1. Juvenile angiofibroma(sbo-2)
Juvenile angiofibroma is an uncommon,benign &extremely vascular tumour that arises in the tissues
within the sphenopalatine foramen.It develops almost exclusively in adolencent males,though there
are reports of this tumour being found in children,elderly,young,even pregnant women.Juvenile
angiofibroma are locally invasive.
Pathogenesis
Juvenile angiofibroma present as well-defined,lobulated tumour that are covered by nasopharyngeal
mucosa.The tumour consists of proliferating,irregular vascular channels wthin a fibrous
stroma.Tumour blood vessels typically lack smooth muscle &elastin fibres.The stromal component is
made up of plump of cells that can be spindle or stellate in shape& give rise to varing amount of
collagen.It makes the tumour firm to hard while others may be soft.
Androgen receptors are present in at least 75% of the tumour.Progesterone receptors may present
in small proportion of tumour.
Juvenile angiofibroma have also been reported to develop 25 times more frequently in patient with
familial adenomatous polyposis.
Presentation
Recurrent severe epistaxes acompaned by progressive nasal obstruction are the classical symptoms
of juvenile angiofibroma.These tumours don’t grow fast &so many months even years may pass
before it appear to the patients.In most cases,there is a delay of at least 6 to 7 months between the
onset of symptoms & presentation.By that time, it is usual for the youth to have other signs&
symptoms of tumour growth &extension. Swelling of check ,trismus, hearing loss & nasal
intonationof voice.
Anterior rhinoscopy is likely to confirm the presence of aboundant mucopurulent secretion in the
nasal cavity that obscure the vision.
Soft palate is often displaced inferiorly by the bulk of tumour which can be seen pink or reddish
mass.
Assessment
x-ray of skull lateral view shows anterior bowing of the posterior wall of the maxillary sinus.
CT imaging allows for delineation of tumour extent:Classic CT finding include erosion of the medial
pterygoid plate & indentation of the posterior wall of the maxillary sinus(Halman-muller sign).
MRI is helpful in defining tumour extension in pterygopalatine fossa ,infratemporal fossa, or
intracranial extension.
Angiography is performed within 48hrs of surgery to define the tumour blood supply & to
embolisation of the feeding.
2. Several staging systems have been proposed but that of FISCH is the most robust & practical.
Type Details
1 Tumour limited to the nasopharyngeal
cavity;bone destruction negligible or limited to
the sphenopalatine foramen.
2 Tumour invading the pterygopalatine fossa or
the maxillary ,ethmoid or sphenoid sinus with
bone destruction.
3 Tumour invading the infratemporal or orbital
region: a) without intracranial involvement.
b) with intracranial extradural involvement.
4 Intracranial intradural tumour.a)without
infiltration of the cavernus sinus ,pituitary fossa
or optic chiasma.b) with infiltration of the
cavernous sinus ,pituitary fossa or optic chiasma.
Treatment:
Recognized by ancient times by Hippocrates & called Hard nasal polyp.
Preoperative embolization;The role of preoperative embolization is controversial.More extensive
tumour acquire a blood supply from others vessels.The maxillary or external carotid artery can be
controlled by ligation relatively easy.Recurrence rate is increased by preoperative
embolizaton,Perhaps shrinkage of the tumour makes it more difficult to define its entirety at the
bottom of a deep& bloody operative field.
Peoperative chemotherapy
Ostrogens shrinkage in some but effect is variable &secondary feminizaion effect by an adolescent
boy.
Androgen receptor blocker Flutamide,tumour shrinkage of upto 44% used in the management of
prostate cancer.
Surgical resections
Most small tumour can resected through a transpalatal approach,Lateral rhinotomy approach or
Mid-facial degloving approach.
Now a days stage Fisch 1,2&some type 3 tumours are suitable for endoscopic approach using one or
two surgeon techniques. Advantage of endonasal endoscopic techniques are reduced intraoperative
bleeding,fewer postoperative complications,& reduced length of hospital stay.
3. Endoscopic endonasal techniques
Under G/A the nose is prepared with vasoconstrictor solution,the anterior end of middle turbinate is
resected>anterior ethmoidectomy+removal of the medial wall of the maxillary sinus to get access to
the posterior wall of the antrum.>Dissection into the sphenoidal rostrum.
Second surgeon through contralateral nostril aids resection of large tumour>apply traction to the
tumour.
Open approaches
Most surgeons now have adopted the technique of mid-facial degloving for the resection of
angiofbromas.Using the exposure afforded by this approach ,anterior ,medial,lateral&posterior walls
of the maxillary antrum can be removed preserving a rim of bone around the nasal aperture & infra-obital
nerve.This produces a very large cavity that is confluent with nasal cavity & post-nasal space.
Radiotherapy
External beam radiation in several fractions to achieve a total dose of 30-55Gy.
Regression of angiofibrioma is very slow,often taking 2 to3 yrs before radiological stabilization.
Complications
Recurrence of the tumour 25% regardless of method of treatment.Further recurrence may develop
in up to 40% of the patients.
Recurrence is more with advanced disease.preoperative embolizaton& young patients.
Surgical standpoint,most recurrence develop invasion of basisphenoid,drilling out of the
basisphenoid ensure that no recurrence.
Infra-orbital nerve sensory & vestibular stenosis deficit are recognised in mid-facial degloving
approach.Prolonged nasal crusting is also common>regular nasal douching with saline &use of 25%
glucose in glycerine can alleviate this.