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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 19  |  Issue : 4  |  Page : 366-369
Extended osteoplastic maxillotomy for total excision of giant multicompartmental juvenile nasopharyngeal angiofibroma

1 Department of Dental and Oral Surgery, Christian Medical College, Vellore, India
2 Department of Otorhinolaryngology, Christian Medical College, Vellore, India
3 Department of Plastic and Reconstructive Surgery, Christian Medical College, Vellore, India
4 Department of Neurological Sciences, Christian Medical College, Vellore, India

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Date of Submission27-Feb-2008
Date of Decision20-Jun-2008
Date of Acceptance06-Jul-2008


Juvenile nasopharyngeal angiofibroma (JNA) is a rare vascular neoplasm occurring almost exclusively in adolescent males. Although benign, it is often locally aggressive and can erode into surrounding tissues and structures resulting in significant morbidity and mortality. In 20% of cases, there is intracranial extension. In this paper, we report on the total excision of a large, recurrent JNA with intracranial extension into the middle cranial fossa encroaching into the cavernous sinus, by right temporal craniotomy and extended osteoplastic maxillotomy.

Keywords: Extended osteoplastic maxillotomy, juvenile nasopharyngeal angiofibroma

How to cite this article:
Koshy S, George M, Gupta A, Daniel RT. Extended osteoplastic maxillotomy for total excision of giant multicompartmental juvenile nasopharyngeal angiofibroma. Indian J Dent Res 2008;19:366-9

How to cite this URL:
Koshy S, George M, Gupta A, Daniel RT. Extended osteoplastic maxillotomy for total excision of giant multicompartmental juvenile nasopharyngeal angiofibroma. Indian J Dent Res [serial online] 2008 [cited 2023 Mar 22];19:366-9. Available from:
Juvenile nasopharyngeal angiofibroma (JNA) is a benign, vascular tumor occurring almost exclusively in pre-pubescent or pubescent males. The triad of epistaxis, nasal obstruction, and a nasopharyngeal mass are characteristic of the lesion, especially when seen in an adolescent male. The incidence of JNA has been stated to be as low as one in 50,000 new otolaryngological patients, accounting for less than 0.05% of all head and neck tumors. Recent reports, however, suggest that the tumor may be more common in the Indian subcontinent than reported in the West. [1] Intracranial extension has been observed in 20-30% patients with JNA, [2],[3],[3],[4] and this is thought to represent advanced disease.

Though histologically a benign tumor, JNA can become life-threatening due to excessive bleeding or intracranial extension. It is locally aggressive, eroding adjacent bone and growing through natural foramina and fissures, thus gaining easy access into the cranium and the infratemporal region. Intracranial extension usually occurs either through erosion of the sphenoid sinus through the sella medial to the carotid artery and lateral to the pituitary gland, or via erosion of the greater wing of the sphenoid through the middle cranial fossa anterior to the foramen lacerum and lateral to the cavernous sinus and artery.

In this paper, we report on the total excision of a large recurrent JNA with intracranial extension into the middle cranial fossa encroaching into the cavernous sinus, by right temporal craniotomy and extended osteoplastic maxillotomy.

   Case Report Top

A 15-year-old boy presented with right nasal obstruction and intermittent right nasal epistaxis for the past one year. On examination, there was pallor. There was mild proptosis of the right eye. Eye movements were normal, there was no nystagmus. Pupils were equal and reacting to light. Fundi were normal. Examinations of the other cranial nerves were normal. Motor and sensory examination was normal. There were no signs of cerebellar dysfunction or meningeal irritation. Anterior rhinoscopy showed a large reddish lobulated mass in the right nasal cavity, extending into the oropharynx behind the palate to about 2 cm. This was confirmed by nasal endoscopy.

Axial, coronal, and sagittal views on contrast magnetic resonance imaging revealed a large well-defined moderately enhancing nasopharyngeal mass extending into the right nasal cavity with widening of the pterygopalatine fossa laterally, into the sphenoidal sinus, right optic canal, and middle cranial fossa encroaching on the posterior and medial aspect of the right cavernous sinus superiorly, and into the oropharynx inferiorly [Figure 1].

A diagnosis of JNA with intracranial extension was made and the patient was advised surgery. He underwent bilateral arterial embolization, followed in a few hours by right temporal craniotomy, extended osteoplastic maxillotomy, and total excision of the tumor. Skin incisions for the extended osteoplastic maxillotomy included a standard Weber Ferguson and a subciliary incision, a frontotemporal, and a transverse communicating incision that merged with the previous lower lid incision. The midline lip incision was taken vertically through the attached mucosa/gingiva between the central incisors. The palatal incision was extended posteriorly till the junction of the soft and hard palates. A joining transverse mucosal incision was then made, approximately 5 mm superior to the junction of the hard and soft palates. Bone cuts were made with fissure burrs and completed with osteotomes. Osteotomies performed through the anterior maxillary alveolus between the teeth were continued paramedially through the length of the hard palate into the nasal floor, laterally from the piriform fossa to the infraorbital rim, and then continued along the floor of the orbit posterior to the inferior orbital rim, with care not to section the infraorbital nerve. The cut was extended toward the lateral orbital rim below the frontozygomatic suture. The zygomatic arch was sectioned where it joined the body of the zygoma [Figure 2]. The maxilla was separated from the pterygoid plates with a curved pterygoid chisel. The maxilla along with the zygomatic complex was then outfractured and pedicled to the soft tissue. This provided an excellent approach to the central skull base facilitating en-bloc resection of the tumor. Reconstruction of cranial base defects was achieved using a pedicled temporalis muscle flap. While closing, the osteoplastic unit was positioned by the prefabricated splint and fixation was done over the 4-point areas.

Postoperatively, the patient was managed with a lumbar subarachnoid drain and bed rest. Other than mild sixth nerve paresis on the right, there were no other complications encountered during the postoperative course. Biopsy confirmed the diagnosis of JNA. The patient remains disease-free after one year.

   Discussion Top

JNA is a locally aggressive benign vascular tumor. These tumors are most likely androgen dependent, explaining their almost exclusive presentation in males, and are thought to arise from a residual vascular plexus from the involution of the first branchial arch artery. [5] The initial symptoms characteristically include nasal obstruction, epistaxis, pain, nasal discharge, and hearing impairment. [6] The diagnosis and assessment of tumor extension are made on the basis of clinical symptoms and radiographic investigations including CT and angiography.

There are several systems of classification for JNA that are in current common use [7] [Table 1]. For tumors with intracranial extension, surgical excision is recommended with or without additional radiation and hormonal therapy.

Several surgical approaches have been established for the excision of small, localized extracranial JNAs, including pre-operative arterial embolization, lateral rhinotomy, transpalatal, craniofacial, and midface degloving procedures, and more recently, less invasive endonasal approaches. [8] Opinion, however, varies on the management of more aggressive, advanced tumors with intracranial extension. [9] Treatment options in such cases include surgery and irradiation. Tumors invading the cavernous sinus have generally been considered inoperable because of the high likelihood of bleeding from the cavernous venous plexus or potential injury to the internal carotid artery or one of the cranial nerves. In addition, surgical defects in the dura surrounding the cavernous sinus can be difficult to repair, leading to the possibility of an uncontrollable cerebrospinal fluid leak. Based largely on these concerns, most surgeons addressing the treatment of intracranial angiofibromas have advocated either nonsurgical treatment or incomplete resection of the tumor.

The extended osteoplastic maxillotomy [10],[11],[12],[13] uses a combined maxillofacial, otolaryngological, and neurosurgical approach in order to assess the extent of the tumor and to isolate feeding vessels. This method maintains the vascularity to the maxillofacial skeleton, unlike the facial translocation procedure. It provides greater exposure of the skull base and intracranial structures compared to the infratemporal fossa approaches, the subtotal maxillectomy, the mandibular swing, and the pre-auricular temporal-zygomatic approaches. Unlike the transotic approaches, this procedure preserves middle and inner ear and facial nerve function. Exclusive endoscopic approaches have been used in tumors extending up to the medial aspect of infratemporal fossa and retromaxillary space. [14],[15] The osteoplastic maxillotomy allows for restoration of form and function to the maxilla and potential for reconstruction of the cranial base. The disadvantages of the procedure include midfacial incisions, the potential for lower eyelid ectropion, and contamination of the surgical site by the oral cavity. [10] In this particular case, despite the extensive tumor, the patient had no symptoms that suggested intracranial or cavernous sinus involvement. The surgical approach used allowed en block excision of the tumor.

   Conclusion Top

The extended osteoplastic maxillotomy is an approach that provides extensive exposure of the central skull base and infratemporal fossa. Surgical access is achieved through the mobilization of an osteoplastic unit. The surgical approach used allows en block excision of the tumor. The procedure allows the preservation of vascularity to the maxillofacial skeleton as well as hearing and facial nerve functions. Facial incisions have satisfactory cosmesis compared with alternative surgical approaches, and morbidity associated with the procedure is low.

   References Top

1.Biswas D, Saha S, Bera SP. Relative distribution of the tumours of ear, nose and throat in the paediatric patients. Int J Pediatr Otorhinolaryngol 2007;71:801-5.   Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Jafek BW, Krekorian EA, Kirsch WM, Wood RP. Juvenile nasopharyngeal angiofibroma: Management of intracranial extension. Head 1979;2:119-28.   Back to cited text no. 2    
3.Cummings BJ, Blend R, Keane T, Fitzpatrick P, Beale F, Clark R, et al . Primary radiation therapy for juvenile nasopharyngeal angiofibroma. Laryngoscope 1984;94:1599-605.   Back to cited text no. 3  [PUBMED]  
4.Mannai C, Schwartz HC. Juvenile nasopharyngeal angiofibroma presenting as a facial swelling: A case report. Maxillofac Surg 1986;14:329-31.  Back to cited text no. 4    
5.Schick B, Urbschat S. New aspects of pathogenesis of juvenile angiofibroma. Hosp Med 2004;65:269-73.  Back to cited text no. 5  [PUBMED]  
6.Jacobsson M, Petruson B, Svendsen P, Berthelsen B. Juvenile nasopharyngeal angiofibroma: A report of eighteen cases. Acta Otolaryngol 1988;105:132-9.  Back to cited text no. 6  [PUBMED]  
7.Marshall AH, Bradley PJ. Management dilemmas in the treatment and follow-up of advanced juvenile nasopharyngeal angiofibroma. J Otorhinolaryngol Relat Spec 2006;68:273-8.  Back to cited text no. 7    
8.Mann WJ, Jecker P, Amedee RG. Juvenile angiofibromas: Changing surgical concept over the last 20 years. Laryngoscope 2004;114:291-3.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]
9.Bales C, Kotapka M, Loevner LA, Al-Rawi M, Weinstein G, Hurst R, et al . Craniofacial resection of advanced juvenile nasopharyngeal angiofibroma. Arch Otolaryngol Head Neck Surg 2002;128:1071-8.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]
10.Catalano PJ, Biller HF. Extended osteoplastic maxillotomy: A versatile new procedure for wide access to the central skull base and infratemporal fossa. Arch Otolaryngol Head Neck Surg 1993;119:394-400.  Back to cited text no. 10  [PUBMED]  
11.Marks P. The maxillofacial /neurosurgical team approach. In: Langdon JD, Patel MF, editors. Operative Maxillofacial Surgery. UK: Chapman and Hall Medical; 1998. p. 437-44.   Back to cited text no. 11    
12.Carrillo JF, Celis MA, Ramirez-Ortega M, Rivas B, Ochoa FJ. Osteoplastic maxillotomy for treatment of neoplasms of the nasopharynx and infratemporal fossa. Ann Otol Rhinol Laryngol 2005;114:58-64.  Back to cited text no. 12  [PUBMED]  
13.Karkas AA, Schmerber SA, Bettega GV, Reyt EP, Righini CA. Osteoplastic maxillotomy approach for infraorbital nerve schwannoma: A case report. Head Neck 2008;30:401-4.  Back to cited text no. 13  [PUBMED]  [FULLTEXT]
14.Roger G, Tran Ba Huy P, Froehlich P, Van Den Abbeele T, Klossek JM, Serrano E, et al . Exclusively endoscopic removal of juvenile nasopharyngeal angiofibroma: Trends and limits. Arch Otolaryngol Head Neck Surg 2002;128:928-35.  Back to cited text no. 14  [PUBMED]  [FULLTEXT]
15.Hofmann T, Bernal-Sprekelsen M, Koele W, Reittner P, Klein E, Stammberger H. Endoscopic resection of juvenile angiofibromas - long term results. Rhinology 2005;43:282-90.  Back to cited text no. 15  [PUBMED]  

Correspondence Address:
Santosh Koshy
Department of Dental and Oral Surgery, Christian Medical College, Vellore
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0970-9290.44526

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  [Figure 1], [Figure 2]

  [Table 1]

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