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Juvenile nasopharyngeal angiofibroma cantly less blood loss and facilitates endo-
(JNA) is a rare tumour representing only scopic resection.3
about 0.05% of head and neck tumours.1
The most common presentation is a prepu- Traditionally, several open approaches are
bescent or adolescent male with severe, employed, including lateral rhinotomy,
recurrent epistaxis and nasal obstruction. midfacial degloving, transmaxillary (Cald-
The epistaxis may even require a blood well-Luc), transpalatal, Le Fort 1 and in-
transfusion. Since JNAs occur exclusively fratemporal fossa approaches. Extensive
in males, a hormonal theory has been im- tumours, such as those with lateral infra-
plicated. temporal fossa involvement or significant
optic canal or intracranial extension may
As these tumours are quite rare, many necessitate an open or a combined open
patients may have been treated conserva- and endoscopic approach. Radiotherapy
tively by a primary care provider before and anti-androgen therapy are reserved for
being referred to an otolaryngologist. Pa- tumours deemed inoperable.4
tients may have undergone a trial of nasal
steroids and antihistamines or been mista- Pertinent Anatomy
ken for having simple anterior epistaxis.
An adolescent male with recurrent epista- It is essential that a surgeon be familiar
xis and chronic nasal obstruction is highly with the detailed vascular anatomy of the
suspicious for a JNA. The epistaxis and maxillary artery and its terminal branches,
nasal obstruction progressively worsen. and that of the maxilla, paranasal sinuses,
Unilateral nasal obstruction may progress pterygoplatine fossa, orbit and anterior
to bilateral obstruction as the tumour skull base. Studying a cadaver skull and
grows to fill the nasopharynx. Other com- having it available in the operating room is
mon symptoms include headache, facial of great value.
swelling, unilateral rhinorrhoea, hyposmia,
and ipsilateral conductive hearing loss due Vascular anatomy
to Eustachian tube dysfunction.
JNAs typically arise from the spheno-
JNAs originate from the sphenopalatine palatine artery, which is a terminal branch
artery near the sphenopalatine foramen, an of the internal maxillary artery. The inter-
anatomic area usually readily accessible nal maxillary artery branches off the exter-
via endoscopic technique. Hence most of nal carotid artery (Figures 1, 2). The sphe-
these tumours are now removed via an nopalatine artery usually contains two or
endoscopic approach by surgeons skilled more branches. Larger tumours can how-
in endoscopic surgery working in properly ever have arterial supply from the ascen-
equipped centres. Compared to open ap- ding pharyngeal, contralateral internal
proaches, the endoscopic approach results maxillary artery, and be supplied by the
in less intraoperative blood loss, fewer cavernous portion of the internal carotid
complications, lower rates of recurrence, artery at the lateral wall of sphenoid sinus.
and shorter hospital stays.2 Due to the
vascularity of these tumours, preoperative
embolization of major feeding vessels by
interventional radiology leads to signifi-
eight foramina.5 It communicates laterally
with the infratemporal fossa via the ptery-
gomaxillary fissure, and medially with the
Infraorbital
artery
nasal cavity via the sphenopalatine fora-
men (Figures 3, 4). Immediately posterior
Internal
maxillary are the pterygoid plates (Figures 3-9).
artery
Figures 7 & 8 show axial views of the ana-
tomy of the pterygopalatine fossa, pterygo-
maxillary fissure, and maxillary sinus.
2
tine fossa. It also illustrates how a JNA
may extend superiorly though the inferior
orbital fissure into the orbit, medially into
the nasal cavity and sphenoid, and laterally
into the infratemporal fossa. Posterior wall of
maxillary sinus
Pterygomaxillary
fissure
Sphenopalatine
artery
Christa ethmoidalis
Inferior turbinate
Nerves
Figure 6: Internal maxillary artery (red The maxillary division of trigeminal (V2)
arrow) traverses the pterygomaxillary fis- enters the pterygopalatine fossa via for a-
sure to enter the pterygopalatine fossa men rotundum (Figures 7, 10). The infra-
orbital nerve is a terminal branch of V2
and runs in the floor of the orbit/roof of the
antrum to exit the infraorbital foramen.
3
Figure 10: V2, pterygopalatine ganglion Figure 11: View of right nasal cavity
and infraorbital nerve (pterygopalatine showing large, vascular mass
fossa in red)
Preoperative Evaluation a
Clinical Evaluation
Radiologic Evaluation
Figure 13a: MRI: No orbital or intracra- Table 2: Radkowski Staging for JNA
nial extension
Stage Description of Tumour Involvement
IA: Limited to nose or nasopharynx
b I
IB: Same as above but involving ≥ 1 sinus
IIA: Minimal extension through
sphenopalatine foramen and into medial
pterygomaxillary fossa
IIB: Full occupation of pterygomaxillary
II fossa displacing posterior wall of
maxillary sinus forward, orbit erosion,
displacement of maxillary artery branches
IIC: Involvement of infratemporal fossa or
cheek, or posterior to pterygoid plates
Erosion of skull base
IIIA: Minimal intracranial involvement
III
IIIB: Extensive intracranial involvement or
any cavernous sinus extension
5
Angiography
6
Anaesthesia considerations • Intraoperative image guidance (if avai-
lable)
• Patients are placed supine in reverse
Trendelenburg position Procedure
• Oral RAE® endotracheal tube permits
unobstructed access to the nose • Inject Lidocaine with epinephrine into
• Hypotensive general anaesthesia the greater palatine foramina, septum,
• Type and crossmatch 2 units of blood uncinate and middle turbinate on the
as rapid blood loss can occur; consider side with the tumour
banking 2 units of autologous blood • Pack both nasal cavities for 10min with
one week before surgery cottonoid pledgets soaked in oxymeta-
• Intraoperative blood salvage (autolo- zoline
gous blood transfusion/cell salvage/cell • On the side with the tumour, amputate
saver technique) can be employed to the inferior aspect of the middle turbi-
recover blood lost during surgery nate with scissors (Figure 15)
which is reinfused into the patient
Endoscopic resection
Indications
7
Figure 16: Intraoperative photo (right
nose) following middle meatal antrostomy, Figure 18: The posterior wall of the maxil-
showing crista ethmoidalis and posterior lary sinus is removed along the spheno-
maxillary sinus wall palatine artery (Reprinted with permission from Wormald PJ,
Van Hasselt A. Endoscopic removal of juvenile angiofibromas.
Otolaryngol Head Neck Surg. 2003;129(6):684-91. SAGE Publications)
8
• Nasal saline irrigations are started on
the 1st postoperative day, at least twice
daily, for nasal toilet
• The patient is instructed not to blow
the nose
• The 1st postoperative visit is scheduled
at 1 week
Complications
Nasopharynx
Intranasal
Ethmoids
Sphenoid
Pt Pal Fossa
Medial ITF
Lateral ITF
Medial cav
sinus
Lateral cav
sinus
Middle cranial
fossa
Figure 22: Lateral rhinotomy incision.
Very rarely is a lip split extension
Table 4: Access provided by different sur- required for access
gical approaches 9
• Inspect the antrum to determine the
Medial maxillectomy
tumour extent and to plan the subse-
quent bony cuts
Medial maxillectomy is suited to tumours
• The extent of the subsequent bony
limited to the nose, nasopharynx, sphenoid,
resection is tailored to the JNA
pterygopalatine fossa, medial infratempo-
• A medial maxillectomy can now been
ral fossa and medial cavernous sinus
(Table 4). Unless an ethmoidectomy is done (Figure 23); Figures 24a-c illu-
required, the medial maxillectomy is more strate the extent of the bone resection
limited than that described in the chapter with the limited medial maxillectomy
on medial maxillectomy. generally required for JNAs
10
Figure 24b: Coronal CT through mid-
antrum demonstrates resected lateral nasal
wall including inferior turbinate, uncinate
process and trimmed middle turbinate
11
bone nibbler is used to cut along starting superiorly at the posterior
the thick inferior orbital rim just end of the previous osteotomy, and
medial to the infraorbital nerve ending at the level of the nasal
(Figures 23, 24) floor
2. Osteotomy connecting antrostomy
with nasal vestibule: A sharp os- • The medial maxillectomy specimen is
teotome is used to connect the then removed by gently levering it in-
anterior antrostomy with the floor feriorly and laterally with the Mayo
of the nasal vestibule (Figures 23, scissors while completing the posterior
24) osteotomy, remaining lateral to and
3. Osteotomy across frontal process preserving middle turbinate
of maxilla: This part of the dissec- • An external ethmoidectomy may now
tion is often best done with a Kerri- safely be completed under direct vision
son’s rongeur or oscillating saw. up to cribriform plate if required
There is often persistent minor • Carefully remove the paper-thin poste-
bleeding from the bone that may be rior wall of the maxillary antrum to
controlled with bone wax or caut- expose the JNA and the sphenopalatine
ery (Figure 23) and/or internal maxillary artery
4. Osteotomy along floor of nose: A • Clip/ligate/bipolar the sphenopalatine /
sharp osteotome or heavy scissors internal maxillary artery even if it has
is used to divide the lateral wall of been embolised
the nose/medial wall of the antrum • Proceed with the resection using blunt
along the floor of the nasal cavity and bipolar dissection; suction bipolar
up to the posterior wall of the electrocautery is first used to ablate
antrum. When doing this dissection feeding vessels along the surface of the
with an osteotome, the dissection is tumour; a suction Freer elevator or
halted when the osteotome hits up knife is used to release adhesions
against the solid pterygoid bone • Dissect tumour off adjacent structures;
(signalled by change in the sound) often it is adherent to septum, sphenoid
5. Osteotomy through lacrimal bone, rostrum, skull base, and nasopharynx
and anterior ethmoids: This osteo- • If tumour extends laterally beyond the
tomy is made at the level of the pterygopalatine fossa into the infra-
roof of the antrum (Figures 24b, c). temporal fossa, then remove the poste-
The osteotomy is done by gently rolateral antral wall for additional
tapping on an osteotome or with exposure
heavy curved scissors with tips
• Inspect the entire area that was invol-
pointed inferiorly. The osteotomy
ved with tumour; this may be aided by
stops at the posterior wall of the
use of an endoscope
antrum
• Obtain meticulous haemostasis
6. Vertical posterior osteotomy
through posterior end of medial • Apply haemostatic sinus material, such
wall of antrum anterior to ptery- as Surgicel to bleeding surfaces
gopalatine fossa: The final poste- • At the conclusion of surgery, the
rior vertical cut is made with heavy transected lacrimal sac (Figure 24a) is
curved (Mayo) scissors as a down- slit along its longitudinal axis and the
ward continuation of the osteotomy edges are sutured to the surrounding
in Point 5. It runs through the tissues or stented to avoid epiphora
medial wall of the maxillary sinus,
12
Le Fort 1 osteotomy
b
Figure 25: Le Fort 1 osteotomy; posterior-
ly it passes through the pterygomaxillary Figure 27b: Maxilla down fractured to
fissure expose JNA 11
Posterior wall of
antrum obscuring
pterygopalatine fossa
Pterygoid plates
13
d
Transpalatal approach
Figure 29: Mucosal incision in hard palate
This approach can be used for JNAs con-
(yellow line) to elevate palatal flap; bone
fined to the nasopharynx, sphenoid and
removal to expose tumour (chequered
nasal cavity (Table 4). The bony anatomy
area)
of the hard palate is illustrated in Figure
28.
Maxillary swing approach (Figure 30)
Sphenoid
Pterygoid plates
14
Infratemporal fossa approach
15
This may require forceful inferior
retraction of the soft tissues with a
Pterygomaxillary Langenbeck retractor (Figure 39)
fissure & pterygo-
palatine fossa
Internal maxillary
artery
Pterygoid plates
16
• Incise the two layers of deep temporal es for excision of juvenile naso-
fascia along the superior margin of the pharyngeal angiofibroma. Laryngo-
zygoma, and free the zygoma from the scope 2005; 115: 1201-7
insertion of the masseter muscle 3. Schroth G, Haldemann AR, Mariani L,
• Osteotomise and remove the zygomatic Remonda L, Raveh J. Preoperative
arch, and preserve it in saline so that it embolization of paragangliomas and
can be plated/wired back later in the angiofibromas. Measurement of intra-
procedure tumoral arteriovenous shunts. Arch
• Elevate the temporalis muscle from the Otolaryngol Head Neck Surg 1996;
bone of the temporal fossa using either 122:1320-5
diathermy or a periosteal elevator 4. Lee JT, Chen P, Safa A, Juillard G,
while remaining hard on the bone Calcaterra TC. The role of radiation in
(Figure 40) the treatment of advanced juvenile
angiofibroma. Laryngoscope 2002;
112:1213-20
5. Osborn AG. Radiology of the pterygoid
plates and pterygopalatine fossa. AJR
Am J Roentgenol 1979; 132:389-94
6. Andrews JC, Fisch U, Valavanis A,
Aeppli U, Makek MS. The surgical
management of extensive nasopharyn-
geal angiofibromas with the infra-
temporal fossa approach. Laryngoscope
1989; 99:429-37
7. Radkowski D, McGill T, Healy GB,
Ohlms L, Jones DT. Angiofibroma.
Changes in staging and treatment. Arch
Otolaryngol Head Neck Surg 1996;
Figure 40: Flap completely elevated 122:122-9
from temporal fossa 8. Snyderman CH, Pant H, Carrau RL,
Gardner P. A new endoscopic staging
• Extend the dissection medial to the system for angiofibromas. Arch Oto-
coronoid process of the mandible that laryngol Head Neck Surg 2010;
is now readily palpable; the coronoid 136:588-94
process of the mandible can be divided 9. Fagan JJ, Snyderman CH, Carrau RL,
and reflected inferiorly for additional Janecka IP. Nasopharyngeal angiofibro-
exposure mas: selecting a surgical approach.
Head Neck. 1997 Aug;19(5):391-9
10. Lowlicht RA, Jassin B, Kim M, Sasaki
References CT. Long-term Effects of Le Fort I
Osteotomy for Resection of Juvenile
1. Herman P, Lot G, Chapot R, Salvan D, Nasopharyngeal Angiofibroma on
Huy PT. Long-term follow-up of Maxillary Growth and Dental Sensa-
juvenile nasopharyngeal angiofibromas: tion. Arch Otolaryngol Head Neck Surg.
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1999; 109:140-7 11. Avelar RL, de Santana Santos T,
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17
proach for Resection of Juvenile Naso- johannes.fagan@uct.ac.za
pharyngeal Angiofibroma. J Craniofac
Surg 2011;22: 1027-30 THE OPEN ACCESS ATLAS OF
OTOLARYNGOLOGY, HEAD &
Suggested video
NECK OPERATIVE SURGERY
www.entdev.uct.ac.za
Goncalves N, Lubbe DE. Juvenile Nasal
Angiofibroma (JNA): A case presentation
and review of endoscopic surgical techni-
que. UCT-Africa ENT Virtual video
channel The Open Access Atlas of Otolaryngology, Head &
Neck Operative Surgery by Johan Fagan (Editor)
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Author
Derek J. Rogers, MD
Pediatric Otolaryngology
Harvard Medical School
Massachusetts Eye and Ear Infirmary,
Boston, MA, USA
Derek_Rogers@meei.harvard.edu
[Disclaimer: The views expressed in this chapter are those of the authors
and do not necessarily reflect the official policy or position of the
Department of the Army, the Department of Defense, or the US
government. MAJ Rogers is a military service member. This work was
prepared as part of his official duties. Title 17 U.S.C. 105 provides that
‘Copyright protection under this title is not available for any work of the
United States Government.’ Title 17 U.S.C. defines a “United States
Government work” as a work prepared by a military service member or
employee of the United States Government as part of that person’s official
duties]
Author
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