Clinical Neurology and Neurosurgery 117 (2014) 116– 119
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Clinical Neurology and Neurosurgery
journa l h om epa ge : www.elsev ier .com/ locate /c l ineuro
ase Report
uccessful endoscopic endonasal management of a transclivalerebrospinal fluid fistula secondary to ecchordosis physaliphora – Anctopic remnant of primitive notochord tissue in the clivus
uis Augusto Diasa, Márcio Nakanishib, João Mangussi-Gomesb,∗, Marcelo Canutoc,ustavo Takanod, Carlos Augusto Oliveirab
Department of Neurosurgery of the University Hospital of Brasília (HUB), University of Brasília (UNB), Brasília-DF, BrazilDepartment of Otolaryngology & Head and Neck Surgery of the University Hospital of Brasília (HUB), University of Brasília (UNB), Brasília-DF, BrazilDepartment of Radiology of the Federal District Base Hospital (HBDF), Brasília-DF, BrazilDepartment of Pathology of the University Hospital of Brasília (HUB), University of Brasília (UNB), Brasília-DF, Brazil
r t i c l e i n f o
rticle history:ccepted 29 November 2013vailable online 7 December 2013
Ecchordosis physaliphora (EP) is a rare congenital jelly-like lesion,mall in size, found more often on the dorsal midline surface ofhe clivus. Thought to be derived from ectopic remnants of prim-tive notochord tissue, it is usually asymptomatic and found onlyncidentally in post-mortem or radiological studies [1]. Few symp-omatic presentations, however, have already been described in theiterature [2].
We present an unusual case of EP associated to transclivalSF fistula which was successfully managed by an endoscopicndonasal skull base surgery technique. We subsequently present
brief discussion about EP and its main differential diagnosis.
. Case report
A 54-year-old female patient presented to the emergency
nity of a tertiary hospital with an acute history of high fever,evere headache, neck stiffness, vomiting and confusion. Lumbaruncture was promptly performed and CSF analysis confirmed the
hypothesis of acute bacterial meningitis. Proper treatment withantibiotic and corticosteroid regimens was started.
After recovering normal mental status, she referred a 6-monthhistory of intermittent, right-sided, watery rhinorrhea. She deniedco-morbidities and use of medications. Her physical, neurologicaland otolaryngological examinations were otherwise unremarkableat that moment.
She was then submitted to a complete radiological inves-tigation. Computed tomography-cisternography (CT-C) imagesdemonstrated a cystic lesion inside the sphenoid sinus. Thelesion communicated with the subarachnoid space trough a subtleosseous defect in the superior part of the clivus (Fig. 1A). Fur-ther computed tomography (CT) images evidenced that the tinyhole had regular margins and there was no bone erosion (Fig. 1B).On magnetic resonance (MR) scans the intrasphenoidal lesion washypointense and hyperintense on T1 and T2-weighted images,respectively (Fig. 1C and D). The lesion did not show enhancementafter contrast administration on CT and MR images.
Based on these benign clinical and radiological characteristics, adiagnostic hypothesis of transclival CSF fistula secondary to ecchor-dosis physaliphora (EP) was made.
An endoscopic endonasal surgery was performed. During theprocedure, after intrathecal fluorescein injection, a fluorescein-colored cystic lesion was clearly visualized on the posterior wallof the sphenoid sinus. The lesion was excised and two small bone
L.A. Dias et al. / Clinical Neurology and Neurosurgery 117 (2014) 116– 119 117
Fig. 1. Computed tomography (CT) and magnetic resonance (MR) images: (A) Midline sagittal CT-cisternography scan demonstrates a cystic lesion inside the sphenoidsinus (green arrow); contrast medium passage from the subarachnoid space to the interior of the sphenoid sinus (red arrow) through a subtle osseous clival defect (yellowarrow) can also be seen. (B) Sagittal CT image shows an enlarged cystic lesion inside the sphenoid sinus (green arrow). A clearly defined osseous defect in the clivus (yellowarrow) can also be seen. (C) Sagittal T2-weighted MR image (FIESTA sequence) demonstrates a homogeneous and hyperintense cystic lesion inside the sphenoid sinus(green arrow) and a small orifice in the clivus (yellow arrow), communicating the pre-pontine cistern within the mentioned sinus. (D) The clival lesion was hypointense onT1-weighted images and revealed no enhancement after Gadolinium (Gd) administration. (For interpretation of the references to color in this figure legend, the reader isr
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efects were found in the upper third of the clivus. The orificesere corrected using hydroxyapatite bone cement (BoneSource®)
nd additionally repaired with a pedicled nasoseptal flap. A layerf polyethylene glycol hydrogel sealant (Duraseal®) was finallypplied over the mucosal flap (Fig. 2A–D).
Histopathological examination of the lesion revealed a wellircumscribed aggregate of cells, without surrounding tissue inva-ion or bone involvement (Fig. 2E). The cells were immersed in
myxoid and amphophilic matrix and presented numerous andarge intracytoplasmic mucin-containing vacuoles – the so calledhysaliphorous cells (Fig. 2F). No architectural disarray, mitotic fig-res, cellular atypia or pleomorphism could be seen in the wholepecimen (Fig. 2E and F).
Immunohistochemical assays supported the hypothesis of aotochord derived lesion. The tissue was positive for pancytoker-tins (AE1 and AE3 clones) and epithelial membrane antigen (EMA)Fig. 2G and H) and negative for S-100 protein.
The patient did well after surgery and has been asymp-omatic during a 2-year follow-up period. Recent radiologic examsvidenced no lesion recurrence.
. Discussion
Ecchordosis physaliphora (EP) is a benign notochord-derivedesion most often found in the dorsal midline surface of the clivus. It
as first described by Luschka, in 1856. However, it was only almost0 years later that Ribbert experimentally demonstrated its embry-logical origin and coined the name “ecchordosis physaliphora”3,4].
EP is rarely symptomatic and is usually found only incidentallyn 0.4–2% of all post-mortem or radiological studies [1]. However,ome few symptomatic cases have already been reported in theiterature [2].
Similar cases of EP associated to CSF fistula had been describednly twice before [3,5]. MacDonald et al. described the case of a6-year-old woman with CSF fistula secondary to a radiologicallyuspected EP. She was operated via microscopic transsphenoidalpproach. The diagnosis was supported by the anatomopatholog-cal study [5]. For their turn, Alli et al. reported a 52-year-old
oman who also presented with the hypothesis of CSF fistulaecondary to EP. She was primarily submitted to an endoscopicndonasal surgical procedure, which was not sufficient for the
omplete resection of the lesion and prevention of CSF leakage.
microscopic transsphenoidal approach was therefore necessaryo treat the patient. The hypothesis of EP was not confirmed afterurgery [3] (Table 1).
The main differential diagnosis of EP is chordoma. It is alsoa notochord derived tumor which is considered the malignantcounterpart of EP by some authors [1,3,5]. Differentiating EP fromchordomas is often challenging and has very important prognosticand therapeutic implications [3,5]. As no single feature may reliablydifferentiate both lesions, all clinical, radiological, histological andimmunohistochemical characteristics must be taken into accountto the correct diagnosis [6].
In the case presented, the hypothesis of EP was initiallysuspected by radiological findings. Radiology is very useful indemonstrating the indolence and benignity of EP [1,4,6]. Computedtomography (CT) scans usually evidence a subtle and small soft tis-sue mass posterior to the clivus. Although not always present, anosseous stalk-like projection connecting the lesion to the dorsalaspect of the clivus is considered the pathological hallmark of EPby some authors [1,3,4]. Focal bony dissolution may be seen aroundEP, depending on its size and on its relationship to the clivus [1].
Magnetic resonance (MR) is very useful in characterizing EPlesions. MR scans often evidence homogeneous masses that presenthypointense and hyperintense signals on T1 and T2-weightedimages, respectively [1]. Because EP is a scarcely vascularizedlesion, enhancement after contrast administration is usually absent[1,4].
Chordomas, for their turn, are considered locally aggressive andhighly recurrent neoplasms. Patients with chordomas normallypresent with much more aggressive symptoms, like focal neuro-logical deficits and severe headache. Distant metastases are alsonot rare at the moment of diagnosis [1,5]. Radiological imagesoften show heterogeneous masses with irregular intratumoral cal-cification, bone destruction and surrounding tissue invasion [4].MR scans may evidence large heterogeneous masses with low T1and T2-weighted signals [1]. Enhancement after contrast admin-istration on CT and MR scans should always be considered as afundamental feature in the differentiation of EP from chordomas.Chordomas are highly vascularized lesions and present importantheterogeneous enhancement after contrast administration [1,2,4].
Primitive notochord tissue and its variants present strik-ing similar histological and immunohistochemical features [5].Physaliphorous cells, which are characterized by large mucin-containing intracytoplasmic vacuoles, are typically present in allthese lesions. The cells are often immersed in a myxoid amphophilicmatrix. Staining positive for vimentin, low molecular weight cytok-eratins, epithelial membrane antigen (EMA) and S-100 protein is
also common for all these tissues [3,5,6]. Because of all that, thesefeatures may point to the embryological origin of the tumors, butare not useful in differentiating benign from malignant masses[2,3,5].
118 L.A. Dias et al. / Clinical Neurology and Neurosurgery 117 (2014) 116– 119
Fig. 2. Intraoperative endoscopic endonasal views of (A) a gelatinous fluorescein-colored cystic lesion protruding trough the posterior wall of the sphenoid sinus, aftersuboccipital puncture and intrathecal fluorescein injection; (B) two small bone defects in the upper third of the clivus (black arrows); (C) the pedicled nasoseptal flap in place,after the bone defects were corrected; (D) the layer of polyethylene glycol hydrogel sealant applied over the previously placed nasoseptal flap. Histopathological imagesof the lesion after Hematoxylin and Eosin (HE) staining: (E) (HE 40×) and (F) (HE 100×) – a well circumscribed aggregate of basophilic cells, without surrounding tissueinvasion or bone involvement is evidenced; the cells are immersed in a myxoid and amphophilic intercellular matrix and are characterized by the presence numerous andlarge intracytoplasmic mucin-containing vacuoles – the so called physaliphorous cells (red arrow); note the absence of architectural disarray, mitotic figures and/or cellulara nd (H)m r in th
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expansion of EP or the migration of its cells toward the interior ofthe sphenoid sinus [3]. Similarly, pulsations within the pre-pontine
typia or pleomorphism. Immunohistochemical analysis of the excised lesion: (G) aembrane antigen (EMA), respectively. (For interpretation of the references to colo
Some histopathological aspects, however, are more commonn one type of lesion than another. Consonant to its benig-ity, EP is normally characterized by regular aggregates of cellsith no surrounding tissue infiltration, hypercellularity, cellularleomorphism and/or atypia [3,5]. Additionally, proliferationarkers, like mitotic figures and the Ki-67 index, are normally high
n the case of chordomas [2]. Although Ki-67 index was not mea-
ured in the case presented, no mitotic figures were seen withinhe whole specimen studied, supporting the benignity if the lesion.
the lesion stained positive for pancytokeratins (AE1 and AE3 clones) and epithelialis figure legend, the reader is referred to the web version of this article.)
In the case reported, all clinical, radiological, histopathologi-cal and immunohistochemical findings led the authors to the finaldiagnosis. The reason why a benign lesion would cause a CSF fis-tula remains only speculative, however. It could be supposed thatthe surrounding clival bone would be dissoluted by the progressive
cistern could cause bone atrophy and subsequent enlargement ofa previously existing congenital tract related to EP [5]. Indeed,
L.A. Dias et al. / Clinical Neurology and Neurosurgery 117 (2014) 116– 119 119
Table 1Comparison between the case reports of CSF fistula secondary to EP registered in the literature and the case presented.
Case report MacDonald et al. [5] Alli et al. [3] Case presented
Gender F F FAge (years) 66 52 54Symptoms Right-sided watery rhinorrhea Left-sided watery rhinorrhea Right-sided watery rhinorrheaDuration of symptoms before presentation (months) 24 2 6Confirmation of CSF leakage Dosage of chloride and glucose
[fistula secondary to ecchordosis physaliphora. Neurosurgery 1990;26:515–8
bbreviations and symbols: CSF, cerebrospinal fluid; EP, ecchordosis physaliphora; F,, hypointense.a Studies not performed/mentioned.
t really seems that an indolent and progressive mechanism ofone “resorption” is much more probably involved in this case, asvidenced by radiological findings. Findings of osseous “destruc-ion” or “erosion” would be more common in malignant lesions.
Finally, it was opted to perform an endoscopic endonasalpproach to treat the patient in the case reported. With thisechnique, it was possible to completely remove the lesion and suc-essfully correct the clival defect, preventing further CSF leakage.his procedure provided the advantage of closely visualizing theesion and the clival defect. No major complications were reportednd it proved to be the best option for the patient. This is the firstase of CSF fistula secondary to EP to be successfully managed byhis technique [2,3,5].
. Conclusion
EP is a rare lesion which is asymptomatic in most of theases. Differentiating it from chordomas is often challengingnd has important therapeutic and prognostic implications. Clin-cal, radiological, histopathological and immunohistochemical
[
e; CT, computed tomography; MRI, magnetic resonance imaging; ↑, hyperintense;
characteristics must all be considered to the correct diagnosis.Endoscopic endonasal approach should always be considered forthe successful management of EP.
References
1] Mehnert F, Beschorner R, Küker W, Hahn U, Nägele T. Retroclival ecchordosisphysaliphora: MR imaging and review of the literature. AJNR Am J Neuroradiol2004;25:1851–5.
2] Yamamoto T, Yano S, Hide T, Kuratsu J-I. A case of ecchordosis physaliphorapresenting with an abducens nerve palsy: a rare symptomatic case man-aged with endoscopic endonasal transsphenoidal surgery. Surg Neurol Int2013;4:13.
3] Alli A, Clark M, Mansell NJ. Cerebrospinal fluid rhinorrhea secondary to ecchor-dosis physaliphora. Skull Base 2008;18:395–9.
4] Srinivasan A, Goyal M, Kingstone M. Case 133: ecchordosis physaliphora. Radi-ology 2008;247:585–8.
