- Department of Neurosurgery, Kumamoto University School of Medicine, Kumamoto, Japan
- Center of Neurosurgery, Nobeoka Hospital, Nobeoka, Japan
Correspondence Address:
Shigetoshi Yano
Department of Neurosurgery, Kumamoto University School of Medicine, Kumamoto, Japan
DOI:10.4103/2152-7806.106562
Copyright: © 2013 Yamamoto T This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.How to cite this article: Yamamoto T, Yano S, Hide T, Kuratsu J. A case of ecchordosis physaliphora presenting with an abducens nerve palsy: A rare symptomatic case managed with endoscopic endonasal transsphenoidal surgery. Surg Neurol Int 28-Jan-2013;4:13
How to cite this URL: Yamamoto T, Yano S, Hide T, Kuratsu J. A case of ecchordosis physaliphora presenting with an abducens nerve palsy: A rare symptomatic case managed with endoscopic endonasal transsphenoidal surgery. Surg Neurol Int 28-Jan-2013;4:13. Available from: http://sni.wpengine.com/surgicalint_articles/a-case-of-ecchordosis-physaliphora-presenting-with-an-abducens-nerve-palsy-a-rare-symptomatic-case-managed-with-endoscopic-endonasal-transsphenoidal-surgery/
Abstract
Background:Ecchordosis physaliphora (EP) is a benign notochordal remnant that is usually asymptomatic; symptomatic cases are extremely rare. Most of the reported symptomatic cases were managed by resection via craniotomy.
Case Description:We report a case of a 20-year-old male presenting with abducens nerve palsy. Magnetic resonance imaging performed on admission demonstrated a mass in the retroclival prepontine location. The patient was treated successfully by endoscopic endonasal trans-sphenoidal surgery (ETSS), his postoperative course was uneventful, and the abducens nerve palsy disappeared.
Conclusion:ETSS has advantages not only for treatment but also for differentiation between EP and intradural chordoma. This is the first case of symptomatic EP successfully treated solely by ETSS.
Keywords: Abducens nerve palsy, chordoma, ecchordosis physaliphora, endoscopic endonasal transsphenoidal surgery
INTRODUCTION
Ecchordosis physaliphora (EP) is a benign notochordal remnant that is usually asymptomatic and requires no intervention.[
CASE REPORT
History and examination
A 20-year-old healthy male was admitted to our hospital for sudden onset of diplopia. Physical examination at admission revealed right abducens nerve palsy but no other neurological disorder. The diplopia arose when he gazed straight ahead or to the right. Computed tomography (CT) and magnetic resonance imaging (MRI) demonstrated a lesion, measuring 22 mm in diameter, which was isodense and had no enhancement on CT. On MRI, the lesion appeared heterogeneously hyperintense on a T1-weighted image, more hyperintense on a T2-weighted image, and had slightly restricted diffusion on diffusion-weighted images. No contrast enhancement was observed after the addition of gadolinium diethylenetriamine pentaacetic acid. The lesion compressed the patient's pons and right abducens nerve [
Figure 1
(a) Unenhanced cranial CT performed on admission. The mass is isodense with no visible bone destruction, (b) T1-weighted MRI performed on admission. The mass appears heterogeneously hyperintense, (c) T2-weighted MRI performed on admission. The mass appears more hyperintense than by T1-weighted MRI, (d) Diffusion-weighted images demonstrate that the mass has slightly restricted diffusion, (e) Gadolinium-enhanced axial T1-weighted MRI performed on admission showing no contrast enhancement, (f) Gadolinium-enhanced sagittal T1-weighted MRI performed on admission demonstrate a lesion in the retroclival prepontine location with no contrast enhancement
Treatment
ETSS was performed. A gelatinous mass was partially exposed after removing the mucosa of the sphenoid sinus. The clival bone was drilled to entirely expose the mass, which was mainly located in the extradural space and partially located intradurally, accompanied by a small dural defect. The intradural mass had compressed the arachnoid mater, which had a yellowish discoloration [
Figure 2
Operative findings. (a) An image obtained before drilling the clival recess. The patient underwent ETSS, (b) After removing the mucosa of the sphenoid sinus, the clival bone was drilled to entirely expose the mass. The gelatinous and yellowish-brown mass mainly located in the extradural space, (c) The mass is partially located intradurally accompanied with a small dural defect, (d) An image obtained after removal of the mass. The mass is partially removed and decompression is performed. OCR = opticocarotid recess, CP = carotid protuberance, EP = ecchordosis physaliphora, BA = basilar artery
Postoperative course
The patient's postoperative course was uneventful. Postoperatively, the right abducens nerve palsy improved, but his diplopia persisted when he gazed to the right. Postoperative MRI demonstrated partial removal of the mass, adequate for decompression [
Pathological findings
Pathological examination of the mass showed hypocellular physaliphorous cells with a lobular growth pattern and eosinophilic cytoplasm with vacuolated mucous droplets. Neither mitosis nor dyskaryosis were visible [
DISCUSSION
EP is an ectopic notochordal remnant,[
Symptomatic EP cases are extremely rare and only a few cases have been reported to date. To the best of our knowledge, only 11 cases of symptomatic EP, located in the retroclival prepontine region have been reported, but features of EP remain to be elucidated. Of the 11 previously reported cases and our present patient with symptomatic EP found in the retroclival prepontine location, 5 were male and 7 were female with a mean age of 40.9 years (range, 12-75 years). The major symptoms presented were headache (33.3%), diplopia (25%), and subarachnoid hemorrhage (16.6%) [
To date, nine cases of the reported symptomatic EP cases were resected by craniotomy, one case was treated by craniotomy with endoscopic assist, and one case was treated by endoscopic transthirdventricle approach.[
In our case, the residual mass disappeared after 4 months follow-up. From our review, two of three followed-up cases, which had residual mass after treatment, showed spontaneous loss.[
Next, we wish to distinguish EP from intradural chordoma. Interventions of intradural chordomas should be aimed at total removal of the tumor, which may require adjuvant radiotherapy for the residual tissue.[
From a review of the literature, gadolinium-enhanced T1-weighted MRI, the Ki-67 index, and surgical findings of a gelatinous nodule were the most available differential criteria. In our case, the mass was gelatinous, had no enhancement on MRI, and the Ki-67 index was not increased. Moreover, the residual mass disappeared after 4 months. These findings and the clinical course suggested a diagnosis of EP in our case. Operative findings are important for a diagnosis of EP and to differentiate from an intradural chordoma. Therefore, ETSS has advantages not only for resection but also for diagnosis.
CONCLUSION
Here we present a rare case of symptomatic EP that was managed successfully by ETSS without resection via craniotomy. ETSS has advantages over resection by craniotomy, but additional studies are needed to clarify the features of EP and to conveniently differentiate EP from an intradural chordoma.
References
1. Akimoto J, Takeda H, Hashimoto T, Haraoka J, Ito H. A surgical case of ecchordosis physaliphora [in Japanese]. No Shinkei Geka. 1996. 24: 1021-5
2. Alkan O, Yildirim T, Kizilkiliç O, Tan M, Cekinmez M. A case of ecchordosis physaliphora presenting with an intratumoral hemorrhage. Turk Neurosurg. 2009. 19: 293-6
3. Amer HZ, Hameed M. Intraosseous benign notochordal cell tumor. Arch Pathol Lab Med. 2010. 134: 283-8
4. Cha ST, Jarrahy R, Yong WH, Eby T, Shahinian HK. A rare symptomatic presentation of ecchordosis physaliphora and unique endoscope-assisted surgical management. Minim Invasive Neurosurg. 2002. 45: 36-40
5. Ciarpaglini R, Pasquini E, Mazzatenta D, Ambrosini-Spaltro A, Sciarretta V, Frank G. Intradural clival chordoma and ecchordosis physaliphora: A challenging differential diagnosis: Case report. Neurosurgery. 2009. 64: E387-8
6. Fracasso T, Brinkmann B, Paulus W. Sudden death due to subarachnoid bleeding from ecchordosis physaliphora. Int J Legal Med. 2008. 122: 225-7
7. Ling SS, Sader C, Robbins P, Rajan GP. A case of giant ecchordosis physaliphora: A case report and literature review. Otol Neurotol. 2007. 28: 931-3
8. Macdonald RL, Cusimano MD, Deck JH, Gullane PJ, Dolan EJ. Cerebrospinal fluid fistula secondary to ecchordosis physaliphora. Neurosurgery. 1990. 26: 515-8
9. Mehnert F, Beschorner R, Küker W, Hahn U, Nägele T. Retroclival ecchordosis physaliphora: MR imaging and review of the literature. AJNR Am J Neuroradiol. 2004. 25: 1851-5
10. Miki T, Nakajima N, Akimoto J, Wada J, Haraoka J. Neuroendoscopic trans-third ventricle approach for lesions of the ventral brainstem surface. Minim Invasive Neurosurg. 2008. 51: 313-8
11. Rotondo M, Natale M, Mirone G, Cirillo M, Conforti R, Scuotto A. A rare symptomatic presentation of ecchordosis physaliphora: Neuroradiological and surgical management. J Neurol Neurosurg Psychiatry. 2007. 78: 647-9
12. Stam FC, Kamphorst W. Ecchordosis physaliphora as a cause of fatal pontine hemorrhage. Eur Neurol. 1982. 21: 90-3
13. Stewart MJ, Burrow Jle F. Ecchordosis physaliphora spheno-occipitalis. J Neurol Psychopathol. 1923. 4: 218-20
14. Takeyama J, Hayashi T, Shirane R. Notochordal remnant-derived mass: Ecchordosis physaliphora or chordoma?. Pathology. 2006. 38: 599-600
15. Toda H, Kondo A, Iwasaki K. Neuroradiological characteristics of ecchordosis physaliphora. Case report and review of the literature. J Neurosurg. 1998. 89: 830-4
16. Wyatt RB, Schochet SS, McCormick WF. Ecchordosis physaliphora. An electron microscopic study. J Neurosurg. 1971. 34: 672-7