Reminder of important clinical lesson


Cavernous sinus syndrome: need for early diagnosis Jaime Toro,1,2,3 Lisseth Estefania Burbano,1 Saúl Reyes,1,2 Paula Barreras3 1

Department of Neurology, Hospital Universitario– Fundación Santa Fe de Bogotá, Bogotá, Colombia 2 School of Medicine, Universidad El Bosque, Bogotá, Colombia 3 School of Medicine, Universidad de Los Andes, Bogotá, Colombia Correspondence to Dr Jaime Toro; [email protected] Accepted 26 February 2015

SUMMARY Cavernous sinus syndrome (CSS) is a rare condition characterised by ophthalmoplegia, proptosis, ocular and conjunctival congestion, trigeminal sensory loss and Horner’s syndrome. These signs and symptoms result from the involvement of the cranial nerves passing through the cavernous sinus. We report the case of a 53-year-old man with a history of daily stabbing headache associated with dizziness, progressive blurred vision, right ocular pain, ptosis and ophthalmoplegia. After working up the patient, a meningioma was identified as the cause of the CSS. Despite advances in neuroimaging techniques, in some cases, the aetiology of CSS remains difficult to determine. We highlight the clinical and radiological features of a meningioma, one of the causes of CSS. Early diagnosis and treatment of CSS play a key role in a better prognosis.

BACKGROUND The cavernous sinus (CS) is a venous plexus that lies between the periosteal and dural layers of the dura mater. It is located at the central base of the skull, on either side of the sella turcica.1 2 Structures passing through the CS include the internal carotid artery, its sympathetic plexus and the abducens nerve. The oculomotor and trochlear nerves are embedded in its lateral wall as is the ophthalmic division of the trigeminal nerve. The maxillary nerve runs through the lower border of the sinus.1 2 CS syndrome (CSS) is characterised by signs and symptoms resulting from the compromise of the cranial nerves passing through the CS. Common clinical manifestations include ophthalmoplegia, proptosis, ocular and conjunctival congestion, trigeminal sensory loss and Horner’s syndrome.1 2 Causes can be divided into vascular, traumatic, neoplastic, infectious and miscellaneous inflammatory disorders.1 2 CSS frequently presents with overlapping clinical and radiological features, making its diagnosis difficult and often significantly delayed.3 4 It is important to have a high degree of suspicion in order to avoid misdiagnosis of this condition, and to initiate proper and timely treatment.


To cite: Toro J, Burbano LE, Reyes S, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2014206999

A 53-year-old right-handed man presented to our neurology clinic with a 2-year history of mild daily stabbing right temporal-parietal headache, associated with dizziness and blurred vision. Symptoms progressed over the 4 months prior to referral to a right ptosis, which raised the probability of a spaceoccupying lesion. At this time, the patient presented to his primary care provider who treated him with analgesics and referred him to the

ophthalmologist. Later, he progressively lost vision in his right eye, and developed complete ophthalmoplegia. Owing to worsening of symptoms he was referred to neurology. The patient had a history of occasional alcohol intake, but denied consuming drugs or smoking. No other relevant medical history was reported. The general physical examination was unremarkable; the patient’s blood pressure was 120/70 mm Hg, pulse rate 70 bpm, respiratory rate 16 breaths/min and his temperature was 37°C. The neurological examination revealed a complete right ptosis (figure 1A) and palsy of extraocular muscles (figure 1C–F). The right pupil was fixed at 6 mm diameter and unresponsive to light, while the left pupil was 3 mm in diameter and reactive (figure 1B). The patient’s best-corrected visual acuity was no light perception in the right eye and 20/30 in the left. Funduscopic examination was normal. The right corneal reflex was absent and hypoesthesia in the territory of the first and second divisions of the right trigeminal nerve was also noted. No periorbital murmurs were auscultated.

INVESTIGATIONS Prior to referral to our hospital, a complete blood cell count, chemistry panel, levels of serum electrolytes, thyroid function tests, erythrocyte sedimentation rate and C reactive protein were carried out and results were within normal limits. Complement levels and results of assays for autoantibodies (antinuclear antibodies, antineutrophil cytoplasmic antibodies and antibodies to antiextractable nuclear antigens (anti-Smith, anti-Ro, anti-La, antiribonucleoprotein)) were also performed, and results were normal. A chest X-ray was also unremarkable. MRI of the brain with gadolinium was ordered at our hospital. T2-weighted images showed a predominantly isointense and partially hyperintense extra-axial mass. The lesion extended from the right CS into the orbital apex (figure 2A), compressing the right temporal lobe and the lateral–dorsal aspect of the pons (figure 2B). The lesion was homogeneously enhanced after gadolinium administration on T1-weighted images (figure 2C, D). These findings were consistent with a meningioma.

TREATMENT The patient was scheduled for surgery.

OUTCOME AND FOLLOW-UP Surgery was performed but the tumour could not be completely resected. The histopathological diagnosis was a meningothelial meningioma corresponding to WHO grade 1.

Toro J, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-206999


Reminder of important clinical lesson

Figure 1 Ocular manifestations of cavernous sinus syndrome. Right ptosis (A), absent right pupillary response (B), and limited adduction (C), adduction (D), supraduction (E) and infraduction (F) of the right eye.


CS pathology accounts for 5% of all cases of ophthalmoplegia. As seen in our patient, the most common cause of CSS is a neoplastic lesion, which can be secondary, result from local spread, or be primary in origin.5–7 Other causes of CSS include: thrombophlebitis, aspergillosis, Tolosa-Hunt syndrome, inflammatory pseudotumour, aneurysm of the internal carotid artery, carotid–cavernous fistula and dural arteriovenous shunt.1 2 5 8 Also, there are many conditions that can produce a painful ophthalmoplegia and mimic a CSS such as vasculitis, basal meningitis, diabetes mellitus and ophthalmoplegic migraine.4 8

CSS usually presents with multiple cranial neuropathies.1 2 9 Indirect signs and symptoms such as proptosis, conjunctival injection, amaurosis and signs of intracranial hypertension can also occur due to an obstruction of arterial or venous structures and a possible mass effect.3 4 Other symptoms include: retroorbital pain, headache, disturbances of the hypothalamicpituitary axis and seizures caused by posterior or lateral extension of the lesion.10 Certain clinical features may be helpful in determining the workup and diagnosis, although in some cases the manifestations can be non-specific and the aetiology of the CSS cannot be confirmed until a biopsy is performed.11–13

Figure 2 Brain MRI. T2-weighted axial (A) and coronal (B) images showing a predominantly isointense and partially hyperintense extra-axial mass extending from the right cavernous sinus into the orbital apex and sphenoid sinus. The lesion was homogeneously enhanced after gadolinium administration on T1-weighted axial (C) and coronal (D) images. A distinctive dural ‘tail’ (arrow) is seen on the coronal (D) image.


Toro J, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-206999

Reminder of important clinical lesson A contrast-enhanced MRI is the most effective technique to study a mass lesion of the CS.2 6 A meningioma is usually hypointense to isointense with respect to grey matter in all MRI sequences, and enhances intensely and homogeneously.1 2 Meningiomas can arise primarily in the CS or grow into it as part of a larger tumour involving the medial sphenoid wing, orbit, the middle fossa, clivus or petrous bone.14 15 As seen in our patient, new onset and slowly evolving headache is common during the initial presentation of a meningioma and it is usually unassociated with other symptoms suggestive of intracranial hypertension.16 This insidious presentation reflects the slow growth of these tumours, which can delay the diagnosis. For example, our patient did not seek medical attention until neurophthalmological symptoms were present. The ideal treatment of benign meningiomas is surgical resection, especially when they are symptomatic.17 Radiotherapy is mainly used as adjuvant therapy for incompletely resected, highgrade or recurrent tumours.18 For CS meningiomas there is still an ongoing debate regarding when to operate and how aggressive the resection should be. New strategies for the treatment of CS meningiomas attempt to preserve neurological function and limit tumour growth by using surgery and radiation therapy safely.19 CS meningiomas should always be considered as a differential diagnosis in a patient with a painful ophthalmoplegia. A careful approach to the evaluation of these patients can lead to early recognition of serious disorders that, if left untreated, can be associated with a poor prognosis.8

Jenny M Macheta (School of Medicine, Universidad de Los Andes, provided technical assistance) for her assistance in the search for relevant literature. Contributors JT drafted and revised the manuscript for intellectual content. LEB prepared the figures and drafted the manuscript. SR and PB drafted the manuscript. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.


3 4 5 6 7 8 9 10 11


Learning points ▸ Facial pain or hypoesthesia associated with ophthalmoplegia should raise suspicion for a cavernous sinus lesion. ▸ Cavernous sinus syndrome (CSS) meningiomas may initially present with symptoms of insidious onset, which can delay the diagnosis. ▸ Contrast-enhanced MRI is recommended in all patients with a CSS.



15 16 17 18

Acknowledgements The authors would like to express their gratitude to Dr Nicolas Useche (Neuroradiology, Hospital Universitario−Fundación Santa Fe de Bogotá, scientific advisor) for his assistance in reviewing the neuroimaging and to


Lee JH, Lee HK, Park JK, et al. Cavernous sinus syndrome: clinical features and differential diagnosis with MR imaging. AJR Am J Roentgenol 2003;181:583–90. Bone I, Hadley D. Syndromes of the orbital fissure, cavernous sinus, cerebello-pontine angle, and skull base. J Neurol Neurosurg Psychiatry 2005;76 (Suppl 3):iii29–38. Kriss TC, Kriss VM, Warf BC. Cavernous sinus thrombophlebitis: case report. Neurosurgery 1996;39:385–9. La Mantia L, Erbetta A, Bussone G. Painful ophthalmoplegia: an unresolved clinical problem. Neurol Sci 2005;26:s79–82. Fernández S, Godino O, Martínez-Yélamos S, et al. Cavernous sinus syndrome: a series of 126 patients. Medicine (Baltimore) 2007;86:278–81. Keane J. Cavernous sinus syndrome: analysis of 151 cases. Arch Neurol 1996;53:967–71. Post MJ, Mendez DR, Kline LB, et al. Metastatic disease to the cavernous sinus: clinical syndrome and CT diagnosis. J Comput Assist Tomogr 1985;9:115–20. Gladstone J, Dodick D. Painful ophthalmoplegia: overview with a focus on Tolosa-Hunt syndrome. Curr Pain Headache Rep 2004;8:321–9. Keane JR. Multiple cranial nerve palsies. Arch Neurol 2005;62:1714–17. Charbonneau F, Williams M, Lafitte F, et al. No more fear of the cavernous sinuses! Diagn Interv Imaging 2013;94:1003–16. Boutarbouch M, Arkha Y, Gana R, et al. Tuberculoma of the cavernous sinus mimicking a meningioma: case report and review of the literature. J Neurol Sci 2009;15:123–6. Noel CB, Moeketsi K, Kies B. Cavernous sinus syndrome, an atypical presentation of tertiary syphilis: case report and review of the literature. Clin Neurol Neurosurg 2011;113:65–7. Messerer M, Dubourg J, Saint-Pierre G, et al. Percutaneous biopsy of lesions in the cavernous sinus region through the foramen ovale: diagnostic accuracy and limits in 50 patients. J Neurosurg 2012;116:390–8. Bhat AR, Wani MA, Kirmani AR, et al. Histological-subtypes and anatomical location correlated in meningeal brain tumors (meningiomas) J Neurosci Rural Pract. 2014;5:244–9. Heth JA, Al-Mefty O. Cavernous sinus meningiomas. Neurosurg Focus 2003;14:e13. Rockhill J, Mrugala M, Chamberlain M. Intracranial meningiomas: an overview of diagnosis and treatment. Neurosurg Focus 2007;23:E1. Norden AD, Drappatz J, Wen PY. Advances in meningioma therapy. Curr Neurol Neurosci Rep 2009;9:231–40. Mirimanoff RO. New radiotherapy technologies for meningiomas: 3D conformal radiotherapy? Radiosurgery? Stereotactic radiotherapy? Intensity-modulated radiotherapy? Proton beam radiotherapy? Spot scanning proton radiation therapy... or nothing at all? Radiother Oncol 2004;71:247–9. Klinger DR, Flores BC, Lewis JJ, et al. The treatment of cavernous sinus meningiomas: evolution of a modern approach. Neurosurg Focus 2013;35:E8.

Copyright 2015 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit for more articles like this and to become a Fellow

Toro J, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-206999


Cavernous sinus syndrome: need for early diagnosis.

Cavernous sinus syndrome (CSS) is a rare condition characterised by ophthalmoplegia, proptosis, ocular and conjunctival congestion, trigeminal sensory...
299KB Sizes 0 Downloads 13 Views