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CC BY 4.0 · J Neurol Surg Rep 2025; 86(04): e230-e239
DOI: 10.1055/a-2740-7947
Review Report

Unraveling the Diagnostic Challenge of Arachnoiditis Ossificans in Association with Syringomyelia: A Review of the Literature and Two Case Reports

Authors

  • Fatemeh Khafaji 

    1   Department of Neurosurgery, Medical Campus Upper Franconia, Friedrich-Alexander-University Erlangen-Nuremberg, Bayreuth, Germany

  • Jochen Tuettenberg

    2   Department of Neurosurgery, SHG Klinikum Idar-Oberstein, Idar-Oberstein, Germany

  • Clemens Sommer

    3   Institute of Neuropathology, University Medical Center of the Johannes Gutenberg-University Mainz, Mainz, Germany

  • Bernardo Reyes Medina

    4   Department of Neurosurgery, Medical Campus Upper Franconia, Friedrich-Alexander-University Erlangen-Nuremberg, Bayreuth, Germany

  • Frank Hertel

    5   Department of Neurosurgery, Centre Hospitalier de Luxembourg, Luxembourg City, Luxembourg
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Abstract

Introduction

Arachnoiditis ossificans (AO) associated with syringomyelia (SM) is a rare pathology. Its clinical and image-based diagnostic features are challenging to identify. Only a limited number of cases have been published thus far. We present two new cases and offer a review of the literature.

Materials and Methods

We conducted a systematic literature search using PubMed, Web of Science, and Google Scholar with the following keywords: Arachnoiditis ossificans, leptomeningeal calcification, and spinal meningeal calcification, in combination with syrinx, syringomyelia, hydromyelia, cord cavitation, and cystic necrosis of the spinal cord.

Results

AO-SM predominantly affected females (12 F, 7 M), with a mean age of 55.84 ± 14.7 years. The mean follow-up was 14.07 ± 9.01 months postoperatively. The main complaints included low back pain and progressive para-/tetraparesis, with or without urinary disturbances. Potentially causative events occurred 25.07 ± 13.75 years prior to diagnosis. Based on imaging findings, patients primarily experienced thoracic AO-SM. In seven studies, arachnoid cysts were reported in association with AO-SM. Surgical treatment mainly involved microsurgical AO resection, shunting, or draining of the SM, along with duraplasty and cystectomy or fenestration of the arachnoid cyst. A second surgical intervention was conducted on five patients. Approximately 57% of the patients showed improvement.

Discussion and Conclusion

AO-SM remains one of the least understood causes of myelopathy. Clinical and imaging diagnostics continue to pose challenges. Preoperative evaluation using magnetic resonance imaging (MRI) and native CT may be regarded as the gold standard. CT myelography and, occasionally, Cine MRI should be considered to determine the best surgical option. Surgical treatment continues to be a dilemma.

Keywords

arachnoiditis ossificans - CSF block - syringomyelia

Contributors' Statement

F.K., J.T., C.S., and F.H. contributed to the study's conception and design, data acquisition, analysis, and interpretation, as well as manuscript revision, editing, and final approval of the submitted versions. F.K. additionally drafted the initial version of the article and approved it for submission and revision. B.R-M. contributed to the manuscript's revision and editing and provided final approval for submission.




Publication History

Received: 01 July 2025

Accepted: 21 September 2025

Accepted Manuscript online:
07 November 2025

Article published online:
17 November 2025

© 2025. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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