Journal of Cranio-Maxillo-FacialSur~erv
(1994] 22. 311 316
The surgical approaches to nasopharyngeal angiofibroma Giorgio Iannetti 1, Evaristo Bell?, Francesco De Ponte 1, Andrea CicconettP, Roberto Delfini 2
Department of Maxillo-Facial Surgery, 2Department of Neurosurgery, University of Rome ' La Sapienza', Italy
S U M M A R Y. After a short summary of the aetiopathogenesis, the routes of extension and the diagnostic features of nasopharyngeal angiofibroma, the importance of early diagnosis and careful surgical planning is underlined. In particular the extension and topographic localization allows the choice of the best approach to optimize surgical radicality--the prime concern in the treatment of nasopharyngeal angiofibroma. Different surgical approaches are proposed for the tumour removal according to our experience in 17 patients.
INTRODUCTION
prefer to treat patients with nasopharyngeal angiofibroma by surgical therapy only. The goal is the radical removal of the neoformation. This result can be attained through a wide operating field, secondary to the choice of surgical approach. Firstly, this will allow removal of the entire neoplasm with its vascular pedicle and ensure careful haemostasis. Moreover, with the choice of the surgical technique, it is necessary to keep in mind the patient's age and also to limit as much as possible the aesthetic and functional impairment of cranio-facial aesthetics.
The nasopharyngeal angiofibroma is a benign tumour with strong local invasivity, found especially in adolescent males, with a tendency to local recurrence after incomplete resection. It has its origin in the posterior roof of the nasal cavity and it extends to the neighbouring parts of the nasopharynx, and more exactly, according to the majority of authors, it originates from the vascular structures in the basisphenoid region, particularly in the region of the sphenopalatine foramen (Jacobsson et al., 1988; Mishra et al., 1989). The age of appearance is around 10 to 12 years of age, although usually clinical symptoms present at a later age, about 14 to 15 years of age. This fact can be related to the involvement of anatomical structures such as the nasopharynx, ethmoid, sphenoid, paranasal sinuses and infratemporal fossa, presenting a high level of functional compensation which hides the neoformation until it reaches significant dimensions (Ross et al., 1966). The clinical period can be extremely variable related to two important factors"
MATERIALS AND METHODS This study encompasses the 17 patients with nasopharyngeal angiofibromas evaluated and treated in the Department of Maxillofacia! Surgery University of Rome 'La Sapienza' from 1986 to 1992. All 17 patients had extensive lesions, while the age range was from 16 to 36 years. The extent of tumour involvement was established by clinical and radiological evaluation. : The therapeutic approach was based on an exact diagnosis of the tumour's extent and location and on its relation to the neighbouring anatomical structures and it was further clarified by study of the neoplastic vascularization. This information is obtained by CT and NMR, together with carotidography that allows choice of the correct surgical approach (Jacobsson et al., 1988). Additionally, carotidography represents a useful therapeutic aid associated with selective embolization of the neoplasm, preferably performed 24 to 48 h before surgery (Standefer et al., 1983).
1. The tendency to progressive growth 2. The rich vascularity. In the first case nasopharyngeal angiofibroma extends from the region of origin preferentially through loci minoris resistentiae (Mishra et al., 1989), such as the facial skeletal and the skull base openings or alternatively it extends directly through the skeletal structures invading the neighbouring region; in the second case some large, not easy to manage, haemorrhages may occur during the period of its development as well as during the intraoperative period. Surgery and radiotherapy (Comming et al., 1984) represent the two main therapeutic options although the literature reports an almost total preference for surgery as the main choice of treatment. Due to the contraindications to radiotherapy (high risk of development of malignancy in the tumour) we
SURGICAL PROCEDURE The surgical approaches used in our department can be separated into trans-facial and trans-basal, relating to the need for exposure and/or removal of the skull base. 311
312 Journalof Cranio-Maxillo-FacialSurgery
Fig. 1- Trans-naso-ethmoidalapproach. It is possible by means of this approach to expose the nasal-ethmoidalregion and the rhinopharynx. In the first case, we can separate the approach into three types: the trans-oral, trans-naso-ethmoidal and trans-maxillary approaches (Belmont et al., 1988); in the second case into two types: the anterior and lateral approaches. The trans-oral approach is indicated when the neoplasm extension is paramedian and antero-inferior, involving the nasal cavity and the maxillary sinus. Osteotomy of the anterior wall of the maxillary sinus is performed after an initial incision located in the gingivolabial sulcus (Conley et al., 1979) extended to the pyriform aperture and carried down to the periosteum. The maxillary sinus and the nasal cavity are thus exposed, permitting radical removal of the tumour. The repositioning and fixing of the osteotomized bone segments permits complete reconstruction of the patient's face. For a median tumour, with an antero-inferior extension, involving the ethmoid, nasal region and the rhinopharynx, it is possible to perform a lateral rhinotomy (Bremeret al., 1986; Popeet al., 1978) and a mono or bilateral reflection of the nasal pyramid. It may be useful to perform an inferior a n d / o r middle turbinectomy to increase the possibility of exposing and removing the tumour. The osteotomized segment is repositioned to restore the nasal pyramid integrity. Where lesions involve the entire nasal cavity, the maxillary sinus with erosion of the posterior wall of the maxilla can be approached by a trans-maxillary view using the Weber-Fergusonincision (Fig. 1). The anterior wall of the maxillary sinus with the homolateral nasal bones are removed by an osteotomy performed over the teeth apices. The wide exposure of the naso-ethmoido-maxillo-rhinopharynx complex allows removal of the tumour.
Fig. 2-The trans-basal anterior approach. This is reserved for lesions with the main ethmoido-sphenoidalextensioninvolving the anterior skull base.
The trans-basal anterior approach is reserved for lesions with the main ethmoido-sphenoidal extension involving the anterior skull base (Fig. 2). A bifrontal craniotomy (Standefer et al., 1983) is performed after bicoronal incision and after the preparation of a
The surgical approaches to nasopharyngeal angiofibroma 313
Fig. 3-Subfronto-subtemporal infratemporal approach. It is possible by means of this approach to expose the apex of the orbit, the chiasma and the postero-lateral portion of the anterior half of the skull base.
Fig. 4-Case 1. Patient affectedby a nasopharyngeal angiofibroma. The CT scan in axial and coronal view shows the involvement of the nasopharynx, the nasal cavity and the infratemporal fossa.
peduncled pericranial flap. An adequate exposure of the supero-medial orbital margins and of the glabellonasal region is performed, preserving the infraorbital nerve and the lacrimal duct. After retraction of the frontal lobes, the removal of a fronto-orbito-glabello-nasal segment is performed, thus allowing a tangential exposure of the anterior skull base as far as the clinoid processes and the entire naso-ethmoidal region. This tangential approach allows the removal of the ethmoidal planum with minimal retraction of the brain lobes, limiting the risk of dural laceration and if
Fig. 5-The postoperative CT scan shows the total removal of the neoplasm.
Fig. 6-Case 2. Young patient affectedby a nasopharyngeal angiofibroma on the left side.
necessary permitting easier repair. The skull base can be rebuilt by reversal of the pericranial flap, and in this case associated with a bone autotransplant, after repositioning and fixation of the osteotomized bone segment. Fixation by means of micro and mini plates permits a perfect spatial repositioning of the osteotomized segments. As reported in the literature by various authors, the involvement of the infratemporal fossa and the orbital apex is a relatively rare possibility for tumours, originating in the sphenopalatine region, that find a preferential route to extend into the inferior orbital fissure (Figs 4, 6, 7, 8).
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Figs 7 & 8 - T h e CT scan, in axial and coronal view demonstrated the involvement of the nasopharynx, the nasal cavities and the infratemporal fossa (arrows).
The lateral trans-basal approach possibly extended to the superior maxilla and to the orbita is best indicated in such cases (Andrews et al., 1989; Fish et al., 1983; Goldenberg et al., 1984). By fronto-temporal craniotomy and osteotomy of the supero-lateral orbital rim and of the zygomatic arch, the infratemporal fossa, the frontal and temporal lobes, the ocular, globe and the orbital apex as far as the chiasma are exposed (Fig. 3). Osteotomy of the inferior orbital rim and of the anterior maxillary wall by means of a Weber-Fergusonincision allows total exposure and removal of the tumour (Fig. 5, 9, 10). Repositioning of the osteotomized segments and stabilizing with rigid fixation permits a good immediate facial reconstruction (Fig. 11). The temporal muscle is repositioned in the temporal fossa to protect the brain lobes and the orbital contents.
Figs 9 & 10-The postoperative CT scan shows the radicality of the surgical treatment.
DISCUSSION Nasopharyngeal angiofibroma comprises about 0.5 % (Jacobsson et al., 1988) of all head and neck tumours. Its anatomical point of origin is the sphenopalatine region. Tumour growth may involve the nasopharynx, maxillary sinus, sphenoid, infratemporal fossa, ethmoid, orbital and oral cavity; intracranial extension occurs in about 20 to 36 % (Close et al., 1989). Clinical symptoms (Fitzpatrick et al., 1980), generally presenting at an advanced stage of the tumour, include epistaxis, nasal obstruction and discharge, pain and swelling of soft tissues.
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Vascularization can be via the internal maxillary artery, the internal or external carotid artery, the common carotid artery and finally the ascending pharyngeal artery (Jacobsson et al., 1988). In order to apply correct surgical treatment to a nasopharyngeal angiofibroma, an exact evaluation of the turnout extent, or erosion or bone displacement is necessary, by using the most modern radiological techniques such as CT scan and N M R ; particularly, we agree with the classification proposed by Andrews and Fish et al., 1983 (Table 1 and 2) based on the growth pattern of this turnout, Presurgical angiography associated with the selective embolization of tumour's nutrient arteries, performed not more than 24 h before surgery, allows: 1. Evaluation of the pathological vascularity of the tumour 2. Neoplasm reduction 3. Reduction of the intraoperative blood loss.
Fig. 11-The postoperative view of the patient shows the satisfactory aesthetic result after a Weber-Ferguson approach associated with a lateral approach to the infratemporal fossa.
Table 1
Patients (no.)
Age (years)
Sex
Type
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17
20 18 16 18 16 17 16 16 22 25 26 18 21 33 22 36 19
M M M M M M M M F M M M F F M M F
III I IIIb II II II III II II IIIb III I II II II IIIa II
Table 2-Classification of nasopharyngeal angiofibromas Type 1 Type I I Type I I I a Type IIIb Type I V a Type V b -
Turnout limited to the nasopharynx and nasal cavity. Bone destruction negligible or limited to the sphenopalatine foramen. Tumour invading the pterygopalatine fossa or the maxillary, ethmoid or sphenoid sinus with bone destruction. Turnout invading the infratemporal fossa or orbital region without intracranial involvement. Tumour invading the infratemporal fossa or orbital region intracranial extradural (parasellar) involvement. Intracranial intradural tumour without infiltration of the cavernous sinus, pituitary fossa or optic chiasma. Intracranial intradural tumour with infiltration of the cavernous sinus, pituitary fossa or optic chiasma.
CONCLUSION In our experience and according to the international literature, the treatment of choice for nasopharyngeal angiofibroma is surgery; it must be carefully planned for the best approach (De Fries et al., 1988) as this is fundamentally important for the exposure of the tumour, radical removal, control of possible intraoperative haemorrhage and for the best aestheticfunctional results. Early diagnosis, together with the preoperative embolization of the nasopharyngeal angiofibroma, permits a less aggressive surgical approach and makes radical removal of the neoformation easier. The concept of surgical radicality minimizes the use of complementary therapeutic methods such as chemotherapy (Goepfert, 1985), radiotherapy and hormone therapy. These are not utilized in our centre on account of the high risk of malignancy of nasopharyngeal angiofibroma especially with radiotherapy. Because of the biological characteristics and of the young age of the patient affected by nasopharyngeal angiofibroma, it is extremely important that the surgical approach be executed in such a way to minimize the aesthetic problems. The possibility of osteotomizing the different parts of the cranio-facial bones allows access to deep regions and reduction of surgical risks consequent on inadequate exposure. Moreover, the immediate reconstruction by the repositioning of the osteotomized bone segments by means of mini and micro systems of rigid fixation, enhances the aesthetic and functional results.
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316 Journal of Cranio-Maxillo-Facial Surgery Belmont, J..' The Le Fort osteotomy approach for
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juvenile nasopharyngeal angiofibroma. Arch. Otolaryngol. 111 (1985) 285-289 Jacobsson, M., B. Petruson, Svendsen, et al.: Juvenile nasopharyngeal angiofibroma: a report of eighteen cases. Acta Otolaryngol. 105 (1988) 132-139 Mishra, S. C., G. K. Shukla, et al.: A rationale classification of angiofibromas of the post nasal space. J. Laryngol. and Otology. 103 (1989) 912-916 Pope, T. et al. : Surgical approach to tumors of the nasal cavity. Laryngoscope 88 (1978) 1743-1748 Ross, D. et al.: Nasopharyngeal tumors. Am. J. of Surg. 3 (1966) 524-530. Standefer, J., G. R. Holt, W. E. Brown, et al.. Combined intracranial and extracranial excision of nasopharyngeal angiofibroma. Laryngoscope 93 (1983) 772-778
Prof. G. Iannetti
Dept of Maxillo-Facial-Surgery Viale Regina Elena 287/A Rome 00161 Italy Paper received: 29 March 1993 Accepted: 4 May 1994