Juvenile nasopharyngeal angiofibroma.pptx

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

5. 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.

6. Endoscopic anatomy Juvenile nasopharyngeal angiofibroma – narayanan janakiram

7. Fisch staging of jna

8. Path of spread

9. 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,

10. 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

11. 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

12. 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

13. 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

14. 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

15. Involvement of pterygoid wedge and sphenoid Juvenile nasopharyngeal angiofibroma – narayanan janakiram

16. Tumour involving pterygoid wedge , nasopharynx and sphenoid Juvenile nasopharyngeal angiofibroma – narayanan janakiram

17. Minimal involvement of pterygopalatine fossa

18. Cont .. Juvenile nasopharyngeal angiofibroma – narayanan janakiram

19. Pterygopalatine fossa/ infratemporal fossa

20. Cont …. Juvenile nasopharyngeal angiofibroma – narayanan janakiram

21. Extension to cheek – modified endoscopic denkers procedure

22. Cont..

23. Cont..

24. 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

25. Open approaches Lateral rhinotomy approach Transpalatal approach, Sublabial trans-maxillary approach Maxillary swing Midfacial de-gloving ( LeFort I) Infratemporal fossa resection Also , several combinations

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

27. 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

28. Osteotomies of medial maxillectomy

29. Medial maxillectomy Early complication Late complication Haemorrhage Orbital oedema CSF Leak Meningitis Vestibular stenosis Diplopia Epiphora Frontal sinus obstruction and mucocele

30. 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

31. Midfacial degloving

32. Midfacial degloving Early complication Late complication Haemorrhage Facial bruising Infraorbital paresthesia Vestibular stenosis Oro antral fistula Epiphora Septal perforation Upward tip rotation

33. Transpalatal approach This approach can be used for JNAs confined to the nasopharynx, sphenoid and nasal cavity

34. 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

35. 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.

36. 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

37. Infratemporal fossa approach Significant involvement of the infratemporal fossa, cavernous sinus, or middle cranial fossa requires infratemporal fossa or subtemporal approaches

38. 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.

39. 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%.

40. 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.

41. 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

42. complications Juvenile nasopharyngeal angiofibroma – narayanan janakiram

43. Intra – op complications Trigemino cardiac reflex- arterial hypotension , bradycardia, asystole ICA spasm Blood loss CSF Leak Cranial nerve palsy

44. Juvenile nasopharyngeal angiofibroma – narayanan janakiram

45. Post op complications Erosion of nares Custing of cavity Orbital haematoma Infra – orbital nerve paraesthesia Infection Collapse of ala Recurrence

46. Thank you