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Surgical approaches to juvenile nasopharyngeal angiofibroma
ARTICLE IN PRESS Journal of Cranio-Maxillofacial Surgery (2006) 34, 3–8 r 2005 European Association for Cranio-Maxillofacial Surgery doi:10.1016/j.jcms.2005.08.006, available online at http://www.sciencedirect.com
Surgical approaches to juvenile nasopharyngeal angiofibroma Harun CANSIZ, M. Gu¨ven GU¨VENC¸, Nihat S- EKERCI˙OG˘LU Department of Otorhinolaryngology (Chairperson: Dr. O+ . Enver), Cerrahpas- a Medical Faculty, Istanbul University, Turkey
SUMMARY. Introduction: Juvenile nasopharyngeal angiofibromas are highly vascular, non-encapsulated tumours affecting predominantly young males. These lesions are benign histologically but they may become lifethreatening with excessive bleeding or intracranial extension. Material and methods: The surgical approaches to 22 male patients with nasopharyngeal angiofibromas are reviewed. A modification of midfacial degloving performed without rhinoplasty incisions and lateral osteotomies is described. Results: The patients’ ages ranged between 9 and 26 years (mean 14.9). Three stage I tumours, 8 stage II tumours, 6 stage III tumours and 5 stage IV tumours were included into this study. All stage I lesions and one stage II lesion were treated via transnasal endoscopic approach. A modified midfacial degloving approach was used for the removal of seven other stage II lesions, all six stage III lesions, and three stage IV lesions. A combined midfacial degloving-infratemporal fossa Fisch C-transcranial approach was the route chosen for the remaining two stage IV lesions. The complications encountered in the postoperative course include temporary facial palsy in one patient (following a Fisch C infratemporal resection), mild crusting in the nasal cavity in 8 patients, and facial paraesthesia in 6 patients whose tumours were resected via midfacial degloving, and rupture of the subpetrous part of the internal carotid artery in one patient. Conclusion: The suggested treatment of juvenile nasopharyngeal angiofibroma consists of an endoscopic transnasal approach for early stage lesions, and a modified midfacial degloving for almost all of the advanced lesions. The latter approach is very useful considering surgical exposure, duration of surgery, cosmetic outcome, and morbidity. It can be combined with an infratemporal approach or craniotomy if necessary. r 2005 European Association for Cranio-Maxillofacial Surgery
Keywords: juvenile nasopharyngeal angiofibroma; surgical approach; modified midfacial degloving
radiotherapy on craniofacial growth and its potential carcinogenic effects limit its use, thus surgery is the mainstay of treatment. Location and extension of the tumour and the experience of the surgical team are important factors when choosing the appropriate surgical approach. The most frequently used surgical routes include transpalatal, Le Fort I maxillotomy, medial maxillotomy, facial translocation, infratemporal, intranasal endoscopic approaches and midfacial degloving (Fagan et al., 1997; Howard and Lund, 1992).
INTRODUCTION Juvenile nasopharyngeal angiofibromas are highly vascular, non-encapsulated tumours affecting predominantly young males, most commonly between the ages of 14 and 25 years (Fagan et al., 1997). They originate from vascular structures in the basisphenoid region, particularly in the region of the sphenopalatine foramen and have a tendency to local recurrence when resected incompletely (Jacobsson et al., 1988; Mishra et al., 1989). Even though these lesions are benign histologically, they are very locally invasive, spread submucosally and thus may easily become lifethreatening due to excessive bleeding or by intracranial extension (Fagan et al., 1997). Spontaneous regression is reported only for residual angiofibromas (Neel et al., 1973; Stansbie and Phelps, 1986). Neverthless, the tendency to bleed diminishes due to an increasing proportion of fibrous elements (Girgis and Fahmy, 1973). The most common presenting symptoms are usually painless, unilateral nasal obstruction and epistaxis (Fagan et al., 1997). The treatment options consist of surgery, radiotherapy, electrocoagulation, oestrogen therapy, embolization, injection of sclerosing agents and cryotherapy (Jacobsson et al., 1988; Standefer et al., 1983). The negative effects of
PATIENTS AND METHODS Twenty-two patients diagnosed with juvenile nasopharyngeal angiofibroma and operated on in this department by two surgeons between 1988 and 2004 were reviewed. They were all male and the mean age at the time of the diagnosis was 14.9 (range: 9–26 years). The patients were initially evaluated with case history, general examination and a complete otorhinolaryngological examination including nasal endoscopy. Computed tomography (CT) scans were taken for each patient, and some also underwent magnetic 3
ARTICLE IN PRESS 4 Journal of Cranio-Maxillofacial Surgery
resonance imaging (MRI). Superselective digital subtraction angiography and preoperative embolization were performed 24–72 h before surgery in 13 cases. The tumours were classified according to Fisch (1983), stage I tumours being limited to the nasal cavity and nasopharynx, with no bone destruction. Stage II tumours invade the pterygomaxillary fossa and/or the paranasal sinuses with bone destruction. Stage III tumours invade the infratemporal fossa, orbit and/or the parasellar region, but remain lateral to the cavernous sinus. Stage IV tumours invade the cavernous sinus, optic chiasma region, and/or the pituitary fossa. All patients were treated solely with surgery, radiotherapy was not given. All stage I lesions were treated via a transnasal endoscopic approach. This approach was also used for the removal of one stage II lesion. Seven other stage II lesions were operated on using the modified midfacial degloving (MMFD) approach. All six stage III lesions were also operated on with MMFD. One of these cases had previously been operated in another institution with a lateral rhinotomy. Three stage IV tumours (Fig. 1) were operated with MMFD, among whom one case had previously been operated on using a lateral rhinotomy approach in another hospital. Combined MMFD-infratemporal fossa Fisch C-transcranial (frontotemporal) approach was the route chosen for the remaining two stage IV lesions (Table 1).
RESULTS Table 2 lists the preoperative and postoperative courses of all 22 patients. Three stage I tumours, 8 stage II tumours, 6 stage III tumours and 5 tumours with intracranial extension (stage IV) were included in this study. All patients underwent a CT scan while 9 also had MRI. Revisional surgery had to be performed in four cases for a recurrence. Two of these had been operated on previously in another institution while the other two patients’ first operations were performed in this hospital. One other patient had undergone radiotherapy previously in another institution before his admission to this hospital. Followup ranged from 5 months to 10 years. The complications encountered in the postoperative course were temporary facial palsy in one patient (following a Fisch C infratemporal resection), mild crusting in the nasal cavity (which disappeared within 3 months) in 8 patients whose tumours were resected via midfacial degloving. Facial paraesthesia was seen in 6 patients also operated on using MMFD. In one patient, the subpetrous part of the internal carotid artery was ruptured after removal of the tumour. This complication was managed by an interventional neuroradiologist with an intravascular covered stent (Fig. 2). There was no other complication.
Fig. 1 – (a) Enhanced CT scan (coronal plane) demonstrating a bulky nasopharyngeal (hypervascular) mass extending into the sphenoid sinus, cavernous sinus and right infratemporal fossa. (Patient 2). (b) Coronal T2 weighted MRI of the patient after surgical excision via modified midfacial degloving.
DISCUSSION Juvenile nasopharyngeal angiofibromas are benign, affecting especially adolescents and young adult males, and comprise about 0.5% of all head and neck tumours (Jacobsson et al., 1988). They originate from the vascular structures in the basisphenoid region, particularly the sphenopalatine foramen, and the roof of the anterior nasopharynx or from the posterior nasal fossa (Jacobsson et al., 1988; Mishra et al., 1989; Standefer et al., 1983). They have a
ARTICLE IN PRESS Surgical approaches to juvenile nasopharyngeal angiofibroma 5 Table 1 – Surgical approach Tumour stage
Endoscopic transnasal
Stage Stage Stage Stage
3 1
1 2 3 4
Total
MMFD
4
Combined MMFD-ITF-craniotomy
Total
7 6 3
2
3 8 6 5
16
2
22
MMFD: modified midfacial degloving. ITF: infratemporal fossa Fisch C approach.
Table 2 – Data regarding patients, lesions, treatment, approach and complications Patient no.
1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
18
19 20
21
22
Age Location Nasopharynx Nasal cavity Maxillary sinus Ethmoids Sphenoid sinus Pterygopalatine fossa Infratemporal fossa Orbit Cavernous sinus Middle cranial fossa Tumour stage Prior surgery Prior radiotherapy Preoperative embolization Surgical approach Endoscopic Transnasal Modified midfacial degloving Infratemporal fossa Craniotomy Complications Mild crusting Facial paraesthesia Temporary facial palsy Carotid artery rupture
9
17
11
14
26
13
15
14
10
18
17
12
13
19
15
14
21
14
15 13
17
10
X X
X X
X X
X X
X X
X X
X X X
X X X
X X X
X X X
X X
X
X X
X X X
X
X X
X
X X X
X X X X X X X IV
X X X
X
X X X
X
X X
X X X
X X X
X X X X X
X X X X X X X
X X X
X X X X X
X X X X X X X X X X III X
a
III Xa
II
II
X
II
X X X X X III
II
X
X
X
X
X
X
X
X
X
X
II
X X IV
X
II
X
X
X X X
X X X X
X X III
X X IV
III
III
IV X
X X
X
X
X
X
X
X
X
X
X X X
X X
X
X X
X
X
X
X X
X
X
X
X
X
X X X
X
X X
X X X
X
X II
I
II
I
I
X X
X
X X
X
X
X
IV Xa X
X X
X
X X
Previous operation with lateral rhinotomy.
pseudocapsule of fibrous tissue produced by compression of the surrounding (normal) tissue. The blood vessels of these highly vascularized tumours lack a complete muscular layer. This can lead to difficulty in controlling bleeding (Standefer et al., 1983). The vascularization is achieved most commonly by the internal maxillary artery but also by the internal or external carotid, the common carotid artery and/or finally the ascending pharyngeal artery (Jacobsson et al.,1988). These tumours can grow anteriorly into the nasal cavity, superiorly into the sphenoid bone or sella, or laterally through the sphenopalatine foramen into the pterygopalatine fossa (Mitskavich et al., 1998). An intracranial extension is found in about 10–20% (Standefer et al., 1983). This intracranial extension can occur from the infratemporal fossa through the floor of the middle cranial fossa, from the pterygomaxillary fissure along the foramen rotundum into
the cavernous sinus, from the sphenoid sinus via the superior wall into the cavernous sinus and/or pituitary fossa, and from the pterygopalatine fossa through the vidian canal (Mitskavich et al., 1998). The most common presenting symptoms are unilateral nasal obstruction and epistaxis (Fagan et al., 1997). Other symptoms include conductive hearing loss, swelling of the cheek, and dacryocystitis (Jamal, 1994). The diagnosis is based on case history, physical examination, and radiological study. Plain radiographs of the nasopharynx and sinuses are not diagnostic. Nasal endoscopy, CT scan and MRI are helpful to delineate the exact location of the lesion, to show any intracranial or infratemporal spread, and to determine the relationship between the tumour and vital structures (Jamal, 1994; Hendrix et al., 1990). Digital subtraction angiography can demonstrate the blood supply, which is variable. The principal aims of the preoperative work-up are to establish a correct
ARTICLE IN PRESS 6 Journal of Cranio-Maxillofacial Surgery
Fig. 2 – Right internal carotid A–P arteriograms of patient 2. (A) Filling of pseudoaneurysm located in subpetrosal segment (arrow). (B) Occlusion of the pseudoaneurysm achieved after placement of covered stent to the affected segment.
diagnosis, to select the optimal surgical approach, and to be prepared for the intraoperative blood loss. Superselective angiography with embolization is performed within two days preoperatively. The biopsy is not recommended unless the diagnosis cannot be achieved with CT or angiography (Jamal, 1994). The tumour usually regresses when the patient reaches the ages of 20–25 years, but complete regression does not occur in every patient. Mishra et al. (1989) and Neel et al. (1973) stated that spontaneous regression is only valuable for residual tumours. Surgery is the mainstay of the treatment even though occasionally it can become quite challenging. The endoscopic transnasal approach is usually preferred for small tumours confined to nasopharynx, nasal cavity, ethmoid and sphenoid sinuses. Nicolai et al. (2003) stated that this approach is also valuable for intermediate sized lesions. It carries the advantage of minimal soft-tissue and bony disruption. The disadvantages with this technique are restricted access and loss of visibility if bleeding
occurs during surgery. The transnasal approach can be used for tumours limited to the nasopharynx, nasal cavity and sphenoid sinus but lateral exposure is very limited with this technique. According to Mann et al. (2004), contraindications for the endonasal approach are stage IV angiofibromas and some stage III cases with major extension into the middle cranial fossa. In the group of patients described here, the transnasal approach was chosen in four cases; three of them were stage I tumours, and the other was a stage II tumour. We believe that this approach can be used in combination with other techniques as an auxiliary tool for more advanced tumours. The Le Fort I maxillotomy approach affords access to tumours limited to the nasopharynx, nasal cavity, paranasal sinuses, pterygopalatine fossa, and to tumours with minor extensions into the infratemporal fossa. Tumours in the nasopharynx, orbit, ethmoids, sphenoid sinus, pterygopalatine fossa, infratemporal fossa, and the medial part of the cavernous sinus can be reached via a medial maxillotomy. The latter can be performed through a lateral rhinotomy, or Weber–Ferguson approach, or by midfacial degloving. Lateral rhinotomy plus Weber–Ferguson approach has the disadvantage to leave a scar in the face, this inconvenience must be considered. The infratemporal approach is best suited for tumours extending to the infratemporal fossa, and for tumours involving the middle cranial fossa and the lateral part of the cavernous sinus. This approach permits ligation of the internal maxillary artery early in the dissection thus limiting intra-operative blood loss. The facial translocation approach provides maximal exposure of the nasopharynx, sphenoid sinus, pterygopalatine fossa, and cavernous sinus. However, it may affect facial growth due to extensive soft-tissue dissection and multiple osteotomies. The facial translocation approach was not used in any of these patients. 18 patients were operated upon via the MMFD which is our favourite approach. Incisions through the gingivobuccal sulcus, septal transfixation, intercartilaginous, circumferential vestibular and pyriform aperture areas are performed for the standard midfacial degloving approach. This carries the advantage of an excellent exposure of the lesion and direct control of the internal maxillary artery in the pterygopalatine fossa and it provides a very satisfactory cosmetic outcome (Fig. 3; Howard and Lund, 1992). Possible immediate complications of this procedure are haemorrhage and paraesthesia of the overlying skin. Crusting of the cavity is frequent but usually resolves within three months. Other complications include oro-antral fistula, epiphora and vestibular stenosis. Several modifications of this technique have been described in order to avoid vestibular stenosis. In the techniques described by Buchwald et al. (1995), and Krause and Jafek (1999), the intercartilaginous incision and circumferential vestibular incisions are
ARTICLE IN PRESS Surgical approaches to juvenile nasopharyngeal angiofibroma 7
possibility of incomplete tumour removal (Mann et al., 2004). The recurrence rate is reported to be between 6% and 27.5% (Zhang et al., 1998; Herman et al., 1999). We had to perform revision surgery in four out of 22 cases. This was probably due to incomplete resection.
CONCLUSION
Fig. 3 – Postoperative view of patient no. 13 whose tumour was resected using modified midfacial degloving. Note that there is no cosmetic deformity.
not performed, and the nasal pyramid is mobilized by lateral osteotomies. Jeon et al. (2003) reported the technique of hemifacial degloving approach involving a gingivobuccal incision, pyriform aperture incision and one sided lateral osteotomy which may be relevant for unilateral lesions. In this department neither rhinoplasty incisions, nor lateral osteotomies were performed. The septum was detached from the maxillopalatine crest using a chisel following extended gingivobuccal incision. The anterior and medial bony walls of one or both maxillary sinuses were removed, and a quite wide exposure was obtained. These bony walls were not reconstructed. The complications encountered with this technique were mild crusting and facial paraesthesia. This approach of MMFD provides a wide exposure in almost every case, if necessary. It can be combined with facial translocation or transcranial techniques when there is intracranial extension. MMFD shortens the operative time, and provides good postoperative healing. As the mucosa in the nasal cavity and turbinates are often left intact, less crusting is encountered. Facial translocation could be more relevant functionally for advanced cases. But this approach requires a longer surgical time and specific material for reconstruction. Interdisciplinary cooperation among experienced otolaryngologists, maxillofacial surgeons, neurosurgeons, neuroradiologists, and anesthesiologists is extremely important. This was obvious in the case with carotid artery rupture treated with a cover stent by the neuroradiologists. Preoperative embolization had been performed in 13 patients. The purpose of embolization was not simply to obstruct the feeder artery, but rather to provoke progressive development of thrombosis in the small distal vessels of the tumour (Standefer et al., 1983). It is believed that this procedure substantially reduces the need for blood transfusion. However, massive operative bleeding can still occur despite embolization. On the other hand, it has been reported that embolization can increase the
Surgery is the treatment of choice for juvenile nasopharyngeal angiofibroma. Early diagnosis and preoperative embolization are important adjuncts. The most appropriate surgical route must be determined considering size and location of the tumour, extension to adjacent structures, and experience of the surgical team. In this department, the treatment consists of endoscopic transnasal approach for early stage lesions, and modified midfacial degloving for almost all of the advanced lesions. The latter approach proved helpful regarding exposure, duration of surgery, cosmetic outcome, and morbidity. It can be combined with an infratemporal approach or craniotomy if necessary. References Buchwald C, Bonding P, Kirkby B, Fallentin E: Modified midfacial degloving: a practical approach to extensive bilateral benign tumours of the nasal cavity and paranasal sinuses. Rhinology 33: 39–42, 1995 Fagan JJ, Snyderman CH, Carrau RL, Janecka IP: Nasopharyngeal angiofibromas: selecting a surgical approach. Head Neck 19: 391–399, 1997 Fisch U: The infratemporal fossa approach for nasopharyngeal tumours. Laryngoscope 93: 36–44, 1983 Girgis IH, Fahmy SA: Nasopharyngeal fibroma: its histopathological nature. J Laryngol Otol 87: 1107–1123, 1973 Hendrix RA, Lenkinski RE, Vogele K, Bloch P, McKenna WG: 31P localized magnetic resonance spectroscopy of head and neck tumours—preliminary findings. Otolaryngol Head Neck Surg 103: 775–783, 1990 Herman P, Lot G, Chapot R, Salvan D, Huy PT: Long-term follow-up of juvenile nasopharyngeal angiofibromas: analysis of recurrences. Laryngoscope 109: 140–147, 1999 Howard DJ, Lund VJ: The midfacial degloving approach to sinonasal disease. J Laryngol Otol 106: 1059–1062, 1992 Jacobsson M, Petruson B, Svendsen P, Berthelsen B: Juvenile nasopharyngeal angiofibroma: a report of eighteen cases. Acta Otolaryngol 105: 132–139, 1988 Jamal MN: Imaging and management of angiofibroma. Eur Arch Otorhinolaryngol 251: 241–245, 1994 Jeon SY, Jeong JH, Kim HS, Ahn SK, Kim JP: Hemifacial degloving approach for medial maxillectomy: a modification of midfacial degloving approach. Laryngoscope 113: 754–756, 2003 Krause GE, Jafek BW: A modification of the midface degloving technique. Laryngoscope 109: 1781–1784, 1999 Mann WJ, Jecker P, Amedee RG: Juvenile angiofibromas: changing surgical concept over the last 20 years. Laryngoscope 114: 291–293, 2004 Mishra SC, Shukla GK, Bhatia N, Pant MC: A rational classification of angiofibromas of the postnasal space. J Laryngol Otol 103: 912–916, 1989 Mitskavich MT, Carrau RL, Snyderman CH, Weissman JL, Fagan JJ: Intranasal endoscopic excision of a juvenile angiofibroma. Auris Nasus Larynx 25: 39–44, 1998
ARTICLE IN PRESS 8 Journal of Cranio-Maxillofacial Surgery Neel III. HB, Whicker JH, Devine KD, Weiland LH: Juvenile angiofibroma. Review of 120 cases. Am J Surg 126: 547–556, 1973 Nicolai P, Berlucchi M, Tomenzoli D, Cappiello J, Trimarchi M, Maroldi R, Battaglia G, Antonelli AR: Endoscopic surgery for juvenile angiofibroma: when and how. Laryngoscope 113: 775–782, 2003 Standefer J, Holt GR, Brown Jr. WE, Gates GA: Combined intracranial and extracranial excision of nasopharyngeal angiofibroma. Laryngoscope 93: 772–779, 1983 Stansbie JM, Phelps PD: Involution of residual juvenile nasopharyngeal angiofibroma (a case report). J Laryngol Otol 100: 599–603, 1986 Zhang M, Garvis W, Linder T, Fisch U: Update on the infratemporal fossa approaches to nasopharyngeal angiofibroma. Laryngoscope 108: 1717–1723, 1998
M. Gu¨ven GU¨VENC¸ Og˘uzhan Cad. Ug˘urpalas Ap. No: 21/5 34270 Fındıkzade/Istanbul Turkey Tel.: +90 212 5234344 Fax: +90 212 4143408 E-mail: [email protected] Paper received 1 February 2005 Accepted 18 August 2005
Surgical approaches to juvenile nasopharyngeal angiofibroma Harun CANSIZ, M. Gu¨ven GU¨VENC¸, Nihat S- EKERCI˙OG˘LU Department of Otorhinolaryngology (Chairperson: Dr. O+ . Enver), Cerrahpas- a Medical Faculty, Istanbul University, Turkey
SUMMARY. Introduction: Juvenile nasopharyngeal angiofibromas are highly vascular, non-encapsulated tumours affecting predominantly young males. These lesions are benign histologically but they may become lifethreatening with excessive bleeding or intracranial extension. Material and methods: The surgical approaches to 22 male patients with nasopharyngeal angiofibromas are reviewed. A modification of midfacial degloving performed without rhinoplasty incisions and lateral osteotomies is described. Results: The patients’ ages ranged between 9 and 26 years (mean 14.9). Three stage I tumours, 8 stage II tumours, 6 stage III tumours and 5 stage IV tumours were included into this study. All stage I lesions and one stage II lesion were treated via transnasal endoscopic approach. A modified midfacial degloving approach was used for the removal of seven other stage II lesions, all six stage III lesions, and three stage IV lesions. A combined midfacial degloving-infratemporal fossa Fisch C-transcranial approach was the route chosen for the remaining two stage IV lesions. The complications encountered in the postoperative course include temporary facial palsy in one patient (following a Fisch C infratemporal resection), mild crusting in the nasal cavity in 8 patients, and facial paraesthesia in 6 patients whose tumours were resected via midfacial degloving, and rupture of the subpetrous part of the internal carotid artery in one patient. Conclusion: The suggested treatment of juvenile nasopharyngeal angiofibroma consists of an endoscopic transnasal approach for early stage lesions, and a modified midfacial degloving for almost all of the advanced lesions. The latter approach is very useful considering surgical exposure, duration of surgery, cosmetic outcome, and morbidity. It can be combined with an infratemporal approach or craniotomy if necessary. r 2005 European Association for Cranio-Maxillofacial Surgery
Keywords: juvenile nasopharyngeal angiofibroma; surgical approach; modified midfacial degloving
radiotherapy on craniofacial growth and its potential carcinogenic effects limit its use, thus surgery is the mainstay of treatment. Location and extension of the tumour and the experience of the surgical team are important factors when choosing the appropriate surgical approach. The most frequently used surgical routes include transpalatal, Le Fort I maxillotomy, medial maxillotomy, facial translocation, infratemporal, intranasal endoscopic approaches and midfacial degloving (Fagan et al., 1997; Howard and Lund, 1992).
INTRODUCTION Juvenile nasopharyngeal angiofibromas are highly vascular, non-encapsulated tumours affecting predominantly young males, most commonly between the ages of 14 and 25 years (Fagan et al., 1997). They originate from vascular structures in the basisphenoid region, particularly in the region of the sphenopalatine foramen and have a tendency to local recurrence when resected incompletely (Jacobsson et al., 1988; Mishra et al., 1989). Even though these lesions are benign histologically, they are very locally invasive, spread submucosally and thus may easily become lifethreatening due to excessive bleeding or by intracranial extension (Fagan et al., 1997). Spontaneous regression is reported only for residual angiofibromas (Neel et al., 1973; Stansbie and Phelps, 1986). Neverthless, the tendency to bleed diminishes due to an increasing proportion of fibrous elements (Girgis and Fahmy, 1973). The most common presenting symptoms are usually painless, unilateral nasal obstruction and epistaxis (Fagan et al., 1997). The treatment options consist of surgery, radiotherapy, electrocoagulation, oestrogen therapy, embolization, injection of sclerosing agents and cryotherapy (Jacobsson et al., 1988; Standefer et al., 1983). The negative effects of
PATIENTS AND METHODS Twenty-two patients diagnosed with juvenile nasopharyngeal angiofibroma and operated on in this department by two surgeons between 1988 and 2004 were reviewed. They were all male and the mean age at the time of the diagnosis was 14.9 (range: 9–26 years). The patients were initially evaluated with case history, general examination and a complete otorhinolaryngological examination including nasal endoscopy. Computed tomography (CT) scans were taken for each patient, and some also underwent magnetic 3
ARTICLE IN PRESS 4 Journal of Cranio-Maxillofacial Surgery
resonance imaging (MRI). Superselective digital subtraction angiography and preoperative embolization were performed 24–72 h before surgery in 13 cases. The tumours were classified according to Fisch (1983), stage I tumours being limited to the nasal cavity and nasopharynx, with no bone destruction. Stage II tumours invade the pterygomaxillary fossa and/or the paranasal sinuses with bone destruction. Stage III tumours invade the infratemporal fossa, orbit and/or the parasellar region, but remain lateral to the cavernous sinus. Stage IV tumours invade the cavernous sinus, optic chiasma region, and/or the pituitary fossa. All patients were treated solely with surgery, radiotherapy was not given. All stage I lesions were treated via a transnasal endoscopic approach. This approach was also used for the removal of one stage II lesion. Seven other stage II lesions were operated on using the modified midfacial degloving (MMFD) approach. All six stage III lesions were also operated on with MMFD. One of these cases had previously been operated in another institution with a lateral rhinotomy. Three stage IV tumours (Fig. 1) were operated with MMFD, among whom one case had previously been operated on using a lateral rhinotomy approach in another hospital. Combined MMFD-infratemporal fossa Fisch C-transcranial (frontotemporal) approach was the route chosen for the remaining two stage IV lesions (Table 1).
RESULTS Table 2 lists the preoperative and postoperative courses of all 22 patients. Three stage I tumours, 8 stage II tumours, 6 stage III tumours and 5 tumours with intracranial extension (stage IV) were included in this study. All patients underwent a CT scan while 9 also had MRI. Revisional surgery had to be performed in four cases for a recurrence. Two of these had been operated on previously in another institution while the other two patients’ first operations were performed in this hospital. One other patient had undergone radiotherapy previously in another institution before his admission to this hospital. Followup ranged from 5 months to 10 years. The complications encountered in the postoperative course were temporary facial palsy in one patient (following a Fisch C infratemporal resection), mild crusting in the nasal cavity (which disappeared within 3 months) in 8 patients whose tumours were resected via midfacial degloving. Facial paraesthesia was seen in 6 patients also operated on using MMFD. In one patient, the subpetrous part of the internal carotid artery was ruptured after removal of the tumour. This complication was managed by an interventional neuroradiologist with an intravascular covered stent (Fig. 2). There was no other complication.
Fig. 1 – (a) Enhanced CT scan (coronal plane) demonstrating a bulky nasopharyngeal (hypervascular) mass extending into the sphenoid sinus, cavernous sinus and right infratemporal fossa. (Patient 2). (b) Coronal T2 weighted MRI of the patient after surgical excision via modified midfacial degloving.
DISCUSSION Juvenile nasopharyngeal angiofibromas are benign, affecting especially adolescents and young adult males, and comprise about 0.5% of all head and neck tumours (Jacobsson et al., 1988). They originate from the vascular structures in the basisphenoid region, particularly the sphenopalatine foramen, and the roof of the anterior nasopharynx or from the posterior nasal fossa (Jacobsson et al., 1988; Mishra et al., 1989; Standefer et al., 1983). They have a
ARTICLE IN PRESS Surgical approaches to juvenile nasopharyngeal angiofibroma 5 Table 1 – Surgical approach Tumour stage
Endoscopic transnasal
Stage Stage Stage Stage
3 1
1 2 3 4
Total
MMFD
4
Combined MMFD-ITF-craniotomy
Total
7 6 3
2
3 8 6 5
16
2
22
MMFD: modified midfacial degloving. ITF: infratemporal fossa Fisch C approach.
Table 2 – Data regarding patients, lesions, treatment, approach and complications Patient no.
1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
18
19 20
21
22
Age Location Nasopharynx Nasal cavity Maxillary sinus Ethmoids Sphenoid sinus Pterygopalatine fossa Infratemporal fossa Orbit Cavernous sinus Middle cranial fossa Tumour stage Prior surgery Prior radiotherapy Preoperative embolization Surgical approach Endoscopic Transnasal Modified midfacial degloving Infratemporal fossa Craniotomy Complications Mild crusting Facial paraesthesia Temporary facial palsy Carotid artery rupture
9
17
11
14
26
13
15
14
10
18
17
12
13
19
15
14
21
14
15 13
17
10
X X
X X
X X
X X
X X
X X
X X X
X X X
X X X
X X X
X X
X
X X
X X X
X
X X
X
X X X
X X X X X X X IV
X X X
X
X X X
X
X X
X X X
X X X
X X X X X
X X X X X X X
X X X
X X X X X
X X X X X X X X X X III X
a
III Xa
II
II
X
II
X X X X X III
II
X
X
X
X
X
X
X
X
X
X
II
X X IV
X
II
X
X
X X X
X X X X
X X III
X X IV
III
III
IV X
X X
X
X
X
X
X
X
X
X
X X X
X X
X
X X
X
X
X
X X
X
X
X
X
X
X X X
X
X X
X X X
X
X II
I
II
I
I
X X
X
X X
X
X
X
IV Xa X
X X
X
X X
Previous operation with lateral rhinotomy.
pseudocapsule of fibrous tissue produced by compression of the surrounding (normal) tissue. The blood vessels of these highly vascularized tumours lack a complete muscular layer. This can lead to difficulty in controlling bleeding (Standefer et al., 1983). The vascularization is achieved most commonly by the internal maxillary artery but also by the internal or external carotid, the common carotid artery and/or finally the ascending pharyngeal artery (Jacobsson et al.,1988). These tumours can grow anteriorly into the nasal cavity, superiorly into the sphenoid bone or sella, or laterally through the sphenopalatine foramen into the pterygopalatine fossa (Mitskavich et al., 1998). An intracranial extension is found in about 10–20% (Standefer et al., 1983). This intracranial extension can occur from the infratemporal fossa through the floor of the middle cranial fossa, from the pterygomaxillary fissure along the foramen rotundum into
the cavernous sinus, from the sphenoid sinus via the superior wall into the cavernous sinus and/or pituitary fossa, and from the pterygopalatine fossa through the vidian canal (Mitskavich et al., 1998). The most common presenting symptoms are unilateral nasal obstruction and epistaxis (Fagan et al., 1997). Other symptoms include conductive hearing loss, swelling of the cheek, and dacryocystitis (Jamal, 1994). The diagnosis is based on case history, physical examination, and radiological study. Plain radiographs of the nasopharynx and sinuses are not diagnostic. Nasal endoscopy, CT scan and MRI are helpful to delineate the exact location of the lesion, to show any intracranial or infratemporal spread, and to determine the relationship between the tumour and vital structures (Jamal, 1994; Hendrix et al., 1990). Digital subtraction angiography can demonstrate the blood supply, which is variable. The principal aims of the preoperative work-up are to establish a correct
ARTICLE IN PRESS 6 Journal of Cranio-Maxillofacial Surgery
Fig. 2 – Right internal carotid A–P arteriograms of patient 2. (A) Filling of pseudoaneurysm located in subpetrosal segment (arrow). (B) Occlusion of the pseudoaneurysm achieved after placement of covered stent to the affected segment.
diagnosis, to select the optimal surgical approach, and to be prepared for the intraoperative blood loss. Superselective angiography with embolization is performed within two days preoperatively. The biopsy is not recommended unless the diagnosis cannot be achieved with CT or angiography (Jamal, 1994). The tumour usually regresses when the patient reaches the ages of 20–25 years, but complete regression does not occur in every patient. Mishra et al. (1989) and Neel et al. (1973) stated that spontaneous regression is only valuable for residual tumours. Surgery is the mainstay of the treatment even though occasionally it can become quite challenging. The endoscopic transnasal approach is usually preferred for small tumours confined to nasopharynx, nasal cavity, ethmoid and sphenoid sinuses. Nicolai et al. (2003) stated that this approach is also valuable for intermediate sized lesions. It carries the advantage of minimal soft-tissue and bony disruption. The disadvantages with this technique are restricted access and loss of visibility if bleeding
occurs during surgery. The transnasal approach can be used for tumours limited to the nasopharynx, nasal cavity and sphenoid sinus but lateral exposure is very limited with this technique. According to Mann et al. (2004), contraindications for the endonasal approach are stage IV angiofibromas and some stage III cases with major extension into the middle cranial fossa. In the group of patients described here, the transnasal approach was chosen in four cases; three of them were stage I tumours, and the other was a stage II tumour. We believe that this approach can be used in combination with other techniques as an auxiliary tool for more advanced tumours. The Le Fort I maxillotomy approach affords access to tumours limited to the nasopharynx, nasal cavity, paranasal sinuses, pterygopalatine fossa, and to tumours with minor extensions into the infratemporal fossa. Tumours in the nasopharynx, orbit, ethmoids, sphenoid sinus, pterygopalatine fossa, infratemporal fossa, and the medial part of the cavernous sinus can be reached via a medial maxillotomy. The latter can be performed through a lateral rhinotomy, or Weber–Ferguson approach, or by midfacial degloving. Lateral rhinotomy plus Weber–Ferguson approach has the disadvantage to leave a scar in the face, this inconvenience must be considered. The infratemporal approach is best suited for tumours extending to the infratemporal fossa, and for tumours involving the middle cranial fossa and the lateral part of the cavernous sinus. This approach permits ligation of the internal maxillary artery early in the dissection thus limiting intra-operative blood loss. The facial translocation approach provides maximal exposure of the nasopharynx, sphenoid sinus, pterygopalatine fossa, and cavernous sinus. However, it may affect facial growth due to extensive soft-tissue dissection and multiple osteotomies. The facial translocation approach was not used in any of these patients. 18 patients were operated upon via the MMFD which is our favourite approach. Incisions through the gingivobuccal sulcus, septal transfixation, intercartilaginous, circumferential vestibular and pyriform aperture areas are performed for the standard midfacial degloving approach. This carries the advantage of an excellent exposure of the lesion and direct control of the internal maxillary artery in the pterygopalatine fossa and it provides a very satisfactory cosmetic outcome (Fig. 3; Howard and Lund, 1992). Possible immediate complications of this procedure are haemorrhage and paraesthesia of the overlying skin. Crusting of the cavity is frequent but usually resolves within three months. Other complications include oro-antral fistula, epiphora and vestibular stenosis. Several modifications of this technique have been described in order to avoid vestibular stenosis. In the techniques described by Buchwald et al. (1995), and Krause and Jafek (1999), the intercartilaginous incision and circumferential vestibular incisions are
ARTICLE IN PRESS Surgical approaches to juvenile nasopharyngeal angiofibroma 7
possibility of incomplete tumour removal (Mann et al., 2004). The recurrence rate is reported to be between 6% and 27.5% (Zhang et al., 1998; Herman et al., 1999). We had to perform revision surgery in four out of 22 cases. This was probably due to incomplete resection.
CONCLUSION
Fig. 3 – Postoperative view of patient no. 13 whose tumour was resected using modified midfacial degloving. Note that there is no cosmetic deformity.
not performed, and the nasal pyramid is mobilized by lateral osteotomies. Jeon et al. (2003) reported the technique of hemifacial degloving approach involving a gingivobuccal incision, pyriform aperture incision and one sided lateral osteotomy which may be relevant for unilateral lesions. In this department neither rhinoplasty incisions, nor lateral osteotomies were performed. The septum was detached from the maxillopalatine crest using a chisel following extended gingivobuccal incision. The anterior and medial bony walls of one or both maxillary sinuses were removed, and a quite wide exposure was obtained. These bony walls were not reconstructed. The complications encountered with this technique were mild crusting and facial paraesthesia. This approach of MMFD provides a wide exposure in almost every case, if necessary. It can be combined with facial translocation or transcranial techniques when there is intracranial extension. MMFD shortens the operative time, and provides good postoperative healing. As the mucosa in the nasal cavity and turbinates are often left intact, less crusting is encountered. Facial translocation could be more relevant functionally for advanced cases. But this approach requires a longer surgical time and specific material for reconstruction. Interdisciplinary cooperation among experienced otolaryngologists, maxillofacial surgeons, neurosurgeons, neuroradiologists, and anesthesiologists is extremely important. This was obvious in the case with carotid artery rupture treated with a cover stent by the neuroradiologists. Preoperative embolization had been performed in 13 patients. The purpose of embolization was not simply to obstruct the feeder artery, but rather to provoke progressive development of thrombosis in the small distal vessels of the tumour (Standefer et al., 1983). It is believed that this procedure substantially reduces the need for blood transfusion. However, massive operative bleeding can still occur despite embolization. On the other hand, it has been reported that embolization can increase the
Surgery is the treatment of choice for juvenile nasopharyngeal angiofibroma. Early diagnosis and preoperative embolization are important adjuncts. The most appropriate surgical route must be determined considering size and location of the tumour, extension to adjacent structures, and experience of the surgical team. In this department, the treatment consists of endoscopic transnasal approach for early stage lesions, and modified midfacial degloving for almost all of the advanced lesions. The latter approach proved helpful regarding exposure, duration of surgery, cosmetic outcome, and morbidity. It can be combined with an infratemporal approach or craniotomy if necessary. References Buchwald C, Bonding P, Kirkby B, Fallentin E: Modified midfacial degloving: a practical approach to extensive bilateral benign tumours of the nasal cavity and paranasal sinuses. Rhinology 33: 39–42, 1995 Fagan JJ, Snyderman CH, Carrau RL, Janecka IP: Nasopharyngeal angiofibromas: selecting a surgical approach. Head Neck 19: 391–399, 1997 Fisch U: The infratemporal fossa approach for nasopharyngeal tumours. Laryngoscope 93: 36–44, 1983 Girgis IH, Fahmy SA: Nasopharyngeal fibroma: its histopathological nature. J Laryngol Otol 87: 1107–1123, 1973 Hendrix RA, Lenkinski RE, Vogele K, Bloch P, McKenna WG: 31P localized magnetic resonance spectroscopy of head and neck tumours—preliminary findings. Otolaryngol Head Neck Surg 103: 775–783, 1990 Herman P, Lot G, Chapot R, Salvan D, Huy PT: Long-term follow-up of juvenile nasopharyngeal angiofibromas: analysis of recurrences. Laryngoscope 109: 140–147, 1999 Howard DJ, Lund VJ: The midfacial degloving approach to sinonasal disease. J Laryngol Otol 106: 1059–1062, 1992 Jacobsson M, Petruson B, Svendsen P, Berthelsen B: Juvenile nasopharyngeal angiofibroma: a report of eighteen cases. Acta Otolaryngol 105: 132–139, 1988 Jamal MN: Imaging and management of angiofibroma. Eur Arch Otorhinolaryngol 251: 241–245, 1994 Jeon SY, Jeong JH, Kim HS, Ahn SK, Kim JP: Hemifacial degloving approach for medial maxillectomy: a modification of midfacial degloving approach. Laryngoscope 113: 754–756, 2003 Krause GE, Jafek BW: A modification of the midface degloving technique. Laryngoscope 109: 1781–1784, 1999 Mann WJ, Jecker P, Amedee RG: Juvenile angiofibromas: changing surgical concept over the last 20 years. Laryngoscope 114: 291–293, 2004 Mishra SC, Shukla GK, Bhatia N, Pant MC: A rational classification of angiofibromas of the postnasal space. J Laryngol Otol 103: 912–916, 1989 Mitskavich MT, Carrau RL, Snyderman CH, Weissman JL, Fagan JJ: Intranasal endoscopic excision of a juvenile angiofibroma. Auris Nasus Larynx 25: 39–44, 1998
ARTICLE IN PRESS 8 Journal of Cranio-Maxillofacial Surgery Neel III. HB, Whicker JH, Devine KD, Weiland LH: Juvenile angiofibroma. Review of 120 cases. Am J Surg 126: 547–556, 1973 Nicolai P, Berlucchi M, Tomenzoli D, Cappiello J, Trimarchi M, Maroldi R, Battaglia G, Antonelli AR: Endoscopic surgery for juvenile angiofibroma: when and how. Laryngoscope 113: 775–782, 2003 Standefer J, Holt GR, Brown Jr. WE, Gates GA: Combined intracranial and extracranial excision of nasopharyngeal angiofibroma. Laryngoscope 93: 772–779, 1983 Stansbie JM, Phelps PD: Involution of residual juvenile nasopharyngeal angiofibroma (a case report). J Laryngol Otol 100: 599–603, 1986 Zhang M, Garvis W, Linder T, Fisch U: Update on the infratemporal fossa approaches to nasopharyngeal angiofibroma. Laryngoscope 108: 1717–1723, 1998
M. Gu¨ven GU¨VENC¸ Og˘uzhan Cad. Ug˘urpalas Ap. No: 21/5 34270 Fındıkzade/Istanbul Turkey Tel.: +90 212 5234344 Fax: +90 212 4143408 E-mail: [email protected] Paper received 1 February 2005 Accepted 18 August 2005