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Juvenile nasopharyngeal angiofibroma (JNA) are locally growing highly vascular tumours treated primarily by surgical excision (open approach as wide as a mid facial degloving or endoscopic approach). All our patients underwent exclusive endoscopic tumour excision after a pre-operative embolisation. The tumours were completely resected with acceptable blood loss and no recurrences or residual masses were seen. Post-operative morbidity was minimal without external scar-marks. To conclude endoscopic excision is a very effective method to resect JNA even for extensive tumours.
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ORIGINAL ARTICLE
Endoscopic Approach to Fisch Stage II to III-b Juvenile
Nasopharyngeal Angiofibroma
Brajendra Baser Shenal Kothari Arvind Kinger
Received: 25 August 2010 / Accepted: 10 October 2010 / Published online: 13 January 2011
ÓAssociation of Otolaryngologists of India 2011
Abstract Juvenile nasopharyngeal angiofibroma (JNA)
are locally growing highly vascular tumours treated pri-
marily by surgical excision (open approach as wide as a
mid facial degloving or endoscopic approach). All our
patients underwent exclusive endoscopic tumour excision
after a pre-operative embolisation. The tumours were
completely resected with acceptable blood loss and no
recurrences or residual masses were seen. Post-operative
morbidity was minimal without external scar-marks. To
conclude endoscopic excision is a very effective method to
resect JNA even for extensive tumours.
Keywords Endoscopy Juvenile nasopharyngeal
angiofibroma Intracranial Extradural Antral window
Septal window
Introduction
Juvenile nasopharyngeal angiofibroma (JNA) is a benign
vascular tumour forming about 0.05% of all head and neck
tumours [1]. Grossly, these are pale red to blue smooth
masses often lobulated, non-encapsulated, sessile or
pedunculated arising in pterygopalatine fossa. They grow
along the tissue planes following the path of least resis-
tance and this is of benefit in surgical clearance. Out of
various theories described for their pathogenesis androgen
dependence theory is most accepted.
The patient presents with recurrent epistaxis and nasal
blockage [2] with intranasal mass. Cheek swelling, visual
changes, hearing impairment and neurological deficits may
be present some times.
Various management options for control of JNA,
include radiotherapy, chemotherapy, hormonal therapy,
electrocoagulation and surgery. Surgery remains the
mainstay of treatment [2]. Surgical approaches range from
extensive mid facial degloving to minimally invasive
transnasal endoscopic excision.
The knowledge of wide maxillary antrostomies, anterior
ethmoidectomies, middle turbinate removal, maxillecto-
mies, and accessibility to pterygomaxillary fissure is a pre-
requisite.
Although open approaches give the benefit of wider
exposure high morbidity, more hospitalisation, more
bleeding, facial disfigurement, sometimes delayed bone
growth in children, is a cause of concern. All these draw-
backs are preventable by endoscopic resection without
compromising the tumour excision. Pre-operative emboli-
sations help in blocking the feeding vessel (internal max-
illary artery) and thus decrease tumour vascularity. The
sinoscope gives better illumination and a panoramic view
and without disturbing the functional anatomy of the
sinuses. A residual mass may be left behind in extensive
tumours but this is rare.
Management of these patients is essentially based on a
thorough clinical and radiological study. The radiograph of
the paranasal sinuses and nasopharynx reveal presence of a
nasopharyngeal mass, anterior bowing of the posterior wall
of maxillary antrum (pathognomonic) and nasal septal
displacement. CT-MRI with contrast gives a better vision
of the extent of the tumour and a good delineation of the
tumour margins and a r e therefore more informative and
diagnostic tools. Angiography and embolisation pre-oper-
atively help to assess tumour vascularity and reduce it
respectively.
B. Baser S. Kothari (&)A. Kinger
SAIMS Medical College, Indore-Ujjain State Highway,
Bhanwarsala, Sanwer, Indore, India
e-mail: drshenal@gmail.com
123
Indian J Otolaryngol Head Neck Surg
(January–March 2011) 63(1):1–4; DOI 10.1007/s12070-010-0061-1
Recurrences and relapses can be avoided by proper
evaluation, staging and good surgical plan. The recurrence
is common when tumours reach skull base [3], roof of
infratemporal fossa, cavernous sinus or body of sphenoid.
Fortunately these tumour remnants either regress or remain
stable [4,5]. If clinically or radiologically the tumour is
stable a close surveillance is all that is required.
Materials and Methods
Between Jan 2007 and Mar 2010, 12 patients with JNA
ranging from Stage II to Stage III-B (Fisch classification), all
young males in the age group of 10–17 years, were diag-
nosed and operated by a transnasal endoscopic excision with
pre-operative embolisation. Post-operative CT scan and
endoscopy did not reveal any residual disease or recurrences.
Surgical Technique
After adequate decongestion, tumour exposure was the first
requirement and following steps were adopted, the lower
part of middle turbinate amputated, creation of septal
window, removal of uncinate process, the maxillary ostia
widening, and removal of the medial wall of maxillary
sinus. Smaller tumours could be accessed with this expo-
sure. To create a septal window the posterior part of the
bony septum was removed. In this way a two surgeon-two
nostril approach was adopted and a wider working space
created. For larger tumours further exposure was achieved
by removing medial and posterior maxillary walls as per
the case. When the maxillary artery was encountered it was
coagulated. The tumour was mobilised from the most lat-
eral part of the pterygopalatine fossa. Then the sphenoid
extension was mobilised and displaced inferiorly. The
plane of dissection was further followed into the naso-
pharyngeal region and the tumour delivered orally. During
the entire operation bone removal was facilitated by use of
Kerrison’s punches and a microdebrider with a sharp burr.
In Case no. 5 the entire medial wall was removed along
with the posterior wall far laterally to get access to the
infratemporal fossa. The tumour in this case was reaching
up to the cavernous sinus intracranially but was safely
extradural which could be accessed and removed by trac-
tion and blunt dissection under endoscopic vision reaching
up to lateral aspect of the sphenoid.
For case no. 12 the posterior wall removal was through
transantral window and it was possible to access the most
lateral extension in the infratemporal fossa. The endoscope
was introduced through the maxillary sinus and the tumour
dealt through the nasal cavity.
Anterior nasal packing was done for haemostasis which
was removed the second post-op day and discharged
between the third and fifth post-operative day. First follow-
up was after 2 weeks and then every month for 3 months.
Overall, the length of follow-up was between 6 and
26 months.
There were no recurrences. Two patients developed
mucocele which were endoscopically excised.
Results
All patients were discharged within a period of 2–5 days
post-operatively as against 10–15 days in an open
approach.
The tumours ranged from stage II to III-B as per Fisch
classification (Table 1).
Pre-operative embolisation (Figs. 1,2,3) was done in all
patients. This helped to minimize the intraoperative blood
loss. Only two patients needed one unit of blood, one
patient needed two units of blood and rest did well without
blood transfusion.
There were no recurrences or complication.
Discussion
A young male presenting with complaints of epistaxis,
nasal obstruction and a nasopharyngeal mass is almost
diagnostic of JNA. The presence of anterior bowing of the
posterior wall of the maxillary sinus on imaging (Holman-
Miller sign) is a known pathognomonic finding [6].
These tumours grow slowly and do not erode sur-
rounding structures. All our patients had a history of nasal
obstruction for last 2–4 years and none had symptoms of
surrounding structures involvement. Intracranial extension
is very rare as in one of our cases (stage III-B) is rare which
was also excised by an exclusive endoscopic approach and
tumour delivered orally. Almost all, (if not all) JNAs with
intracranial extension remain extradural and extra cavern-
ous and this property allows for a safer dissection.
Endoscopic removal of JNA has been limited up to II-A
stage as per literature [7]. In an article by Jorrisen et al. [8]
endoscopic treatment for more than II-A stage has been
done. Carrau et al. [9] considered that the sphenoid
involvement can be treated with endoscopy alone.
Endoscopic resection in management of angiofibromas
is now a established approach. Our experience also sub-
stantiates that JFA can be safely and effectively excised by
endoscopic approach, with negligible morbidity. Several
studies which favour this approach as it is true with our
series also.
2 Indian J Otolaryngol Head Neck Surg (January–March 2011) 63(1):1–4
123
For tumours reaching infratemporal fossa an endonasal
approach assisted by transantral window as was done for
case no. 4 helps to achieve a wider exposure and improves
visualisation as wide angle endoscopes give a panoramic
view. Case no. 5 had an intracranial extradural extension
and this was completely removed under endoscopic guid-
ance by removing the medial maxillary sinus wall and a
part of medial pterygoid plate. In our study also we could
access the intracranial extension endonasally and there was
no recurrence. Onerci et al. recommended endoscopic
resection for lesions with minimal extension into the cra-
nium [10].
Pre-operative embolisation was instrumental for good
haemostasis. All patients in this study were embolised
24–36 h pre-operatively. There are studies in which
authors have reported less bleeding with hypotensive
anaesthesia alone without pre-operative embolisation.
Haemostasis was further achieved with harmonic and lig-
asure cautery.
Table 1 Details of observations made for endoscopically excised extensive angiofibromas
S No. Stage Nose & Npx S S PPF ITF Retro pter ext Intra Cr. Extra dural Pre-op embo Av. Bld loss (ml)
1II???-- --?300
2 III-a ????? ?-?1200
3II???-? --?400
4 III-a ????- --?300
5 III-b ????? ?-?700
6 III-a ????- --?500
7II???-- --?350
8II???-- --?400
9II???-- --?550
10 II ???-- --?1200
11 II ???-- --?500
12 III-A ????? ?-?900
Npx Nasopharynx, SS Sphenoid Sinus, PPF Pterygopalatine Fossa, ITF Infratemporal Fossa, Retro pter ext Retro pterygoid extension, Intr/Cr
Intracranial Extension, Extr/dur Extradural, Preop/embo Pre-operative embolisation, Av. Bld loss Average blood loss
Fig. 1 MRI showing enhancing mass (angiofibroma) in the sphenoid
extending intracranially (extradural) into right cavernous sinus of case
no. 5
Fig. 2 Case-12 exposure of most lateral Part of tumour in infratem-
poral fossa
Indian J Otolaryngol Head Neck Surg (January–March 2011) 63(1):1–4 3
123
Recurrence or any other complication was not seen in
any of the patients. Extensive tumours were also excised
endonasally without any external incision irrespective of
stage. Recurrence however if seen, as these masses are
inactive, is followed by a close surveillance alone and
considered for revision surgery only when they become
symptomatic.
References
1. Wormald PJ, Van Hasselt A (2003) Endoscopic removal of
juvenile angiofibroma. Otolaryngol Head Neck Surg
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2. Brentani MM, Butugan O (2003) Endoscopic laser assisted
excision of juvenile nasopharyngeal angiofibroma. Arch Otolar-
yngol Head Neck Surg 129:454–459
3. Bothwell MR, Piccirillo JF, Lusk RB, Ridenour BD (2002)
Longterm follow-up of facial growth after FESSO to laryngology.
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4. Herman P, Lot G, Chapot R, Salvan D, Tran Ba, Hey P (1999)
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analysis of recurrences. Laryngoscope 109:140–147
5. Jacobson M, Petruson B, Ruth M (1989) Involution of residual
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sion, a case report with C T assessment. Arch Otolaryngol Head
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6. Cummings CW (ed) (1998) Otolaryngology-head and neck sur-
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7. Radwoski D, McGill T, Heady GB et al (1996) Angiofibroma
changes in staging and treatment. Arch Otolaryngol Head Neck
Surg 122:122–129
8. Jorrisen M, Eloy P, Bachert C, Daele J (2000) Endoscopics in us
surgery for juvenile Nasopharyngeal angiofibroma. Acta Otolar-
yngol 54:201–219
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Fig. 3 One year post-operative endoscopic picture showing optic
chiasma in case no. 5
4 Indian J Otolaryngol Head Neck Surg (January–March 2011) 63(1):1–4
123
... JNA is a formidable, locally aggressive, benign tumor of nasopharynx that spreads submucosally. It occurs almost exclusively in adolescent males and forms 0.05% of all head and neck neoplasms [1,2]. This decade has witnessed a new armamentarium of diagnostic techniques that have revolutionized the management of JNA. ...
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