Transactions 29th European Strabismological Association Meeting – de Faber (ed) © 2005 European Strabismological Association, ISBN 04 1537 211 9

Glomus jugulare tumour presenting with VIth nerve palsy

A.G. Sainani, E. Dawson & J.P. Lee

Moorfields eye hospital, London, UK

ABSTRACT:

Introduction: Tumours of the glomus jugulare are a rare entity. The typical presentation is with middle ear symptoms or dysfunction of cranial nerves VIIth, VIIIth, Xth, XIIth, and IXth.

We report two cases of glomus jugulare tumour with diplopia secondary to VIth nerve palsy as the presenting feature.

Methods: Patients included were, one female with an age of presentation of 52 years and one male of 55 years. Both patients underwent resection surgery for the tumour. Both patients were managed initially with Botulinum toxin followed by appropriate squint surgery for the ocular motility problem. One patient regained binocular vision with stereopsis of 240.

Conclusion: This case report is to highlight the rare presentation of sixth nerve palsy as a result of glomus jugulare tumour. To our knowledge, VIth nerve palsy as the presenting feature of glomus jugulare tumour has not been reported before.

1INTRODUCTION

The glomus jugulare is a chemoreceptor located in the adventitia of the superior bulb of the internal jugular vein. Tumours of the glomus jugulare are a rare entity. It is a slow growing tumour, which initially presents with middle ear symptoms. Depending on location and tumour growth, it can cause dysfunction of the VII, IX, X, XI and XII cranial nerves.

We report two cases of glomus jugulare tumour with associated VIth nerve palsies.

2CASE REPORTS

2.1Case No. 1

A 52 year old lady presented with a 3-year history of double vision and difficulty in swallowing. She initially had left sensorineural deafness 5 years ago. She was then diagnosed to have a left glomus jugulare tumour with IX and Xth nerve palsies 2 years ago. She underwent embolization of tumour followed by excision 1 year ago. She also had external beam radiation therapy at the time. After the surgery the double vision worsened and XIIth nerve was paralysed.

On cover test, she had a left convergent squint measuring 70PD. On examining the ocular movements she had grossly limited abduction beyond midline in the left eye indicating a total VIth nerve palsy. The patient initially had botulinum toxin A injected into her left medial rectus, which was followed by a lateral transposition of the superior and inferior recti, to the upper and lower borders of the lateral rectus. She was overcorrected to a 45PD exotropia in primary position on her first post-operative visit. In the next 4 years she slowly drifted back to a 25PD esotropia with diplopia in primary gaze. She then underwent left medial rectus recession on adjustable sutures and a right lateral rectus faden at 7 mm for the residual esotropia. Post operatively she was left with a 4PD esotropia for near and 6PD exotropia for distance with diplopia only on laevoversion.

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2.2Case No. 2

A 55 years old gentleman presented with a 6-month history of double vision in left gaze. Past medical history revealed that he was diagnosed with a left glomus jugulare tumour 13 years ago. He had undergone 6 excision operations for the tumour. His last operation was 2 years ago. He also developed a left facial paresis after the 3rd operation.

On examination he had a left sided face turn. On cover test he had an esophoria. Prism bar cover test revealed a primary deviation of 14PD esotropia and a secondary deviation of 25PD esotropia. Ocular movements suggested a partial VIth nerve palsy.

He was initially managed with prisms and also had botulinium toxin A injected into the ipsilateral medial rectus as a postoperative diplopia test. He then underwent a left medial rectus recession and lateral recession on adjustable sutures. Post operatively he corrected to a 2PD exotropia in primary gaze. Although he was asymptomatic in primary gaze, he had double vision on both lateral gazes. This incomitancy worsened in the subsequent years. Neurological assessment revealed that VII, IX and XII nerves were also affected now. Repeat substraction MRI did not show any recurrence, although revealed a significant amount of fibrosis at the apex of the temporal bone in the region of the excised tumour.

He subsequently underwent Scott procedures on the medial and lateral rectii of the fellow eye. This operation involves the resection followed by recession of a muscle by the same amount to effectively weaken the action of the muscle and was placed on an adjustable suture with the aim to match the eye movements on lateral gaze. Post operatively the diplopia improved on lateral gaze and he regained binocular vision with stereopsis of 240.

3DISCUSSION

In 1941, Stacy Guildi (Guild 1941), of Johns Hopkins Medical School, described an anatomical structure he called a glomus body. The glomus jugularis also known as a jugulotympanic paraganglionii (Glenner 1974) occurs in the jugular bulb of the internal jugular vein. It is a chemoreceptor and when stimulated by hypoxia elicits a reflex increase in ventilation via its connections with the brainstem respiratory centres. Rossenwasseriii (Rossenwasser 1945) was the first to realize the origins of a vascular tumour of the glomus body in the middle ear. Most paragangliomas arise from the paraganglion situated in the wall of the jugular bulb; these are the so-called jugular paragangliomas. A minority of the tumours arise from the paraganglion situated near the middle ear surface of the promontory; these are called tympanic paragangliomas.

The glomus jugulare tumour is the most common tumour of the middle eariv (Spector 1979) and is the second most common tumour of the temporal bonev(Spector 1980). Females are 4 to 6 times more commonly affected than males vi(Alford 1962). Patients generally present in the 5th and 6th decades of life.

The progression of manifest symptomatology depends on the tumour location and local growth,

which commonly progresses as follows. Growth in the middle ear can cause pulsatile tinnitusvii (House 1968) and conductive hearing lossviii,ix (Spector 1975, Brown 1967). The tumour can simulate

an aural polyp that can haemorrhage spontaneouslyix (Brown 1967). Medial extension of the tumour

disrupts the facial nervex (Spector 1975). Invasion of the labyrinth causes a sensorineural hearing lossvi,ix(Alford 1962, Brown 1967). It has been reported that 1–3% of paragangliomas secrete clin-

ically significant levels of catecholaminesxi (Schwaber 1984). The overall incidencex (Spector 1975) of cranial nerve palsy with glomus jugulare tumour is reported to be 35%. The IX, X, XI, XII nerves and sympathetic chain are more commonly involved at the level of the neck as they enter the brain through their respective foramina in the skull. Thus the patient may present with hoarseness of voice, difficulty in swallowing, tongue atrophy or Horners syndrome depending on the nerves affected.

It is very uncommon for VIth nerve to be affected in a case of glomus jugulare tumourxii (Gulia 1993). It is distantly related to the jugular foramen lying medial to it as it ascends anterior to the brainstem in the posterior cranial fossa.

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We propose the following theories for the possible cause of VIth nerve palsy in a case of Glomus jugulare tumour:

The sixth nerve has been reported to be affected post surgically, after excision of the tumour. It can be affected directly during the excision of a large tumour with intracranial extension or due to post surgical fibrous tissue formation in the area of the VIth nerve. The later is the most probable cause in our second patient who had undergone multiple surgical procedures for the tumour prior to the VIth nerve palsy and a subsequent MRI showed scarring over the petrous part of the temporal bone.

Large tumours have been known to spread to the posterior cranial fossax (Spector 1975) through the jugular and hypoglossal foramina. Access to the middle cranial fossa is provided by the carotid artery through the foramen lacerum. Intracranial extension pressing on the VIth nerve directly can thus be a cause of the palsy. Raised intracranial pressure may result in papilloedemavi (Alford 1962) with VIth nerve palsy as a false localising sign.

Distant metastasis is reported to occur only in 4% of casesxiii (Borsanyi 1962). The sites of metastasis in decreasing order are lungs, lymph nodes, liver and bonexiv. A small metastatic lesion near the intracranial part of VIth nerve can be a theoretically rare possibility.

Jugulotympanic tumours have a tendency towards multicentricityxv, xvi(Rossenwasser 1968, Spector 1973). Multicentricity is the simultaneous occurrence of synchronous tumours in the different craniocervical paraganglia. These synchronous lesions may arise as a carotid body tumour, a glomus intravagalexvii (Ervin 1984) or another jugulotympanic paraganglioma. Although a synchronous tumour has been reported to affect the Xth nerve, it has not yet been found in the area of the VIth nerve.

The management of the ocular motility problems and the diplopia due to VIth nerve palsy secondary to this tumour is not considered to be any different from that, of any other VIth nerve palsy.

4CONCLUSION

This case report highlights the rare presentation of sixth nerve palsy as a result of glomus jugulare tumour. VIth nerve palsy has been reported to occur secondary to neurosurgery for the tumour, as was the case in our second patient. In our first patient although, VIth nerve palsy was a presenting feature. To our knowledge, VIth nerve palsy as the presenting feature of glomus jugulare tumour has not been reported before.

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