Detail description of pathology & management of JNA.

1. JNA : SURGICAL APPROACHES & NEWER
TREATMENT OPTIONS
DR UTKAL MISHRA
AIIMS, BHOPAL

2. JUVENILE NASOPHARYNGEAL ANGIOFIBROMA
 Most common benign tumor of nasopharynx.
 Seen almost exclusively in Adolescent Males of 10-20 years
 It is encapsulated , slow-growing ,vascular tumor
 Although benign it is locally aggressive and has a high recurrence
rate

3. EPIDEMIOLOGY
 Accounts for 0.05 to 0.5% of all head & neck tumours.
 Intracranial extension found in 20 % cases.
 Incidence – 1/6000 Harma et al to 1/50,000 Hondousa et al
 In India incidence is increasing.

4. PATHOLOGY
 Gross : - Sessile, Firm, Lobulated, Pink – Red in colour
 Histology : -
1. Encapsulated, composed of vascular tissue & fibrous stroma.
2. Vessels are thin-walled, endothelium lined with no muscle or elastic coat.

5. THEORIES OF ORIGIN
 Ringertz theory: JNA always arose from the periosteum of the skull base.
 Bensch & Ewing (1941): Origin from embryoninc fibro cartilage between the basi occiput and basi sphenoid.
 Brunner (1942): Origin from conjoined pharyngobasilar and buccopharyngeal fascia.
 Marten (1948): Tumors resulted from deficiency of androgens or over activity of estrogens
 Sternberg (1954): Hamartoma
 Osborn (1959): Hamartomatous origin
 Girgis & Fahmy (1973): They considered JNA to be a paraganglionoma.
 Mild & Mauris theory: Origin from midline erectile tissue/ androgen dependent hamartoma

6. SITE OF ORIGIN
 Most common site - Superior Margin Of Sphenopalatine Foramen
 Pterygoid wedge
 Vidians canal
 Basisphenoid

7. EXTRANASOPHARYNGEAL ANGIOFIBROMA
 Do not originate from the area around the sphenopalatine foramen.
 Common in older Females
 Less vascular
 Commonest site – Maxillary sinus
 Other sites – Ethmoid sinus, Inferior Turbinates, Frontal Recess, Tonsil, RMT

8. MOLECULAR ANALYSIS
 Androgen receptors - 75%
 VEGF – 80%
 Progesterone receptors
 SOMATOSTATIN Receptor (SSTR 2)
 IGF II
 APC gene - 25 times more frequent in FAP patients
 ß catenin
 CD 34
 Loss of expression of GSTM 1

9. CLINICAL FEATURES
 Commonest Symptom - Profuse, Unprovoked, Recurrent and Spontaneous
Epistaxis.
 Progressive nasal obstruction and denasal speech
 Conductive hearing loss and otitis media with effusion.
 Mass in the nasopharynx, Palatal Bulge
 Broadening of Nasal Bridge, Proptosis, Swelling of Cheek

10. EXAMINATION OF NOSE
 Smooth Reddish Lobulated mass filling the nasal cavity & choana at times.
 Accumulations of secretions anterior to mass – CHOANAL BANKING EFFECT
 DNS to contralatertal side may be present.

11. SPREAD OF JNA
Sphenopalatine
foramen
Pterygopalatine
fossa
Infratemporal
fossa
Inferior orbital fissure
Orbit
Maxillary sinus
Cheek
Sphenoid sinus
Middle Cranial
fossa
Pituitary
Cavernous sinus
Nasal
cavity
Nasopharyn
x

12. SIGNIFICANCE OF PTERYGOID WEDGE
 It is defined as the anterior junction of the medial & lateral pterygoid plates.
 Involvement of pterygoid wedge is found in 99% cases.
 Pterygoid wedge is the Epicenter of tumour.
 Most common site of residual & recurrent disease – pterygoid wedge (45%)
 Most important step in JNA surgery to prevent recurrence - Drilling of pterygoid wedge

14. FISCH STAGING
Courtesy : Scott Browns Otolaryngology & Head & Neck Surgery 7th
edition

15. RADKOWSKI STAGING
Courtesy : Scott Browns Otolaryngology & Head & Neck Surgery 7th
edition

16. OTHER STAGING SYSTEMS
 Andrews staging
 Chandlier’s staging
 Session’s staging
 Onerci staging
 Tondon staging

18. DIAGNOSIS
 BIOPSY CONTRAINDICATED
 Investigation of choice – Contrast Enhanced CT scan
 MRI – Intracranial extension, Orbit, Infratemporal fossa
 Carotid Angiography with Embolization

20. HOLMAN MILLER SIGN

21. HONDOUSA SIGN
 HONDOUSA SIGN – Widening of gap between ramus of
mandible & maxillary body

22. RAM HARAN SIGN
 RAM HARAN SIGN – Quadrilateral appearance of pterygoid wedge

23. CHOP STICK SIGN
 CHOP STICK SIGN – Post op appearance of medial & lateral pterygoid plates as
two separate sticks due to drilling & removal of pterygoid wedge.

24. MRI
 Characteristic – Salt & Pepper appearance due to flow voids
 It aids in differentiation of tumour in – Orbit , Cavernous sinus , Middle cranial fossa , Infratemporal
region

25. MRI

26. DIGITAL SUBSTRACTION ANGIOGRAPHY
 Commonest feeding vessel – Internal Maxillary Artery
 In large tumours –
1. Ascending Pharyngeal Artery
2. Contralateral ECA branches
3. ICA branches - Ophthalmic, Meningo-hypophyseal, Vidian Artery

28. EMBOLIZATION
 Planned 24-48 hrs before surgery to avoid revascularization.
 No anesthesia required for cooperative patients
 Done under DSA guidance.

29. DISADVANTAGE
 Advantage – Reduction in blood loss, Less operative time, Improved visualisation of tumour margins
 Disadvantage –
1. Neurological complications, - Stroke, Cranial N. palsy, Blindness
2. Recurrence
3. Friable
4. Obscure tumour front in cracks & crevices.

30. TYPES
 2 types –
1. TRANSARTERIAL EMBOLIZATION WITH PVA
2. DIRECT PERCUTANEOUS EMBOLIZATION WITH ONYX –
Advantage : Solidifies slowly & infiltrates small vessels with excellent penetration of parenchyma

32. TREATMENT MODALITIES
 Surgery – Treatment of choice
 Radiotherapy
 Hormonal therapy
 Chemotherapy

33. PRINCIPLES OF JNA SURGERY
 Analyze the coronal CT thoroughly & plan the approach.
 Adequate tumour exposure.
 Don’t touch the tumour until feeding vessels are controlled.
 Drilling of pterygoid wedge is must.

34. ANAESTHETIC CONSIDERATIONS
 TIVA – Ramifentanyl + Propofol
 Controlled hypotension by Nitroglycerine infusion
 Maintain MAP → 60 – 70 mm Hg
 Positioning – Reverse Trendelenberg position

35. SURGICAL
APPROACHES
ENDOSCOPIC
APPROACH
OPEN
APPROACH

36. TREATMENT

37. ENDOSCOPIC APPROACH
INDICATIONS -
 Fisch 1& II tumours
 Fisch III tumours with limited medial invasion of infratemporal fossa

38. BINOSTRIL 4 HANDED SURGERY
 1 st described by – MAY et al in 1990.
 Posterior septectomy done as 1st step.
 Requires 2 surgeons
 Surgeon 1 – Holds endoscope at 11 o clock position + Irrigation
 Surgeon 2 – Suction same nostril + Instruments opposite nostril

39. ENDOSCOPIC ENDONASAL TECHNIQUE
 Nose is prepared with 4% Cocaine & adrenaline 1:10,000
 Resection of anterior end of middle turbinate
 Anterior ethmoidectomy + Removal of medial wall of maxillary sinus
 Removal of posterior wall of maxillary antrum to achieve complete lateral exposure of tumor
 Ligating SPA + DPA
 Dissection continues till rostrum of sphenoid
 Tumor is peeled inferiorly
 Drilling of basisphenoid & pterygoid wedge to remove residual tumour.

40. MODIFIED DENKERS APPROACH

41. THE FOUR-PORT BRADOO TECHNIQUE
 4 ports –
(A) The ipsilateral nostril.
(B) The contralateral nostril after doing a posterior septectomy.
(C) An antral window in the canine fossa.
(D) An incision of one inch in the gingivobuccal sulcus adjacent to the last molar.
 Advantage – Avoids removal of frontonasal process of maxilla

42. POST OP MANAGEMENT
 Merocel pack removed after 48 hrs.
 Saline irrigation started after pack removal
 Endoscopic cleaning of nose every weekly until crusting subsides.
 CECT done after 36 hrs to rule out residual disease.

43. FOLLOW UP
 Endoscopic examination of nose every 3 months
 Routine CECT every year for at least 3 years

44. OPEN APPROACHES
1. Transpalatine
2. Transpalatine + Sublabial (Sardana’s approach)
3. Lateral rhinotomy with medial maxillectomy
4. Midfacial degloving approach
5. Transmaxillary (Le Fort I) approach
6. Maxillary swing approach or facial translocation approach (Wei’s operation)
7. Infratemporal fossa approach
8. Intracranial–extracranial approach

45. WILSONS TRANSPALATAL APPROACH
 Indication - For tumour restricted to nasopharynx
 Advantage – Excellent cosmesis
 Disadvantage – Limited exposure, Palatal fistula

46. WILSONS TRANSPALATAL APPROACH

47. LATERAL RHINOTOMY + MEDIAL MAXILLECTOMY
 Suited for growth in nasal cavity extending to maxillary sinus, pterygopalatine
fossa, medial part of infratemporal fossa.
 Advantage – Wide exposure, Feeding vessels easily controlled
 Disadvantage – Scar, Bleeding

48. LATERAL RHINOTOMY + MEDIAL MAXILLECTOMY

49. MIDFACIAL DEGLOVING APPROACH
 4 INCISIONS –
1. Sublabial incision 3rd molar
2. Transfixation incision
3. Intercartilagenous incision
4. Circumvestibular incision
 Commonest complication – Vestibular stenosis, Infraorbital N.
Injury

50. LE FORT 1 OSTEOTOMY
 Wide access to Nasopharynx, Maxillary sinus, Sphenoid
sinus
 Complication – Malocclusion, Necrosis of maxilla

51. MAXILLARY SWING OR FACIAL TRANSLOCATION (WEI’S)

53. HEMOSTASIS IN JNA
 Reverse trendelenberg position with 200 head elevation – Improves venous drainage from brain.
 Direct pressure
 Liga clips
 Bipolar forceps
 Warm saline irrigation 400c
 1:1000 topical adrenaline
 Surgicel
 Floseal – Bovine Collagen + Human Thrombin

54. MANAGEMENT OF ICA INJURY
 Don’t panic Don’t pack
 Use 2 suctions
 1 – 2 cm3 muscle harvested from thigh or abdomen
 Crushed & placed over bleeding point for atleast 3-5 min. → Activates platelet fibrin plug
 Reinforce with surgicel
 If still not controlled → Endovascular intervention by angiography team

55. TRIGEMINO-CARDIAC REFLEX
 Characterized by –
1. Bradycardia / Asystole
2. Hypotension
3. Apnea
4. Gastric Hypermotility
 Incidence – 4 %
 Cause – Manipulation of PPF, ITF, NP Mucosa
 To prevent – 4% Xylocaine pack in PPF , ITF
 If occurs – Stop all manipulation, IV Crystalloids, wait for 10-15 min

56. EARLY POST OP
 Nasal Crusting
 Orbital hematoma
 Infraorbital nerve paraesthesia

57. LATE COMPLICATIONS
 Alar collapse – Modified denkers due to drilling of pyriform aperture
 Vestibular stenosis
 Fistula of palate
 Caroticocavernous fistula
 Recurrence

58. RECURRENCE
 Defined as subsequent tumour after negative immediate post op scan at 36 hours
 Incidence – 32 %
 Factors responsible-
1. Extensive Disease
2. Young Age
3. Pre op Embolization
4. Inexprienced Surgeon
 MOST IMPORTANT STEP TO PREVENT RECURRENCE – Drilling the cancellous bone of pterygoid wedge

60. INDICATIONS
 Extensive primary disease with intracranial extension
 Unresectable residual disease
 Medically unfit

61. TYPES
 Megavoltage EBRT
 IMRT
 GAMMA KNIFE & CYBER KNIFE

62. DOSE
 3000 to 5500 cGy in 15–18 fractions is delivered in 3–3.5 weeks.
 Tumour regression is very slow (over 2-3 year).
 Tumor regression by radiation vasculitis and occlusion of vessels by perivascular fibrosis.

63. COMPLICATIONS
 Occular – Cataract, Glaucoma, Endophthalmitis, Optic N. Atrophy
 Cranial N. Palsy
 Pan Hypopituitarism
 Temporal lobe necrosis
 Malignant transformation of JNA
 Xerostomia, Hyposmia, Crusting

64. HORMONAL THERAPY
 Flutamide - 10mg/kg/day in 3 divided doses x 6 weeks – 44% tumour shrinkage
 Diethylstilbestrol – 5 mg TID
 Bevacizumab – Mab against VEGF
 Sirolimus / Rapamycin

65. CHEMOTHERAPY
 Doxorubicin
 Dacarbazine
 Vincristine
 Dactinomycin
 Cyclophophamide
 Cisplatine

66. THANK
YOU