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Tonsillectomy with modified reconstruction of the cisterna magna with and without craniectomy for the treatment of adult Chiari malformation type I with syringomyelia

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Abstract

Background

In light of the controversies regarding the surgical treatment of adult Chiari malformation type I (CM-I) with syringomyelia, a retrospective study was conducted to evaluate the safety and efficacy of tonsillectomy followed by modified reconstruction of the cisterna magna with or without craniectomy.

Methods

Between 2008 and 2017, 78 adult CM-I patients (36 males and 42 females, mean age 40.6 years old) with syringomyelia were treated with posterior fossa decompression (PFD) with tonsillectomy and modified reconstruction of the cisterna magna. Patients were divided into two study groups: group A (n = 40) underwent cranioplasty with replacement of the bone flap; group B (n = 38) underwent suboccipital craniectomy. Neurological outcomes were evaluated by traditional physician assessment (improved, unchanged, and worsened) and the Chicago Chiari Outcome Scale (CCOS). Syringomyelia outcomes were assessed radiologically.

Results

The procedure was successfully performed in all patients, and restoration of normal cerebrospinal fluid (CSF) flow was confirmed by intraoperative ultrasonography. The median postoperative follow-up was 20.3 months (range 18–60 months). Clinical improvement was evident in 66 (84.6%) patients, with no significant differences between the two groups (85.0% vs. 84.2%, P = 0.897). According to the CCOS, 36 patients (90.0%) in group A were labeled as “good” outcome, compared with that of 34 (86.8%) in group B (P = 0.734). Improvement of syringomyelia was also comparable between the groups, which was observed in 35 (87.5%) vs. 33 (86.8%) patients (P = 0.887). The postoperative overall (7.5% vs. 23.7%, P = 0.048) and CSF-related (2.5% vs. 18.4%, P = 0.027) complication rates were significantly lower in group A than group B.

Conclusions

Tonsillectomy with modified reconstruction of the cisterna magna without craniectomy seems to be a safe and effective surgical option to treat adult CM-I patients with syringomyelia, though future well-powered prospective randomized studies are warranted to validate these findings.

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Author notes

  1. Bolin Liu, Yuan Wang and Shujuan Liu contributed equally to this work.

Authors and Affiliations

  1. Department of Neurosurgery, Xi’an International Medical Center, No.1 Xitai Road, Xi’an, 710100, Shaanxi Province, China

    Bolin Liu, Dan Lu, Lei Chen, Tao Zheng & Shiming He

  2. Department of Neurosurgery, Tangdu Hospital, Fourth Military Medical University, Xi’an, Shaanxi Province, China

    Bolin Liu, Yuan Wang, Yufu Zhang, Dan Lu, Lei Chen, Tao Zheng, Tianzhi Zhao, Lanfu Zhao, Guodong Gao, Yan Qu & Shiming He

  3. Department of Obstetrics and Gynecology, Xijing Hospital, Fourth Military Medical University, Xi’an, Shaanxi Province, China

    Shujuan Liu

  4. Department of Neurosurgery, Duke University Medical Center, Durham, NC, 27710, USA

    Eric W. Sankey

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Contributions

Conception and design: B Liu, Y Wang, and S He. Acquisition of data: B Liu, Y Wang, S Liu, Y Zhang, D Lu, L Chen, T Zheng, T Zhao, L Zhao, and S He. Analysis and interpretation of data: B Liu, Y Wang, S Liu, and S He. Drafting the article: B Liu, Y Wang, S Liu, and S He. Critically revising the article: all. Reviewed submitted version of manuscript: all. Approved the final version of the manuscript: all. Statistical analysis: B Liu, Y Wang, and S Liu. Administrative/technical/material support: Y Zhang, D Lu, L Chen, T Zheng, T Zhao, L Zhao, G Gao, and Y Qu. Study supervision: G Gao, Y Qu, and S He.

Corresponding author

Correspondence to Shiming He.

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The authors declare that they have no conflict of interest.

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All procedures performed in this study were in accordance with the ethical standards of the institutional research committee (Ethical Committee of Tangdu Hospital) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. For this type of study, formal consent is not required.

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Supplementary Figure 1

Tonsillectomy and modified reconstruction of cisterna magna. A: Open the arachnoid membrane. B: Gently dissect the adhesions among the cerebellar tonsil, arachnoid membrane, and brainstem. C: Resect the cerebellar tonsil subpially. D: Flip the pia superiolaterally and suture. E: Reconstruct the cisterna magna and ensure the foramen of Magendie was unobstructed. F: Suture the arachnoid membrane (PNG 1922 kb)

High resolution image (TIF 4078 kb)

Supplementary Figure 2

Neuropathological findings of resected cerebellar tonsils. Purkinje cell loss and gliosis were commonly observed. Other histological changes included atrophic cerebellar cortex, internal granular layer loss, focal degenerative changes and neuronal eosinophilic change. A: ×100; B: ×200; C: ×400 (PNG 4221 kb)

High resolution image (TIF 16669 kb)

Supplementary Table 1

(DOCX 26 kb)

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Liu, B., Wang, Y., Liu, S. et al. Tonsillectomy with modified reconstruction of the cisterna magna with and without craniectomy for the treatment of adult Chiari malformation type I with syringomyelia. Acta Neurochir 162, 1585–1595 (2020). https://doi.org/10.1007/s00701-019-04177-9

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