JUVENILE NASOPHARYNGEAL ANGIOFIBROMA

MANAGED BY MIDFACIAL DEGLOVING

APPROACH: A CASE REPORT

Case Report
Silvia, Ashri Yudistira
 INTRODUCTION

• Juvenile Nasopharyngeal Angiofibroma (JNA) represents 0,05 to 0,5% of all

head & neck tumors, but is the common tumor of nasopharynx.
• It affects almost exclusively male adolescents.
• Benign appereance is counterbalanced by malignant clinical course due to
severe epistaxis, high incidence of recurrence, etc.
• Patients usually present at late stage, severe epistaxis accompanied by
proggresive nasal obstruction are the classical symptoms.
• There are a variety of staging criteria: by Radkowski, Fisch, Andrews,
Onerci, Sessions.
• Clasical treatment is surgery, however there are cases which may indicate
radiotheraphy & gamma knife surgery.
• Right now we can use nasal endoscopic surgery to remove some tumors
that would traditionally have been extracted by using an open surgical
approach.
 CASE REPORT

• B, 15 years old boy came to HAM General Hospital on August 20th 2014 with
spontaneus right sided epistaxis intermitently since 9 months ago.
• Patient also complained of nasal congestion & a mass on right nasal cavity
realized since 6 months ago.
• No history of trauma or previous nasal surgery.
• Physical examination, laboratory & chest x-ray: Normal.
• Ear & oropharynx within normal limit.
• Anterior rhinoscopy: Reddish mass that filled right nasal cavity.
• Posterior rhinoscopy: Reddish mass that filled nasopharynx.
• Nasal endoscopic: Reddish mass that filled right nasal cavity & nasopharynx,
nasal septum push to the left.
• CT scan: Nasopharyngeal mass extending towards choanae, right maxillary
sinus & nasal cavity, did not extend intracranially and the skull base was
intact
CASE REPORT
CASE REPORT
 CASE REPORT

• Patient was laid down with general anasthesia

on the operating table.
• Desinfection on the operating field with
betadine and 70% alcohol.

• We did infiltration into upper labial sulcus, then

we made a marker in the frenulum.

• Sublabial incision was done across the midline

extending laterally to maxilla tuberosity to the
periosteum.
CASE REPORT

• Midface was degloved subperiosteally.

• Septum cartilage was cut start from
nasal spine up to nasofrontal suture

• We evaluated nasopharynx, the mass was

detached from its surrounding tissue, and extracted
completely.
• The bleeding was controlled.

• Nasal septum was sutured.

• Frenulum was carefully approximated
with the marker as a guidance and
then sutured
 CASE REPORT

• We inserted posterior tamponade and

followed by anterior tamponade.

• Mass was succesfully extracted

completelly with 11x4x2 cm in size.

• Total blood loss during operation was 700 ml, and

then mass was sent to anatomy pathologist
 CASE REPORT

• After surgery: Patient was given antibiotic, anti hemorrhage, steroid &
analgetic injections.
• 2 days after surgery the tamponade was taken off.
• Patient recovered completely without any complications and discharged 5
days after surgery.
• Histopathologic examination showed blood vessels structure, dense stroma
composed of fibrous connective tissue and collagen.
• There was no evidence of tumour recurrence after 3 months of follow up, and
after that the patient never came back again.
 DISCUSSION

• Angiofibroma are histopathologically benign but potentially locally destructive

vascular tumors.
• Angiofibroma originate predominantly in the posterior-lateral nasopharynx wall.
• Angiofibroma expand commonly beyond the nasopharynx into cranium, nose
and paranasal sinuses.

• JNA are age- & sex-linked.

• JNA accounts for less than 0,5% of all benign lesions that originate in
nasopharynx.
• Despite low incidence, JNA is the most common benign tumor originating in
nasopharynx of young males.
• In this case the patient was a 15 years old boy.
 DISCUSSION

• Clinical presentation of JNA usually consists of nasal obstruction & intermitent

epistaxis. It is important to mention that the bleeding is unrelated to trauma &
is completely spontaneous.
• Signs & symptoms of tumour growth and extension: Swelling of the cheek,
trismus, hearing loss, anosmia, a nasal intonation, etc.
• Anterior rhinoscopy will show abundant mucopurulent secretions as well as
bowing of the nasal septum to the tumor free side.
• The soft case:
In this palate is often displaced inferiorly by the bulk of the tumour which can
be seen clearly as a pink or reddish mass that fills nasopharynx.
• Patient complained spontaneous right sided epistaxis intermitently & also
complained nasal congestion and a mass on right nasal cavity.
• Anterior rhinoscopy: Reddis mass that filled right nasal cavity.
• Posterior rhinoscopy: Reddish mass that filled nasopharynx.
• Nasal endoscopic: Reddish mass that filled right nasal cavity & nasopharynx,
nasal septum push to left.
 DISCUSSION

• CT is the most important pre operative test because it is usefull for showing
destruction of bony structures & widening of foramen & fissures at the skull
base due to spread of tumour.
• MRI is usefull to show presence of intracranial extension of tumour, MRI is
superior for soft tissues.
• Selective angiography identifies the feeding vessels & allows option of
preoperative embolization for vascular control. The arterial supply to JNA is
primarily from distal IMA branches.

In this case:
CT scan with contrast: Nasopharyngeal mass extending towards choanae, right
maxillary sinus & nasal cavity, it did not extend intracranially and skull base was
Intact.
 DISCUSSION

• Differential diagnosis: Antrochoanal polyp, inflammatory sinonasal polyp,

neurofibroma, adenoidal hypertrophy, nasopharyngeal cyst, pyogenic
granuloma, chordoma and malignant neoplasms.
• Treatment: Surgical resection, embolization, external beam radiation,
hormonal theraphy and combinations. Surgery is a good form of treatment,
especially when there is no intracranial involvement.
• The most important principle in surgery is exposure of the tumour, as this is
the key for complete & safe removal.

In this case:

The patient underwent surgery with midfacial degloving approach

 DISCUSSION

• Spontaneous regresion is rare, recurrence is a common problem in JNA

treatment.
• Recurrance rates ranged from 6% to 60%. Recurrence can occur as early as
3-4 months after surgery.
• Malignant transformation is usually not a concern when dealing with JNA, but
there have been some rare reports of fibrosarcoma transformation. This is
thought to be associated with prior radiation theraphy.
 CONCLUSION

• JNA should be suspected in a boy or adolescent male with history of chronic

nasal obstruction or recurrent epistaxis without history of trauma & a soft
tissue mass in the nose or nasopharynx.

• Prognosis of this disease is extremely good if diagnosed well in time and if

has not extended intracranially.

• Midfacial degloving approach is an excellent approach for removal of

nasopharyngeal angiofibroma with less bleeding and also no facial scar.

• We report a case of JNA managed by midfacial degloving approach without

any complications.
THANK YOU