Volume 3, Issue 3 , Pages 128-131, September 2008
Juvenile nasopharyngeal angiofibroma involving the cavernous sinus: Does surgery have a role?
Article Outline
Summary
Juvenile nasopharyngeal angiofibroma involving the cavernous sinus poses a difficult dilemma. In the UKJ practice, radiotherapy is often recommended but has significant implications due to tumour volume and patient age. We present two cases of JNA involving the cavernous sinus treated by surgical excision. The surgical approaches and literature are discussed. In these cases, it was possible to separate the disease from the cavernous sinus without neurological sequelae. In advanced juvenile nasopharyngeal angiofibroma involving the cavernous sinus, surgical treatment remains an important option.
Keywords: Angiofibroma, Sinonasal tumours, Cavernous sinus
Introduction
Juvenile nasopharyngeal angiofibroma (JNA) comprises 0.5% of all neoplasms of the head and neck and usually presents with epistaxis, nasal obstruction followed by the confirmation of a posterior nasal mass in a young adult male and surgical excision is the recommended management. Advanced JNA, although a benign condition, can invade many of the surrounding vital structures including the infratemporal fossa, carotico-venous systems, dura and cavernous sinus. In the advanced cases the role of radiotherapy, surgery and possibly chemotherapy are less clear.
The cavernous sinus is a small venous space, within leaves of dura and periosteum, situated either side of the sella turcica and intimately associated with cranial nerves (III, IV, VI) and the internal carotid artery. Traditionally, JNA invasion of the cavernous sinuses has prompted external beam irradiation therapy to be the primary modality of treatment based on radiological staging of disease. We present two cases of advanced JNA invading the cavernous sinus on MR imaging, but treated surgically through different approaches.
Case-reports
Case 1
A 15-year-old Caucasian boy presented with a 3-month history of clotted blood in the nose. There was a longer history of nasal obstruction but this had been associated with a nasal fracture incurred approximately a year ago. On examination there was a fleshy vascular mass totally obstructing the nasal cavity. There was mild left eye proptosis with normal ocular movements and visual acuity. An MR scan confirmed a very extensive skull base mass eroding the clivus and involving the right cavernous sinus and anterior part of the middle cranial fossa (Fig. 1). Following a multi-disciplinary cranio-facial meeting, surgical excision was advocated. Post-operative external beam radiotherapy was recommended due to the size of the tumour and possible residual disease. The operation was performed jointly by a paediatric head & neck surgeon, cranio-facial surgeon and a plastic & reconstructive surgeon. Pre-operative embolisation was successfully carried out. Initial access was obtained through a mid-facial degloving approach for the anterior part of the lesion. A LeFort I osteotomy provided only limited access and so a left maxillary swing procedure was performed. This gave excellent exposure allowing complete macroscopic tumour removal Figure 2, Figure 3.
Figure 1.
Axial T1W post-contrast MRI scan shows an intensely enhancing mass centred on the left sphenopalatine foramen which is expanded (white arrows) and extending into the posterior nasal cavity, pterygopalatine fossa and infratemporal fossa. There are dark structures within it in keeping with vascular flow voids (red arrow).
Figure 2.
Images at time of presentation (a,b) show the lesion extends posteriorly into Meckel's cave which is bulky (arrow a) and from there to the level of the cavernous sinus (arrow b). Note that the cavernous segment of the left ICA is displaced slightly medially therefore it is difficult to assess true invasion of the cavernous sinus. On a later post-embolisation scan (c,d) the tumour has progressed at this site. The medial and lateral margins of the cavernous sinus are not clearly seen and tumour appears to lie within it. Note also prominent flow voids related to the cavernous segment of the ICA providing arterial supply to the tumour (arrow).
Figure 3.
Left internal carotid cerebral angiogram (AP and lateral projections, (a and b)) showing that part of the tumour is supplied by branches from the cavernous segment of the internal carotid artery (arrow).
The potentially inoperable skull base involvement was peeled away using blunt dissection allowing macroscopically complete removal of the tumour from the skull base, clivus and cavernous sinus. Reconstruction was done with 1.5
mm plates in pre-drilled holes and closure in layers. Various haemostatic packs were used to control any bleeding in the nasal cavity. A further short procedure was carried out a week later to remove all nasal packing and assess the healing cavity. Recovery has been uneventful and radiotherapy commenced locally.
Case 2
A 10-year-old boy presented with a gradually enlarging lesion of the left side of the face over 1 year. He had normal visual acuity but developed increasing difficulty with the airway necessitating a tracheostomy. Following referral to our institution's multi-disciplinary meeting, embolisation was performed, revealing a rich supply from both the internal and external carotid artery systems. MR imaging confirmed a large mass involving the maxillary, ethmoidal and sphenoidal sinuses and extending up into the left cavernous sinus enveloping the cavernous segment of the carotid artery. A left Weber–Ferguson incision was used to gain access to the tumour. The intranasal tumour was debulked. Again, the skull base resection was managed completely by blunt dissection with any bleeding controlled by packing. The post-operative recovery was unremarkable and the tracheostomy was successfully removed. MR imaging at 1 year detected recurrent disease in the nasopharynx and infratemporal fossa. This was resected via a mid-facial degloving approach combined with a Le Fort I osteotomy. Post-operative radiotherapy has been organised.
Discussion
Reports from the literature suggest that JNAs encroaching or invading surrounding structures may be better treated by radiotherapy [1], [2]. The surgical experience with cavernous sinus involvement is limited. Traditionally, true invasion of the cavernous sinus would make the condition inoperable due to the risks of torrential bleeding, cranial nerve injury and CSF leak. Jafek et al. [3] and Fisch [4] have both recommended that, in these cases, treatment should be incomplete excision followed by radiotherapy.
Interestingly, in both of our cases, MR imaging suggested cavernous sinus invasion. However, during the operation we were able to peel the tumour away from the dura leaving the sinus intact. This potential plane between tumour and dura has been described before [5] and remains the principle reason why this group of tumours should be approached surgically.
Commonly used classifications include Fisch's, Chandler's and Session's systems [6]. The latter has been further modified by Radowski and is considered to be the most frequently employed. It is interesting to note that in Radowski's modifications staging is not affected by cavernous sinus involvement, whereas in Fisch's classification [7], cavernous sinus involvement upstages the disease. This variability has implications when comparing studies from different centres. The numerous current staging systems for JNAs do not allow for modifications based on intraoperative findings. In Danesi et al. [7] series’, 50% of radiologically staged advanced JNAs were downgraded by operative findings. This confusion arises because it is often not possible to determine radiologically whether a tumour extends intradurally or not. Further, there are few, if any, predictors that can ascertain the degree of resection pre-operatively. Angiography may help determine vascular invasion but is unlikely to demonstrate whether tumour is removable from the sinus wall. It is well recognised that the tumour/dura interface is difficult to demonstrate but some MR criteria have been developed from work on cavernous sinus invasion by pituitary adenoma to assist in the diagnosis [8]. A multimodal approach with specific criteria may allow a better assessment of tumour progression in advanced cases of JNA.
Approaches for advanced JNA require a multidisciplinary assessment with careful planning. Each tumour needs its own management plan based on volume of disease, extent of involvement of surrounding structures, surgical experience and co-morbidity. We have described two approaches for advanced JNA which were felt to be the most effective in gaining maximum control of the disease process. The preferred soft-tissue approach is mid-facial degloving which gives satisfactory access for the majority of JNAs. These two children underwent Weber–Ferguson incisions and Le Fort I osteotomy to give extended access. The bony work required in the anterior trans-facial approaches used is splendidly offset by the excellent wound healing in this age group. Further benefits include the absence of a visible scar coupled with the avoidance of potential risks of neuropathy and facial asymmetry seen after radiotherapy.
We feel that our experience supported by cogent anecdotal evidence in the literature endorses surgery as the primary choice of treatment in JNA involving the cavernous sinus. The benefits of this include complete tumour removal if it peels away from the dura or significant debulking of tumour volume followed by radiotherapy. However, all treatment options need to be carefully put forward as part of informed consent and the patient's preference remains the key in the decision-making process.
References
- . Juvenile angiofibroma: a more rational therapeutic approach based upon clinical and experimental evidence. Laryngoscope. 1974;84(Dec 12):2181–2194
- . Radiotherapy and radiosurgery for skull base tumors. Otolaryngol. Clin. North Am. 2001;34(Dec 6):1065–1077
- . Juvenile nasopharyngeal angiofibroma: management of intracranial extension. Head Neck Surg. 1979;2(Nov–Dec 2):119–128
- . The infratemporal fossa approach for nasopharyngeal tumors. Laryngoscope. 1983;93(Jan 1):36–44
- . Operative management of tumors involving the cavernous sinus. J. Neurosurg. 1986;64(Jun 6):879–889
- . Juvenile nasopharyngeal angiofibroma—current thoughts on management. ENT News. 2007;15(6):62–64
- . Anterior approaches in juvenile nasopharyngeal angiofibromas with intracranial extension. Otolaryngol. Head Neck Surg. 2000;122(Feb 2):277–283
- . Evaluation of magnetic resonance imaging criteria for cavernous sinus invasion in patients with pituitary adenomas: logistic regression analysis and correlation with surgical findings. Surg. Neurol. 2006;65(Feb 2):130–135
PII: S1871-4048(08)00006-3
doi:10.1016/j.pedex.2008.01.004
© 2008 Elsevier Ireland Ltd. All rights reserved.
Volume 3, Issue 3 , Pages 128-131, September 2008
