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Year : 2001  |  Volume : 49  |  Issue : 2  |  Page : 166--9

Giant ocular nerve neurofibroma of the cavernous sinus : a series of 5 cases.

PS Chandra, AK Mahapatra 
 Department of Neurosurgery, CN Centre, All India Institute of Medical Sciences, New Delhi, 110 029, India., India

Correspondence Address:
P S Chandra
Department of Neurosurgery, CN Centre, All India Institute of Medical Sciences, New Delhi, 110 029, India.


Five cases of giant cavernous sinus neurofibroma arising from the ocular cranial nerves are reported. These patients collected over a period of 5 years consisted of 3 males and 2 females with an age range of 9 to 40 years and a mean of 20.6 years. Clinically, all patients presented with ocular palsies over a long period (mean 3.4 years). All of them underwent a frontotemporal craniotomy along with an orbito-zygomatic osteotomy and excision of the tumour. In patients with extension of the tumour into the orbit, the superior orbital fissure was drilled, the tenon«SQ»s capsule was cut and the intraorbital portion was excised. The management of these tumours is discussed and the literature reviewed.

How to cite this article:
Chandra P S, Mahapatra A K. Giant ocular nerve neurofibroma of the cavernous sinus : a series of 5 cases. Neurol India 2001;49:166-9

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Chandra P S, Mahapatra A K. Giant ocular nerve neurofibroma of the cavernous sinus : a series of 5 cases. Neurol India [serial online] 2001 [cited 2022 Jan 27 ];49:166-9
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  ::   IntroductionTop

Primary intracranial schwannomas usually arise from the vestibular portion of the 8th cranial nerve. Less commonly, they may arise from trigeminal nerve root and the ganglion.[1],[2] Neurofibromas arising from the ocular cranial nerves, that too within the cavernous sinus are very uncommon, unless associated with von Recklinghausen's disease. In small tumours, it is possible to identify the nerve of origin precisely, however, this becomes difficult in large lesions. We report 5 cases of giant neurofibromas arising from the ocular cranial nerves within the cavernous sinus.

  ::   Material and methodsTop

Five patients with giant cavernous sinus neurofibroma (CSN) were treated surgically at the All India Institute of Medical Sciences (AIIMS) over the past 5 years. Their clinical features are summarised in [Table I]. There were 2 females and 3 male patients in the study, with an age range from 9- 40 years (mean 20.6 years). Four patients presented with third nerve palsies, while one had only isolated sixth cranial nerve paresis. Two patients had extra-axial proptosis, but there was no chemosis or congestion. The duration of symptoms varied from one month to 10 years with a mean of 3.4 years. Surprisingly, none of the patients had features suggestive of raised intracranial tension, although irregular dull aching headaches were a common symptom in all the patients. One patient presented with 3rd nerve paresis along with recurrent attacks of sinusitis (case 5). An ENT surgeon outside evaluated him and mass within the sphenoid sinus was detected, biopsy of which resulted in severe bleeding. Following nasal packing, the patient was referred to us and was operated.

Investigations : All the patients were referred to us with CT scans already done outside. MRI revealed the size of the tumour to be more than 3 cms in all the cases, maximum being about 6.5 cms in diameter. The tumours were iso to mildly hyperintense on T1WI, hyperintense on T2WI and enhanced well with contrast injection [Figure 1] and [Figure 2]. In three cases, the tumour was extending into the orbit through superior orbital fissure [Figure 3]. In the remaining two cases, a small wedge of tissue extended until the opening of the fissure. The carotid was displaced medially in two cases and encased in three. Intra-sphenoidal extension was seen in one case with an abnormal loop of the carotid within the tumour and extending into the sphenoid sinus (case 5). Angiogram was performed in the case 5, where a sphenoid mass biopsy had been attempted outside, to look for any injury to the carotid.

Management : All the patients underwent frontotemporal craniotomy along with orbitozygomatic osteotomy. The tumour was located epidurally, within the cavernous sinus in all the cases and was approached through both subtemporal and transsylvian routes. Tumour decompression was performed using microneurosurgical techniques. CUSA and micro-CUSA was used in all the cases. Microvascular doppler was useful to ascertain the position of the internal carotid artery during tumour decompression. Care was taken to preserve the cranial nerves whenever possible. Radical excision (>90%) of the tumour was performed in 4 cases and partial excision in one case (case 4). In the latter, radical excision was not possible due to high vascularity of the tumour. In all the cases, the tumour was shaved around the carotid artery to the extent possible. In 3 cases, where the tumour was extending into the orbit, the superior orbital fissure was drilled epidurally and the incision over the dura was extended into the orbit over the tenon's capsule. Following this, excision of the intraorbital portion was achieved. Histopathology, in all the cases was suggestive of neurofibroma.

Results and followup : Postoperatively patients were put on steroids and anticonvulsants. Antibiotics were administered in patients where surgery lasted for more than 6 hours. All the patients developed complete ophthalmoplegia following surgery, which improved subsequently, beginning from the 2nd week. The follow-up ranged from 6 months to 4 years. During the follow-up, cranial nerve palsies improved completely in two patients (cases 1 and 3). Partial improvement in 3rd nerve paresis was noted in one patient, while there was no improvement in the other two. There was no other preoperative morbidity and mortality. Postoperative imaging was performed in all cases (MRI in 3 cases and CT scans in 2 cases). There was no residual tumour in 2 cases (cases 1 and 3), a small residual tumour was seen around the carotid in two cases, while significant residual tumour was present in one case (case 4). The latter is being followed up now for more than 1 year with repeat CT scans, which do not show any increase in the size of the tumour.

  ::   DiscussionTop

Neurinomas arising from the intracavernous portion of ocular cranial nerves are very rare. Usually, an association of von Recklinghausen's disease is found in cases where a purely motor nerve is involved. Celli et al reviewed 38 cases of neurinomas arising from ocular cranial nerves and included one of their own cases of trochlear nerve schwannoma.[3] More cases have been reported since then.[4],[5],[6],[7],[8],[9] Celli et al[3] divided these tumours into 3 groups i.e. cisternal, cisternocavernous and cavernous. Of the cavernous sinus neurinomas described in the literature,[6],[9],[10],[11],[12],[13] in one group, where the tumour was mainly confined to the cavernous sinus, the clinical profile consisted of the ocular nerve paresis due to involvement of cranial nerves by the tumour. In another group,[14],[15] where there was extension of the tumour into the orbit the clinical profile consisted of single or multiple ocular cranial nerve palsies due to compression of these nerves at the superior orbital fissure or at the orbital apex, usually accompanied by proptosis. In the present series, 3 cases had intraorbital extension of the tumour. Of these, only two patients had proptosis. All of them had only single cranial nerve palsy. This seems surprising, as the tumours were of significant size. Possibly, this may be due to the slow growth of the tumour, which had spared or had caused only subclinical involvement of other cranial nerves. This may be analogous to the slow growing large cerebellopontine angle vestibular schwannomas, where the other cranial nerves are minimally involved, inspite of large size of the tumour.

MRI is the investigation of choice. This not only shows the extent of the tumour, but also provides information regarding the displacement or encasement of the carotid artery.[7] In most of the cases, the preoperative diagnosis was that of a meningioma. However, we feel that if MRI shows a 'tail' of tissue extending into the superior orbital fissure or the orbit, a high possibility of a neurofibroma should be considered. Investigations like CT scan and angiography are rarely required.

Sindou and Pelissou[16] in a review, quoted 50% chance of complete removal of parasellar neurinomas of ocular nerves, similar to that reported for parasellar neurinomas of the fifth nerve. However, with the present microneurosurgical techniques, these tumours may be removed radically. Furthermore, the addition of orbitozygomatic osteotomy provides maximum exposure and minimum retraction. It is not always possible to determine the nerve of origin especially with large sized tumours. In all our patients ophthalmoplegia was present in the immediate postoperative period but recovered completely in 2 patients and partially in 1 patient.


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