Rhinology Flashcards
How often does the alternating cyclic engorgement of nasal turbinates occur?
Every 2-4 hours
Increased obstruction when lying down or on dependent side with lateral recumbent position
What type of mucosa is in the paranasal sinuses?
Pseudostratified ciliated columnar epithelium
Limen nasi
Mucosal ridge between the nasal cavity proper and the nasal vestibule
Inborn disorders of mucociliary transport can occur because of what two defects?
- Ciliary dysfunction as in primary ciliary dyskinesia
- Increased viscosity of respiratory secretions as in cystic fibrosis
What can impair mucociliary transport?
Inflammation, infection, exposure to ciliotoxic agents
Describe the double layered mucus blanket
It consists of 2 layers: (1) an inner serous layer (ie, sol phase) in which cilia recover from their active beat and (2) an outer, more viscous layer (ie, gel phase), which is transported by the ciliary beat. Proper balance between the inner sol phase and outer gel phase is of critical importance for normal mucociliary clearance.
Ethmoturbinals
First - ascending portion is the agger nasi and the descending portion is the uncinate process
Second - middle turbinate
Third - superior turbinate
Fourth/Fifth- usually degenerate but can form supreme turbinate
Five anterior to posterior bony lamina encountered in endoscopic sinus surgery
- Uncinate process
- Ethmoid bulla
- Vertical portion of basal lamella of middle turbinate
- Vertical portion of lamella of superior turbinate
- Anterior wall of sphenoid sinus
Structures within the ostiomeatal complex
- Uncinate process: sickle shaped bone running anteriosuperior to posterioinferior with attachments along the lateral nasal wall. First structure encountered with MT medialized
- Ethmoid bulla
- Hiatus semilunaris; two dimensional slit that lies between the free edge of the uncinate process and the ethmoid bulla; connects the middle meauts into the infundibulum laterally
- Infundibulum: funnel shaped three dimensional space between the uncinate process medially and the lamina papyracea laterally
- Middle turbinate
- Maxillary sinus ostium
Variable sites of superior attachment of the unicinate process
- Laterally to lamina papyracea: most commonly resulting in a recessus terminalis; frontal recess drains medially to the uncinate and directly into the middle meatus
- Superiorly onto the skull base: frontal recess drains laterally into the infundibulum
- Medially to the middle turbinate: frontal recess drains laterally into the infundibulum
Parts of the middle turbinate
Anterior part: oriented in the sagittal place (vertical) and attaches to the agger nasi region anteriorly and the cribiform plate superiorly
Middle: oriented in the coronal plane obliquely and attached to the lamina papyracea
Posterior: oriented in the axial plane (horizontal) and attached to the lateral nasal wall at the lamina papyracea, maxilla and perpendicular process of the palatine bone.
The middle oblique part is the only part that can be sacrificed without compromising the integrity of the turbinate, Injury to the vertical or horizontal part will lateralize it and obstruct the middle meatus and posterior ethmoid complex
Where do the anterior and posterior ethmoid air cells drain into?
Anterior: middle meatus
Posterior: superior meatus
Describe the nasal fontanelles
Two areas along the lateral nasal wall where bone is absent. Anterior fontanelle is anterior to the uncinate bone and posterior fontanelle is posterior to uncinate
Describe where to find the sphenoid os
- Halfway to two thirds up the anterior wall of the sinus
- Medial to the posterior end of the superior turbinate in the majority of cases
- On average 7cm from the nasal spine at an angle of 30 degrees from the floor
Boundaries of the frontal recess
Medial: middle turbinate
Lateral: lamina papyracea
Anterior: posterior wall of agger nasi
Posterior: ethmoid bulla
Types of frontal cells
Type I: a single cell superior to the agger nasi
Type II: a tier of two or more cells above the agger nasi
Type III: a single cell extends from the agger nasi into the frontal sinus, above the floor of the frontal sinus but <50% of the frontal sinus height
Type IV: an isolated cell within the frontal sinus (Kuhn) or a single cell that extends into the fronal sinus for >50% of the frontal sinus height (Wormald)
All frontal cells lie anterior to the frontal recess
Supraorbital ethmoid cell
Cells posterior to the frontal sinus, pneumatizing superiorly to the orbital roof
Interfrontal sinus cell
Pneumatized intersinus septum and drains into frontal sinus, medially to the frontal osteum
Suprabullar cell
Cell superior to the ethmoid bulla
Frontal bulla cell
Cell superior to the ethmoid bull pneumatizing into the posterior frontal table (anterior skull base)
Suprabullar recess
Air cell space left between the ethmoid bulla and the fovea ethmoidalis when the bulla does not extend up to the fovea
Sinus lateralis/retrobulla recess
Air cell space found between the posterior surface of the ethmoid bulla and the vertical portion of the basal lamella
Sinus terminalis
Uncinate process terminates in the lamina papyracea; frontal recess drains medially to the uncinate process; this sinus is essentially a superior ending of the infundibulum
Keros classification of lateral lamella of cribiform height
Type I: cribiform plate 1-3mm below the fovea ethmoidalis
Type 2: cribiform plate 4-7mm below the fovea
Type 3: cribiform plate 8-16mm below the fovea (highest risk of skull base penetration)
Churg Strauss Syndrome
- Asthma, eosinophilia (>10%), allergic rhinosinusitis, pulmonary infiltrates, small to medium vessel vasculitis, mononeuritis multiplex or polyneuropathy
- P- ANCA antibodies often present
- Treat with oral steroids, cyclophosphamide and management of sinonasal symptoms
- Doesn’t usually present with epistaxis
- Clinical history follows 3 phases: 1) myalgias, arthralgias, malaise, fever, weight loss, asthma refractory to conventional tx 2) eosinophilia 3) vasculitis
Rhinoscleroma
- Infectious cause of rhinitis due to klebsiella resulting in a chronic granulomatous disease. Treat with ciprofloxacin or tetracycline.
- Histolopathology shows Mikulicz cells and Russel bodies
Rhinosporidiosis
- Painless, friable “strawberry” lesion caused by rhinosporidium seeberi from contaminated water (endemic in Africa and India)
- Histopathology shows pseudoepitheliomatous hyperplasia on histopathology
- Treat with excision, antifungals and dapsone
Rhinitis Sicca Anterior
- Dry, raw, nasal mucosa caused by changes in temperature/humidity, nose picking and dust
- Symptoms include dryness, crusting and epistaxis
- Treat with saline irrigation, topical antibiotics and oil based nasal ointments
What arteries supply Kisselbach’s plexus?
Anterior ethmoid, sphenopalatine, greater palatine, superior labial artery
Curacao criteria for HHT
- Epistaxis
- Mucosal telangectasias (oral or sinonasal)
- Visceral lesions (pulmonary arteriovenous malformation, cerebral AVM, hepatic AVM, spinal AVM)
- family history (first degree relative) ; disease is autosomal dominant
Definite diagnoses if 3; suspected if 2; unlikely if <2
Airflow through the nasal cavity
Middle meatus - 50%
Inferior meatus - 35%
Olfactory cleft - 15%
Types of olfactory disorders
- Transport or conductive (nasal inflammation, polyps, neoplasm, mass effect, septal deviation or nasal obstruction)
- Sensory (damage to neuroepithelium caused by drugs, neoplasms, radiation, toxic chemical exposure, viral URI)
- Neural olfactory loss (alcohol, tobacco, HIV, age, neurologic disorders like Parkinson’s, Kallman syndrome, Korsakoff psychosis, metabolic disorders like diabetes or malnutrition, trauma)
Causes of CSF rhinorrhea
- Traumatic (accidental or iatrogenic)
- Spontaneous ( may be related in increased intracranial pressure)
- Neoplasm
- Congenital
- Infection
Acute rhinosinusitis (ARS)
Any inflammation of the nose and sinus mucosa lasting 4 weeks or less with complete resolution
Recurrent ARS
Four or more annual episodes of rhinosinusitis without persistent symptoms in between
Chronic rhinosinusitis
Rhinosinusitis lasting longer than 12 weeks
Hallmark symptoms of acute rhinosinusitis
- Purulent rhinorrhea
- Nasal obstruction
- Facial pain and or pressure
Diagnosis of acute bacterial rhinosinusitis
Can be differentiated from viral sinusitis when hallmark symptoms persist without evidence of improvement for 10 days or symptoms worsen within 10 days after an initial period of improvement.
Treatment of acute bacterial rhinosinusitis
- Observation
- First line antibiotic: Augmentin
- If penicillin allergic then fluroquinolone (levofloxacin or moxifloxacin) or doxycycline
- Symptomatic treatment with nasal saline irrigation, nasal steroids and short term decongestants
Chandler classification of orbital complications during ARS
- Preseptal cellulitis
- Orbital cellulitis
- Subperiosteal abscess
- Orbital abscess
- Cavernous sinus thrombosis
Pathophysiologic contributors to chronic rhinosinusitis
- Predominance of T helper 2 cytokines
- Predominance of esoinophils
- Staphlococcal super antigen
- Deficiency in innate immunity
- Impaired mucociliary clearance
- Alterations in microbiome
Symptoms of CRS
- Nasal obstruction/congestion
- Hyposmia/anosmia
- Nasal discharge/postnasal drip
- Facial pressure
- Cough
- Wheeze (asthma)
Diagnosis of rhinosinusitis - major and minor symptoms
Diagnosis requires two major or one major and two minor symptoms
Major symptoms: facial pain/pressure; facial congestion/fullness; nasal obstruction/blockage; nasal discharge/purulence; hyposmia/anosmia; purulence on nasal exam; fever (acute rhinosinusitis only)
Minor symptoms: headache, fever (non acute), halitosis, fatigue, dental pain, cough, ear pain/pressure/fullness
Bent Kuhn criteria for allergic fungal sinusitis
Major criteria: type 1 hypersensitivity, nasal polyposis, characteristic CT findings (expansion of sinuses, asymmetry, heterogenously dense material in sinuses), positive fungal smear, eosinophilic mucin without tissue invasion
Minor criteria: asthma, unilateral predominance, serum eosinophilia, radiographic bone erosion, fungal culture, Charcot-Leyden crystals
Aspirin exacerbated respiratory disease
- Presence of polyps, asthma and asthma exacerbated by Cox-1 inhibitors (aspirin and NSAIDS)
- Caused by abnormality of arachidonic acid cascade that leads to increased production of proinflammatory leukotrienes
- Cox-1 inhibitors lead to increased production of leukotrienes, leading to severe worsening of asthma symptoms and or allergy like symptoms
Pathophysiology of Cystic Fibrosis
- Autosomal recessive defect in chloride ion channel
- Multiple different mutation in CF conductance transmembrane conductance regulator (CFTR)
- F508 deletion is most common
Granulomatosis with Polyangiitis
- Small medium vessel vasculitis
- C ANCA antibodies often present
- Can present with severe chronic inflammation, granulation tissue, chronic mucosal crusting, septal perforation and erosion of sinonasal structures, saddle nose deformity
Sarcoidosis
- Noncaseating granulomatous disease
- Systemic manifestations: bihilary lymphadenopathy on chest xray, fatigue, night seats, weight loss, erythema nodosum, uveiitis, peripheral lymphadenopathy, Heerford syndrome (facial nerve palsy, uveitis, fever, enlarged parotid glands)
- Sinus symptoms: polyps, chornic inflammation, mucosal nodules or cobblestoning of mucosa, mucosal crusting
Three phases of Churg Strauss
- Prodromal - asthma and upper respiratory involvement
- Peripheral eosinophilia (pulmonary or GI involvement)
- Disseminated/vasculitic phase (lung, CNS, kidney, GI, skin)
Ddx of Benign Sinonasal Neoplasms
- Osteoma
- Fibrous dysplasia
- Ossifying fibroma
- Chordoma
- Cementoma
- Hemangioma
- Inverted papilloma
- JNA
- Pleomorphic adenoma
- Schwannoma
- Neurofibroma
Ddx of Malignant Sinonasal Neoplasms
- Esthesioneuroblastoma
- Lymphoma
- Ewing sarcoma
- Chondrosarcoma
- Rhabdomyosarcoma
- Nasopharyngeal carcinoma
- Small cell carcinoma
- Squamous cell carcinoma
- Sinonasal undifferentiated carcinoma (SNUC)
- Adenocarcinoma
- Adenocystic carcinoma
- Mucoepidermoid carcinoma
- Hemangiopericytoma (both benign and malignant types)
- Peripheral nerve sheath tumor
Ddx of Congenital Sinonasal Neoplasms
- Dermoid
- Teratoma
- Glioma
- Encephalocele
Ddx of Inflammatory Sinonasal Neoplasms
- Polyp
- Antrochoanal polyp
- Inverting papilloma
Ddx for Sinonasal “Blue Cell” Tumors
- Rhabdomyosarcoma
- Esthesioneuroblastoma
- Lymphoma
- Melanoma
- SNUC
- Hemangiopericytoma
- Immature teratoma
- Carcinoid
- Peripheral nerve sheath tumor
Radkowski staging for JNA
Stage IA: limited to nose or nasopharynx
Stage IB: extension into at least one paranasal sinus
Stage IIA: Minimal extension through sphenopalatine formaen, includes minimal part of medial pterygomaxillary fossa
Stage IIB: full occupation of pterygomaxillary fossa with bowing of posterior wall of maxillary sinus on CT (Holman-Miller sign); lateral or anterior displacement of maxillary artery branches; may have superior extension with orbital bone erosion
Stage IIC: extension through pterygomaxillary fossa into cheek, temporal fossa or posterior to pterygoids
Stage IIIA: skull base erosion with minimal intracranial extension
Stage IIIB: skull base erosion with extensive intracranial +/- cavernous sinus
Krouse staging system for Inverted Papilloma
T1 - limited to one area of nasal cavity
T2 - involvement of medial wall of maxillary or ethmoid sinuses and or the osteomeatal complex
T3- involvement of superior, inferior, posterior, anterior or lateral walls of maxillary sinus
T4- Tumors with extrasinonasal spread or malignancy
Kadish staging of Esthesioneuroblastoma
Stage A: tumor limited to nasal cavity
Stage B: extension to paranasal sinuses
Stage C: extension beyond nasal cavity/sinuses
Stage D: cervical nodes or metastatic disease
Frontal Sinus Draf procedures
Draf I: Complete ethmoidectomy with removal of bulla and suprabullar cells
Draf IIa: enlargement of frontal sinus outflow tract with removal of all occupying cells (frontal sinusotomy)
Draf IIb (unilateral frontal sinus drill out): removal of floor of frontal sinus from lamina papyracea to the septum, to produce the largest possible unilateral outflow tract
Draf III (modified Lothrup): complete drill out of floor of the frontal sinus, frontal beak and intersinus septum and adjacent part of nasal septum
What cells contribute to formation of the nose during 4th week of embryogenesis?
Neural crest cells
Before closure during embryogenesis what are the following spaces called?
Between frontal and nasal bones
Between frontal and ethmoid bones
Between nasal bones and nasal capsule
Fronticulus nasofrontalis, foramen cecum, prenasal space
Into what structures do the medial and lateral processes of the nasal pits and the maxillary process of the maxilla develop?
Medial nasal pit: nasal septum, philtrum, premaxilla
Lateral nasal pit: Nasal alae
Maxillary process: lateral nasal side wall
What embryologic membrane separates the nasal and oral cavities and normally degenerates to allow open passages as the choanae are formed by deepening olfactory pits?
Nasobuccal membrane
Nasal bones attach to what structures in the facial skeleton
Frontal bone, nasal process of maxilla, upper lateral cartilages, contralateral nasal bone, perpendicular plate of ethmoid, cartilagenous septum
What structure does the frontal process of the maxilla, nasal floor and lateral fibrofatty tissue form?
Piriform aperture
Boundaries of external nasal valve
Caudal septum, lower lateral cartilages (caudal edge of lateral crus junction with upper lateral cartilage), piriform aperture
Components of nasal septum
Perpendicular plate of ethmoid bone, quadrangular cartilage, vomer, maxillary crest, palatine bone
Blood supply of nasal septum
- Anterior and posterior ethmoid arteries (superior septum)
- Sphenopalatine artery branches/posterior septal branch (posterior/inferior septum)
- Greater palatine artery (posteriorly)
- Superior labial artery (anteriorly)
The uncinate process is an extension of what bone
Ethmoid
What is the opening to the space between the uncinate and the ethmoid bulla?
Semilunar hiatus
The uncinate process covers the medial aspect of which space that provides a common drainage pathway for some of the anterior sinuses?
Infundibulum
Attachment sites of uncinate
Ethmoid crest of maxilla, lacrimal bone, ethmoidal process of inferior turbinate bone, palatine bone via the lamina perpendicularis
The lamina paprycea is formed by which bone
Ethmoid
Nasolacrimal duct empties under which structure?
Inferior turbinate (via Hasner valve)
What structure separates the anterior and posterior ethmoid sinuses?
Basal lamella
What is the horizontal plate of the ethmoid bone that forms the roof of the ethmoid sinus and separates the ethmoid air cells form the anterior cranial fossa?
Fovea ethmoidalis
What are the three infundibular cells that are anterior ethmoid air cells?
Agger nasi, Terminal cell (recessus terminalis), suprainfundibular cell
Which cell is the most anterior of the ethmoid cells and forms near the attachment of the middle turbinate to the lateral nasal wall?
Agger nasi
What arterial structure usually runs through the roof of the ethmoid bulla?
Anterior ethmoid artery
An infraorbital ethmoid cell that pneumatizes into the maxillary sinus and can narrow the maxillary sinus ostium
Haller cell
Where do the posterior ethmoidal cells drain
Superior meatus
Air cells that pneumatize lateral or posterior to the anterior wall of the sphenoid sinus
Onodi cells
First sinus to develop embryologically?
Maxillary
Where is the most common location for the maxillary ostium within the infundibulum?
Inferior third (65%)
What structure runs through the roof of the maxillary sinus?
Infraorbital nerve
A series of three or four frontal furrows arise out of the ventral middle meatus and give rise to what?
First frontal furrow = agger nasi
Second frontal furrow = frontal sinus
Third/fourth frontal furrow = anterior ethmoid cells
What is the last sinus to fully develop and at what age has it typically reached full size?
Frontal sinus, late teens
The spread of frontal sinus infections intracranially is commonly thought to pass through what structures?
Foramina of Breschet (small venules that drain the frontal sinus mucosa to the dural veins)
When is sphenoid sinus finish developing
Around age 9-12
Surgical landmarks to identify sphenoid sinus
- 6.2-8.0cm from anterior nasal spine
- 30-40 degrees from nasal floor
- Medial to posterior end of superior turbinate
- Halway up anterior sphenoid wall
In what % of patients is carotid artery dehiscent in sphenoid sinus?
~15%
What nerve is most medial in cavernous sinus?
VI
- This explains why abducens palys may be preferentially affected in sphenoid disease
The intersinus septum of the sphenoid sinus can be attached to what critical structure?
Internal carotid artery
What is the space between the internal carotid artery and the optic nerve within the sphenoid sinus called?
Opticocarotid recess
What neurovascular structures are set within the parasellar cavernous sinus?
- Internal carotid artery
- Cranial nerves III, IV, VI, V1, V2
What structures pass through the optic canal?
Optic nerve, ophthalmic artery and vein
The vidian nerve is formed by whcih two nerves before it runs through the vidian canal and exits into the pterygopalatine fossa?
- Greater superficial petrosal nerve from the geniculate ganglion of facial nerve (parasympathetic fibers from superior salivary nucleus)
- Deep petrosal nerve from the sympathetic plexus of internal carotid artery
Sternberg canal
Lateral craniopharyngeal canal that may persist in the adult patient and lead to encephalocele formation and CSF leak and most commonly is noted in patients with significant lateral pneumatization of sphenoid sinus.
What major branches of internal maxillary artery provide artery blood supply to the nose?
- Sphenopalatine artery
- Descending palatine artery -> greater and lesser palatine arteries
The sphenopalatine foramen is located posterior to attachment of middle turbinate to lateral nasal wall, may have several foramina and almost always is demarcated by what small, raised bony crest just anterior or anteroinferior to the foramen?
Crista ethmoidalis of palatine bone
The SPA can exit the foramen in up to 10 separate branches. What are the most common branches and their distribution?
- Lateral nasal artery: lateral nasal wall including turbinates
- Posterior septal artery: posterior/inferior septum
When ligating the anterior ethmoid artery via an external approach, the vessel can be found running in what suture line?
Frontoethmoid suture
What is the distance between the anterior lacrimal crest of the maxilla’s frontal process to the anterior ethmoidal artery
20-25mm
What is the average distance between the anterior and posterior ethmoidal arteries?
10-19mm
What is the average distance from posterior ethmoid artery to optic nerve?
3-7mm
What intranasal vessels are branches of the internal carotid artery?
Anterior and posterior ethmoid arteries
Woodruff plexus
Arterial plexus formed along posterior lateral nasal wall just under the inferior turbinate by branches from the ascending pharyngeal, posterior ethmoid, SPA and lateral nasal arteries.
True or false the venules within the respiratory mucosa of the nasal and paranasal cavities do not have valves.
true
Where do the sphenopalatine (1), ethmoid (2), angular (3), and anterior facial veins (4) drain?
1 - pterygoid plexus
2- superior ophthalmic vein
3 - opthalmic vein -> cavernous sinus
4 - common facial vein -> internal jugular
Primary blood supply to external nose
Angular artery (facial artery) Superior labial artery (facial artery)
What major nerve branches arise from nasociliary nerve (V1) and what regions of the nose do they supply?
- Infratrochlear nerve -> medial eyelid skin
- Anterior ethmoid nerve -> anterior/superior nasal cavity, lateral nasal wall, and septum, external skin of nasal tip
What runs in foramen rotundum
V2
After exiting the foramen rotundum V2 contributes fibers to the pterygopalatine (sphenopalatine) ganglion, which then supplies innervation to the nose via which branches?
- Infraorbital nerve -> anterior area of inferior meatus, anterior nasal floor, nasal vestibule
- Superior nasal branches -> posterior superior/middle turbinates, posterior ethmoid sinuses, face of sphenoid, nasal vault, posterior septum
- Nasopalatine nerve -> anterior hard palate
- Greater palatine nerve -> middle/inferior meatus, posterior aspect of inferior turbinate
Where do the parasympathetic fibers that provide vasodilation and secretomotor stimulation to nasal mucus glands synapse?
Pterygopalatine (sphenopalatine) ganglion
Superior salivatory nucleus -> nervus intermedius -> geniculate ganglion -> vidian nerve -> pterygopalatine ganglion -> sphenopalatine nerve branches -> vasodilation/secretomotor function
Postganglionic sympathetic fibers that ultimately control vasoconstriction in the nose arise from what ganglion?
Superior cervical ganglion
T1-T3 -> superior cervical ganglion -> internal carotid artery plexus -> join greater superficial petrosal nerve -> vidian nerve -> pterygopalatine ganglion -> sphenopalatine nerve branches -> vasoconstriction
Where do olfactory neruons synapse?
Olfactory bulb
Bones of the orbit
lacrimal, ethmoid, frontal, maxillary, sphenoid, zygomatic, palatine
What extraocular muscle is at highest risk during medial orbital decompression for Graves opthalmopathy?
medial rectus
What epithelium covers the cribiform plate bilaterally, extending to the superior and middle turbinates?
Olfacotry neuroepithelium: pseudostratified columnar epithelium
What part of the nasal cavity is composed of stratified keratinizing squamous epithelium, hair follicles, sebaceous glands and sweat glands?
Nasal vestibule
What is another name for the ciliated pseudostratified columnar epithelium that lines the nasal and paranasal cavities?
Schneiderian membrane (ectodermally derived)
Length of average nasal cycle and factors that may contribute to increase or decrease in congestion on a given side?
- Average cycle 2-4 hours
- Decrease in cycle length: excersize, increase in heart rate
- Increase in cycle length: on down side when lying down on one’s side
What results in the change in nasal cycle
- Changes in sympathetic tone result in normal nasal cycle
- Sympathetic tone leads to vasoconstriction. When tone decreases there is increased vasodilation
The nose filters out particles from the air larger than what size?
5 micormeters
- Particles smaller than this size are able to reach the alveoli of the lungs
What are the two mucous layers associated with the nasal mucociliary system?
- Upper gel layer: trap inhaled particle; formed by goblet cells and submucosal glands
- Lower sol layer: surround cilia of epithelium, formed by microvilli
What cells are responsible for producing airway mucus?
Goblet cells: secrete mucins
Submucosal seromucous glands: secrete mucins
Epithelial cells: hydration of mucus via active transepithelial transport systems
Venultes: plasma proteins
What test can be used to measure mucociliary transport time in the nose?
Saccharin test: a saccharin pellet is placed in the anterior nasal cavity and dissolves, passing toward the oropharynx via the mucociliary system and resulting in the sensation of a sweet taste. Time for placement to sensation: < 20 minutes
Which nerves contribute to the overall experience of an odor?
- Olfactory nerve
- Trigeminal nerve
- Vagus nerve
- Glossopharyngeal nerve
What type of cell is responsible for olfaction?
Olfactory resceptor cells (bipolar ciliated neurons)
Vomeronasal organ (Jacobson organ)
Small pit in anterioinferior nasal septum whose function in humans is unknown but in other mammals is thought to be related to detection of pheromones
How often does olfactory loss occur after head trauma
5-10%
- Less common in pediatric patients
What is thought to be the mechanism associated with olfactory dysfunction resulting from head trauma?
Shearing of olfactory nerve axons, contusion/hemorrhage within the olfactory regions of the brain or structural alteration of sinonasal tract
What percentage of adults will recover their sense of smell after experiencing anosmia from head trauma?
5-10%
What is most common cause of olfactory loss?
Persistent olfactory dysfunction after URI. This is more common in women (typically age 50+)
What % of patients will recover their sense of smell following postviral URI regardless of treatment
33%
In what two neurologic diseases is olfactory loss thought to be one of the earliest signs?
Parkinson disease and Alzheimer disease
What disorder is associated with anosmia and hypogonadism?
Kallmann (can be X linked or autosomal dominant)
Describe Kallmann syndrome’s relation to congenital olfactory dysfunction
Gonadotropin releasing hormone neurons fail to migrate from olfactory placode to the hypothalamus. MRI will demonstrate absense of olfactory bulb.
In what familial autosomal dominant condition to patients develop anosmia, early baldness and bilateral vascular headaches?
Familial anosmia
Most common side effects of second generation histamine type I (H1) blockers?
headache, urinary retention, dry mouth, blurry vision, GI upset
Most common side effects of pseudoephedrine?
Nervousness, hypertension, urinary retention
Onset of action of cocaine?
5-10 minutes
Duration of action of cocaine?
6 hours
Maximum recommended dose of cocaine?
1-3 mg/kg
Commonly comes in 4% solution and it is estimated that <40% is truly absorbed
What percentage of the population will have a concha bullosa?
25%
What size septal perforation has a high risk of failed surgical closure?
Large perforations. > 2cm
What pathogen is often associated with facial or nasal folliculitis?
S. aureus
Why are incision and drainage of nasal furuncles, if necessary, deferred for at least 24 hours after initiating antistaphylococcal antibotics?
Cavernous sinus thrombosis risk
External ligation of the anterior ethmoid artery is obtained through what approach?
Lynch incision - located ~ 24 mm posterior to anterior lacrimal crest along frontoethmoid suture line
Describe location of SPA for endoscopic ligation
Posterior to the inferior attachment of the middle turbinate, submucosal on the lateral nasal sidewall near posterior maxillary sinus wall.
What autosomal dominant disorder results in punctate hemangiomas or vascular sinuses that are irregularly shaped, associated with thin epithelium, and have no muscular or elastic layers resulting in easy bleeding?
Osler-Weber-Rendu disease (HHT)
Organs most commonly affected in HHT
Nasal cavity, oral cavity, GI tract, lungs, liver
Young’s procedure
When the nasal cavities are closed by creating two layered flaps (nasal mucosa and skin), thus eliminating airflow through the nasal cavities. Used for extreme recurrent epistaxis patients.
What are the main forms of rhinitis?
Allergic and nonallergic
Primary subtypes of allergic rhinitis
Seasonal allergic rhinitis
Perennial allergic rhinitis
Mixed allergic rhinitis
Classic symptoms of allergic rhinitis
- Sneezing
- Rhinorrhea
- Nasal congestion
- Pruritus
- Watery eyes
- PND
- Anosmia/hyposmia
Common comorbidities associated with allergic rhinitis
OSA/SDB, asthma, acute rhinosinusitis, otitis media with effusion,
Subtypes of nonallergic rhinitis
- Vasomotor rhinitis
- Infectious rhinitis
- Nonallergic rhinitis of eosinophilia syndrome (NARES)
- Gustatory rhinitis
- Occupational
- Hormonally induced
- Medication induced
- Atrophic
- Inflammatory/immune related disorders
Symptoms associated with nonallergic rhinitis
- Sneezing
- Rhinorrhea
- Nasal congestion
- PND
Vasomotor rhinitis
Excess parasympathetic tone resulting in vasodilation, which can be triggered by cold temperatures and strong smells resulting in nonallergic rhinits
Surgical intervention (although controversial) that can be considered for patients with vasomotor rhinitis
Vidian neurectomy
NARES
Nonallergic rhinits of eosinohpilia syndrome
Presents with perennial symptoms of sneezing, watery rhinorrhea, nasal pruritus, intermittent hyposmia/anosmia. Demonstrates 10-20% eosinophils on nasal smear and is associated with negative in vivo and in vitro allergy testing
Underlying pathophysiology of gustatory rhinitis
Vagally (cholinergically) mediated vasodilation after eating (especially with hot or spicy foods)
Common medications that can cause rhinitis
ACE inhibitors, beta blockers and other antihypertensives, sildenafil, oral contraceptives, aspirin in sensitive individuals, alcohol
What organism commonly colonizes the nasal mucosa in patients suffering from atrophic rhinitis?
Klebsiella ozaenae
Clinic exam findings associated with atrophic rhinitis?
Foul odor, yellow/green crusting, atrophic/fibrotic mucosa
Management options for nonallergic rhinitis
- Intranasal glucocorticoids (flonase - fluticasone, nasocort - triamcinolone, nasonex - mometasone, rhinocort budesinide)
- Intranasal antihistamine (astelin, astepro - second generation H1 antagonists)
- Intranasal ipratroprium bromide (anticholinergic) (atrovent)
- Nasal irrigations
- Adjunctive oral medications (antihistamines, decongestants)
- Dymista (combination of azelastine and fluticasone)
- For allergic rhinitis use combination of all the above. Can also use cromyln sodium nasal spray (mast cell stabilizer)
What is proposed reason an intranasal antihistamine nasal spray would benefit someone with nonallergic rhinitis?
Anti-inflammatory. Decreased esosinophil activation, expression of adhesion molecules and cytokine production. Potentially decreases neurogenic excitation from olfactory stimuli.
Subacute rhinosinusitis
Rhinosinusitis lasting 4-12 weeks
Two most common pathogens associated with acute viral rhinosinusitis
Rhinovirus and influenza virus
What % of viral rhinosinusitis is estimated to progress to bacterial sinusitis?
0.5-2%
What do some guidelines on acute rhinosinusitis recommend against using mucus color to dictate antibiotic use?
Mucus color is driven by neutrophils, not bacteria
Classic triad associated with Kartagener syndrome
Situs inversus, Bronchiectasis, CRS
Caused by dynein arm defect, autosomal recessive
What percentage of patients with CRS will also have asthma?
50%
What cytokine or proinflammatory mediator is thought to be primarily involved in Samter triad patients?
Leukotrienes
In patients with refractory rhinosinusitis what underlying immunodeficiencies may be identified?
- common variable immunodeficiency (10%)
- Selective IgA deficiency (6%)
- Low titers of IgG (18%), IgA (17%), or IgM (5%)
What is prevalence of rhinosinusitis in HIV population?
20-70%. Patients with HIV are increased risk because of lymphocyte dysfunction and increased mucociliary transport time.
What lab tests should be considered for refractory CRS or recurrent acute to evaluate for underlying immunodeficiency?
- Quantitative immunoglobulin assays (IgG, IgA, IgM)
- Antibody response to tetanus toxoid and pneumococcal vaccines (before and after vaccination)
- T cell number and function
Lund Mackay system
Staging system that grades the amount of mucosal disease present in left and right frontal, anterior/posterior ethmoid, maxillary and sphenoid sinuses (0=clear, 1= partial opacification, 2= complete opacification) and ostiomeatal complex (0=clear, 2=occluded)
Overtime more rare anaerobic infections can occur in CRS. What are three such pathogens?
- Fusobacterium spp
- Peptostreptococcus spp
- Prevotella spp
Three primary subtypes of CRS
- CRS with NP
- CRS without NP
- Allergic fungal rhinosinusitis
Four macrolides that can be considered for long term antibiotic therapy in CRS without NP?
- Azithromycin
- Clarithromycin
- Roxithromycin
- Erthyromycin
What is the only FDA approve topical corticosteroid spray for nasal polyposis?
Mometasone
Most common sites for extrasinus complications associated with rhinosinusitis
- Orbital (60-75%)
- Intracranial (15-20%)
- Bony (5-10%)
Name the valveless veins that allow retrograde spread of thrombophlebitis from mucosal veins to emissary veins which pass through the diploe between the anterior and posterior tables of the cranial cancellous bone to subdural veins and ultimately to cerebral veins
Veins of Breschet (aka diploic veins)
What approaches can be used for surgical intervention of subperiosteal abscess (chandler group 3)
- Endonasal endoscopic drainage for medial abscess
- External ethmoidectomy via a Lynch incision
- Transcaruncular transconjunctival approach
Superior orbital fissure syndrome
- Can result from an orbital abscess
- Associated with ptosis, proptosis, opthalmoplegia, fixed and dilated pupil, V1 anesthesia
Orbital apex syndrome
- Can result from an orbital abscess
- Associated with ptosis, proptosis, opthalmoplegia, fixed and dilated pupil, V1 anesthesia and vision loss
Cavernous sinus thrombosis symptoms
Orbital pain, conjunctival and lid edema, vision loss, proptosis, exophthalmosis, photophobia, involvement of CN II, III, IV, V1 and VI
Intracranial complications associated with rhinosinusitis
- Meningitis
- Epidural abscess
- Subdural abscess
- Intracerebral abscess
- Cavernous sinus thrombosis
What paranasal sinuses are most frequently involved by mucoceles?
The frontal sinus is most common followed by ethmoid sinus. Maxillary and sphenoid sinus are less frequently involved
Characteristic imaging findings for allergic fungal rhinosinusitis
CT: hyperdense central mucin surrounded by rim of hypointensity with speckled areas of increased attenuation resulting from ferromagnetic fungal elements. Unilateral > bilateral. May have bony expansion of the paranasal sinuses
MRI: T1 and T2 show central hypointensity surrounded by hyperintensity and T2 may show a central void
Characteristics of eosinophilic mucin
- Necrotic inflammatory cells
- Eosinophils
- Charcot Leyden crystals
- Fungal hyphae
What fungi are commonly implicated in allergic fungal rhinosinusitis
Alternaria, aspergillus, bipolaris, curvularia, cladosprium, dreschlera
What is the most common fungus isolated in a fungus ball
Aspergillus fumigatus
Define the distribution of paranasal sinus fungus balls
Maxillary> sphenoid> ethmoid> frontal sinuses
Paranasal fungus ball exhibit what imaging characteristics?
- Complete or subtotal opacification, usually of a single sinus
- Osteal thickening or sclerosis
- Noncontrast CT shows hyperattenuating lesion with punctate calcifications. Fungus ball is hypointense on T1 weighted and T2 weighted images due to abscence of free water
- Calcifications and paramagnetic metals generate areas of signal void on T2 weighted images
Treatment of fungus ball
Surgical debriedment and postop irrigations
What are the most common physical exam findings suggestive of acute invasive fungal sinusitis
Early: pale, boggy mucosa, petechia, or areas of ischemia
Late: black eschar, sloughing mucosa, gross hyphae, decreased sensation, mucosa does not bleed
- The middle turbinate is the most commonly affected site followed by the septum and inferior turbinate
Findings on CT scan suggestive of invasive fungal sinusitis
- Unilateral sinus involvement (more common than bilateral)
- Severe soft tissue edema of the nasal cavity mucosa
- Paranasal sinus mucoperiosteal thickening
- Bone erosion
- Facial soft tissue swelling
- Retroantral fat pad thickening
- Orbital invasion
- Intracranial invasion
Which fungal organisms have nonseptate twisted hyphae that branch at 90 degree angles, are seen in a necrotic background and often demonstrate angioinvasion, which most commonly occurs in diabetic keotacidosis?
Mucormycoses
What is the antifungal of choice for IFS (mucor and aspergillus)
Mucor - amphotericin
Aspergillus - Voriconazole
What patient populations are most at risk for development of chornic invasive fungal rhinosinusitis
- Mildly immunocompromised
- Elderly
- Diabetic
- Glucocorticoid use
- AIDS
What are the most common CT findings associated with chronic invasive fungal rhinosinusitis
Thickened mucosa associated with bony erosions and a mass lesion in a single paransal sinus (most common in the sphenoid or ethmoid)
What are the findings are histopathology that suggest chronic invasive fungal sinusitis?
- Dense accumulation of hyphae
- +/- vascular invasion
- Little to no inflammatory reaction to invasion and destruction of local structures
What is the most common fungal species associated with chronic IFS
Aspergillus fumigatus
Recommended treatment for chornic IFS
- Rapid diagnosis and intervention with IV antifungals, aggressive surgical resection and reverse underlying immune dysfunction
What form of invasive fungal rhinosinusitis is diagnosed after 12 weeks duration, most commonly in immunocompetent patients from Sudan, India, Pakistan and Saudi Arabia
Chronic granulomatous invasive fungal sinusitis
What is the most common manifestation seen in patients with chronic granulomatous invasive fungal rhinosinusitis
- Enlarging mass (cheek, orbit, nose, paranasal sinus)
- Gradual onset of symptoms
What are histopathologic findings most commonly seen in chronic granulomatous invasive fungal rhinosinusitis?
- Significant fibrosis
- Noncaseating granulmoas
- Foreign body type reactions
- Langerhans type multinucleated giant cells
- Possible vasculitis, vascular proliferation, perivascular fibrosis
What fungal species is most often implicated in chronic granulomatous invasive fungal rhinosinusitis?
Aspergillus flavus
Recommended treatment for chronic granulomatous invasive fungal rhinosinusitis
Surgical biopsy and debridement, system antifungals
What type of papilloma arises within the nasal vestibule and nostril from the stratified squamous epithelium, is more common in males, and can be treated by simple excision or cauterization?
Vestibular (keratotic) papilloma
What three types of papillomas arise in the nasal cavity?
- Fungiform papilloma (also called exophytic, septal or everted papilloma)
- Inverted papilloma (also called Schneiderian, epithelial, transitional cell, or Ringertz papilloma)
- Oncocytic papilloma (also called cylindrical)
Where do everted papillomas of the nasal cavity most commonly arise?
Nasal septum (rarely: inferior turbinate, nasal vestibule, nostril)
Describe the gross and microscopic characteristics of everted papillomas of the nasal cavity
- Gross: raised, verrucous, 1-15mm in diameter, single, unilateral, attached to mucosa via a broad base
Micro: branching fronts of mucosa with a connective tissue core with stratified squamous epithelium; koilocytosis is common
What HPV subtypes are commonly associated with everted nasal paillomas
HPV 6 and 11
What is the treatment for everted papillomas of the nasal cavity?
Simple excision or cauterization. Rare recurrence or transformation possible
Where do inverted papillomas most commonly arise within the nasal cavity and from what epithelial subtype do they originate?
Lateral nasal wall. May also arise from ethmoid air cells or maxillary sinus. Unilateral more common than bilateral. Come from Schneiderian epithelium
What patient demographic most commonly has inverted papilloma
White men (1M:0.75F) in their 60s and 70s
Describe the gross and microscopic characteristics of inverted papilloma
Gross: Unilateral, pale pink to reddish gray, polypoid (“mulberry”) mass arising from a stalk (can be broad or narrow), irregular, friable, often firmer than inflammatory polyps, although may be difficult to differentiate
Microscopic: Hyperplastic ribbons of basement membrane and epithelium invaginating into the underlying stroma. Stroma demonstrates inflammatory changes containing fibrosis and edema. Multilayered squamous, columnar or transitional cell epithelium (or a combination) containing mucocytes and intraepithelial mucous cysts
Which HPV subtypes are most commonly associated with inverted papilloma?
HPV 16, 18 although 6 and 11 have as well
What is the incidence of malignant degeneration of IP to SCC?
5-10%
Although the risk of recurrence of IP is high, what might increase the risk of recurrence?
Surgical approach and multicentricity of the tumor
What two imaging modalities are used most commonly during the workup of inverted papillomas?
Contrast CT: demonstrates bony destruction including erosion, remodeling and sclerosis; may demonstrate areas of calcification within the lesion
Contrast MRI: Can differentiate inspissated secretions, mucoperiosteal thickening, and inflammatory changes. T1 lesion is ~hyperintense to muscle; T2 inspissated secretions and inflammatory polyps are hyperintense
What are the treatment options available for sinonasal IP?
- Complete surgical excision
- Radiation therapy
- Observation
When is radiation therapy for IP recommended?
- For aggressive, multifocal disease
- For SCC
- If patient cannot tolerate surgery or if the functional or cosmetic result of surgical resection are not acceptable
How long should patients be followed for surveillance of IP and when should CT scans be ordered?
Minimum of 5 years. Recurrence most often occurs between 2 and 10 years. CT scans should be ordered if scarring limits full visualization of resection cavity, if patient is symptomatic or evidence of recurrence
What is the least common unilateral Schneiderian nasal papilloma?
Oncocytic (cylindrical) papilloma, 3-5% of all sinonasal papillomas
- Treat with complete surgical excision
Oncocytic papilloma histopathology
Endophytic invaginations of tall columnar, multilayered epithelium composed of oncocytes and containing microcysts laden with mucin and neutrophils
What is reported malignant potential of oncocytic papillomas
10-17%
- Have a relatively high rate of recurrence after resection
What is the most common benign nasopharyngeal tumor that most commonly affects prepubescent males at an average age of 14-15% (range: 10-15)
Juvenile nasopharyngeal angiofibroma
Describe the major hypothesis put forth to explain the development of JNA
- Incomplete regression of the first branchial arch artery
- Development from embryologic chondrocartilage of the skull base at the junction of the palatine bone, horizontal ala of the vomer and root of the pteryoid process
- Abnormality of the pituitary androgen estrogen axis
What is the most common blood supply to JNAs
- Internal maxillary artery
- May also arise from ascending pharyngeal, external/internal carotid artery and occasionally from the contralateral supply
Characteristic gross appearance of a JNA
- Welll circumscribed, smooth, lobulated, purple to reddish hue compressible
Characteristic routes of spread of patterns of growth of JNA
- Pterygopalatine (sphenopalatine) fossa
- Orbit -> middle cranial fossa
- Masticator space -> intracranial cavity
- Infratemporal fossa -> cheek or intracranial cavity
- Nasal cavity
- Paranasal sinus (sphenoid sinus -> intracranial cavity)
- Nasopharynx
- Dural invasion is rare
What specific findings can be seen on CT and MRI that help distinguish a JNA?
CT: bony remodeling without frank bony destruction
MRI: flow voids on bone T1 and T2 weighted imaging
Holman Miller sign
Anterior bowing of the posterior maxillary sinus associated with JNA
What arguments are made against using preop embolization for JNA
- It can potentially obscure the tumor boundaries resulting in higher rate of recurrence
- Complications associated with embolization are not benign: stroke, blindness, facial paralysis, skin and soft tissue necrosis
- Some argue that tumor hypoxia can decrease the radiosensitivity of these tumors
What is the risk of recurrence for JNA and what increases the risk?
- up to 40%
- Large tumors, intracranial extension, involvement of sphenoid, base of pterygoids or clivus, failure to remove all tumor, or primary radiation treatment
Between cavernous and capillary sinonasal hemangiomas which is more common in adults?
Cavernous hemangiomas
Where do sinonasal hemangiomas most commonly arise within the nasal cavity and or sinuses?
- Cavernous: lateral nasal wall or medial maxillary wall
- Capillary: septum
What are the most common symptoms associated with sinonasal hemangiomas?
Epistaxis and nasal obstruction
Imaging characteristics of sinonasal hemangiomas
CT: enhance on CT with contrast. Often indistinct from turbiantes and can be associated with bony destruction
MRI: enhance with contrast, intermediate signal intensity, +/- flow voids. If intraosseous it may have a soap bubble, sun ray or honeycomb appearance
Management of choice for sinonasal hemangiomas
Surgical resection. Can consider preop embolizations
What pathologic lesion can be seen in the sinonasal or oral cavity as a rapidly growing mass resulting in nasal obstruction and epistaxis and is composed of proliferating capillaries separated into lobules by loose connective stroma?
Lobular capillary hemangiomas (previously known as pyogenic granulomas)
What theories have been presented as explanations for the formation of lobular capillary hemangiomas?
- Local trauma
- Hormonal fluctuations
- Infection
- AVM
- Local production of angiogenic growth factors
What benign neoplasm can develop from Zimmerman pericytes (contractile cells surrounding small vessels) and can be seen in the sinonasal tract, retroperitoneum, lower extremities and pelvis?
Hemangiopericytoma
Typical clinical presentation and gross appearance of a sinonasal hemangiopericytoma?
- Nasal obstruction and epistaxis
- Gray/tan mass that is spongy, vascular, and polypod in appearance and involving the sinonasal cavity and often one or more paranasal sinuses
Imaging characteristics associated with sinonasal hemangiopericytoma
CT: homogenous, enhancing lesion, expansiale, resulting in bony remodeling
MRI: Enhance on T2 weighted imaging, intermediate inhancement on T1 weighted
Most common bening tumor of the sinonasal tract that occurs as an expansile proliferation of mature bone within the membranous bones of the skull and face
Osteoma
Which sinuses are most commonly involved with osteomas
Frontal> ethmoid> >maxillary»_space; > sphenoid
Most likely diagnosis for a patient with multiple osteomas, intestinal polyps and epidermoid inclusion cysts and desmoid tumors?
Gardner syndrome (autosomal dominant)
Explanations of osteoma development
- Embryologic theory (development from the junction of the frontal bone, which is membranous and the ethmoid bone which is cartilaginous)
- Inflammation/infection theory
- Traumatic theory
- Slow growing osseous hamartomas
Describe the three subtypes of osteomas
- Ivory (eburnated): compact, dense bone, minimal fibrous tissue
- Mature (spongiosum): spongy, mature bony, tracebulum composed of fibrous tissue
- Mixed: components of both ivorty and mature osteomas
Average growth rate of an osteoma
1.6mm/year
Imaging characteristics assocaited with osteomas?
CT: range from high density lesion to a less dense ground glass appearing lesion based on the amount of mineralized bone
MRI: necessary only to evaluate adjacent soft tissue, trapped secretions or mucoceles
Hyperostosis frontalis interna
Hyperostosis of the inner table of the skull, thickened cancellous bone and intact cortex sparing the midline and frequently seen in post menopausal women
Morgagni syndrome (metabolic craniopathy)
Hyperostosis in postmenopausal omen in the presence of obesity and hirsutism
What characteristics can be used to differentiate an ossifying fibroma from fibrous dysplasia?
An ossifying fibroma typically has a capsule and more mature bone and is more common in black women in their 30s-40s
Imaging characteristics common to ossifying fibroma
CT: well defined, multiloculated lesion, surrounded by a bony capsule
MRI: hyperintense T2 signal, T1 signal is intermediately intense centrally and hyperintense peripherally
Treatment of choice for ossifying fibromas?
Radical surgical resection
What bony disorder is due to the replacement of normal bone with fibro-osseous tissue (women-ossified tissue, increased bony matrix, abnormal mineralization, marrow fibrosis) that can occur in monostotic, polyostotic or syndromic (McCune Albright) forms?
Fibrous dysplasia
Radiographic and histologic appearance of fibrous dysplasia
Radiographic: expansile lesion with relatively homogenous smooth ground glass appearance
Histology: irregularly shaped, patternless woven trabecular bone with vascularized fibrous stroma; the irregular shaped spicules can occur as Chinese writing type, pagetoid type and hypercellular type
Although rare (<4%) malignant transformation of fibrous dysplasia can occur. What is the most common cancer associated with this transformation?
Osteosarcoma
What is the name given to the congenital dehiscence seen in some patients in the lateral sphenoid roof and is thought to be due to a persistence of the lateral craniopharyngeal canal?
Sternberg canal
How much CSF is required to test for beta-2 transferrin
0.4 mL
Potential side effects associated with low dose intrathecal fluorescein
- Malise, headache, dizziness, nausea, vomting, back pain, lower extremity weakness, tinnitus, neck pain, fever
Higher doses can cause seizures and cranial neuropathies
In patients with known idiopathic intracranial hypertension and history of CSF leak what additional interventions can decrease risk of recurrence?
- Weight loss
- Acetazolamide
- Ventriculoperitoneal shunt
Three primary granulomatous diseases that can involve the nasal airway?
- Sarcoid
- GPA
- Churg Strauss syndrome
Classic clinical triad seen in GPA
- Necrotizing granulomas of the respiratory tract (lung most commonly)
- Vasculitis
- Glomerulonephritis
Otologic manifestations of GPA
- Sinonasal: obstruction, rhinorrhea, crusting, sinusitis, epistaxis, septal perforation, saddle nose deformity
- Otologic: conductive hearing loss with serous otitis media, less commonly SNHL and vertigo
- Subglottic: stridor, dyspnea, subglottic stenosis, crusting
The antibodies used for diagnosis of GPA are directed at what protein?
PR3
What blood tests are highly sensitive for GPA but if negative do not completely exclude the diagnosis?
C-ANCA, PR3, anti myeloperoxidase (AMO)
What pathologic findings are seen on biopsies of a patient with GPA
- Necrotizing, noncaseating giant cell granulomas
- Small and medium vessel vasculitis
- Microabscesses
What is the most common nasal symptom associated with sarcoidosis?
Nasal obstruction. Other symptoms include epistaxis, epiphora, nasal pain and dyspnea. Nasal symptoms are present in <5%.
Where does sarcoid most commonly manifest in the nose?
Septum and inferior turbiantes and commonly involved with thick crusting. At later stages yellow subcutaneous nodules can be seen
Typical disease course of sarcoidosis
Spontaneous resolution within 2 years of disease onset. ~10% will have progressive disease and pulmonary fibrosis
What laboratory test can be used in the diagnosis of sarcoidosis?
Serum angiotensin converting enzyme
What % of patients with active sarcoidosis will have a positive SACE test?
80%
Treatment for sinonasal sarcoidosis?
Nasal saline irrigations and topical nasal steroids
Criteria for diagnosis of Churg Strauss syndrome
Four of the following must be present in a patient with known vasculitis
- Asthma
- More than 10% eosinophils in peripheral blood
- Neuropathy
- Pulmonary opacities on chest x ray
- Sinonasal disease
- biopsy of blood vessel showing eosinophil accumulation
What are the two most common causes of midline granuloma syndrome?
- GPA
- T cell lymphoma
What criteria are needed to make a diagnosis of relapsing polychondritis?
- Chondritis of two of three sites (auricle, nasal or laryngotracheal) or chondritis of one of the above listed sites with two other features (ocular inflammation, seronegative inflammatory arthritis or vestibular dysfunction, hearing loss)
What is the method of inheritance associated with the most common form of anhidrotic ectodermal hypoplasia?
X linked recessive
What % of patients with primary ciliary dyskinesia will have situs inversus?
50%
Rhinophyma
Results from hypertrophy of the sebaceous glands in both the nasal skin and fibrosis
Two early signs of rhinophyma
- Dilated (patulous) pores
- Telangiectatic vessels on the distal nose
Rhinophyma may manifest as the final stage of what other skin disease?
Acne rosacea, although not all patients with rhinophyma have a history of rosacea
What malignant condition can be associated with rhinophyma?
BCC
How is rhinophyma managed?
Inflammation can be managed conservately, similar to rosacea. For significant hypertrophy, deformity and nasal obstruction, surgical recontouring can be performed using CO2 laser with or without dermabrasion
What is the infectious cause of rhinoscleroma, an infectious granulomatous disease that is endemic to Africa, southeast Asia, and Central America and affects the nasal cavity and occasionally the larynx, paranasal sinuses and nasopharynx?
Klebsiella rhinoscleromatis
Clinical stages associated with rhinoscleroma?
- Catarrhal (atrophic): purulent rhinorrhea, rhinitis, honey nasal crusting
- Granulomatous (hypertrophic): painless granulomatous nodules in the upper respiratory tract
- Sclerotic: healing with extensive scar tissue formation and nasal stenosis
Histologic findings associated with rhinoscleroma?
Mikulics cells (foamy histiocytes) and Russel bodies (intracellular inclusions associated with excessive immunoglobulin synthesis)
Management of choice for treating rhinoscleroma?
Long term culture specific antibiotics and surgical debriedment
What granulomatous infection is endemic to Africa, Pakison, Sri Lanka, and India and manifests with strawberry red, friable, polypoid lesions of the nose and eye causing nasal obstruction and epistaxis?
Rhinosporidiosis
What is the classic histopathologic findings associated with rhinosporidiosis
Fungal sporangia with chitinous elements in the setting of pseudoepitheliomatous hyperplasia and submucosal cystic structures
What areas of the sinonasal cavity most commonly manifest acute invasive fungal sinusitis?
The middle turbinate most common followed by the septum and inferior turbinate. The palate and oral cavity must also be evaluated
CT scan findings suggestive of invasive fungal sinusitis
- unilateral sinus involvement
- severe soft tissue edema of the nasal cavity mucosa
- paranasal sinus mucoperiosteal thickening
- bone erosion
- facial soft tissue swelling
- retroantral fat pad thickening
- orbital invasiona
- intracranial invasion
What fungal organisms are known to have septate hyphae that branch 45 degree angles and are best seen by using methenamine sliver stain?
Aspergillus spp
UPSIT scoring
A multiple choice test of smell. 40 questions with 4 multiple choice answers.
0-5: malingering (should get at least a 10 with just random guessing)
5-18: anosmia
19-33: hyposmia
34-40: normal smell
Pediatric versus adult allergic fungal sinusitis
Both are likely to grow curvularia and bipolaris species. Children are more like to present with obvious facial abnormalities (proptosis) and unilateral disease. Adults are more likely to grow aspergillus.
