approach to a JNA patient

1. JUVENILE NASOPHARYNGEAL
ANGIOFIBROMA
Dr Safika Zaman
Dept of ENT and HNS
RKMSP, VIMS

2. INTRODUCTION
• JNA is a rare, benign, vascular tumour that
is almost exclusively found in the male
population.
• Origin is at the sphenopalatine foramen
or, vidian canal.
• Diagnosis is based on clinical and
radiological evaluation.

3. HISTOLOGY
JNA is an abundantly vascular
tumor in a fibrous connective
stroma that lacks a capsule.
A single layer of flat endothelial
cells line its vessels which only
sporadically contain smooth
muscle in their walls

4. BOUNDARIES

6. VASCULAR ANATOMY
• JNAs typically arise from the
sphenopalatine artery, which is a
terminal branch of the internal maxillary
artery.
• Larger tumours - ascending pharyngeal,
contralateral internal maxillary artery,
cavernous portion of the internal carotid
artery.

7. ENDOSCOPIC ANATOMY
Juvenile nasopharyngeal
angiofibroma – narayanan
janakiram

8. FISCH STAGING OF JNA

9. PATH OF SPREAD

10. GENERAL SURGICAL PRINCIPLES
JNAs may be resected by endoscopic, open or combined (endoscopic & open)
techniques
The surgical approach is dependent on - Tumour location and extent
Pattern of vascular supply
Complete all bone work and ensure good access to the tumour before attempting
resection,

11. PATIENT PREPARATION AND ANAESTHESIA
• Pre – op optimization of haemoglobin
• Premedication – anxiolytic , H2 blocker, clonidine / beta blockers
• Positioning – reverse Trendelenberg position
• Controlled hypotension – MAP – around 70 mm o hg , SBP- around 90 mm of hg

12. EMBOLIZATION
Advantage Disadvantage
Reduction in blood flow
Decreased intraoperative time
Improved visualization of tumour margins
Risk of stroke , pain
Cranial nerve injury
Blindness
Changes the consistency of the tumour, lead to
residual tumour

13. EMOLIZATION
Procedures – Trans-arterial embolization (digital subtraction angiography )
Percutaneous embolization
Timing – 24 to 48 hour before the surgery
Materials – particulate - poly vinyl alcohol
microspheres
gelfoam
Liquid – N- butyl 2 cyano acrylate
onyx
Coils

14. PRINCIPLES OF ENDOSCOPIC EXCISION
• Teamwork between – Surgeon ,
Anaesthesiologist , Interventional radiologist
• Bi-nostril four handed surgery – two
surgeons work in unison
• Wide exposure – to visualize tumour
margins, posterior septum is removed to
create a single cavity
Juvenile nasopharyngeal angiofibroma – narayanan janakiram

15. ADVANTAGES OF ENDOSCOPIC TECHNIQUE
• Effective technique
• Resulting in less disability
• Decreases in the duration of hospitalization
• Lower rates of intraoperative bleeding
• Avoidance of surgical scars on the face
• Resection of the least amount of normal soft tissue
• Avoidance of the destruction of facial bones and occurrence of late facial deformity
Juvenile nasopharyngeal angiofibroma – narayanan janakiram

16. INVOLVEMENT OF PTERYGOID WEDGE AND
SPHENOID
Wide MMA
Opening of sphenoid os
Ligation of septal branch of SPA
Removal o sphenoid part of tumour by traction
Pterygoid wedge is drilled to remove residual tumour
Juvenile nasopharyngeal
angiofibroma – narayanan
janakiram

17. TUMOUR INVOLVING PTERYGOID WEDGE ,
NASOPHARYNX AND SPHENOID
Inferior turbinectomy
Wide MMA
ethmoidectomy with sphenoidectomy
Tumour removal , Pterigoid wedge is drilled
Juvenile nasopharyngeal
angiofibroma – narayanan
janakiram

18. MINIMAL INVOLVEMENT OF
PTERYGOPALATINE FOSSA
Inferior turbinectomy
Partial resection of middle turbinate
Uncinnectomy , wide MMA, ethmoidectomy sphenoidectomy
Septectomy or septal window

19. CONT ..
Posterior wall of maxillary sinus is removed
Cauterization of SPA and decending palatine artery
Dissecting tumour from ethmoid , sphenoid and PPF
Dissection of tumour around besi- spenoid attachment site – drilling of
pterygoid wedge
Juvenile nasopharyngeal
angiofibroma – narayanan
janakiram

20. PTERYGOPALATINE FOSSA/ INFRATEMPORAL
FOSSA
Inferior turbinectomy
Partial resection of middle turbinate
Medial maxillectomy
Ethmoidectomy with sphenoidectomy
Posterior septectomy

21. CONT ….
Removal of posterior wall of maxillary sinus by drilling
Upper dissection of tumour from inferior orbital nerve,
lateral dissection from infratemporal fossa , ppf
Segmental resection anterior to pterygoid wedge , drilling
of wedge
Juvenile nasopharyngeal
angiofibroma – narayanan
janakiram

22. EXTENSION TO CHEEK – MODIFIED
ENDOSCOPIC DENKERS PROCEDURE
Anterior edge of pyriform aperture is incised
Expose of anterolateral wall of maxilla
Removal of anterolateral wall of maxillary sinus

23. CONT..
Medial maxillectomy
Removal of posterior wall of maxillary sinus by drilling
Identification and ligation of internal maxillary artery

24. CONT..
Removal of tumour from ITF and cheek by applying gentle
traction
Dissection of tumour from all attachments
Pterygoid wedge drilling

25. HEMOSTASIS IN JNA
Methods Agents
Mechanical methods Direct pressure
Liga clips
Thermal methods Bipolar electrocautery
Warm saline irrigation
Chemical methods Adr soaked gauze packing
Cellolose
Gelatin thrombin products
Fibrin sealants
Albumin and glutaraldehyde
Cyanoacrylate

26. OPEN APPROACHES
• Lateral rhinotomy approach
• Transpalatal approach,
• Sublabial trans-maxillary approach
• Maxillary swing
• Midfacial de-gloving (LeFort I)
• Infratemporal fossa resection
• Also , several combinations

27. CONSIDERATION OF OPEN APPROACH
Lateral infratemporal fossa extension
Intradural extension,
Orbital nerve involvement

28. MEDIAL MAXILLECTOMY
• suited to tumours limited
to the nose, nasopharynx,
sphenoid, pterygopalatine
fossa, medial infratemporal
fossa and medial cavernous
sinus
OPEN ACCESS ATLAS OF OTOLARYNGOLOGY, HEAD &
NECK OPERATIVE SURGERY
JUVENILE NASOPHARYNGEAL ANGIOFIBROMA SURGERY
Derek Rogers, Christopher Hartnick, Johan Fagan

29. OSTEOTOMIES OF MEDIAL MAXILLECTOMY
Inferior orbital rim
Across frontal process of maxilla
Osteotomy along floor of nose
Osteotomy along lacrimal bone and ethmoid
Posterior wall of antrum to pterygopalatine fossa

30. MEDIAL MAXILLECTOMY
Early complication Late complication
Haemorrhage
Orbital oedema
CSF Leak
Meningitis
Vestibular stenosis
Diplopia
Epiphora
Frontal sinus obstruction
and mucocele

31. MIDFACIAL DEGLOVING-LA FORT 1
• Le Fort 1 osteotomy with
down-fracturing of the
palate is suited to tumours
limited to the nose,
nasopharynx, sphenoid,
pterygopalatine fossa,
medial infratemporal fossa
and medial cavernous
sinus

32. MIDFACIAL DEGLOVING

33. MIDFACIAL DEGLOVING
Early complication Late complication
Haemorrhage
Facial bruising
Infraorbital paresthesia
Vestibular stenosis
Oro antral fistula
Epiphora
Septal perforation
Upward tip rotation

34. TRANSPALATAL APPROACH
• This approach can be used for
JNAs confined to the
nasopharynx, sphenoid and
nasal cavity

35. Incision is made in the mucosa of the hard palate
Thick mucosa is stripped off the hard palate,
leaving it attached to the soft palate posteriorly
The soft palate is freed from the posterior edge of
the hard palate to access the nasopharynx.
The horizontal plate of the palatine bone is
removed using Kerrison’s rongeur /drill to expose
the JNA

36. TRANSPALATAL APPROACH
Advantage Disadvantage
Minimal bone removal and no facial incision Tracheostomy requirement
Palatal dehiscence and oronasal fistula.
Limited lateral access to the parapharyngeal space and
risk of palatal fistula.

37. MAXILLARY SWING
• A Weber–Ferguson–Longmire incision is
made.
• The vertical incision limb goes through the
upper lip and is continued between the
central incisors and onto the hard palate.
• The osteotomy is started at anterior maxilla
inf to orbital rim, midline at hard palate, at
ant. Zygoma and finally between post
maxillary wall and pterygoid plate.
• The entire maxilla can be swung laterally
attached to the cheek flap and the masseter
muscle

38. INFRATEMPORAL FOSSA APPROACH
• Significant involvement of the
infratemporal fossa, cavernous sinus, or
middle cranial fossa requires
infratemporal fossa or subtemporal
approaches

39. CONT
• A postauricular C-shaped incision is made that extends superiorly into the temporal
region and inferiorly into the neck .
• The temporalis muscle, mastoid and zygoma are exposed. A periosteal flap is
elevated and the external auditory canal is transected and closed as a blind sac.
• The pinna and skin flap are reflected anteriorly.
• As a precaution, the neck is dissected so that control of the carotid and jugular
vessels can be achieved.

40. CONT
Advantage Limitation
Wide access to infratemporal, parasellar and temporal
region
Direct approach
Short working distance.
Temporary post-operative trismus and malocclusion
Hypaesthesia of the lower half of the face and ipsilateral
tongue (V3)
Permanent post-operative conductive hearing loss
Temporary frontal facial paresis in 30%.

42. RADIATION
• Radiotherapy is primarily an adjuvant
treatment in the setting of residual or
recurrent disease.
• Primary treatment modality if a tumor is
deemed unresectable based on its extent
of invasion and the critical structures that
it involves
• Various case series involving radiotherapy
treatments prescribe a typical dose range
of 30 to 50 Gy, at 1.8 to 2 Gy per daily
fraction.

43. RADIOTHERPY
• External beam radiation is used in the form of IMRT in a
conformal technique to limit radiation exposure and doses to
nearby optic nerves/optic chiasm, lens, retina,
brain/brainstem, spinal cord, and salivary glands as compared
to conventional radiotherapy.
• Stereotactic radiotherapy/radiosurgery has also been used

44. COMPLICATIONS
Juvenile nasopharyngeal
angiofibroma – narayanan
janakiram

45. INTRA – OP COMPLICATIONS
• Trigemino cardiac reflex- arterial hypotension , bradycardia, asystole
• ICA spasm
• Blood loss
• CSF Leak
• Cranial nerve palsy

46. Juvenile nasopharyngeal
angiofibroma – narayanan janakiram

47. POST OP COMPLICATIONS
• Erosion of nares
• Custing of cavity
• Orbital haematoma
• Infra – orbital nerve paraesthesia
• Infection
• Collapse of ala
• Recurrence

48. Thank you