Cavernous angioma as a rare neuroradiologic finding in the cavernous sinus

554 Sharma et al. / Gliemroth et al.

REFERENCES

Fig. 4 Photomicrograph showing fibrous tissue lined by a monolayer of columnar cells with vesicular nuclei and eosinophilic cytoplasm (H & E × 40.

4. Aqueductal ependymal bands and ependyma lined grooves.11 In general, they are believed to arise from the sequestration of the primitive ependymal lining into either the cortical mantle or the perimedullary mesh. Frede and Yasargil1 attributed such ependymal/glioependymal cysts whether intracerebral or subarachnoid to the ectopic displacement of segments of the neural tube wall at the sites of formation of the tela choroidea, whereas Ho and Chasan8 suggested that their subarachnoid example could have originated from leptomeningeal neuroglial heterotopias. According to Rengachary and Kennedy17 columnar or cuboidal cells may line the walls of these cysts with or without cillia. The blepharoplasts may or may not be identifiable. These cysts never communicate with the ventricular system, lying as they do within the white matter of the cerebral hemispheres and in rare instances in the subarachnoid spaces.17,18 The majority of the ependymal cysts occupy the central white matter of the frontal or temporo-parietal lobes, causing progressive neurological deficits with increased intracranial pressure and seizures. Presentations in our cases, growth disturbances in one and hemifacial spasm in another, were unusual. The woman with hemifacial spasm was known to have benign intracranial hypertension. During the microvascular decompressive procedure no arterial loop was noted in direct relationship with the facial nerve; however, a glistening cystic lesion (ependymal cyst) was found between the facial nerve root and the brain stem. This was excised completely with good outcome. The protein content of the cyst fluid is generally greater than that of the cerebrospinal fluid. On the CT brain scan these cysts appear as a low attenuation cystic lesion however the attenuation value of the cyst fluid is slightly higher than the CSF. On the MR scans ependymal cysts appear isointense or slightly hyperintense to the CSF. These cysts usually do not communicate with the cerebral ventricles. The surgical management consists of the drainage of the cyst fluid and excision of its wall with a good outcome.

CONCLUSION Ependymal/glioependymal cysts are rare, benign, congenital, ependyma lined intraparenchymal (common) or extraparenchymal (uncommon) cysts. They may become symptomatic early (i.e. delayed milestones in infants) or later in life (i.e. hemifacial spasms). Microsurgery is curative.

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1. Friede RL, Yasargil MG. Supratentorial intracerebral epithelial (ependymal) cysts: review, case reports and fine structure. J Neurol Neurosurg Psychiatry 1977; 40:127–137. 2. Brackett CE, Rengachary SS. Arachnoid cysts. In: Youman’s neurological surgery, 2nd edn. Vol. 3. Philadelphia: WB Saunders company, 1982: 1436–1446. 3. Haddad FS, Abla A, Allam C. Ependymal brain cyst. Surg Neurol 1982; 18:246–249. 4. Ismail A, Tampieri D, Melanson D et al. Glioependymal cysts: CT and MR findings. J Comput Assist Tomogr 1992; 16:860–864. 5. Sharma RR, Chandy MJ, Lad SD. Perimesenchephalic ependymal cyst (letter). Br J Neurosurg 1990; 4:447–448. 6. Russel DS, Rubinstein LJ. Tumors and tumor like lesions of maldevelopmental origin, 5th edn., London: Edward Arnold 1989: 710–727. 7. Zulch K. Brain tumors. Their biology and pathology, 3rd edn. Berlin: Springer – Verlag 1986: 441. 8. Ho KL, Chason JL. A glioependymal cyst of the cerebello-pontine angle: Immuno histochemical and ultrastructural studies. Acta Neurolopathogica 1987; 74:382. 9. Gherardi R, Lacombe MJ, Poirier J, Roucayrol AM, Wechsler J. Asymptomatic encephalic intra-parenchymatous neuroepithelial cyst. Acta Neuro-pathologica 1984; 63:264. 10. Tandon PN, Roy S, Elvidge A. Subarachnoid ependymal cyst. Report of two cases. J Neurosurg 1972; 47:741. 11. Zulch KJ. Biologie and Pathologie der Hirngeschwiilste. Handbuch der Neurochirurgie, Vol III. Berlin: Springer 1956. 12. Forester O. Einfall Von Agenesie des corpus callosum verbunden mit einem diverticulum paraphysarium des ventriculus tertius. Z Gesamte Neurol Psychiatr 1939; 164:380–391. 13. Moor MT, Book MH. Congenital cervical ependymal cyst. J Neurosurg 1966; 24:558–561. 14. Forester O, Gagel O. Ein fall Von Ependymzyste des 3. Ventrikels. Ein Beitrag Zur Frage der Beziehungen Psychischer Storungen Zum Hirstamm. Z Gesamte. Neurol Psychiatr 1933; 149:312–344. 15. Shuangshoti S, Phisitburr M, Kasantikul V, Netsky MG. Multiple neuroepithelial (colloid) cysts: association with other congenital anomalies. Neurology 1977;m 27:561. 16. Marx P, Kleihues P, Zulch KJ. Normale and Pathologiche Anatomie des Mittelhirns. Radiologe 1968; 8:335–347. 17. Rengachary SS, Kennedy JD. Intracranial arachnoid and ependymal cysts. In: Wilkins RH, Rengachary SS (eds). Neurosurgery, 2nd edn. New York: McGrawHill 1996; 3709–3726. 18. Patric BS. Ependymal cyst of the sylvian fissure: case report. J Neurosurg 1971; 35:751–754.

Cavernous angioma as a rare neuroradiologic finding in the cavernous sinus J. Gliemroth1 MD,* U. Missler2 MD, A. Sepehrnia1 MD 1

Department of Neurosurgery and 2Department of Neuroradiology, Medical University of Lübeck, Germany

Summary We report the case of a 56-year-old female with a pathologically confirmed cavernous angioma of the cavernous sinus. There are only a few reports on cavernous sinus angiomas in the literature. In contrast to typical intracerebral cavernous angiomas, these lesions are characterized by strong contrast enhancement on computed tomography and magnetic resonance imaging. In spite of the problematic location within the cavernous sinus, these angiomas can be completely resected without additional neurologic deficits. The clinical course of the patient and the unusual neuroradiologic imaging findings, as well as the cases from the literature are discussed. © 2000 Harcourt Publishers Ltd

© 2000 Harcourt Publishers Ltd

Cavernous angioma 555 Journal of Clinical Neuroscience (2000) 7(6), 554–557 © 2000 Harcourt Publishers Ltd doi: 10.1054/ jocn.1999.0697, available online at http://www.idealibrary.com on

Keywords: cavernous angioma, cavernous sinus, cavernous meningioma, MRI, CT, angiography Received 7 May 1999 Accepted 4 June 1999

Correspondence to: Jan Gliemroth MD, Department of Neurosurgery, Medical University of Lübeck, Ratzeburger Allee 160, 23538 Lübeck, Germany. Tel.: +49 451 500 2076; Fax: +49 451 500 6191

INTRODUCTION Intra-axial cavernous haemangiomas of the central nervous system (CNS) are uncommon but well described. They can involve any part of the CNS, but tend to be found more often in the cerebral hemispheres. Histopathologically, they consist of a honeycomb of endothelial-lined sinusoidal spaces without intervening neuronal tissue. They usually present with seizures (60%), intracerebral haemorrhage (20–30%) or focal or progressive neurologic deficits (20–25%). In 40% of all cases, they are multiple. They often occur as hyperdense lesions on a nonenhanced computed tomography (CT). They are occasionally calcified. They usually do not enhance on CT following contrast administration. In magnetic resonance imagine (MRI), a mixed pattern with areas of low and high signal is usual. The most sensitive MR modality is gradient echo sequences as they demonstrate the susceptibility effects of haemosiderin as a surrounding low-signal rim. Cavernous haemangiomas frequently show a delayed enhancement following Gadolinium administration. Angiography is usually normal, but occasionally, a faint blush is seen on the capillary or venous phase. The extraaxial cavernous haemangiomas of the cavernous sinus, however, show a different clinical and neuroradiological picture. They present with mass effect and frequently cranial-nerve deficits. Surgery for excision may be difficult with a high morbidity and mortality. The typical neuroradiological pattern will be described and discussed. CASE REPORT The patient is a 56-year-old female presenting with diplopia, who otherwise had an uneventful clinical history. Neurological examination revealed a left sixth cranial-nerve palsy. The patient reported having the same symptoms 5 years previously. MRI showed a lesion in the left cavernous sinus, which was hyperintense on T2-weighted images and isointense on T1-weighted images with strong enhancement following Gadolinium administration (Figs 1a–c). These findings were interpreted as a vascular meningioma with atypical signal behaviour on T2-weighted images. Following this MRI examination, the patient went to surgery at another hospital. An encapsulated, dark red, pulsating soft mass in the cavernous sinus was exposed. Owing to the estimated high risk of excision, the operation was terminated. CT, another MRI and cerebral angiography were performed postoperatively. On non-enhanced CT, the lesion was hyperdense with a strong enhancement after contrast administration (Fig. 2a). Angiography only showed indirect signs of a space occupying lesion (Fig. 2b). The patient was transferred to our hospital for surgery. Neurological examination revealed a complete palsy of the third, fourth and the sixth cranial nerves on the left side and hypoaesthesia of the first branch of the trigeminal nerve. The © 2000 Harcourt Publishers Ltd

Fig. 1 (a) T2-weighted preoperative axial MRI. (b) T1-weighted preoperative non-enhanced coronal MRI. (c) T1-weighted preoperative coronal MRI after Gadolinium administration.

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a

b

c

Fig. 2 (a) Contrast-enhanced preoperative CT scan. (b) Preoperative angiography of the left carotid artery in lateral view.

patient was operated upon again via a left pterional approach. The malformation could be clearly visualized, isolated and completely resected. The third, fourth, first branch of the fifth, and the sixth cranial nerve were destroyed by the lesion and could not be preserved. However, no further neurologic deficits occured. The diagnosis of a cavernous angioma was confirmed by histopathological examination. The postoperative course was uneventful. Complete removal of the cavernoma was documented by CT (Fig. 3a; directly postoperative) and MRI (Figs 3b & c; 9 months later).

DISCUSSION

Fig. 3 (a) Contrast-enhanced CT directly postoperative. (b) T1-weighted coronal non-enhanced MRI (9 months postoperative). (c) T1-weighted coronal MRI after Gadolinium administration (9 months postoperative).

Cavernous angioma is a rare lesion in the cavernous sinus found most commonly in middle-aged women. It is often misdiagnosed as a meningioma. Only a few reports have been published in the recent literature.1–9 Cavernous sinus angiomas typically cause retrobulbar pain and cranial nerve deficits including

ophthalmoplegia and facial numbness. Hypothalamic symptoms occasionally occur. Because of the high bleeding risk during operation, which is occasionally fatal, cavernous angiomas

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Cavernous angioma / Haemorrhage and pituitary apoplexy 557

should be differentiated from other mass lesions involving the cavernous sinus preoperatively.10 In the previous case reports on cavernous angiomas in the cavernous sinus, the neuroradiologic findings are similar: onnon-enhanced CT there is an isodenseto-hyperdense mass with homogeneous enhancement after administration of contrast material, though this may also indicate a meningioma. The lesion is usually well-demarcated. Erosion of the sphenoid bone is present in more than half of all cases. Calcification of the lesion is unusual. In contrast, however, meningiomas of the cavernous sinus frequently demonstrate hyperostosis and calcification. Angiography shows a slight, delayed blush that often has a flecked appearance with pooling of the contrast medium. However, approximately 20–30% of the cases revealed no staining. On the other hand, meningiomas usually show a marked tumour blush. On MRI, these well-demarcated lesions are remarkably hyperintense in T2-weighted and proton density-weighted images. In T1 sequences they are isointense or slightly hypointense with marked contrast enhancement after Gadolinium administration. The hypointense surrounding rim indicative of haemosiderin which is typical in intracerebral cavernous angioma is not seen in extraaxial cavernous angioma. The most difficult differential diagnosis of cavernoma in the cavernous sinus is meningioma.11 Less frequent possibilities are metastasis, granuloma neurofibroma, chemodectoma and lymphoma. These lesions are often less homogeneous and frequently less clearly demarcated on MR images, which, as a rule, makes differentiation from vascular hamartomas less difficult. Aneurysms can be clearly distinguished from the other lesions by MRI and angiography. Accompanying enlargement of the foramen ovale or foramen rotundum favours the diagnosis of the rare trigeminal neurinoma. Because of the considerably different surgical procedures required, it is important to identify cavernomas in the cavernous sinus and to distinguish them from meningiomas prior to surgery.12 Despite its rarity, cavernoma of the cavernous sinus should be kept in mind. Angiography, together with CT and MRI should be used as preoperative diagnostic tools to distinguish meningioma from cavernoma. The cavernomas reported in the literature do not show more than a very slight, delayed and persistent blush. Bony erosion on CT and marked hyperintensity in T2 are also diagnostic hallmarks. Whether the ‘meningeal tail’ sign on CT and MRI is helpful remains questionable.

REFERENCES 1. Namba S. Extracerebral cavernous hemangioma of the middle cranial fossa. Surg Neurol 1983; 19: 379–388. 2. Rosenblum B, Rothman AS, Lanzieri C, Song S. A cavernous sinus cavernous hemangioma. J Neurosurg 1986; 65: 16–18. 3. Kudo T, Ueki S, Kobayashi H, Torigoe H, Tadokoro M. Experience with the ultrasonic surgical aspirator in a cavernous hemangioma of the cavernous sinus. Neurosurgery 1989; 24: 628–631. 4. Savamura Y, de Tribolet N. Cavernous hemangioma in the cavernous sinus: case report. Neurosurgery 1990; 26: 126–128. 5. Sepehrnia A, Tatagiba M, Brandis A, Samii M, Prawitz R. Cavernous hemangioma in the cavernous sinus: case report. Neurosurgery 1990; 27: 151–155. 6. Katayama Y, Tsubokawa T, Miyazaki S, Yoshida K, Himi K. Magnetic resonance imaging of cavernous sinus cavernous hemangiomas. Neuroradiology 1991; 33: 118–122. 7. Lombardi D, Giovanelli M, de Tribolet N. Sellar and parasellar extra-axial cavernous hemangiomas. Acta Neurochir 1994; 130: 47–54. 8. Goel A, Nadkarni TD. Cavernous haemangioma in the cavernous sinus. Brit J Neurosurg 1995; 9: 77–80. 9. Lee AG, Miller NR, Brazis PW, Benson ML. Cavernous sinus hemangioma. J Neuro-Ophthalmol 1995; 15: 225–229.

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10. Linskey ME, Sekhar LN. Cavernous sinus hemangiomas: a series, a review. and an hypothesis. Neurosurgery 1992; 30: 101–107. 11. Bradac GB, Riva A, Schörner W, Stura G. Cavernous sinus meningiomas: An MRI study. Neuroradiology 1987; 29: 578–581. 12. Meyer FB, Lombardi D, Scheithauer B, Nichols DA. Extra-axial cavernous hemangiomas involving the dural sinuses. J Neurosurg 1990; 73: 187–192.

Spontaneous haemorrhage into an empty sella turcica mimicking pituitary apoplexy S. Alatakis MB BS, G. M. Malham MB CHB, G. C. A. Fabinyi FRACS

Department of Neurosurgery, Austin & Repatriation Medical Centre, Studley Road, Heidelberg, 3084, Victoria, Australia

Summary We present a case of spontaneous haemorrhage into an empty sella turcica with the features of subclinical pituitary apoplexy. A 66-year-old woman with a previously resected pituitary adenoma presented four months later with progressive headache and visual deterioration. Cranial MRI demonstrated hyperacute blood products in a recurrent pituitary adenoma. Operative findings were of subacute blood in an empty sella turcica. There was no operative or subsequent histological evidence of tumour recurrence. The intrasellar haemorrhage was evacuated via a trans-sphenoidal approach, resulting in a rapid improvement in visual function. Endocrine deficits required thyroxine, corticosteroid and desmopressin supplementation. Haemorrhage into an empty sella turcica has not been previously described and needs to be suspected as a clinical entity in patients presenting with the features of pituitary apoplexy. Awareness of this clinical condition will prevent preoperative misdiagnosis. © 2000 Harcourt Publishers Ltd Journal of Clinical Neuroscience (2000) 7(6), 557–560 © 2000 Harcourt Publishers Ltd doi: 10.1054/ jocn.1999.0722, available online at http://www.idealibrary.com on

Keywords: empty sella, intracranial haemorrhage, pituitary adenoma, pituitary apoplexy Received 19 August 1999 Accepted 25 October 1999

Correspondence to: Mr. Gavin Fabinyi, Tel: (03) 9496–3049; Fax: (03) 9496–3028.

INTRODUCTION Pituitary apoplexy is caused by the sudden expansion of a pituitary adenoma secondary to haemorrhage, infarction or necrosis.1 Pituitary adenomas are five times more likely to undergo haemorrhagic change than any other type of primary central nervous system tumour.2 This clinical syndrome occurs in approximately 1% of all pituitary adenomas, but clinically silent pituitary apoplexy occurs with a reported frequency of 10–20%.3 The diagnosis of pituitary apoplexy is often difficult, and delayed recognition is common. Pituitary apoplexy should always be considered in the setting of sudden onset severe headache, reduced visual acuity, ophthalmoplegia and altered conscious state.4,5 The clinical manifestations of pituitary apoplexy are caused by haemorrhage or Journal of Clinical Neuroscience (2000) 7(6), 542–560