelia Joshua Pepper 1

&

Ahmed Elhabal 1 & Georgios Tsermoulas 1 & Graham Flint 1

Received: 18 June 2020 / Accepted: 23 October 2020 # Springer-Verlag GmbH Austria, part of Springer Nature 2020

Abstract Background Specific symptom outcomes after craniovertebral decompression for patients with Chiari type 1 malformation, without accompanying syringomyelia, are not well characterised and poorly reported. Methods We performed a retrospective review of all patients diagnosed with Chiari type 1, without syringomyelia, who underwent craniovertebral decompression in our unit. Results We identified 129 individuals with a minimum of 2 years’ follow-up. The most common pre-operative symptoms were pressure dissociation headaches (78%), visual disturbances (33%), dizziness/balance disturbances (24%) and blackouts (17%). The symptoms most likely to respond to surgery included Valsalva-induced headache (74% response, p < 0.0001) and blackouts (86% response, p < 0.001). Conclusions After successful craniovertebral decompression, most patients presenting with pressure dissociation headaches and blackouts will improve. However, the large variety of other symptoms patients often present with may not improve after surgery. Keywords Chiari I . Symptom outcome . Craniovertebral decompression . Foramen magnum decompression

Abbreviations CSF Cerebrospinal fluid CCOS Chicago Chiari Outcome Scale

Introduction Chiari type 1 malformation consists of herniation of the cerebellar tonsils into the upper cervical spinal canal. This cerebellar ectopia impedes normal movement of cerebrospinal fluid (CSF) across the craniovertebral junction, causing the affected individual to suffer from typical CSF pressuredissociation headaches, brought on by coughing, sneezing, laughing, blowing up party balloons or simply bending over. Portions of this work were presented in abstract form at the international conference, Syringomyelia-Chiari 2018, held in Birmingham, UK, in July 2018 This article is part of the Topical Collection on CSF Circulation * Joshua Pepper [email protected] 1

Department of Neurosurgery, Queen Elizabeth Hospital, Edgbaston, Birmingham B29 4AB, UK

Tonsillar pressure on the brain stem and upper cervical cord can also produce a variety of somatic sensory disturbances and subtle motor symptoms, as well as disturbances of bulbar and lower cranial nerve function. The specific responses of these various symptoms to decompressive surgery are not well documented, and when surgical results are reported, data are often derived from small numbers of cases. Furthermore, reported outcomes are often summarised in an understandably general way, into categories such as cured, better, same or worse. The Chicago Chiari Outcome Score (CCOS) does provide a means of quantifying outcomes [1], although the assessments remain somewhat subjective, offering general outcomes in broad categories. In this paper, we have fo