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.
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
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
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
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
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.
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
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
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