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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
transfusion. Since JNAs occur exclusively infratemporal 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. tumours deemed inoperable.4
Patients may have undergone a trial of
nasal steroids and antihistamines or been
mistaken for having simple anterior epi- Pertinent Anatomy
staxis. An adolescent male with recurrent
epistaxis and chronic nasal obstruction is It is essential that a surgeon be familiar
highly suspicious for a JNA. The epistaxis with the detailed vascular anatomy of the
and nasal obstruction progressively wor- maxillary artery and its terminal branches,
sen. Unilateral nasal obstruction may pro- and that of the maxilla, paranasal sinuses,
gress to bilateral obstruction as the tumour pterygoplatine fossa, orbit and anterior
grows to fill the nasopharynx. Other skull base. Studying a cadaver skull, and
common symptoms include headache, having it available in the operating room is
facial swelling, unilateral rhinorrhoea, hyp- of great value.
osmia, and ipsilateral conductive hearing
loss due to Eustachian tube dysfunction. Vascular anatomy
JNAs originate from the sphenopalatine JNAs typically arise from the spheno-
artery near the sphenopalatine foramen, an palatine artery, which is a terminal branch
anatomic area usually readily accessible of the internal maxillary artery. The inter-
via endoscopic technique. Hence most of nal maxillary artery branches off the exter-
these tumours are now removed via an nal carotid artery (Figures 1, 2). The sphe-
endoscopic approach by surgeons skilled nopalatine artery usually contains two or
in endoscopic surgery working in properly more branches. Larger tumours can how-
equipped centres. Compared to open ap- ever have arterial supply from the ascen-
proaches, the endoscopic approach results ding pharyngeal, contralateral internal
in less intraoperative blood loss, fewer maxillary artery, and be supplied by the
complications, lower rates of recurrence, cavernous portion of the internal carotid
and shorter hospital stays.2 Due to the artery at the lateral wall of sphenoid sinus.
vascularity of these tumours, preoperative
embolization of major feeding vessels by
interventional radiology leads to signifi-
middle cranial fossa through eight fora-
mina.5 It communicates laterally with the
infratemporal fossa via the pterygo-
Infraorbital
artery
maxillary fissure, and medially with the
nasal cavity via the sphenopalatine fora-
Internal
maxillary men (Figures 3, 4). Immediately posterior
artery
are the pterygoid plates (Figures 3-9).
Figures 7 & 8 show axial views of the
anatomy of the pterygopalatine fossa,
pterygomaxillary fissure, and maxillary
Figure 1: Internal maxillary artery enter- sinus.
ing pterygopalatine fossa through pterygo-
maxillary fissure (mandible removed)
Ant ethm foramen
Post ethm foramen
Cribriform plate
Optic foramen
Face of sphenoid
Middle turbinate
Inf orbital fissure
Foramen rotundum
Sphenopalatine for
Pterygoid plates
2
sphenopalatine ganglion and its branches
are encountered within the pterygopala-
tine fossa. It also illustrates how a JNA
may extend superiorly though the inferior
orbital fissure into the orbit, medially into Posterior wall of
maxillary sinus
the nasal cavity and sphenoid, and laterally
into the infratemporal fossa. Pterygomaxillary
fissure
Pterygopalatine fossa
Posterior nasal
artery
Sphenopalatine
artery
Infraorbital foramen
Christa ethmoidalis
Inferior turbinate
Posterior choana
Zygoma
Pterygomaxillary fissure
Figure 5: Endoscopic view of posterior
Pterygoid plates
wall of (R) maxillary antrum through a
large middle meatal antrostomy: spheno-
palatine artery is located directly posterior Figure 8: Axial cut at level of infraorbital
to crista ethmoidalis; posterior nasal foramen and pterygoid plates
artery is superior to sphenopalatine artery Orbital apex
(Adapted from Statham MM, Tami TA. Endoscopic anatomy of the
pterygopalatine fossa. Oper Tech Otolaryngol.2006;17(3):197-200.)
Inferior orbital fissure
Sphenopalatine foramen
Pterygopalatine fossa
Pterygomaxillary
fissure & pterygo- Pterygomaxillary fissure
palatine fossa
Nerves
3
Figure 10: V2, pterygopalatine ganglion Figure 11: View of right nasal cavity
and infraorbital nerve (pterygopalatine showing large, vascular mass
fossa in red)
a
Preoperative Evaluation
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
Endoscopic resection
Indications
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 Figure 22: Lateral rhinotomy incision.
Middle cranial
fossa
Very rarely is a lip split extension
required for access
Table 4: Access provided by different sur-
gical approaches 9 • Inspect the antrum to determine the
tumour extent and to plan the subse-
Medial maxillectomy
quent bony cuts
• The extent of the subsequent bony
Medial maxillectomy is suited to tumours
resection is tailored to the JNA
limited to the nose, nasopharynx, sphenoid,
• A medial maxillectomy can now been
pterygopalatine fossa, medial infratempo-
ral fossa and medial cavernous sinus done (Figure 23); Figures 24a-c
(Table 4). Unless an ethmoidectomy is illustrate the extent of the bone
required, the medial maxillectomy is more resection with the limited medial
limited than that described in the chapter maxillectomy generally required for
on medial maxillectomy. 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 through the medial wall of the
the thick inferior orbital rim just maxillary sinus, starting superiorly
medial to the infraorbital nerve at the posterior end of the previous
(Figures 23, 24) osteotomy, and ending at the level
2. Osteotomy connecting antrostomy of the nasal floor
with nasal vestibule: A sharp
osteotome is used to connect the • The medial maxillectomy specimen is
anterior antrostomy with the floor then removed by gently levering it
of the nasal vestibule (Figures 23, inferiorly and laterally with the Mayo
24) scissors while completing the posterior
3. Osteotomy across frontal process osteotomy, remaining lateral to and
of maxilla: This part of the preserving middle turbinate
dissection is often best done with a • An external ethmoidectomy may now
Kerrison’s rongeur or oscillating safely be completed under direct vision
saw. There is often persistent minor up to cribriform plate if required
bleeding from the bone that may be • Carefully remove the paper thin poste-
controlled with bone wax or rior wall of the maxillary antrum to
cautery (Figure 23) expose the JNA and the sphenopalatine
4. Osteotomy along floor of nose: A and/or internal maxillary artery
sharp osteotome or heavy scissors • Clip/ligate/bipolar the sphenopalatine
is used to divide the lateral wall of /internal maxillary artery even if it has
the nose/medial wall of the antrum been embolised
along the floor of the nasal cavity • Proceed with the resection using blunt
up to the posterior wall of the and bipolar dissection; suction bipolar
antrum. When doing this dissection electrocautery is first used to ablate
with an osteotome, the dissection is feeding vessels along the surface of the
halted when the osteotome hits up tumour; a suction Freer elevator or
against the solid pterygoid bone knife is used to release adhesions
(signalled by a change in the • Dissect tumour off adjacent structures;
sound) often it is adherent to septum, sphenoid
5. Osteotomy through lacrimal bone, rostrum, skull base, and nasopharynx
and anterior ethmoids: This • If tumour extends laterally beyond the
osteotomy is made at the level of pterygopalatine fossa into the infra-
the roof of the antrum (Figures temporal fossa, then remove the post-
24b, c). The osteotomy is done by erolateral antral wall for additional
gently tapping on an osteotome or exposure
with heavy curved scissors with
• Inspect the entire area that was invol-
tips pointed inferiorly. The osteo-
ved with tumour; this may be aided by
tomy stops at the posterior wall of
use of an endoscope
the antrum
• Obtain meticulous haemostasis
6. Vertical posterior osteotomy
through posterior end of medial • Apply haemostatic sinus material, such
wall of antrum anterior to as Surgicel to bleeding surfaces
pterygopalatine fossa: The final • At the conclusion of surgery the
posterior vertical cut is made with transected lacrimal sac (Figure 24a) is
heavy curved (Mayo) scissors as a slit along its longitudinal axis and the
downward continuation of the edges are sutured to the surrounding
osteotomy in Point 5. It runs tissues or stented to avoid epiphora
12
Le Fort 1 osteotomy
b
Figure 25: Le Fort 1 osteotomy;
posteriorly it passes through the pterygo- Figure 27b: Maxilla down-fractured to
maxillary fissure expose JNA 11
Posterior wall of
antrum obscuring
pterygopalatine fossa
Pterygoid plates
13
d
Transpalatal approach
Figure 29: Mucosal incision in hard palate
(yellow line) to elevate palatal flap; bone
This approach can be used for JNAs
removal to expose tumour (chequered
confined to the nasopharynx, sphenoid and
area)
nasal cavity (Table 4). The bony anatomy
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.
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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- THE OPEN ACCESS ATLAS OF
pharyngeal Angiofibroma. J Craniofac OTOLARYNGOLOGY, HEAD &
Surg 2011;22: 1027-30
NECK OPERATIVE SURGERY
www.entdev.uct.ac.za
Author
Derek J. Rogers, MD
Pediatric Otolaryngology The Open Access Atlas of Otolaryngology, Head &
Neck Operative Surgery by Johan Fagan (Editor)
Harvard Medical School johannes.fagan@uct.ac.za is licensed under a Creative
Massachusetts Eye and Ear Infirmary, Commons Attribution - Non-Commercial 3.0 Unported
License
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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