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Syringomyelia caused by an arachnoid web in a patient with shunted Dandy-Walker malformation

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Abstract

Introduction

Dandy-Walker malformation (DWM) is a congenital brain anomaly characterized by dysgenesis of the cerebellar vermis and the presence of a posterior fossa cyst. The association of syringomyelia with DWM is extremely rare.

Case report

A 10-year-old patient who was diagnosed with DWM in infancy presented with progressive scoliosis and fecal incontinence. He had been treated with cystoventriculoperitoneal shunting with a Y-connection during infancy, which was followed by a revision 6 years later. During the revision surgery, intraventricular bleeding occurred and was managed conservatively. Imaging studies for the current visit revealed syringomyelia along the cervicothoracic spinal cord and a membranous structure around the cervicomedullary junction. Phase-contrast cine magnetic resonance imaging (MRI) revealed disturbed cerebrospinal fluid (CSF) flow across the membrane. We excised the arachnoid web that was tethering the brainstem and blocking CSF flow. Postoperatively, the patient experienced symptom relief, and the follow-up imaging study demonstrated a dramatic decrease in the size of the syringomyelia.

Discussion

We suggest that syrinx formation in this patient was possibly caused by disturbed CSF flow and tethering of the brainstem.

Conclusion

We experienced an unusual case of DWM with syringomyelia which was caused by an arachnoid web blocking CSF flow and tethering the brainstem. The arachnoid web seems to be formed by previous bleeding which occurred at the time of shunt revision. After excision of the arachnoid web, the patient showed good outcome.

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Acknowledgments

This research was supported by Basic Science Research Program through the National Research Foundation of Korea (NRF) funded by the Ministry of Science, ICT, and Future Planning (2015R1A2A1A15055781).

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Authors and Affiliations

  1. Division of Pediatric Neurosurgery, Department of Neurosurgery, Seoul National University Children’s Hospital and Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 110-744, Republic of Korea

    Hee Chang Lee, Jung Won Choi, Ji Yeoun Lee, Ji Hoon Phi, Seung-Ki Kim, Byung-Kyu Cho & Kyu-Chang Wang

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  1. Hee Chang Lee
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  2. Jung Won Choi
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  3. Ji Yeoun Lee
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  4. Ji Hoon Phi
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Correspondence to Kyu-Chang Wang.

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Lee, H.C., Choi, J.W., Lee, J.Y. et al. Syringomyelia caused by an arachnoid web in a patient with shunted Dandy-Walker malformation. Childs Nerv Syst 33, 665–670 (2017). https://doi.org/10.1007/s00381-016-3293-x

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  • DOI: https://doi.org/10.1007/s00381-016-3293-x

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