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Retrocerebellar arachnoid cyst resulting in syringomyelia in a patient without tonsillar herniation: successful surgical treatment with reconstruction of CSF flow in the foramen magnum region

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Abstract

A retrocerebellar arachnoid cyst causing syringomyelia is extremely rare without tonsillar herniation. The authors present a 44-year-old woman with symptoms of foramen magnum compression and syringomyelia. Magnetic resonance imaging demonstrated a large retrocerebellar arachnoid cyst with a large cervicothoracic syrinx but no signs of tonsillar herniation or hydrocephalus. The patient underwent a foramen magnum decompression with C1 laminectomy, microsurgical fenestration of the cyst, and duraplasty. After successful reconstruction of CSF flow, the patient experienced a relief of symptoms and a significant reduction of the syrinx. The intraoperative findings support the theory of a piston mechanism in the development of syringomyelia. Additional arachnoidal adhesions may also obstruct the CSF flow around the craniocervical junction. We recommend the surgical treatment should consist of an adequate decompression of the foramen magnum, wide microsurgical arachnoidal debridement, and duraplasty with autologous grafts sutured in a watertight way.

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Acknowledgments

The authors thank Stephan Emich, neurosurgeon at the Christian Doppler Klinik of Paracelsus Medical Private University, for his professional suggestions with respect to the case study and language review. The authors also thank Miss Wenzhuo Fu for her editing of the figures and surgical video.

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Authors and Affiliations

  1. Department of Neurosurgery, Xuanwu Hospital, Capital Medical University, Changchun Street 45, Beijing, 100053, China

    Liyong Sun, Zan Chen, Wu Hao, Feng Ling & Fengzeng Jian

  2. Department of Neurosurgery, Christian Doppler Klinik, Paracelsus Medical Private University (PMU), Ignaz Harrer Street 79, 5020, Salzburg, Austria

    Stephan Emich

  3. Medical experiments and testing center, Capital Medical University, Xitoutiao Street 10, Beijing, 100069, China

    Wenzhuo Fu

Authors
  1. Liyong Sun
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  2. Stephan Emich
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  3. Wenzhuo Fu
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  4. Zan Chen
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  5. Wu Hao
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  6. Feng Ling
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  7. Fengzeng Jian
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Correspondence to Fengzeng Jian.

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Comments

Marcelo Galarza, Murcia, Spain

The case is interesting because it concerns cervicomedullary and posterior fossa spinal CSF circulation. As the authors state, decompressing the occipito-cervical junction, without treating the posterior fossa pathology, in this case, a relatively small retrocerebellar arachnoid cyst, would have been inadequate, not to say incomplete.

Caveats concerning this report are the risk to generalize such an approach for all retrocerebellar arachnoid cysts. The eloquence of clinical symptoms and the imaging findings should be the main herald for posterior craniocervical surgery. I am wondering what would have happened if the case had been treated as a straight forward Chiari malformation case.

In any event, the imaging and clinical outcome reported in this particular case were good, so we congratulate the authors for their approach.

H. Selim Karabekir, Izmir, Turkey

As mentioned in the case report of Sun et al., syringomyelia has been associated with many cerebral, cerebellar, and spinal lesions. In the literature, most causes of syringomyelia (Chiari malformations, posterior fossa tumors, or cysts) are the obstruction of CSF flow at the level of the foramen magnum and the outlet of the fourth ventricle. As defined by the Sun et al. study, arachnoid cysts are generally congenital lesions and seen in 1 % of intracranial mass lesions, and 10 % of them take place at the posterior fossa. The pathophysiological origin of syringomyelia remains controversial, and there are various hypotheses related with the syringomyelia formation caused by arachnoid cysts. In the case of Sun et al., the patient underwent a decompression of the foramen magnum, microsurgical fenestration of the cyst, and duraplasty. They applied wide microsurgical fenestration of the cysts and adequate exploration as necessary. This is a good case report and review of the literature and successful surgical approach.

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Video that demonstrates the surgical procedure, 4 min, 160 MB (MP4 64 mb)

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Sun, L., Emich, S., Fu, W. et al. Retrocerebellar arachnoid cyst resulting in syringomyelia in a patient without tonsillar herniation: successful surgical treatment with reconstruction of CSF flow in the foramen magnum region. Neurosurg Rev 39, 341–347 (2016). https://doi.org/10.1007/s10143-015-0680-9

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  • DOI: https://doi.org/10.1007/s10143-015-0680-9

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