NOSE - Nasopharyngeal angiofibroma

What is nasopharyngeal Angiofibroma?
They are benign, very vascular and biologically aggressive tumours, originating almost exclusively from posterior nasal and naso pharyngeal region in adolescent males.

What is the Aetiology of nasopharyngeal Angiofibroma?
Age     :           Usually occurs in the 2nd decade
Sex      :           Common in males.

Theories of origin:
1. Fibroblastic Theory : This theory is suggestive of an abnormal growth or response of connective tissues such as embryonic occipital plate, fasia basalis, periosteum of posterior nasopharyngeal wall prior to ossification at 25 Years.
2. Hormonal Theory: Angiofibromas are hormone dependent tumours due to oestrogen and androgen  imbalance.
3. Hamartomatous Theory:  It suggests of proliferation of vascular aberrant erectile tissue under hormonal influence.
4. Ringertz: Tumour arise from periosteum of the nasal vault.
5. Som and Neffson: Inequalities in the growth of bones forming skull base results in hypertrophy of the underlying periosteum.
6. Bensch and Ewing: Tumour arises from the embryonic fibrocartilage between basi-occiput and basi sphenoid.
7. Brunner: Tumour arises from conjoined pharyngobasilar and buccopharyngeal fascia.
8. Osborn: He suggested that this tumor is either a hamartoma or residues of fetal erectile tissue under hormonal influences.
9. Girgis and Fahmy: They suggested the presence of paragangliomatous tissue around the terminal part of maxillary artery in the pterygopalatine fossa.
Pathogenesis:
The tumor usually arises from the sphenopalatine foramen or the posterior wall of the nasal cavity with adjoining superolateral wall.
The blood vessels are devoid of tunica media, hence the bleeding is torrential.
Blood supply: Major arterial supply is from the epsilateral internal maxillary artery. Collateral supply is from the ascending pharyngeal artery and branches of internal carotid artery.

What are the Clinical features of Nasopharyngeal Angiofibroma?
Symptoms:                                                                      
1. Nasal obstruction
2. Epistaxis
3. Facial swelling
4. Eustachian tube blockage
5. Rhinolalia

Signs:
1. Reddish purple mass in one nostril which bleeds profusely on touch.
2. Proptosis
3. Soft palate pushed forward by mass
4. Pallor

What Investigations are required for the diagnosis of Nasopharyngeal Angiofibroma?
1. Haemogram
2. CT Scan is the most important diagnostic test to know the extent and vascularity of tumour. It can be performed along with angiography.

It usually shows:
a. Nasopharyngeal mass
b. Widening of pterygopalatine fissure
c. Enlargement of superior orbital fissure
d. Distortion of nasal septum with erosion of the paranasal sinuses.

Spread:
Stage of Angiofibroma on CT Scan:
Stage I           :           Tumor confined to posterior nares or nasopharyngeal vault                   or bolt
Stage II a       :           Minimal Internal extension into sphenopalatine foramen,
or ptergomaxillary fossa.
Stage II b       :           Fills pterygomaxillary fossa, displacing posterior wall of the                                 antrum or extending superiorly eroding the bone of orbit or
both.
Stage II C       :           Extends through pterygomaxillary fossa into cheek and
temporal fossa.
Stage III         :           Intracranial extension.

What is the Treatment of Nasopharyngeal Angiofibroma?  
1. Surgical excision using transpalatal and transnasal maxillary approach (Weber-Fergusson Incision).Pre-operative embolization of the tumour drastically reduces the intra-operative haemorrhage.
2. Radiation therapy in usually palliative.



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