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ADENOIDS & JUVENILE NASOPHARYNGEAL ANGIOFIBROMA (JNA)
ENT - MBBS 4th Year
PART 1: ADENOIDS
Embryology
- Adenoids develop at 16 weeks as subepithelial infiltration of lymphocytes in the nasopharynx
- They form part of Waldeyer's ring (along with palatine tonsils, lingual tonsil, tubal tonsils)
(K.J. Lee's Essential Otolaryngology, p. 959)
Anatomy
- Located on the posterior wall of the nasopharynx (roof and posterior wall)
- Covered by ciliated pseudostratified columnar epithelium (respiratory type); luminal aspect has multiciliated and secretory cells
- Unlike palatine tonsils, adenoids have no crypts - they have surface folds/rugae
- They are not encapsulated and have no afferent lymphatics
Physiology / Role
- Part of the mucosal immune system; produce secretory IgA (sIgA)
- Act as first-line defense against inhaled antigens
- Adenoid size peaks at 4-10 years, then undergoes spontaneous involution at puberty
- In children with CRS (chronic rhinosinusitis):
- Adenoids serve as a bacterial reservoir - bacteria from middle meatal swabs and adenoidal core cultures are near-identical (S. pneumoniae, Group A strep, H. influenzae, S. aureus, coagulase-negative staphylococci)
- Positive predictive value of adenoid core culture for predicting middle meatal culture: 91.5%
- Biofilms cover 88-99% of adenoid surface in CRS children vs 0-6.5% in simple obstructive adenoid hypertrophy
- Lower IgA expression in CRS adenoids; higher TGF-β1, MMP-2, MMP-9
(Scott-Brown's ORL, Vol 2; Cummings Otolaryngology)
Adenoid Hypertrophy - Clinical Features
Age group: Most common between 4-10 years
Symptoms:
| System | Symptom |
|---|
| Nasal | Mouth breathing, nasal obstruction, hyponasal voice (rhinolalia clausa) |
| Otological | Recurrent otitis media, serous otitis media (glue ear), conductive hearing loss |
| Sleep | Snoring, obstructive sleep apnoea (OSA), restlessness during sleep, unusual sleep positions |
| Face | "Adenoid facies" - open mouth, high arched palate, elongated face, dull appearance |
| Systemic | Failure to thrive (in severe cases) |
Adenoid facies = characteristic look due to chronic mouth breathing: elongated face, open mouth, pinched nostrils, dental malocclusion.
Investigation:
- Lateral soft tissue X-ray of nasopharynx - shows pad of adenoid tissue in postnasal space (arrow visible on soft tissue radiograph)
Plain lateral radiograph showing a large pad of adenoid tissue (arrow) in the postnasal space - Bailey & Love's Surgery
- Flexible nasendoscopy (gold standard)
- Note: adenoid size alone does not correlate with severity of CRS on CT
Adenoid and Otitis Media with Effusion (OME / Glue Ear)
- Adenoid hypertrophy causes Eustachian tube dysfunction → OME
- Adenoidectomy alone improves OME/recurrent AOM (due to both mechanical obstruction relief and elimination of bacterial reservoir)
- Added for children ≥4 years when distinct indications exist
Medical Treatment
- Intranasal corticosteroids may significantly improve nasal obstruction symptoms in moderate-to-severe adenoid hypertrophy and may reduce adenoid size (Cochrane evidence - limited but supportive)
Indications for Adenoidectomy
(Bailey & Love's Surgery, 28th Ed; KJ Lee's Otolaryngology)
- OSA associated with postnasal obstruction
- Recurrent acute otitis media or prolonged serous otitis media (>3 months duration)
- Recurrent rhinosinusitis
- Postnasal discharge / chronic adenoiditis
- Failure to thrive due to airway obstruction
Tonsillectomy indications (for comparison):
- 6-7 episodes of acute tonsillitis in 1 year
- 5 episodes/year for 2 years
- 3 episodes/year for 3 years (Paradise criteria)
- Peritonsillar abscess
Adenoidectomy - Operative Technique
- Patient supine, oropharynx exposed with Boyle-Davis mouth gag + Doughty's tongue plate
- Nasopharynx palpated; adenoid curette (St. Clair Thompson) used to scrape adenoid pad
- Haemostasis with post-nasal pack or suction cautery
- Important: Avoid sedative premedications and postoperative opiates in OSA patients (risk of respiratory depression)
PART 2: JUVENILE NASOPHARYNGEAL ANGIOFIBROMA (JNA)
Definition
A benign but locally invasive vascular tumour of the nasopharynx occurring almost exclusively in adolescent males. Accounts for <1% of all head and neck tumours.
(Cummings Otolaryngology Head & Neck Surgery)
Epidemiology
- Age: typically 10-25 years, predominantly males
- Extremely rare in females (some cases in maxillary sinus site)
- Androgen-dependent: androgen receptors (esp. estrogen receptors) present in tumour tissue - explains male predominance and puberty-related onset
Site of Origin
- Arises from the sphenopalatine foramen at the root of the pterygoid process on the lateral nasal wall
- This is the most important fact - the tumour originates here
Pathology
Macroscopy:
- Sessile, lobulated, rubbery, dark red to tan-gray
- Unencapsulated, sessile, wide-based attachment to nasopharynx
- No mucosal ulceration
Microscopy:
- Admixture of vascular tissue + fibrous stroma
- Vessel walls lack elastic fibres and have incomplete or absent smooth muscle → accounts for profuse bleeding
- Vascular cavities with endothelial layer but no muscle layer (unlike normal arteries)
- Similarities between nasal mucosal cells, genital erectile tissue, and vascular spaces
Growth Pattern
- Locally infiltrative, slow-growing
- Expands laterally via pterygopalatine fossa → infratemporal fossa
- Extends into nasopharynx and choanal space → anterior nasal cavity and sinuses
- Can expand to: maxillary/ethmoid sinuses, orbit, cheek, cavernous sinus
- Intracranial extension (middle/anterior cranial fossa) - uncommon but serious
Clinical Features
| Feature | Details |
|---|
| Age/sex | Adolescent males, 10-25 years |
| Epistaxis | Most common presentation - recurrent, severe, may lead to anaemia |
| Nasal obstruction | Usually unilateral |
| Other | Blood-stained sputum, serous otitis media, nasal discharge, facial swelling, proptosis, anosmia |
| Duration | Usually long history, often mild and innocuous initially |
Diagnosis
Endoscopy: Rubbery vascular mass protruding into anterior nasal space; bleeds excessively on contact - biopsy avoided due to risk of haemorrhage
CT with contrast:
- Enhancing soft tissue mass from nasopharynx/lateral nasal wall
- Widening of pterygopalatine fossa
- No bone erosion generally
- Holman-Miller sign (classical): anterior bowing of posterior wall of maxillary sinus
CT scan showing widening of the left sphenopalatine foramen from a juvenile angiofibroma - Cummings Otolaryngology
MRI:
- Vascular tumour with flow voids within the mass
- Enhancement on gadolinium imaging
- Best for delineating intracranial extension and follow-up
MR Angiography / DSA (Digital Subtraction Angiography):
- Shows feeding vessels arising from branches of the external carotid artery (mainly internal maxillary artery)
- If intracranial spread: internal carotid artery also feeds the tumour
- DSA shows enlarged intratumoral vessels + intense inhomogeneous blush in venous phase
Histology: Final confirmation - required before definitive treatment
Chandler Staging (1984)
| Stage | Extent |
|---|
| Stage I | Confined to nasopharynx |
| Stage II | Extension into nasal cavity or sphenoid sinus |
| Stage III | Involvement of maxillary sinus, ethmoid sinus, infratemporal fossa, orbit, cheek, cavernous sinus |
| Stage IV | Intracranial extension |
Management
Primary treatment: Surgical excision
Pre-operative embolization:
- Well-established adjunct to induce tumour shrinkage, improve surgical access, reduce intra-operative blood loss
- Done transarterially with particles (PVA) or liquids (NBCA/Onyx)
- For tumours supplied by branches of external carotid - relatively straightforward
- Intracranial supply (internal carotid) increases risk of neurological deficit
- Direct tumour puncture technique with Onyx injection - improves devascularization
Surgical approaches:
- Traditional: Open approaches (transpalatal, lateral rhinotomy, midfacial degloving)
- Modern: Endoscopic surgical resection - now preferred for smaller tumours (Stage I-II) without spread; some consider preoperative embolization unnecessary for smaller endoscopic cases
- Large/intracranial tumours: Combined skull base approach (head and neck surgeon + neurosurgeon)
Radiotherapy:
- Used when surgery is contraindicated or for recurrence
- Primary radiation therapy has been reported
- Gamma knife used in some cases
Hormonal therapy (adjunct):
- Flutamide (androgen antagonist) - shown to reduce tumour size, may have role as pre-operative adjunct
Natural history:
- Spontaneous regression reported after cessation of androgen stimulation (post-puberty)
- Rare malignant transformation reported
Key Exam Points - JNA
| Feature | Value |
|---|
| Tumour type | Benign, locally invasive, vascular |
| Who | Adolescent males (10-25 years) |
| Origin site | Sphenopalatine foramen / lateral nasal wall |
| Classic sign on CT | Holman-Miller sign (anterior bowing of posterior maxillary wall) |
| Classic imaging finding | Flow voids on MRI |
| Feeding artery | Branches of external carotid (internal maxillary artery) |
| Biopsy in clinic | AVOID - risk of catastrophic haemorrhage |
| Pre-op adjunct | Embolization |
| Primary treatment | Surgical excision |
| Histology | Vascular spaces with absent smooth muscle + fibrous stroma |
| Staging | Chandler staging (I-IV) |
Comparison Table: Adenoids vs JNA
| Feature | Adenoids (Hypertrophy) | JNA |
|---|
| Type | Lymphoid hyperplasia | Benign vascular tumour |
| Age | Children, 4-10 years | Adolescent males, 10-25 years |
| Sex | Both | Almost exclusively male |
| Location | Roof/posterior nasopharynx | Sphenopalatine foramen |
| Main symptom | Nasal obstruction, OME | Epistaxis, nasal obstruction |
| Investigation | Lateral X-ray, nasendoscopy | CT, MRI, DSA |
| Classic sign | Adenoid facies | Holman-Miller sign |
| Treatment | Adenoidectomy | Surgery (±embolization) |
| Biopsy | Safe | DANGEROUS |
Sources: Cummings Otolaryngology Head and Neck Surgery; Bailey & Love's Short Practice of Surgery 28th Ed; Scott-Brown's Otorhinolaryngology Head & Neck Surgery; K.J. Lee's Essential Otolaryngology