Authors:   S.K. Pippal*, Madhu Khare**,  Yashveer B.**
*Professor and Head, Department of ENT and Head & Neck Surgery,  **Resident

Institution:  Gandhi Medical College & Hamidia Hospital, Bhopal (M.P.), India

Corresponding Author: 

Prof. Dr. S.K. Pippal (M.S.),
Professor & Head Department of ENT,
Gandhi Medical College & Hamidia Hospital
Bhopal (M.P.), India
E mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

Abstract:

Juvenile nasopharyngeal angiofibroma is a relatively uncommon, benign tumor occurring most often in adolescent males, presenting with spontaneous recurrent epistaxis.  A study was carried out over a period of 2 years in the Department of Otorhinolaryngology and Head Neck Surgery, Gandhi Medical College, Bhopal.  Thirteen cases were reviewed.  Clinical, radiological presentation and surgical management of these 13 patients were studied. Most of the patients in our study had undergone transpalatine excision.  In conclusion, we have found that the transpalatine approach has given good results with minimum morbidity and mortality.

 
Introduction:

Juvenile nasopharyngeal angiofibroma is a relatively uncommon, benign vascular neoplasm accounting for less than 0.5%(1,4) of all head and neck tumors.  It occurs almost exclusively in adolescent males.  It is highly vascular, slow growing but locally invasive, and a destructive tumor.  It arises mainly from the superior margin of the sphenopalatine foramen at the junction of the pterygoid process of the sphenoid bone and sphenoid process of the palatine bone.   It may extend to the nasal cavity, paranasal sinuses, pterygopalatine and infratemporal fossa leading to various symptoms such as recurrent epistaxis, nasal obstruction, anosmia, nasal discharge, rhinolalia clausa, proptosis and facial deformity.

Diagnosis of JNA is essentially based on careful history and nasal endoscopic examination, supplemented by CT scan and MRI to evaluate tumor extent, bone erosion and soft tissue involvement.  Various treatment options for JNA include surgical excision, radiotherapy, chemotherapy and hormonal therapy. Treatment of choice in the vast majority of cases is surgical excision, other modalities being reserved for recurrence and residual tumors.  Several surgical approaches have been described for large tumors, including transpalatal, lateral rhinotomy, midfacial degloving and endoscopic removal. Pre- operative selective arterial embolization of the feeding vessel significantly decreases intraoperative blood loss and facilitates resection of larger tumors.

Methods:

This study was carried out in the Department of Otorhinolaryngology and Head Neck Surgery, Gandhi Medical College, Bhopal. A total of 13 cases were reviewed during the period of 2 years from January 2009 to January 2011.  Cases were evaluated clinically and radiologically.  All the patients underwent contrast enhanced CT for initial assessment and to know the exact extent of the tumor (Fig.6, 7).  They were classified according to Fisch Staging. Out of 13 patients, 11 patients underwent primary surgical resection and two were treated for recurrence following primary surgery elsewhere.  Intraoperative blood loss was evaluated and patients were transfused whole blood intra and postoperatively depending upon the amount of blood lost. Average duration of the hospital stay was assessed.  All the patients were kept in followup for postoperative assessment and recurrence. Operated specimens were sent for histopathological examination for confirmation.

Results:

In our study, most of the patients were in the age group of 15- 20 years (62%).(Fig.1)  Out of 13 patienrts, 12 were male patients (92.5%) and 1 female patient was diagnosed as JNA following histopathological examination.(Fig.2) - Click to Enlarge

Spontaneous recurrent epistaxis was the most common presentation of JNA, seen in 100% of the cases, followed by nasal obstruction (77%), anosmia, denasal voice and snoring (54%) each.(Fig.3, 4) At the time of diagnosis, 70% of the patients were at stage II, 23% stage III and 7% were stage I of Fisch classification(9).(Fig.5)

All 13 patients underwent surgical resection. Transpalatine excision was done in 11 (85 %) patients, followed by one lateral rhinotomy and one endoscopic removal.(Fig.8) Intra-operative blood loss was 300-600 ml in 62% patients, 23% had more than 600 ml and 15% had up to 300 ml blood loss.(Fig.12) The average duration of the hospital stay was 12.5(range 7-14)days in 62% of the patients.(Fig.13) 32% stayed for more than 14 days. It is noteworthy that no major complication occurred in this group of patients as a consequence of surgery for primary tumors. There was no perioperative or postoperative mortality.

Click on Figures to Enlarge

Click on Figures to Enlarge

Discussion:

The clinical presentation and data reviewed from this study are comparable to studies done previously.  The typical case presentation is of an adolescent male with an average age of 18 years presenting with spontaneous, recurrent epistaxis and nasal obstruction(1,2,3,4).

Diagnosis of JNA is essentially based on careful history, diagnostic nasal endoscopy (DNE), and supplemented by imaging studies. Biopsy to establish histological diagnosis, preoperatively, is contraindicated except when the etiology is unknown.

Surgery is the recommended treatment for JNA (1,2,3,4).  Surgical approach depends on the stage and site of the tumor. In our study, all 13 patients underwent surgical resection.  Stage I tumor was excised via transnasal endoscopic approach. Stage II and stage III tumors were treated by transpalatal resection(7,8). Even those tumors extending to the pterygopalitine fossa and with limited extension to the infratemporal fossa were resected through the transpalatine approach. None of the patients who underwent transpalatine resection developed palatal fistula. as the initial incision was made over the hard palate. The average surgical blood loss was 520ml(1)(300-600 ml).  In the postoperative period, most of the patients stayed in the hospital on average for 12.5 day(1). The patient who was operated via the transnasal endoscopic approach had the shortest hospital stay of 4 days.  Five out of the 13 patients were lost to follow up after 15 months.  Three were declared as disease free after two years of followup and were asked to report yearly. The other five patients are still under followup. Patients in our study neither had any recurrence nor complications in the followup period.

Conclusion:

Surgical resection is the recommended modality of treatment for this highly vascular benign tumor of the nasopharynx. The  availability of different modalities for pre-op reduction in the vascularity of tumors have reduced intraoperative bleeding,  However, management of JNA still poses a great challenge to ENT surgeons worldwide.  In our facility where pre-op embolization is not available, adequate exposure in transpalatine approach gives good results with minimum morbidity and mortality.  Even tumors extending to pterygopalatine fossa and limited extension to infratemporal fossa can be managed through the transpalatine approach in experienced hands.

 
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 View Abstract