Spontaneous cerebrospinal fluid rhinorrhoea with meningitis secondary to ecchordosis physaliphora

Galloway, Luke; Hayhurst, Caroline

doi:10.1080/02688697.2017.1297766

Cited by 12 publications

(7 citation statements)

References 3 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A literature review in 2013 identified 23 case reports of symptomatic EP 29 and our literature search revealed an additional 8 cases. 24 30 31 32 33 34 35 Symptoms included diplopia, cranial nerve VI palsy, facial dysesthesia, headache, confusion, dizziness, hearing loss, tinnitus, facial pain, hemiparesis, and sudden death due to intratumoral bleed. 25 36 The reported cases also include six cases of rhinorrhea secondary to spontaneous CSF fistula.…”

Section: Discussionmentioning

confidence: 99%

Do All Notochordal Lesions Require Proton Beam Radiotherapy? A Proposed Reclassification of Ecchordosis Physaliphora as Benign Notochord Cell Tumor

Sooltangos

Bódi

Ghimire

et al. 2021

J Neurol Surg B Skull Base

View full text Add to dashboard Cite

Objectives Ecchordosis physaliphora (EP) is a benign notochord lesion of the clivus arising from the same cell line as chordoma, its malignant counterpart. Although usually asymptomatic, it can cause spontaneous cerebrospinal fluid (CSF) rhinorrhea. Benign notochordal cell tumor (BNCT) is considered another indolent, benign variant of chordoma. Although aggressive forms of chordoma require maximal safe resection followed by proton beam radiotherapy, BNCT and EP can be managed with close imaging surveillance without resection or radiotherapy. However, while BNCT and EP can be distinguished from more aggressive forms of chordoma, differentiating the two is challenging as they are radiologically and histopathologically identical. This case series aims to characterize the clinicopathological features of EP and to propose classifying EP and BNCT together for the purposes of clinical management. Design Case series. Setting Tertiary referral center, United Kingdom. Participants Patients with suspected EP from 2015 to 2019. Main Outcome Measures Diagnosis of EP. Results Seven patients with radiological suspicion of EP were identified. Five presented with CSF rhinorrhea and two were asymptomatic. Magnetic resonance imaging features consistently showed T1-hypointense, T2-hyperintense nonenhancing lesions. Diagnosis was made on biopsy for patients requiring repair and radiologically where no surgery was indicated. The histological features of EP included physaliphorous cells of notochordal origin (positive epithelial membrane antigen, S100, CD10, and/or MNF116) without mitotic activity. Conclusion EP is indistinguishable from BNCT. Both demonstrate markers of notochord cell lines without malignant features. Their management is also identical. We therefore propose grouping EP with BNCT. Close imaging surveillance is required for both as progression to chordoma remains an unquantified risk.

show abstract

Section: Discussionmentioning

confidence: 99%

Do All Notochordal Lesions Require Proton Beam Radiotherapy? A Proposed Reclassification of Ecchordosis Physaliphora as Benign Notochord Cell Tumor

Sooltangos

Bódi

Ghimire

et al. 2021

J Neurol Surg B Skull Base

View full text Add to dashboard Cite

show abstract

“…Previous cases report that the endoscopic endonasal transsphenoidal approach can be used to successfully manage the following complications associated with EP, including repair of clival defects [6,8,9,14,15], management of CSF leaks [6,9,14,15], and decompression of the abducens nerve [14,20]. An endoscopic endonasal approach is not always indicated for clival EP.…”

Section: Discussionmentioning

confidence: 99%

“…An MRI with contrast was obtained and compared with the previous CT and MRI examinations. Imaging ration and extension into the sphenoid sinus [2,3,[6][7][8][9]. The migration of notochord tissue explains the potential for physaliphorous cells to be found in the extradural, subdural, and subarachnoid spaces [2,3,6].…”

Section: Case Presentationmentioning

confidence: 99%

Endoscopic Transsphenoidal Management of Ecchordosis Physaliphora in the Sphenoid Sinus: A Case Report and Review of Approaches to Resection

Lindemann¹,

Kamrava²,

Chakraborty³

et al. 2019

J Head Neck Surg

View full text Add to dashboard Cite

Background: Ecchordosis Physaliphora (EP) is a benign lesion originating from the developing notochord, similar to chordoma. We report complete resection of an asymptomatic, extradural EP in the sphenoid sinus using an endoscopic transsphenoidal approach and review surgical approaches to resection. Case description:A 35-year-old woman presented with headache and a family history of multiple sclerosis. Radiologic imaging revealed a 2.6 cm T1 hypointense, T2 hyperintense lesion extending from the dorsal clivus into the posterior aspect of the sphenoid sinus. An endoscopic endonasal transsphenoidal approach facilitated inspection and complete resection of the extradural lesion. Combined clinical, radiographic, surgical, and histopathologic findings confirmed the diagnosis of EP.Conclusions: Endoscopic approaches to resection allow for excellent visualization and resection of clival EPs. Endoscopic endonasal transsphenoidal approaches are minimally invasive options for resection of EP of the anterior clivus, particularly for lesions extending into the sphenoid sinus. While total resection is desirable, there are currently no reported EP recurrences after resection.

show abstract

“…However, there are several case reports describing symptomatic ecchordosis physaliphora as well. The most common symptoms include headache, pneumocephalus, spontaneous cerebrospinal fluid (CSF) rhinorrhea with meningitis, and abducens nerve palsy [3][4][5][6][7]. The differential diagnoses of retroclival intradural lesions consist mainly of chordoma, ecchordosis physaliphora, skull base metastasis, dermoid cyst, epidermoid cyst, and arachnoid cyst.…”

Section: Introductionmentioning

confidence: 99%

A Rare Case of Ecchordosis Physaliphora Presenting With Headache, Abducens Nerve Palsy, and Intracranial Hypertension

Sun

Ajam

Campbell

et al. 2020

Cureus

View full text Add to dashboard Cite

We report a rare case of ecchordosis physaliphora presenting with headache, nausea, and diplopia. On neurological examination, the patient was found to have left abducens nerve palsy. CT of the head without contrast was unremarkable. Brain MRI demonstrated a non-enhancing retroclival mass with a mass effect upon the ventral pons. The mass had increased signal intensity on T2 and decreased signal intensity on T1-weighted sequences. Lumbar puncture revealed an opening pressure of 37 cm H 2 O. The patient underwent an endoscopic endonasal approach for retroclival mass resection three weeks later. The tissue analysis of the mass was consistent with ecchordosis physaliphora. This could have been misdiagnosed as idiopathic intracranial hypertension had the MRI of the brain not been performed.

show abstract

Spontaneous cerebrospinal fluid rhinorrhoea with meningitis secondary to ecchordosis physaliphora

Abstract: Transclival CSF fistulae are rare. We report a 40-year-old female who presented with meningitis after a short history of CSF rhinorrhoea. The defect resulted from the rare notochordal remnant, ecchordosis physaliphora (EP), and was successfully resected and repaired endoscopically.

Cited by 12 publications

References 3 publications

Do All Notochordal Lesions Require Proton Beam Radiotherapy? A Proposed Reclassification of Ecchordosis Physaliphora as Benign Notochord Cell Tumor

Do All Notochordal Lesions Require Proton Beam Radiotherapy? A Proposed Reclassification of Ecchordosis Physaliphora as Benign Notochord Cell Tumor

Endoscopic Transsphenoidal Management of Ecchordosis Physaliphora in the Sphenoid Sinus: A Case Report and Review of Approaches to Resection

A Rare Case of Ecchordosis Physaliphora Presenting With Headache, Abducens Nerve Palsy, and Intracranial Hypertension

Contact Info

Product

Resources

About