Choanal Atresia

Choanal Atresia

What would be the most appropriate next course of action in managing a newborn baby who exhibits irritability and cyanosis that improves when crying, and is unable to pass a nasogastric tube during examination with no mist formation observed on the laryngeal mirror test?
A. Endotracheal intubation
B. Oro pharyngeal airway
C. Oxygen inhalation
D. Epinephrine nebulization

ANS

Oro pharyngeal airway

Pathology

• Blockage of the nasal passages
• due to persistence of buccopharyngeal membrane

• Choana:
• Outlet of the nose.
• Communicates nasal cavity with nasopharynx.
• Choanal atresia is no communication.

• Two types:
• Bony.
• Membranous.

• Can be unilateral or bilateral.

• Associated with CHARGE syndrome:
• C - Coloboma Iridis.
• H - Heart defects: ASD/VSD.
• A - Atresia.
• R - Retardation: Growth/mental.
• G - Genital malformations: Hypospadias.
• E - Ear abnormalities.

• Cyclical cyanosis:
• Cyanosis at rest.
• Disappears on crying.
• Seen only in bilateral (b/l) choanal atresia.
• Child is an obligate nose breather until 3 months.
• Crying creates negative intrathoracic pressure, pulling air in.
• B/l choanal atresia is an emergency.

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Keyhole defects	Seen in
Keyhole shaped Visual field	LGB
Keyhole vision with macular sparing	Occipital lobe lesion
Keyhole shape defect	Coloboma Iris

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Symptoms

• Difficulty in breathing and feeding

• Cyanosis

• Irritability

Initial management:

• Facilitate oral breathing by:
• Keeping the mouth open
• Pulling the tongue forward manually using oral digital manipulation

Further management:

• Placement of:
• Plastic oropharyngeal airway or
• McGovern open-tip nipple

Treatment for b/l choanal atresia:

• Introduce McGovern nipple (oral airway and feeding nipple).

• Follow with tracheostomy.

• Correction of atresia at 1.5 - 2 years.
• Cannot correct at birth due to:
• Small nasal cavity.
• Low child weight.

Unilateral (U/l) choanal atresia:

• Not an emergency.

• Can be operated at any time.

• Child breathes through patent nostril.

• Not present at birth.

• Presents with constant nasal discharge or unilateral rhinorrhea.
• Absence of air bubble in nasal discharge.

• Diagnosis:
• Resistance felt on inserting infant feeding tube.
• X-ray after radio opaque dye.
• CT showing Choanal atresia.

Meningocele

• Herniation of meninges +/- brain tissue (meningoencephalocele) into the nasal cavity.

• Improper bone fusion during development creates a gap for herniation.

• Bones of nose roof:

Position	Bone
Anteriorly	Frontal bone
Middle	Cribriform plate
Posteriorly	Sphenoid bone

• Swelling is soft.

• Compressibility test: Positive.

• Reducibility test: Positive.

• Cough impulse test: Positive.
• Furstenberg test + → Cry/cough → ↑ Mass size
• Frustration - cry

• Transillumination test: Positive.

On spine

• On T1, appears dark.

• On T2, appears uniformly white.

• There is a protrusion of a meninges sac
• containing only clear fluid
• no neural components.

• Treatment:
• Excision of herniated mass.
• Reconstruction of defect.

Feature	Meningocele	Glioma	Dermoid
Cause	Improper fusion of bones of base of skull or roof of nasal cavity	No fusion → herniation → delayed fusion	Hamartoma: A congenital anomaly at line of bone fusion.
Consistency of swelling	Soft / Cystic	Firm	Variable
Compressibility test	Positive	Negative	Positive D- Compressible
Reducibility test	Positive	Negative	Negative
Cough impulse test	Positive	Negative	Negative
Transillumination test	Positive	Negative	Negative
Treatment	Excision + Reconstruction	Excision	Excision

 

Glioma

• Improper bone fusion → Herniation of CNS glial tissue into Nasal cavity → Delayed fusion → separates cranial and nasal cavities → CNS tissue left behind → Antigen-antibody reaction → Fibrosis → Firm swelling

• Compressibility test: Negative.

• Reducibility test: Negative.

• Cough impulse test: Negative.

• Transillumination test: Negative.

• Treatment: Excision without reconstruction.

Note:

Optic meningioma Vs Optic glioma

Dermoid

• Congenital swellings at the line of bone fusion.

• A subset of hamartoma.

• Contains all 3 germ layers:
• Ectoderm.
• Endoderm.
• Mesoderm.

• Can form teeth, hair follicles, cheesy sebaceous material.

• Variable consistency.

• Compressibility test: Positive.

• Reducibility test: Negative (most do not communicate with cranial cavity).

• Cough impulse test: Negative.

• Transillumination test: Negative.

• Treatment: Excision.

Table: Comparison of Meningocele, Glioma, and Dermoid

Feature	Meningocele	Glioma	Dermoid
Cause	Improper fusion of bones of base of skull or roof of nasal cavity	No fusion → herniation → delayed fusion	Hamartoma: A congenital anomaly at line of bone fusion.
Consistency of swelling	Soft / Cystic	Firm	Variable
Compressibility test	Positive	Negative	Positive D- Compressible
Reducibility test	Positive	Negative	Negative
Cough impulse test	Positive	Negative	Negative
Transillumination test	Positive	Negative	Negative
Treatment	Excision + Reconstruction	Excision	Excision

 

Septal Disease

Deviated Nasal Septum (DNS)

• Septum is not in the midline.

Types:

• Concave DNS.

• Convex DNS.

• S-shaped septum.

• Spur (sharp bony projection).

• Anterior dislocation.

Indication for surgery:

• Not all patients require surgery.

• DNS + symptoms.

• Symptoms:
• External deformity of nose.
• Nasal obstruction.
• Difficulty breathing.
• Headache.
• Facial pain.
• Sinusitis.
• Smell disturbance.
• Reduced eustachian tube ventilation causing middle ear diseases.

Surgeries:

• Septoplasty.

• SMR.

Cottles line:

• Imaginary line from frontal spine to anterior nasal spine.

• Deviations infront line: Septoplasty only.

• Deviations behind line: Septoplasty or SMR.

Cottles test:

• Identifies patency of the internal nasal valve.

• Cottles test procedure:
• Patient breathes to identify obstruction side.
• On obstructed side, cheek is pulled away.
• Improvement in breathing suggests internal nasal valve obstruction.

Internal nasal valve boundaries:

• Angle between medial and superior border is
• 10 - 15 degrees.

Septoplasty

• Freer’s/Hemitransfixion incision :
• Over lower/caudal septal border

• Septal perforation risk is lower

• Flap elevated on one side.

• Deviation is corrected.

• Flap is replaced.

• Conservative surgery.

SMR (Submucous Resection)

• Killian incision

• Kill smr

• Flap elevated on both sides.

• Deviation corrected by removing most cartilage and bone.

• Mucosa is replaced.

• Radical surgery.

• Septal surgeries are avoided until 17-18 years of age
• due to bone growth and potential recurrence.

Septal Abscess vs Septal Hematoma

Deviations of nose

Septal Perforation

Can occur in:

• Cartilaginous septum:
• Trauma.
• Drug abuse.
• Nose picking.
• Inhalation of gases (mustard, asbestos).
• Following septal abscess.
• Infection (TB), sarcoidosis, SLE, Leprosy

• Bony septum:
• Syphilis.
• Mnemonic: BOSE

• Total septum:
• Wegener's granulomatosis.
• Cocaine
• Mnemonic: Total wage () → spent on cocaine ()

• Small perforation → whistling sound.

• Large perforation → nasal obstruction, crusting, and blood discharge.

Treatment:

• Septal repair is not mandatory for all.

• Done when symptoms prohibit daily activities or if there is a large bleed.

• Includes septal button and reconstruction

Nasal Polyps

• Two types:
• Antrochoanal polyp.
• Ethmoidal polyp.

Table: Antrochoanal vs Ethmoidal Polyp

Antrochoanal Polyp

• From maxillary antrum → Extends posteriorly to choana
• reaching the nasopharynx.

• Single/unilateral.

• Occurs due to maxillary sinus infection.

• Greyish white mass

• Seen in the young age group.

• Male predominant.

• Organism: Streptococcus pneumoniae.

• Symptoms
• Nasal obstruction.
• Difficulty breathing.
• Headache.
• Sinusitis.
• Smell disturbance.
• Foul smell in the nose.

• Treatment:
• No role for medical therapy.
• Surgery: FESS (Functional Endoscopic Sinus Surgery).
• Pre-op CT scan is needed to identify origin and extension.

Dodd's sign:

Ethmoidal Polyp

• Arise from ethmoidal air cells.

• Etiology: Mainly allergic.

• Seen in adults.

• Bilateral, multiple.

• Bunch of grape appearance.

• Grows anteriorly.

• Symptoms are similar to antrochoanal polyp, but bilateral

Associated with:

• Chronic rhinosinusitis.

• Ciliary motility disorder.

• Asthma.

• Cystic fibrosis.

• Sampter’s triad: Asthma, Aspirin intolerance, Nasal polyp (Ethmoidal)

• Kartagener's syndrome: Bronchiectasis, sinusitis, situs inversus.

• Young syndrome: Sinopulmonary syndrome, azoospermia.

• Churg-Strauss syndrome: Asthma, fever, eosinophilia, vasculitis, granuloma.

Triads

• Gradenigo's Syndrome:
• Petrous apicitis
1. Persistence of ear discharge (after cortical mastoidectomy)
2. Deep seated retro-orbital pain → D/t CN 5
3. Diplegia - Lateral rectus palsy due to CSOM → D/t CN 6
• Mnemonic: Pettennu (Petrositis) Granede (Gradenigo) 5,6 thavana itt → Kannilum cheviyilum kond → Eye pain + ↓ movement - case eduth(CSOM)

• Sampter's triad
• (Mnemonic: AAP): SAM → MAS → AS, AS, NAS
• AS - Asthma
• AS - Aspirin intolerance (& other NSAIDS that block COX1)
• NAS - Nasal polyp (Ethmoidal)

• Trotter's Triad
• Diagnostic of Nasopharyngeal Carcinoma
• NPC
• Temporoparietal neuralgia.
• Palatal paralysis.
• CN 10
• Tensor and Levator Veli Palatini involvement
• Unilateral conductive hearing loss.

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• CSOM → Lateral sinus thrombophlebitis signs



• Hectic picket fence type
• Intermittent septic emboli enter bloodstream
• Fever + rigors
• Fever does not touch baseline
• Tobey-Ayre's test:
• Compression of IJV on healthy side raises CSF pressure.
• Mnemonic: Tobey has lot of pressure in his head
• Crowe-Beck test:
• Pressure on IJV on healthy side causes engorgement of retinal veins.
• Mnemonic: Crow with red eyes
• Griesinger sign
• Mastoid emissary veins cannot drain
• Edema and bluish discoloration of the mastoid
• Similar to battle sign
• Mnemonic: Blue color grease

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• Grisel syndrome:
• Seen in Downs
• Non-traumatic inflammatory atlanto-axial subluxation
• Neck stiffness
• Torticollis
• Severe neck pain
• Due to paraspinal spasm from inflammation

Case scenario:

• Q. Patient with asthma, presents with b/l nasal obstruction. On examination, multiple grape-like masses in the nasal cavity are seen. What is the drug to avoid in this patient?
Ans
• Sampters triad: Aspirin has to be avoided due to intolerance.

Diagnosis:

• Anterior rhinoscopy.

• Shows multiple grape-like mass.

• Do not bleed on touch.

• CT scan can also be done.

Treatment:

• Conservative:
• Antihistamines.
• Steroids.
• Nasal decongestants.

• Surgery:
• Done if conservative treatment fails.
• FESS.

Rhinosporidiosis vs Rhinoscleroma

Rhinosporidiosis

• Organism: Rhinosporidium seeberi

• Aquatic parasite, history of swimming (South India)

• Mulberry nose

• Site: Nasal cavity

• Gross: Strawberry polyp

• Microscopy: Sporangia with endospores

• Non-cultivable organism

• Treatment: Excision + Dapsone

 

Rhinitis

Allergic Rhinitis vs Non Allergic Rhinitis (Vasomotor Rhinitis)

Atrophic Rhinitis

• F > M (Starts during puberty).

• Atrophy of all nose structures (nasal/sinus mucosa, olfactory fibers).

• Caused by: Klebsiella ozaenae (Perez bacillus)

• Other causes:
• Hereditary,
• endocrinal,
• nutritional deficiency (Vit D, B12, iron),
• racial, autoimmune.

• Signs:
• Wide roomy nasal cavity.
• Dry nose.
• Crusty nose (greenish, foul smelling).
• Merciful anosmia (patient cannot smell the odor).

Medical Treatment:

• Alkaline nasal douching.
• Parts
• Sodium chloride (2),
• sodium bicarbonate (1),
• sodium biborate (1)
• mixed in 280 ml of water,
• patient is asked to flush the nose with this solution

• Glycerol.

• Glucose in glycerin.

• Kemicetine antiozena solution
• Chloromycetin
• Estradiol
• Placental extracts
• Mnemonic: Kemidennu chloroform manapichitt, estrogen, placental extract edukkum

Surgery (if medical therapy fails):

• Young's operation
• Complete closure of nasal cavity

• Modified Young's surgery.

• Lautenslager surgery (medialization of lateral wall).
• Submucous teflon injection
• Also in VUR → Sting procedure

Rhinitis Sicca

• Involves only anterior 1/3rd of the nose.

• Seen in bakers or people exposed to hot, humid air.

• Signs (anterior 1/3rd only):
• Dry nose.
• Crusting of nose.

• Treatment: Similar to atrophic rhinitis.

• Surgical management is not required.

• Sicca in sick environment

Rhinitis Medicamentosa and Drug Induced Rhinitis

Sinusitis and its Complications

• Inflammation of sinus mucosa.

• Types:
• Acute (<4 weeks).
• Chronic (>12 weeks).
• Subacute (4-12 weeks).

One Liners (Sinusitis)

• Most common sinus in adults: Maxillary sinus.

• Most common sinus in children: Ethmoidal sinus (most developed).

• Least common sinus: Sphenoid sinus (posterior most).

Etiology (Sinusitis)

• Exciting factors:
• Nasal infection.
• Swimming and diving.
• Trauma.
• Dental infection
• especially maxillary sinus:
• 2nd premolar,
• 1st molar

• Predisposing factors:
• Obstruction of sinus drainage leading to retained secretions.

Bacteriology (Sinusitis)

• Most common: Streptococcus pneumoniae.

• Dental origin: Anaerobic and mixed infections.

Headache Types and Associated Symptoms

Diagnosis

• Requires:
• 2 major symptoms OR
• 1 major + 2 minor symptoms.

• Postural test:
• Differentiates
• maxillary vs
• frontal & anterior ethmoidal sinusitis.
• Mop middle meatus discharge, patient lies on opposite side.
• Pus reappearance → maxillary sinusitis.

• For acute sinusitis:
• First investigation: X-ray.
• Gold standard:
• Sinus puncture
• bacterial culture.
• CT scan for immunocompromised or anticipating complications.

• For chronic sinusitis:
• IOC: Non contrast CT scan.

Treatment (Sinusitis)

Acute Sinusitis

• Medical:
• Antibiotics,
• decongestants,
• analgesics,
• hot fomentation.

• Surgical:
• If unresponsive to medical therapy.
• FESS.

Chronic Sinusitis

• Lasts >12 weeks.

• Cause: Failure of resolution of acute infection.

• Maxillary sinus most commonly involved.

• Symptoms:
• Purulent nasal discharge (most common),
• nasal obstruction.

• Medical:
• Antibiotics with antral irrigation.

• Surgery:
• If medical therapy fails.
• FESS.

AFRS (Allergic Fungal Rhinosinusitis)

Non-invasive:

• Types: Fungal ball, Allergic fungal rhinosinusitis

• Seen in immunocompetent and immunocompromised

• Cause: Aspergillus

• Peanut butter discharge

• Mnemonic: HIV AIDS → Kuninj bent cheythapo sinusitis vann

• Bent and Kuhn criteria for AFRS:
• Major Criteria
• Mnemonic: Mr KUHN
• Test Mucus
• Eosinophilic Mucin
• KOH Fungal smear: Positive

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• CT scan:
• Hazy sinuses + Heterogeneous opacities
• Double density sign / Serpiginous sign
• Gray mucous with white fungal matter





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• Endoscope
• Nasal polyps → Ethmoidal polyps more associated
• Serum
• Uno → 1 → Type 1 hypersensitivity (↑IgE levels)
• Minor Criteria
• Mnemonic: CURE AF
• no major features like culture, asthma
• Charcot–Leyden crystals
• Unilateral predominance
• Radiological Bony erosion
• Serum Eosinophilia
• Asthma
• Fungal culture: Positive

• Treatment:
• FESS
• Endoscopic surgical drainage, along with drainage and ventilation.
• Steroids
• Pre- and post-operatively.
• Antifungal therapy: Itraconazole.

NOTE

Invasive:

• History of woody injury (+)

• Types:
1. Invasive aspergillosis
2. Invasive mucormycosis

Mucormycosis Nose

• aka Phycomycosis

• Angioinvasive

• Presents with blackish eschar

• Fungal thrombus causes tissue necrosis

• MRI shows Black turbinate sign

• Black turbinate sign:
• Due to necrosis- no enhancement.

• Biopsy
• Foreign body granuloma
• Gomori’s Methanamine Silver stain + PAS positive

• IOC: Contrast enhanced MRI.

• CT: Bony spread.

• Spread: Blood.

Treatment:

• Extensive debridement of tissue.

• Liposomal form of Amphotericin B (lyophilised form is nephrotoxic).