Juvenile nasopharyngeal angiofibroma

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Juvenile nasopharyngeal angiofibroma

  1. 1. JUVENILE NASOPHARYNGEAL ANGIOFIBROMA - Dr. Praneeth
  2. 2. Introduction  Uncommon, benign and extremely vascular tumour  Up to 0.5% of head and neck tumours  Occurring almost exclusively in males  Average age of onset - 15 years old JNA Facts and Statistics  Intracranial Extension between 10-20%  Recurrence Rates as high as 50% Origin -  Posterolateral nasal wall at the sphenopalatine foramen
  3. 3. Anatomy Sphenopalatine foramen –  Contents – sphenopalatine artery, nerves – nasopalatine, posterior superior nasal.
  4. 4. Anatomy cont…  Locally invasive.  Spread – submucosally Extension -  Medially into nasopharynx or nasal cavity  Laterally into  Infratemporal fossa middle cranial fossa  Pterygopalatine fossa infraorbital fissure orbit
  5. 5. Anatomy cont… Vascular supply –  Most commonly from internal maxillary artery  Also : internal carotid, external carotid, common carotid, ascending pharyngeal
  6. 6. Anatomy cont… Fossa of rosenmuller –  Situated at the corner between lateral and dorsal walls of nasopharynx.  Not obvious in infants  Measures upto 1.5 cm in depth in adults  Opens into nasopharynx at a point below foramen lacerum.
  7. 7. Anatomy cont… Boundaries –  Ant – Eustachian tube & Levator veli palatini  Post – Pharyngeal wall mucosa overlying pharyngobasilar fascia & retropharyngeal space containing nodes of rouviere  Med – nasopharyngeal cavity  Posterolateral (apex) – carotid canal opening and petrous apex posteriorly, foramen ovale & spinosum laterally  Lat – Tensor veli palatini, mandibular nerve, prestyloid compartment of parapharyngeal space.
  8. 8. Anatomy cont… Roof of the fossa of rosenmuller –  Formed by foramen lacerum  Structures passing near it –  Internal carotid artery  Greater superficial petrosal nerve  Ascending palatine artery  Through this foramen, tumours from fossa of rosenmuller invades intracranial structures.  III, IV, V, VI cranial nerves are located near to this foramen, which are commonly involved when tumours invade intracranial structures via the foramen lacerum.
  9. 9. PATHOGENESIS
  10. 10. Pathology Macroscopic –  Well-defined, spongy lobulated tumours with nodules covered by nasopharyngeal mucosa (squamous epithelium).  Nodularity increases with age.  Color varies from pink (part seen in nasopharynx) to white or grey (extrapharyngeal areas).  On section, tumour is reticulated, whorled or spongy in appearance lacking a true capsule.  Edges are sharply demarcated and easily distinguishable from the surrounding tissues.
  11. 11. Pathology cont… Microscopic –  Consists of proliferating, irregular vascular channels within a fibrous stroma.  Vascular component is more in young tumours and as age increases, collagen content increases.  Fibrous tissue increases towards periphery and vascular element tends to be more central.
  12. 12. Pathology cont…  Tumour blood vessels typically lack smooth muscle and elastic fibres, this is the reason for sustained bleeding.  Cellular infiltration with plasma cells, lymphocytes, polymorphs, eosinophils can be present.  Stromal compartment consists spindle or stellate plump cells that give rise to varying amounts of collagen which makes some tumours very hard or firm.  Mucous glands can be seen in superficial parts of the tumour underneath the epithelial covering.
  13. 13. Pathogenesis  This tumour is almost exclusively found in adolescent boys, so there is much speculation and indirect evidence that sex-hormone receptors play some part in its development. Theories associated with its aetiopathogenesis -  Ringertz theory (1938) – JNA always arose from the periosteum of the skull base.  Som & Neffson (1940) – inequalities in the growth of bones forming skull base resulted in hypertrophy of the underlying periosteum in response to hormonal influence.  Bensch & Ewing (1941) – tumour probably arose from embryonic fibro cartilage between basi-occiput and basi-sphenoid.  Brunner (1942) – suggested origin from conjoined pharyngobasilar and buccopharyngeal fascia.
  14. 14. Pathogenesis cont…  Marten et al (1948) – proposed a hormonal theory suggesting that these tumours resulted from deficiency of androgens or overactivity of estrogens and that the hormonal stimulation is responsible for angiomatous components seen in JNA.  Sternberg (1954) – proposed that JNA could be a type of hemangioma like a cutaneous hemangioma seen in children which regresses with age.  Osborn (1959) – it could be due to either a hamartoma or residual fetal erectile tissue which were subjected to hormonal influence.  Girgis & fahmy (1973) – observed cell nests of undifferentiated epitheloid cells or “zellballen” at the growing edge of angiofibromas and so considered it as a paraganglioma.
  15. 15. Pathogenesis cont…  The most accepted theory is that JNAs originate from sex steroid – stimulated hamartomatous tissue located in the turbinate cartilage. The proposed hormonal influence explains why some JNAs involute after puberty. Recent immunocytochemical techniques shown that  androgen receptors are present in at least 75 percent of tumours, these receptors are present in both the vascular and stromal elements.  progesterone receptors were found in some.  In contrast, oestrogen receptors have not been demonstrated ?
  16. 16. Pathogenesis cont… Other factors that play a role in the growth of this tumour are  The angiogenic growth factor (vascular endothelial growth factor (VEGF)) has been found localized on both endothelial and stromal cells, indicating both cell types play a role in tumour development.  Vessel density and both the expression and localization of VEGF correlate with the proliferative marker Ki67.  But both of them do not have any relation to its degree of aggressiveness.  Overexpression of insulin-like growth factor II (IGFII)  found in a large number of juvenile angiofibromas.  IGFII gene is situated on the chromosome 11q.  associated with a tendency to recurrence and poorer prognosis.
  17. 17. Pathogenesis cont…  Sporadic juvenile angiofibromas develop 25 times more frequently in patients with familial adenomatous polyposis (FAP), a condition that is associated with the germline mutations of adenomatous polyposis coli (APC) gene present on chromosome 5q.  This gene regulates the beta-catenin pathway which influences cell to cell adhesion.  Mutations of beta-catenin have been found in recurrent juvenile angiofibromas also.  Beta-catenin localised only in the nuclei of stromal cells suggest that these cells have a critical role in the development of these neoplasms.
  18. 18. Pathophysiology  The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx, usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate.  Fetal histology confirms large areas of endothelial tissues in this region.  Rather than invading surrounding tissue, this tumour displaces and distorts, relying on pressure necrosis to destroy and push through its bony confines.  Intracranial extension is noted in 10-20% of cases.  JNA are seldom seen in children below the age of 8.  Rate of growth of tumour and period of maximum development coincides with rate of erectile tissue of penis, both increasing in size during the period of sexual development.
  19. 19. PRESENTATION
  20. 20. Clinical features  Nasal obstruction  Intermittent unprovoked epistaxes  Chronic anaemia may be present due to repeated epistaxis.  Complete nasal obstruction may cause stasis of secretions and may also lead to sepsis.  Patients may have hyposmia or anosmia. 2 cardinal symptoms
  21. 21. Clinical features cont…  Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down.  Blockage of ET orifice may cause deafness and otalgia.  Headache may be present due to chronic sinusitis history or intracranial extension.  Pressure on optic chaisma due to erosion of mass into the cranial cavity may cause diplopia.  Tenting of the optic nerve by tumour mass may cause failing vision ( as observed by shaheen et al).
  22. 22. Clinical features cont… On Anterior rhinoscopy –  Abundant purulent nasal secretions.  Bowing of nasal septum to uninvolved side. On Posterior rhinoscopy –  Pink or red mass filling the nasopharynx can be seen.
  23. 23. Clinical features cont…  When disease becomes extensive involving nose and infratemporal fossa, gross physical signs will be seen – Nasal bones become spayed out, Swelling in the temple and cheek occur.  Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum.  Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid.  Proptosis is seen if orbital fissures are penetrated.  Frog face appearance may be seen if tumour is extensively spread involving the ethmoidal region.
  24. 24. Extension of the tumour It follows the lines of least resistance –  It mostly arises from SPF area. It may have 2 components – one filling the nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa.  Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone.  1> hangs down in the nasopharynx and may depress the soft palate when large enough  2> may grow into I/L nasal passage towards anterior nares. It can cause pressure on lateral wall and also on septum bending it to opposite side. Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy. Lateral spread into the maxillary sinus may be responsible for the cheek swelling.  3> it can encroach into the orbit through infra orbital fissure.  4> it can erode skull base and cause intracranial problems.
  25. 25. ASSESSMENT
  26. 26. Investigations  Plain lateral view skull x-ray – Opacity in nose and PNS may be seen Mass in the nasopharynx can be seen Anterior bowing of the posterior wall of the maxillary sinus can be seen, called Holman-miller sign.  Now-a-days, the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography.  Biopsy is contra-indicated because of brisk haemorrhage.
  27. 27. Investigations cont…  The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection.  CT is excellent for bone detail. Both plain and contrast (lesion enhances) CT should be done. CT reveals the extent of the lesion and helps in staging of the disease.
  28. 28. Investigations cont…
  29. 29. Investigations cont… Coronal CT: bone window showing  Widening of left sphenopalatine foramen  Lesion filling left choanae  Lesion extending into sphenoid sinus
  30. 30. Investigations cont… Coronal CT: soft tissue window with contrast showing  Homogenous enhancement  Widening of left sphenopalatine foramen  Extension into nasopharynx and pterygopalatine fossa Axial CT: soft tissue window with contrast showing  Homogenous enhancement  Widening of right sphenopalatine foramen  Extension into nasopharynx and pterygopalatine fossa
  31. 31. Investigations cont… MRI reveals the precise extent of the mass. It differentiates tumour from other soft tissue structures.
  32. 32. Investigations cont… Axial MRI : T1 showing • Heterogenous intermediate signal • Flow voids representing enlarged vessels • Extension into nasopharynx and masticator space Coronal MRI : T1 with contrast showing • Diffuse intense enhancement • Multiple flow voids within hypervascular mass • Extension into nasopharynx and pterygopalatine fossa Axial MRI : T2 showing • Heterogenous intermediate to high signal enhancement • Multiple flow voids within hypervascular mass • Extension into nasopharynx and pterygopalatine fossa
  33. 33. Investigations cont…  Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively.
  34. 34. Staging systems Staging is done for prognosis and for therapeutic approaches. Several staging systems have been proposed –  Fisch  Billers  Andrews  Radkowski Fisch staging system –  most robust and practical.  defines clearly which surgical approach is required .
  35. 35. Staging cont…  Type 1 - Tumour limited to the nasopharyngeal cavity; bone destruction negligible or limited to the sphenopalatine foramen  Type 2 - Tumour invading the pterygopalatine fossa or the maxillary, ethmoid or sphenoid sinus with bone destruction  Type 3a - Tumour invading the infratemporal fossa or orbital region: without intracranial involvement  Type 3b - Tumour invading the infratemporal fossa or orbital region: with intracranial extradural (parasellar) involvement  Type 4a - Intracranial intradural tumour: without infiltration of the cavernous sinus, pituitary fossa or optic chiasma  Type 4b - Intracranial intradural tumour: with infiltration of the cavernous sinus, pituitary fossa or optic chiasma
  36. 36. Staging cont… Radkowski staging system  appeals to those involved with the management of smaller tumours as there are more subdivisions but, in reality, this adds little to its utility.  Type 1a - Limited to the nose and nasopharyngeal area  Type 1b - Extension into one or more sinuses  Type 2a - Minimal extension into pterygopalatine fossa  Type 2b - Occupation of the pterygopalatine fossa without orbital erosion  Type 2c - Infratemporal fossa extension without cheek or pterygoid plate involvement  Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids)  Type 3b - Erosion of the skull base with intracranial extension with or without cavernous sinus involvement
  37. 37. Staging cont… Billers staging system -  T1 - Tumour in nose and PNS except sphenoid with or without erosion of bone.  T2 – Tumour extension into orbit or protruding into anterior cranial fossa.  T3 – Tumour involvement in the brain that is resectable with margins.  T4 – Unresectable tumour
  38. 38. Staging cont… Andrews staging system – It is the currently accepted staging system.  Type I – tumour limited to the nasopharyngeal cavity, bone destruction negligible or limited to sphenopalatine foramen.  Type II – Tumour invading the pterygopalatine fossa or the maxillary, ethmoid or sphenoid sinus with bone destruction.  Type III – Tumour invading the infratemporal fossa or orbital margin  (a) without intracranial involvement  (b) with intracranial extradural (parasellar) involvement  Type IV – Intracranial intradural tumour  (a) without infiltration of cavernous sinus, pituitary fossa, optic chaisma  (b) with infiltration of cavernous sinus, pituitary fossa, optic chaisma Upto IVa, surgery is advisable and for IVb, radiotherapy is recommended.
  39. 39. TREATMENT
  40. 40. History Hippocrates (470-410 BC) removed a 'hard nasal polyp' through midline, nose splitting incision and later it was found to be juvenile angiofibroma. Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction.  It extended into his cheek and had eroded the alveolar process.  The patient had experienced a number of severe epistaxes, losing two to three pints of blood on each occasion.  He removed the tumour by performing a total maxillectomy through a Weber Fergusson incision without anaesthesia!  Histopathological examination of the operative specimen showed the tumour had a 'fibro-vascular nature:
  41. 41. Preoperative embolization  Its role is controversial.  For smaller tumours or if the feeder vessels are terminal branches of internal maxillary, then not needed.  For extensive lesions that get blood supply from branches of both ICA & ECA, then it is necessary.  For medium-sized tumours, the benefits of preoperative embolization are doubtful.  Intraoperative blood loss after embolization is definitely less.
  42. 42. Preoperative embolization cont…  The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure, regardless of whether embolization has been undertaken or not.  Recurrence rate is thought to be reduced by this but it was found to be increased.  May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection.
  43. 43. Preoperative embolization cont…  Done 24–72 hours pre-operatively.  Gelfoam or polyvinyl alcohol foam is used.  Gelfoam is resorbed in approximately 2 weeks.  Efficacy – stage 1 reduced from 840ml to 275ml blood loss.
  44. 44. Preoperative chemotherapy Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited. Gates et al observed 44% tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients.  Flutamide is regularly used in the management of prostatic cancer with side effects like nausea, breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy.  So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension).  Unfortunately, Labra et al observed shrinkage in only 7.5% cases in a pilot study and hence it was considered insignificant.
  45. 45. SURGICAL RESECTION
  46. 46. Approaches Various approaches are available to access the area –  Trans-nasal endoscopic  Trans-palatal (wilson)  Lateral rhinotomy  Transhyoid  Transmandibular (kermen)  Sublabial Midfacial degloving (conley)  Transzygomatic (sami & girgis)
  47. 47. Approaches cont… Combined approaches – A combination of various approaches can be attempted depending on the size and extent of the mass.  Trans-palatal sublabial (saldana)  Transpalatal transantral (denker)  Transcervical transmandibular (biller)  Transpalatal + lateral rhinotomy  Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc  Butterfly sub-brow incision or extended lateral rhinotomy  Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen approach for stage IV
  48. 48. ENDOSCOPIC ENDONASAL TECHNIQUES
  49. 49. Endoscopic endonasal  These techniques became more widespread recently as there are advantages like reduced intra-op blood loss, fewer post-op complications, reduced length of hospital stay.  Fisch - type 1, type 2 and some type 3 (with limited medial invasion of the infratem- poral fossa) are done through this technique.  Larger tumours and those extending across or through the skull base are difficult to remove through this technique.
  50. 50. Endoscopic endonasal cont…
  51. 51. Endoscopic endonasal cont… Procedure –  Preoperative embolization is undertaken.  After the induction of anaesthesia, the nose is prepared with a vasoconstrictor solution (4% cocaine or epinephrine 1:10,000).  The anterior end of the middle turbinate is resected.
  52. 52. Endoscopic endonasal cont…  An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done.  Access to the posterior wall of the antrum is gained.  This wall is then removed to achieve complete lateral exposure of the tumour.
  53. 53. Endoscopic endonasal cont…  Dissection is then continued into the sphenoid until its rostrum is reached.  Tumour is peeled off inferiorly.  A similar technique can be used to deliver the lateral extension of the tumour into the operative field.
  54. 54. Endoscopic endonasal cont…  Bipolar diathermy is used throughout the procedure.  Ligaclips are used to control the feeding blood vessels.  A second surgeon can be helpful in aiding the resection of larger tumours by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril.
  55. 55. OPEN APPROACHES
  56. 56. MIDFACIAL DEGLOVING APPROACH
  57. 57. Midfacial degloving  Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives.  It is a bilateral extended transnasal maxillary approach.  Anterior, medial and posterior walls of the maxillary antrum can be removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply.  Extensions into the inferior part of the orbit and infratemporal fossa can also be removed.  There is no visible scarring and so cosmetically most feasible.
  58. 58. Midfacial degloving cont…  A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus, and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches. • Gingivo buccal incision is given. • Nasal intercartilaginous incisions with transfixation incision is given.
  59. 59. Midfacial degloving cont… • Soft tissue elevation is done • Le fort I osteotomy is done. • Mass in the nasopharynx is now accessible for removal.
  60. 60. Midfacial degloving cont… Midfacial degloving approach
  61. 61. Midfacial degloving cont…
  62. 62. TRANSPALATAL APPROACH
  63. 63. Transpalatal Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat- eral to the soft palate on the left- hand side Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left- hand side Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
  64. 64. Transpalatal cont…  General anaesthesia is induced through an orotracheal tube.  A Dingman self-retaining retractor is used to expose the hard palate and the roof of the oral cavity.  An inverted U-shaped incision is outlined, extending from one maxillary tubercle to the other.
  65. 65. Transpalatal excision cont…  The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate.  Using a periosteal elevator, the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated.
  66. 66. Transpalatal excision cont…  The blood supply to the bipedicled flap is derived from the palatine arteries on each side, which are carefully preserved as the elevation of the flap approaches the soft palate.  With use of an osteotome or a highspeed drill with a burr, the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side.
  67. 67. Transpalatal excision cont…  The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx.
  68. 68. Transpalatal excision cont…  A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side.
  69. 69. Transpalatal excision cont…  A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor.
  70. 70. Transpalatal excision cont…  With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors, the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx.
  71. 71. Transpalatal excision cont…  Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate.  The blood supply usually is derived from the sphenopalatine artery, which is electrocoagulated for haemostasis.  The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx.
  72. 72. Transpalatal excision cont…  After the wound is irrigated, the surgical defect in the nasopharynx is left open to epithelialize by secondary intention.  The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures.
  73. 73. Transpalatal excision cont… monobloc excision of the bilobed tumor yellowish-white compact fibromatous tumor
  74. 74. Transpalatal excision cont… Postoperative Care  Intense humidification to avoid crusting and drying of clots in the nasal cavity.  Oral irrigations are started on the first postoperative day, and the patient is allowed to take liquids by mouth approximately 48 hours after surgery.  Irrigations are continued until full epithelialization of the mucosa in the raw areas in the nasopharynx occurs.  Postoperative aesthetic and functional results of this surgical procedure are excellent with no disability in swallowing, speech, or breathing.
  75. 75. Transpalatal excision cont…
  76. 76. MEDIAL MAXILLECTOMY APPROACH
  77. 77. Medial maxillectomy  Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary, ethmoid, or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach.  The patient presented with a history of nasal obstruction and epistaxis of 6 months’ duration. Appearance of the patient at the time of presentation
  78. 78. Medial maxillectomy cont…  Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx.  However,the posterior choana on the right-hand side is within normal limits.
  79. 79. Medial maxillectomy cont…  The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum.
  80. 80. Medial maxillectomy cont…  Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels.  During the venous phase of the angiogram, a highly vascular lesion is demonstrated.  Selective embolization of the feeding vessels is recommended when the angiogram is performed. Venous phase showing tumor blush indicating highly vascular nature
  81. 81. Medial maxillectomy cont…  The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy.  The eye is protected with a corneal shield.  A modified Weber-Ferguson incision with a Lynch extension is preferred.  The incision is extended through the soft tissues of the cheek to expose the anterior wall of the maxilla
  82. 82. Weber – ferguson incision  The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella.  It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity, remaining in the groove of the lateral aspect of the ala, and follows the alar subunit all the way up to the lateral aspect of the nose.  At this point, the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus.
  83. 83. Weber – ferguson incision  The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension. For the Lynch extension, the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow. A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
  84. 84. Medial maxillectomy cont…  An anterior wall antrotomy is made using a high-speed drill with a burr.
  85. 85. Medial maxillectomy cont…  The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum.  Care is taken to prevent injury to the infraorbital nerve, which is carefully preserved.  The opening in the anterior wall is extended up to the frontonasal process of the maxilla.  In a close-up view of the exposure obtained, the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity.
  86. 86. Medial maxillectomy cont…  Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side.  Because this tumor is benign and has a rubbery consistency, it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx.  The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor.  With use of appropriate digital maneuvers, the angiofibroma is removed.  Meticulous attention should be given to gradual, smooth, complete removal of all the lobulations of the tumor, because it is easy to fracture the tumor and leave parts of it behind.
  87. 87. Medial maxillectomy cont…  Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen.  However, as soon as the specimen is delivered, all bleeding can be controlled with packing and electrocoagulation.  The surgical defect shows a large, hollow space in the nasal cavity, nasopharynx, and maxillary antrum.
  88. 88. Medial maxillectomy cont…  All sharp, bony spicules are smoothed out from the edges of the surgical defect.  The wound is irrigated with Bacitracin solution, and Xeroform packing is used in the surgical defect, which is brought out through the left nostril.  The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin.
  89. 89. Medial maxillectomy cont…  The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus, nasopharynx, nasal cavity, and left maxillary antrum.
  90. 90. Medial maxillectomy cont…  Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days.  Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized.  Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity.  The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed, aesthetically acceptable scar.
  91. 91. Medial maxillectomy cont…  A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface.
  92. 92. DENKER’S APPROACH – TRANSPALATAL + TRANSANTRAL
  93. 93. Denker’s  Wide anterior antrostomy is done.  Ascending process of maxilla is removed.  Inferior half of lateral nasal wall is also removed.
  94. 94. RADIOTHERAPY
  95. 95. Radiotherapy  External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy.  Reduction in the size of the tumour takes place, but residual tumour remains.  Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more.  Treatment failure was apparent, usually within the first two to three years, and surgical salvage was generally successful in all these patients.  There are no reports on the efficacy of gamma-knife therapy as yet, though some patients must have been treated by this means.
  96. 96. COMPLICATIONS
  97. 97. Complications Recurrence –  most common complication encountered.  reported in up to 25 percent of patients regardless of the method of treatment.  more likely in patients with advanced disease and in those treated by inexperienced surgeons.  Preoperative embolization is not associated.  The more younger the patient, the more likely that future recurrence will develop.  Most recurrences develop as a consequence of invasion of the basisphenoid, from surgical standpoint.
  98. 98. Complications cont…  drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid.  Prolonged clinical and radiological monitoring is necessary for all these patients in view of the very high incidence of recurrent disease.  Disease-free status five years after primary surgery probably represents cure. Infraorbital nerve sensory deficits induced surgically and also nasal vestibular stenosis are recognized as a potential complication of mid-facial degloving,. Prolonged nasal crusting  may develop into ozaena  regular nasal douching with saline and the use of glucose in glycerine drops can alleviate this unpleasant complication.
  99. 99. Complications cont… Ocular problems -  Displacement of the globe caused by loss of bony support,  Ophthalmoplegia,  Visual loss may also be present. Late complications that may develop after radiotherapy include Growth retardation, panhypopituitarism, temporal lobe necrosis, cataracts, radiation keratopathy, together with skin, thyroid and nasopharyngeal malignancies Some second neoplasms have developed in the radiation field at an even later date.
  100. 100. CONCLUSION
  101. 101. Conclusion  Rare, benign, vascular tumor found almost exclusively in young males  Surgery is the gold standard with a trend towards endoscopic approaches  Frequent follow-up after treatment is necessary
  102. 102. THANK YOU

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