International Journal of Pediatric Otorhinolaryngology 75 (2011) 620–623
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Transnasal endoscopic management of recurrent juvenile nasopharyngeal angiofibroma Asser A. El Sharkawy *, Shawky M. Elmorsy Department of Otorhinolaryngology, Mansoura University, Mansoura, Egypt
A R T I C L E I N F O
A B S T R A C T
Article history: Received 16 December 2010 Received in revised form 19 January 2011 Accepted 21 January 2011 Available online 12 March 2011
Objectives: The purpose of this study was to present our experience with definitive endoscopic surgical management of the recurrent juvenile nasopharyngeal angiofibroma. Study design: Retrospective study. Setting: Tertiary care centre. Materials and methods: This study includes 13 male adolescence patients with recurrent nasopharyngeal angiofibroma who received treatment at our centre between 2005 and 2010. The patient age ranged from 12 to 21 years (mean age, 15.7 years). Endoscopic two surgeons’ technique had been used. Follow up MRI every four months. Results: Complete removal of the recurrent tumor was achieved in 10 cases. Three patients had incomplete removal with further recurrences. These recurrences were two in lateral wall of the sphenoid sinus (2 patients), and soft palate (one patient). Conclusion: Recurrent JNAs are residual disease resulting from incomplete removal of the primary tumor. Transnasal endoscopic sinus surgery is an effective method for treating recurrent JNA. Follow up is essential and integral point in management of JNAs. ß 2011 Elsevier Ireland Ltd. All rights reserved.
Keywords: Recurrent juvenile nasopharyngeal angiofibroma Endoscopic surgery
1. Introduction
2. Materials and methods
Juvenile nasopharyngeal angiofibroma (JNA) is a histologically benign yet locally aggressive vascular head and neck tumor [1]. It accounts for only 0.05% of head and neck tumors and affects only young pubescent males [2]. It originates from the superior margin of the sphenopalatine foramen just posterior to the middle turbinate. Though benign, the tumor causes pressure erosion of the bones with spreading into the pterygopalatine and infratemporal fossae [1]. Recurrence of the JNA is a conspicuous feature of the natural history JNA [3]. Frequent and often rapid recurrence has been reported by many authors. Standard therapeutic options for JNAs include surgery or definitive radiotherapy (RT) [4,5]. Nontraditional treatment approaches have included percutaneous sclerotherapy [6], chemotherapy [7], and hormonal manipulation [8], but studies have shown that surgery and/or RT are the most efficacious treatment modalities [4]. The purpose of this study was to present our experience with definitive endoscopic surgical management of patients with recurrent JNA.
This study included 13 patients with recurrent JNA out of 68 patients who had surgical treatment at our department between 2005 and 2010 .The patients’ age ranged from 12 to 21 years (mean age, 15.7 years) (Table 1). The patients had been previously treated for primary JNA, 12 patients at our department and one patient elsewhere. The primary surgical treatment consisted of transfacial approaches which included lateral rhinotomy in five patients, and mid facial degloving in four patients. A pure transnasal endoscopic approach was used in four patients (Table 1). All patients were assessed by CT scan and MRI (Figs. 1 and 2). Neither arteriography nor embolization was done for any patient preoperatively. The tumors were staged according to Radkowski et al. classification [9].
* Corresponding author at: ORL Department, Mansoura University Hospital, Gomhoria Street, Mansoura 35516, Egypt. Tel.: +20 102223269; fax: +20 22267016. E-mail address:
[email protected] (A.A. El Sharkawy). 0165-5876/$ – see front matter ß 2011 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2011.01.033
2.1. Surgical techniques The patient was placed in supine position with head elevation of 308. Hypotensive general anesthesia was used with the systolic blood pressure being maintained between 70 and 90 mm Hg. The eyes were not covered by drapes. Both nasal cavities were packed with cotton pledges soaked in saline adrenaline solution in a concentration of 1:80,000. After good decongestion of the nose, the tumor extension in both the nasal cavities and nasopharynx was examined carefully using 4 mm 08 and 308 telescope transnasally. Submucosal injection of adrenaline into the mucosa of the nose,
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Table 1 Patients demographics, surgical approach, duration interval of recurrence, tumor stage, result of surgery and follow up. Case
Age
Duration interval of recurrence
Tumor stage
Primary surgery
Result of rev surgery
Follow up
1 2 3 4 5 6 7 8 9 10 11 12 13
12 16 15 18 14 13 13 16 17 18 13 19 21
4 months 5 months 8 months 9 months 4 months 4 months 13 months 6 months 7 months 4 months 5 months 8 months 10 months
IB IIA IIB IIIA IB IB IIA IIB IIC IIA IIA IB IB
Degl. app Degl. app LR LR Deg app LR ESS ESS ESS Deg app LR ESS LR
Complete removal Complete removal Residual tumor Residual tumor Complete removal Complete removal Complete removal Residual tumor Complete removal Complete removal Complete removal Complete removal Complete removal
36 months 30 months 24 months 22 months 20 months 16 months 18 months 12 months 30 months 6 months 10 months 12 months 9 months
years years years years years years years years years years years years years
the anteroinferior rim of the sphenopalatine foramen were removed with a bone punch to expose the tumor in the pterygopalatine fossa and medial part of infratemporal fossa. These extensions were cauterized and dissected meticulously and any bleeding was controlled with adrenaline soaked patties and bipolar diathermy. This released the tumor at the region of the sphenopalatine foramen and from the surrounding structures. Dissection was then continued posteriorly to free the tumor of its attachments to the roof of the nasopharynx and posterior margin of the septum. After ensuring that the tumor was free of all its attachments it was delivered transorally. Bleeding from the sphenopalatine vessels usually occurred at this point and was controlled with bipolar cauterization. After removal of the main mass, the area was examined endoscopically for any tumor remnants and if present, these were removed. After removal of the tumor, anterior nasal pack was placed in the nasal cavity and removed after three days.
septum or middle turbinate was done in a concentration of 1:80,000 using dental syringe to decrease blood loss. Two surgeons’ hands technique was used in all cases. This technique entails the use of four hands; two of the surgeon and two of the assistant to hold the endoscopes and to aspirate under the monitor to give the surgeon the flexibility to deal with the tumor. In IB, IIA cases (9 patients) there were large cavities of previous operations (lateral rhinotomy, degloving or endoscopic) and the tumor affect only nose, nasopharynx and medial part of the pterygopalatine fossa. Any residual of the middle turbinate was excised after cauterizing and dissecting away the tumor attached to it. This was important so as to obtain a good and wide view of the sphenopalatine foramen especially where there was a lateral extension of the tumor. The tumor attachments to various regions, e.g. the middle turbinate, septum and the roof of the posterior choana were carefully identified cauterized with a bipolar diathermy and then dissected using a 3-ram periosteal elevator. Complete ethmoidectomy was done and the sphenoid sinus entered. Extension into the sphenoid sinus was visualized and dissected after removing part of the anterior and inferior sphenoid sinus wall adjacent to the sphenopalatine foramen. In IIB, IIC and IIIA (4 patients) where there is with lateral spread of the tumor to the pterygopalatine fossa and infratemporal fossa, the maxillary sinus was entered through the posterior fontanelle. The posterior half of the medial and the posterolateral wall of the maxillary sinus, the perpendicular plate of the palatine bone and
[()TD$FIG]
Fig. 1. CT scan coronal cut: recurrent juvenile nasopharyngeal angiofibroma (stage Ia).
Fig. 2. Axial MRI with contrast: recurrent juvenile nasopharyngeal angiofibroma of the nasopharynx, and infratemporal fossa.
[()TD$FIG]
2.2. Control of bleeding Preoperative embolization was not done in any patient preoperatively. Therefore control of bleeding is an integral point in this technique. Control of bleeding was achieved by hypotensive anesthesia, submucosal injection of adrenaline into the nasal mucosa, two surgeons hand technique and continuous aspiration
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and wash of the surgical field by warm saline solution. Warm saline solution has many effects; first clean the operative field from any blood or blood clot, gives excellent visualization of tissues, control of bleeding and antifogging effect of the endoscopes.
recommendations of the ethics committee of the faculty of medicine Mansoura University.
2.3. Follow-up
Recurrence or persistence is well reported in JNA despite its benign histology and absence of multifocal origin. Tumor recurrence is primarily seen with high stage tumors [1,9]. The high rate of recurrence may be due to failure to recognize the skull base erosion [1,10]. Recurrence rate for JNA varies from 17% to 46% [11,12]. More frequent recurrence correlates with extension to the sphenoid sinus, the base of pterygoid, and the clivus bone [13]. Recurrence is a conspicuous of the natural history of JNA. The situation was stated accurately by Lafargue who stated in 1947 ‘‘some JNA do not recur at all while others operated on by the same surgeon, by the same methods and under similar conditions recur with astonishing rapidity’’ [14]. This study has shown that recurrent JNA is a residual disease. This could be explained by all recurrences occurred in the same side of the primary tumor. In addition, most recurrences occurred within the first 6 months after surgery (53.8%) and 92.3% within one year. This agrees with Howard et al. [3] who reported recurrence within the same period of time. Therefore, recurrent JNAs result from incomplete removal of the primary tumor with remaining part of the tumor that undergo regrowth and attain a new blood supply. This rapid regrowth of the JNAs could be explained by Lund et al. [15] who stated that JNA are known to undergo a natural cycle of aggressive growth, followed in some cases by regression. The point in the cycle when surgery is undertaken may be the important determinant of ‘‘recurrence’’, as it clears in some cases that small foci of residual disease do not regrowth whereas others behave in an aggressive fashion. Complete surgical extirpation is the treatment of choice for patients with JNA. The traditional surgical approaches include transoral, transfacial, degloving, transpalatal or craniofacial approach [16]. However, advances in endoscopic surgery have provided a minimally invasive means to gain excellent tumor visualization and resection. The advantages of endoscopic approach include better illumination and a magnified field of view. This can lead to more complete inspection of the resection cavity especially for the tumor fronds which may present in the small cavities in the skull base [17]. The two-surgeon technique described by Robinson et al. [18] used in this study enabled free and rapid excision of the tumor with meticulous control of bleeding. The second surgeon can place traction on the tumor during dissection. In addition, the second surgeon can allow better control of brisk hemorrhage by placing high volume suction into the operative field while the primary surgeon bipolar cauterizes or places a clip on the bleeding vessel [15]. The finger like projections into the cancellous bone of the skull base was believed to be the cause of recurrence, therefore careful exploration of the skull base especially at the region of sphenoid and pterygoid was a critical point in our technique. This agrees with Howard et al. [3] who stated that meticulous exploration of the basisphenoid is mandatory because small lobules of angiofibroma were frequently found to be invaginating into the cancellous bone of the sphenoid which may overlooked and may the cause of recurrence. Attachment of the JNA to the soft tissues of posterior pharyngeal wall (longus coli and longus capitis muscles) soft palate and infratemporal fossa muscles was found in this study. Attachment of the JNA to the soft tissues produces difficult dissection of the tumor from the surrounding and increases the bleeding which may prevent complete removal of the tumor. This soft tissue attachment was very important to be considered during
The patients were instructed to attend the out patient clinic every week for inspection of the nasal cavity and cleaning any debris in the cavity. Endoscopic examination of the operative field was done every 2 weeks for the first month then every month after ward for the first three months. Patients’ symptoms were followed up especially nasal bleeding, nasal obstruction ear fullness and headache. MRI with contrast was done 4 months postoperative detect any recurrence. The duration of follow up ranged from 6 months to 36 months years with a mean of 19.5 months (Table 1). 3. Results This was a retrospective study. It included 13 male patients suffering from recurrent nasopharyngeal angiofibroma. The age of the patients ranged from 12 to 21 years with a mean of 15.7 years. Patients’ demographic data are shown in Table 1. The indication of surgery in all cases was the recurrence of symptoms mainly recurrent severe epistaxis in 12 patients (92.3%), nasa1 obstruction in 7 patients (53.8%) and ear fullness in one patient (7.7%). Recurrence of the tumor occurred within the first 6 months in 53.8% of patients and within one year in 92.3% of patients (Table 1). Complete removal of the tumor was achieved in 9 cases with no residual or recurrent tumor or symptoms with average follow up 6–36 months with a mean of 19.5 months. Three cases had residual tumors; one in the soft palate and two in the medial portion of the cavernous sinus. A second operation was needed for two patients with residual tumor in the sphenoid sinus and bony erosion and affection of the medial portion of the cavernous sinus. Severe bleeding occurred with termination of the operation and postoperative radiotherapy was received for the patients. Two patient required insertion of the ventilation tube due to Eustachian tube dysfunction and secretory otitis media (OM). Three cases received postoperative radiotherapy; one for palatal invasion and two for cavernous sinus remnants. One patient developed oroantral fistula after tooth extraction and required surgical closure using buccal bad of fat. 3.1. Mortality and complications No procedure-related mortality was recorded. Complications are shown in Table 2. 3.2. Ethical considerations All procedures including obtaining written informed consents from the patients were conducted in accordance with the Table 2 Mortality and complications. Complication
No of cases (%)
Treatment
Orbital hematoma Nasal bleeding
2(15.4%) 4(30.8%)
Residual tumor Diminusion of vision Oroantral fistula Recurrent dacrycystitis
3(20%) 1(7.7%) 1(7.7%) 1(7.7%)
Conservative treatment Blood transfusion, nasal pack and bipolar diathermy Radiotherapy Megadose of coricosteroid Closure by buccal bad of fat Open DCR
4. Discussion
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revision surgery of JNA as it may be the cause of another recurrence [19]. This concurs with Herman et al. [20] who stated that one of the most important factors that impede complete tumor resection was soft tissue attachment. Although preoperative embolization decreases the bleeding from this vascular tumor plus it decreases the size of the tumor [9]. In this study preoperative embolization was not done. Embolization of the recurrent angiofibroma is a challenge to the radiologist because of disturbed anatomy and vascularity of the tumor after the first interference. In spite of lack of preoperative embolization, control of bleeding was achieved by hypotensive anesthesia, use of bipolar diathermy, and two hand technique. This agrees with ElBanhawy et al. [2] who treated stage III JNA without preoperative embolization. Complete excision of the JNA was achieved in 77% of patients in this study. This was due to meticulous dissection of the tumor from the skull base and proper control of bleeding. Residual tumors were noticed in three cases. First patient had infiltration of the soft palate with difficult complete excision of the tumor. The remaining two patients had infiltration of the medial portion of the cavernous sinus with severe bleeding and incomplete removal of the tumor. These patients had postoperative radiotherapy and do well until now with follow up. Complications of endoscopic sinus surgery have been well characterized (Table 2). Nasal bleeding is a common complaint following endoscopic sinus surgery insertion. In this study, nasal bleeding occurred in 30.8% of cases. The patients were controlled by anterior nasal packs 3 cases and one patient required exploration and bipolar diathermy of the bleeding artery. Orbital complications are possible complications after endoscopic sinus surgery and its incidence is 3 cases (2 orbital haematomas and one diminusion of vision). This diminusion of vision was attributed to nerve edema from thermal effect of extensive cauterization. The patient was managed by mega dose of steroids with marked improvement of degree of vision from hand movement to 6/16. One of the limitations of this study is short follow up period of less than 12 months for 5 patients. 5. Conclusion Recurrent JNAs are residual disease resulting from incomplete removal of the primary tumor. Transnasal endoscopic sinus surgery is an effective method for treating recurrent JNA. It offers good illumination and visualization of the tumor. Also, it gives a direct approach to tumor. Adequate control of intraoperative bleeding, meticulous operative procedures are the keys for complete excision of the tumor. Two surgeons’ hand technique is a reliable technique in endoscopic sinus surgery. Follow up is essential and integral point in management of JNAs.
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Source of funding No source of fund. Conflict of interest No conflict of interest. References [1] I. Yiotakis, A. Eleftheriadou, D. Davilis, E. Giotakis, E. Ferekidou, S. Korres, et al., Juvenile nasopharyngeal angiofibroma stages I and II: a comparative study of surgical approaches, Int. J. Pediatr. Otorhinolaryngol. 72 (2008) 793–800. [2] O.A. El-Banhawy, A. Ragab, M.M. El-Sharnoby, Surgical resection of type III juvenile angiofibroma without preoperative embolization, Int. J. Pediatr. Otorhinolaryngol. 68 (2004) 21–28. [3] D. Howard, G. Lloyd, V. Lund, Recurrence and its avoidance in juvenile angiofibroma, Laryngoscope 111 (2001) 1509–1511. [4] J.T. Lee, P. Chen, A. Safa, G. Juillard, T.C. Calcaterra, The role of radiation in the treatment of advanced juvenile angiofibroma, Laryngoscope 112 (2002) 1213– 1220. [5] J.G. Spector, Management of juvenile angiofibromata, Laryngoscope 98 (1988) 1016–1026. [6] W.L. Chen, Z.Q. Huang, J.S. Li, Q. Chai, D.M. Zhang, Percutaneous sclerotherapy of juvenile nasopharyngeal angiofibroma using fibrin glue combined with OK-432 and bleomycin, Int. J. Pediatr. Otorhinolaryngol. 74 (2010) 422–425. [7] H. Goepfert, A. Cangir, Y.Y. Lee, Chemotherapy for aggressive juvenile nasopharyngeal angiofibroma, Arch. Otolaryngol. 111 (1985) 285–289. [8] A. Labra, R. Chavoll-Magana, A. Lopez-Ugalde, J. Alanis- Calderon, A. HuertaDelgado, Flutamide as a preoperative treatment in juvenile angiofibroma (JA) with intracranial invasion: report of 7 cases, Otolaryngol. Head Neck Surg. 130 (2004) 466–469. [9] D. Radkowski, T. McGill, G.B. Healy, Angiofibroma: changes in staging and treatment, Arch. Otolaryngol. Head Neck Surg. 122 (2) (1996) 122–129. [10] G. Danesi, D.T. Panciera, R.J. Harvey, C. Agostinis, Juvenile nasopharyngeal angiofibroma: evaluation and surgical management of advanced disease, Otolaryngol. Head Neck Surg. 138 (2008) 581–586. [11] G.C. Jones, L.W. DeSanto, J.W. Bremer, H.B. Neel, Juvenile angiofibromas: behaviour and treatment of extensive and residual tumors, Arch. Otolaryngol. Head Neck Surg. 112 (1986) 1191–1193. [12] R.A. Harma, Nasopharyngeal angiofibroma, Acta Otolaryngol. 49 (Suppl. 146) (1958) 7–74. [13] B.S. Bleier, D.W. Kennedy, J.N. Palmer, A.G. Chiu, J.D. Bloom, B.W. O’Malley Jr., Current management of juvenile nasopharyngeal angiofibroma: a tertiary center experience 1999–2007, Am. J. Rhinol. Allergy 23 (2009) 328–330. [14] B. Lafargue, Considerations sur les fibrose nasopharyngiens etude anatomicoclinique, Rev. Laryngol. 68 (1947) 436–465. [15] V. Lund, G.A.S. Lloyd, D.J. Howard, Juvenile angiofibroma – imaging techniques in diagnosis, Rhinology 27 (1989) 179–185. [16] G. Gallia, M. Ramanathan, A.M. Blitz, D.D. Reh, Expanded endonasal endoscopic approach for resection of a juvenile nasopharyngeal angiofibroma with skull base involvement, J. Clin. Neurosci. 17 (2010) 1423–1427. [17] R.H. Kamel, Transnasal endoscopic surgery in juvenile nasopharyngeal angiofibroma, J. Laryngol. Otol. 110 (1996) 962–968. [18] S. Robinson, N. Patel, P.J. Wormald, Endoscopic management of benign tumors extending into the infratemporal fossa: a two surgeons transnasal approach, Laryngoscope 115 (2005) 1818–1822. [19] P.J. Wormald, S. Robinson, Transnasal endoscopic resection of benign tumors occurring from or extending into the infratemporal fossa, Oper. Tech. Otolaryngol. 17 (2006) 97–101. [20] P. Herman, G. Lot, R. Chapot, D. Salvan, P.T. Huy, Long term follow up of juvenile nasopharyngeal angiofibroma: analysis of recurrence, Laryngoscope 109 (1999) 140–146.
