ALLAH IS THE GREATEST ISLAM IS THE TRUE RELIGION

1. Case Discussion
Dr Che Mohd Hilmi
Supervisor: Prof Baha/Mr Rosdi/Mr Rosli

2. History
• 14 years old malay boy
• NKMI
• Main complaint: left nasal blockage and recurrent left nasal bleeding for 3 months.
• Started first with left nasal blockage then followed by left nasal epistaxis.
• Left nasal blockage was persistent, not improve with nasal spray or anti-histamine, not
improve with inspiration or expiration.

3. Left nasal
bleeding/epistaxis:
1st episode
Occur 4 times in last 3 months
Frank blood, profuse bleeding, volume of blood
variable, the highest volume about 10ml (claimed by
using large handkerchief soaked) – during 2nd times.
Stop spontaneously, duration 2-5 minutes.
Exaggerate by exercise and hot weather.

4. Left nasal
bleeding/epistaxis:
➢During the 1st epistaxis - occur during playing ground, his
father seek treatment at KK.
➢The epistaxis was stop once patient arrived at KK, and
advice given for ice packing, avoid hot weather.
➢2nd epistaxis, his father brought him again to KK, however
the epistaxis also stop once arrived at KK. He was refer to
tertiary hospital. Patient was started on oxynase ns and
oral anti-histamine. Diagnosis from KK was epistaxis 2ry
allergic rhinitis.
➢3rd and 4th epistaxis occur during awaiting ENT
appointment.

5. • Otherwise patient did not complaint any nasal mucous discharge, nasal
itchiness, facial pain and hyposmia.
• He also did not complaint of loss of weight, loss of appetite, palpitation,
tiredness, shortness of breath, no PTB contact.
• He denied any habit of nasal rubbing or history of nasal trauma.
• Past medical hx: nil
• Past surgical hx: nil
• Allergic hx: no allergic to seafood or any medication.
• Family: no family history of cancer
• Medication: no herbal medication or anti-coagulant medication

6. Summary
• 14 years old malay boy with nkmi, complaint of persistent
left nasal blockage and recurrent left epistaxis for 3
months. The left nasal blockage was not relieved with nasal
spray and the epistaxis worsening with hot weather or
during exercise.

7. On
examination:
Alert conscious, pink, not tachypnic, not tachycardic
BP 110/60
PR 80
RR 20
T 37
Lung : clear
CVS : DRNM
Perabdomen: soft non tender, no hepatosplenomegaly

8. Nose examination
On inspection:
Front: no swelling, no
deviation, no scar
seen
Superior: no bicoronal
scar, no proptosis,
maxillary prominent
symmetrical
Inferior: no
columellar scar
On palpation: no
sinus tenderness over
maxillary, ethmoid
and frontal sinus.

9. Cold spatula test: reduce fogging on left side.
On lift nasal tip: no columellar scar, no caudal dislocation.
Anterior rhinoscopy: Right nose: mucosa healthy, inferior turbinate not
hypertrophy, septum not deviated, no mass seen. On left nose: noted
mass reddish to bluish medial to inferior turbinate. Inferior turbinate
not hyperthropy.
Cottle test on left nose: -ve (nasal blockage not improve when pull
cheek laterally)

10. 0 degree
endoscopy of
left nose
• Nasoendoscopy revealed the red-bluish
appearance and vascularized mass
occupying the left nasal cavity. It extended
inferiorly till the level of inferior turbinate
and posteriorly till posterior choana. The
origin of the mass cannot be identified. The
mass not pulsatile.
• Inferior turbinate not hypertrophy, left high
DNS, OMC clear, FOR not visualized.
• Olfactory cleft cannot be visualize on
posterior part
• Probing test not done due to afraid of
bleeding

11. Video 0
degree scope
of left nose

12. Right nose
• 0 endoscopy right nose: Inferior turbinate not
hypertrophy, OMC clear, no mass, no polyp, no
discharge seen. Present of posterior septal
deviation with septal spur and hypervascular
mass at posterior choanal which extend from left
nose.

13. Other ENT
examination
• Oral examination: tongue move in all
direction, FOM not raised, uvula center,
gingiva look healthy, no dental carries, tonsil
grade 2, no mass seen.
• Otoscopy examination: Bilateral external ear
canal minimal wax with bilateral TM intact.
• Neck examination: No neck node palpable,
no neck mass.
• Tuning fork test: Bilateral rinne +ve, weber
no lateralization
• All cranial nerve examination normal
• No other node at axilla and inguinal region.

14. Blood
investigation
• Twc: 11, HB 12, Plt 303
• Renal profile: within normal range
• Coagulation profile: within normal range

15. Summary
• 14 years old malay boy with nkmi,
complaint of persistent left nasal blockage
and recurrent left epistaxis for 3 months.
The left nasal blockage was not relieved
with nasal spray and the epistaxis
worsening with hot weather or during
exercise. Otherwise patient did not
complaint any anemic symptoms. 0
endoscopy on left nose revealed the red-
bluish appearance and vascularized mass
occupying the left nasal cavity. It extended
inferiorly till the level of inferior turbinate
and posteriorly till posterior choana. Other
ENT examination and blood investigation
were normal.

16. Differential
diagnosis
• 1. JNA
• 2. AC polyp
• 3. NPC
• 4. Olfactory neuroblastoma
• 5. Inverted papilloma
• 6. Lymphoma
• 7. Meningiocele
• 8. Sinonasal CA

17. Differential Diagnosis Point for Point against
1. JNA Male
Teenage / young average age 15 years old
Profuse recurrent unilateral epistaxis
Unilateral mass with red-bluish vascularized
-
2. AC polyp Children/young age
Trilobed, extend to posterior choanal
the polyp look like peel grape appearance, in this case the
mass look very vascular
Nasal blockage improve with expiration (ball valve effect)
3. NPC Fossa of ronsenmuller cannot be visualized
Bimodal age; youngest 6 years old
80% of case come with neck swelling
4. Olfactory neuroblastoma Unilateral nasal blockage (70%)
Epistaxis (30%)
Olfactory cleft cannot be fully visualize
More in female
Bimodal age 20 and 50

18. Differential Diagnosis Point for Point against
5. Inverted papilloma More in male 4:1
90% from lateral wall
Unilateral mass
Old age ~ 50 y/o
Mulberry appearance
6. Lymphoma Lymphomas are the
most common group of cancers in
teenagers and young adults
No hepatosplenomegaly
FBC normal
7. Meningiocele Children
Unilateral
cyst-like grayish swelling
8. Sinonasal CA - No constitutional symptoms like
loss of weight, loss of appetite.

19. Next step
Due to hypervascular tumour, biopsy was not taken and we proceed with
CT PNS contrast.

20. CT scan
• Computed tomography (CT) scan revealed a well-defined
heterogeneously-enhanced mass arising from the left nasal cavity,
measuring 4.7 cm x 6.0 cm x 4.0 cm.
• It extended into the sphenoid sinus causing erosion of the nasal septum.
It caused bowing of medial wall of the left maxillary sinus.
• The mass also extended to the left maxillary sinus via the expanded
osteo-meatal complex. There was some extension to the left
sphenopalatine foramen.
• However, no expansion of both sphenopalatine foramina seen. No
widening pterygopalatine fossa.
• No anterior bowing of posterior wall of the left maxillary sinus. No
intracranial extension and fossa of Rosenmüller were clear bilaterally.
Mucosal thickenings were present within ethmoid, sphenoid and both
maxillary sinuses.
• Both parapharyngeal spaces and infratemporal spaces were clear. Both
pyriform fossa also were clear. No enhancing lesion noted in oropharynx
or hypopharynx. The visualised brain parenchyma was normal.
• CT scan impression was an aggressive polyp, and malignancy needs to
be ruled out.

21. Video CT
scan

22. Coronal CT showed mass occupied the whole expanded nasal
cavity
Axial CT showed left nasal mass invading the medial wall of
the left maxillary sinus.
CT PNS

23. Next step
• Because of the hypervascular in nature, we
proceeded with embolization even with the
absence of classical features of JNA (No
expansion of both sphenopalatine foramina
seen. No widening pterygopalatine fossa. No
holman miller sign).

24. Embolization of left maxillary
artery
• Because of the hypervascular in nature, we proceeded with
embolization.
• Embolization was done 48 hours before surgical excision of
mass by using intra-arterial polyvinyl alcohol (PVA) under
general anaesthesia of the left maxillary artery with resulted
in 90% reduction of tumour blush.
• Left external carotid artery angiogram showed no abnormal
aneurysmal dilatation or serpiginous vessel seen.

25. Surgery
Video

26. LA given at
axilla, lateral
wall and
middle
turbinate

27. Uncinectomy,
MMA,
Shivering of
lateral part of
middle
turbinate

28. Mid part of
middle
turbinate was
remove to
widen the
surgical field

29. Crista
ethmoidalis
identified,
posterior wall
of maxillary
sinus
removed

30. Feeding
vessel
identified,
bipolar
diathermy
used before
cut

31. Posterior part
of septum
separate
from the
mass till
anterior
border of
sphenoid

32. Left nasal mass
removed en
block with
length 4 cm.

33. Intraoperative
Intraoperatively, the
sphenopalatine artery was
ligated endoscopically before
removal of the mass.
The mass arose from
sphenopalatine foramen with
the posterior extension of
mass till sphenoid sinus.
Medial aspect of mass extends
till posterior part of the
septum.
The anterior sphenoid wall
was brittle and fragile.
The inferior part of mass
extends till nasopharynx and
superior part of adenoid. The
mass removed en bloc.
Merocel was inserted to the
left nose.

34. Post operative
• Post-operatively, there was no any
complication including epistaxis or septal
hematoma. Merocel was kept for 24 hours
and he was discharged on the next day.
• He was seen after a week with
nasoendoscopy demonstrated no blood clot,
no synechiae and no remain or other mass in
the left nostril. Patient was followed-up at
every 3 months.

35. HPE
• Histopathological examination revealed a polypoidal and richly vascular tissue-lined
focally by pseudostratified columnar epithelium. The stroma was fibro cellular composed
of spindle cells with the haphazard arrangement of collagen interspersed with an irregular
vascular channel.
• The blood vessels were of various sizes ranging from slit-like capillaries to irregularly
dilated, and branching vessels identified. Seromucous glands were seen in the periphery.
• No malignancy was seen. Several immunohistochemical stains were done revealed
positive B-catenin. Others immunohistochemical stains like smooth muscle actin and
desmin were negative. The final pathological diagnosis was JNA.

36. 16 points of discussion
1. What is JNA?
2. Pathophysiology of JNA?
3. Anatomy of Pterygopalatine fossa
4. Instead of SPA, can JNA occur in other artery?
5. Role of biopsy in JNA, can it be take in clinic?
6. Can JNA occur in female?
7. What is Hollman miller sign?
8. What is other sign of JNA in CT scan?
9. Staging of JNA
10. Why the surgery should be done >24 hour and before 48 hours after embolization?

37. 16 points of discussion
11. CT vs MRI
12. What are material of choice for embolization?
13. Complication of embolization?
14. Contraindication embolization?
15. Treatment, Endoscopic vs Open approach? How to chose?
16. Role of radiotherapy, chemotherapy ,hormonal therapy in JNA

38. 1. What is JNA?
• Juvenile nasopharyngeal angiofibroma (JNA) is a slow growing benign tumour which arises
from sphenopalatine foramen. It is a highly vascular lesion with tendency to invade
adjacent structures. It is almost exclusively found in teenage males, representing 1% of
head and neck tumour.
• JNA is a rare benign tumour with potential locally destructive of vascular tumours. The
tumour is un-encapsulated and consists of a vascular network within a fibrous stroma.
• It is sex and age-linked. The prevalence is higher in males can be explained by genetic
studies which demonstrated the relationship between the androgen receptor expression
and angioma. It is suggesting that this tumour is androgen-dependent.

39. 2. Pathophysiology
of JNA
• Many theories have been propounded but
none is entirely convincing
• 1. hormonal theory (marten 1948): deficient
of androgen, overactivity of estrogen,
hormonal stimulation
• 2. Hamartoma (Osborn 1959): residual fetal
erectile tissue which subject to hormonal
• 3. Cojoin fascia (brunner 1942): tumour grow
from conjoined pharyngobasillar &
buccopharyngeal fascia
• 4. Fibroblastic (bensch & ewing 1941):
tumour arise from embrayonic fibrocartilage
between the bassiocciput & basisphenoid
The most accepted theory is that JNAs originate
from sex steroid–stimulated hamartomatous
tissue located in the turbinate cartilage. The
proposed hormonal influence may explain why
some JNAs involute after puberty.

40. 3. Anatomy: Boundary of
Pterygopalatine fossa
• Small space, inverted pyramid
shape
• Posterior- greater wing of
sphenoid
• Anterior- posterior wall of
maxilla
• Superior- inferior surface of
body sphenoid
• Medial- perpendicular plate
of palatine
• Lateral- infratemporal fossa
via pterygomaxillary fissure
• Inferior – pyramidal process
of the palatine.

41. Content of
Pterygopalatine fossa
• 1. Neural compartment- pterygopalatine
ganglion and maxillary nerve
• 2. Vascular compartment- terminal part
maxillary a and its branch 3rd part
(pterygopalatine part)
1. Posterior superior alveolar artery
2. Infraorbital artery
3. Greater auricular artery
4. Pharyngeal artery
5. Artery of pterygoid canal
6. Sphenopalatine artery

42. Communications
of
Pterygopalatine
fossa
• Anterior- via inferior orbital fissure with orbit
transmit infraorbital vessel, nerves &
ascending br of pterygopalatine ganglion
• Posterior- via foramen rotundum with
middle cranial fossa transmitting the
maxillary nerve & via pterygoid canal
extending to foramen lacerum
• Medial- via sphenopalatine foramen to nasal
cavity
• Lateral- via pterygomaxillary fissure to
infratemporal fossa, transmit maxillary vessel
& superior alveolar nerve
• Inferior- via greater palatine canal to roof of
mouth, transmit anterior, middle and
posterior palatine nerve and greater and
lesser palatine vessel

43. 4. Instead
of SPA, can
JNA occur
in other
artery?
Ipsilateral maxillary artery 90%
Contralateral maxillary artery
40%
Branch of ICA 20%
Ascending pharyngeal artery 12%
Superficial temporal artery 12%

44. 5. Can we
take biopsy
in clinic if
suspected
of JNA?
• YES!
• But must be done in office hour, good
support blood bank, excellent
communication with anesth and intensive
radiology for kiv massive bleeding, icu
back up and embolization if complication
occur.
• If you don’t have any support, please
don’t do it.
• Know your limit.

45. 6. Can JNA occur
in female?
• YES!
• In 1965, Apostol and Frazell reported
40 cases of JNA in male patients and
suggested that if this diagnosis is
confirmed in a female, sex
• Chromosome studies must be
performed, to investigate for
androgen insensitivity syndrome
(formerly testicular feminization) of a
phenotypic female but genetic male.

46. 7. What is Hollman
miller sign?
• The Holman-Miller sign (also called
the antral sign) is seen in juvenile
nasopharyngeal angiofibroma; it
refers to the anterior bowing of the
posterior wall of the maxillary
antrum
• However, only 81% of JNA present
with these features. Furthermore,
anterior bowing of the posterior wall
of the maxillary sinus known as
‘Holman Miller’ sign or ‘Antral Sign’ is
found only in 87% of JNA.

47. 8. What are
other sign
instead of
‘Holman
Miller’
• Another sign instead of ‘Holman Miller’ sign is ‘Ram-Haran’
sign. It is the broadening of pterygoid wedge which is
estimated 2 times larger than the unaffected side.
• Other classical findings include widening of the
pterygopalatine fossa, bony erosion of sphenopalatine
foramen and medial pterygoid plate.
• In extensive JNA, loss of the fat plane in infratemporal fossa
can be seen. However, none of these signs were present in
our case.
• Lloyd et al described two typical CT features for JNA. The
features include the erosion of the posterior part of the
sphenopalatine foramen, till to the base of the medial
pterygoid plate and present of tissue mass in posterior
nasal cavity or mass in the pterygopalatine fossa.

48. Ram-Haran’ sign :
Pterygoid wedge
which is estimated
2 times larger than
the unaffected
side

49. Other sign
“chop stick”
sign – post
operative due
to removal of
pterygoid
wedge in case
of extensive
JNA

50. 9. Staging –
Fisch
Classification

51. Stage
Rodkowski

52. 10. Why after embolization,
the surgery should be done
>24 hour and before 48
hours?
• Why after 24 hours ?
-Mainly to observe for complications
of embolization and for emboli
• Why before 72 hours?
-Before neovascularization takes
place

53. Radiological
study

54. 11. CT vs MRI
CT MRI
Can evaluate the pattern bony
changes
Can assess intracranial extension,
dura, cavernous sinus involvement,
orbit
Extension of tumour to skull base Best visualize in residual and recurrent
case, differentiate from other soft
tissue structure, mucosal disease,
inflammation.
Feature:
1. Contrast enhancement
2. Anterior bowing posterior
maxillary wall (holman miller
sign)
3. Mass in nose, pterygopalatine
fossa
4. Widening of SPF
5. Erosion of bone at root of
pterygoid plate
Features:
1. T1 low/intermediate signal
intensity
2. T2 hyperintense

55. 12. What are
material for
embolization?
Temporary (Short-term):
1. Gelfoam/gelatin (Absorbable),Collagen,
Thrombin
Permanent (Long Term):
1. Particles: PVA, embospheres
2. Coils (steel/platinum):
Pushables,injectables,detachables
3. Liquids: Glue,alcohol, algel
Autologous (Outdated):
1. Clots,muscle fragments,fascia,dura

56. 13. What are
possible
complication of
embolization in
this case
• Immediate complications of embolization are pain,
embolization of normal vessels, hypersensitivity.
• Delayed complications include fever, pain and infections
• Unintended occlusion lead blindness (From ophthalmic
artery blockage)
• Cranial nerve palsy (Unintended occlusion of vasa
nervorum vessels – nutrient vessel, source of nutrition
that supplies each peripheral nerve from adjacent
blood vessels)
• CVA

57. 14.
Contraindication
embolization?
1. Feeding vessels feed vital structures e.g
brain,eye,spinal cord
2. Complicated vascular anatomy (e.g
exaggerated vascular tortuosity)
3. Significant atherosclerotic disease
4. Life threatening allergy to contrast
5. Coagulation disorder
6. Sepsis

58. 15.
Treatment

59. Choice of
treatment:
Surgical,
Radiotherapy,
Chemotherapy,
Hormonal
• 1. Gold standard : Surgical, with preoperative
embolization
• 2. Approach surgical: Endoscopic vs Open
approach

60. Endoscopic
transnasal Middle
turbinectomy may
be performed for
improved exposure
Middle meatus
antrostomy
Resection of
posterior
maxillary wall
Sphenopalatine
artery ligation
Tumour resection
from
pterygopalatine
fossa

61. Endoscopic
approach.
Pros vs Cons
Pros Cons
Avoid facial and oral incision Restricted assess
Rapid healing Unable to remove to tumour that completely
obliterate nasal cavity and not compressible
Avoid osteotomy, periosteal dissection that
may interfere growth of facial skeleton
Cannot be remove in stage 3 (Fisch)
Shortened hospital stay
Avoid complication related to open surgery:
trimus

62. List of
open
approach

63. List of open
approach
Facial translocation
1. Midface Degloving with Maxillary
Osteotomies
2. Gingivobuccal incision
3. Nasal intercartilaginous incisions with
transfixion incision
4. Soft tissue elevation
5. Le Fort I Osteotomy
Medial maxillectomy
Infratemporal fossa with or without
craniotomy

64. List of open
approach
Alternative approaches:
1. Lateral rhinotomy
2. Weber-Ferguson incision
3. Weber-Ferguson with Lynch extension
4. Weber-Ferguson with lateral subciliary
extension
5. Weber-Ferguson with subciliary
extension and supraciliary extension

65. Surgical
Planning
(Hossenini
2005)- Base
on Rodkowsi
stage
Smaller tumors (Class IA, IB, IIA, IIB, IIC)
1. Trans-nasal
2. Transpalatal
3. Transantral, for lesions extending laterally up
to pterygopalatine fossa
Larger tumors (IIIA, IIIB)
1. Lateral rhinotomy
2. Midface degloving
3. More extensive resections have higher
associated morbidity, however, limited
resections have a higher rate of recurrence

66. Summary
Endoscopic approach
vs Open approach
Endoscopic approach Open approach
Useful in tumour limited to
nasopharynx, nasal cavity, ethmoid and
sphenoid sinus
Transpalatal sublabial (sardana’s
approach)
-if tumour extend to pterygoid & ITF
Suitable for stage 1, 2, 3a Transzygomatic (sami & Girgis 1965)
-if tumour involve the temporal & ITF
Midfacial degloving (conley 1979)
- Most common open approach
- Mainly use for bilateral tumour
Lateral rhinotomy (moure’s incision)
-suitable for tumour restricted to
nasal cavity
ITF approach
-tumour extend laterally
Maxillary swing approach
Transcranial approach

67. 16. Role of Radiotherapy,
Chemotherapy and Hormonal
therapy.
• Radiation therapy has been shown to decrease or
eliminate tumor growth.
• May be useful in treating large, aggressive, or multiple
recurrent tumor in the postoperative.
• External beam radiation -> total tumour dose of 30-55
Gy.
• Radiotherapy can produce some amount of tumour
regression by radiation vasculitis and occlusion of
vessels by perivascular fibrosis.
• Recurrent rate 20%
• Complication: growth retardation, panhypopituitarism,
temporal lobe necrosis, radiation keroratopathy,
secondary malignancy.

68. Chemotherapy
• Chemotherapy has been reported to have
limited role for JNA and can be offered for
patients with extensive disease not
amenable for surgery or radiation therapy.
• If recurrent after resection and failed
radiation

69. Hormonal
therapy

70. Surveillance
• Follow up: Yearly CT/MRI scan to look for
recurrence + Nasal endoscopic assessment.
• Risk of recurrence : More likely in advanced
disease, younger patients, tumour in the
basisphenoid

71. Take home
message
• ‘Holman Miller’ sign is not always a sign for JNA.
• It requires a high index of suspicion to diagnose
JNA.
• JNA mimics other sino-nasal diseases, and
incorrect approach may result in a lethal
consequence.
• Multiple modalities like CT, MRI and angiography
helps to achieve the diagnosis of JNA.
• A biopsy must be avoided due to risk of fatal
haemorrhage.

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