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Ecchordosis physaliphora: a cautionary tale

  • C Georgalas (a1) (a2), D Terzakis (a1), M Tsikna (a1), Z Alatzidou (a1), S de Santi (a3), V Seccia (a3) and I Dallan (a3)...

Abstract

Background

Ecchordosis physaliphora is a congenital, benign lesion originating from notochordal remnants along the craniospinal axis, most frequently located at the level of the clivus and sacrum. Sometimes ecchordosis physaliphora is difficult to recognise and treat, with a total of twenty-six cases described in the literature.

Methods

This study reports on three cases of previously undiagnosed ecchordosis physaliphora presenting with cerebrospinal fluid rhinorrhoea and meningitis.

Conclusion

Endoscopic transclival or transsphenoid surgery including three-layer (fat, fascia and nasoseptal flap) reconstruction was used in all cases with complete resolution of the symptoms.

Copyright

Corresponding author

Author for correspondence: Dr Christos Georgalas, Endoscopic Sinus and Skull Base Center, Hygeia Hospital, Erythrou Stavrou 4, Maroussi15123, Greece E-mail: cgeorgalas@gmail.com

Footnotes

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Dr C Georgalas takes responsibility for the integrity of the content of the paper

Footnotes

References

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1Dias, LA, Nakanishi, M, Mangussi-Gomes, J, Canuto, M, Takano, G, Oliveira, A.Successful endoscopic endonasal management of a transclival cerebrospinal fluid fistula secondary to ecchordosis physaliphora--an ectopic remnant of primitive notochord tissue in the clivus. Clin Neurol Neurosurg 2014;117:116–19
2Lagman, C, Varshneya, K, Sarmiento, J, Turtz, AR, Chitale, RV.Proposed diagnostic criteria, classification schema, and review of literature of notochord-derived ecchordosis physaliphora. Cureus 2016;8:e547
3Alli, A, Clark, M, Mansell, NJ.Cerebrospinal fluid rhinorrhea secondary to ecchordosis physaliphora. Skull Base 2008;18:395–99
4Ferguson, C, Clarke, DB, Sinha, N, Shankar, JJ.A case study of symptomatic retroclival ecchordosis physaliphora: CT and MR Imaging. Can J Neurol Sci 2016;43:210–12
5Galloway, L, Hayhurst, C.Spontaneous cerebrospinal fluid rhinorrhoea with meningitis secondary to ecchordosis physaliphora. Br J Neurosurg 2019;33:99100
6Choudhri, O, Feroze, A, Hwang, P, Vogel, H, Ajlan, A, Harsh, G 4th. Endoscopic resection of a giant intradural retroclival ecchordosis physaliphora: surgical technique and literature review. World Neurosurg 2014;82:912.e21e26
7Park, HH, Lee, K-S, Ahn, SJ, Suh, SH, Hong, CK.Ecchordosis physaliphora: typical and atypical radiologic features. Neurosurg Rev 2017;40:8794
8Bolzoni-Villaret, A, Stefini, R, Fontanella, M, Bottazzoli, M, Turri Zanoni, M, Pistochini, A et al. Transnasal endoscopic resection of symptomatic ecchordosis physaliphora. Laryngoscope 2014;124:1325–8

Keywords

Ecchordosis physaliphora: a cautionary tale

  • C Georgalas (a1) (a2), D Terzakis (a1), M Tsikna (a1), Z Alatzidou (a1), S de Santi (a3), V Seccia (a3) and I Dallan (a3)...

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