Nasal obstruction & masses

Question 1

What are the 5 main etiologies of congenital nasal obstruction?

Answer 1

1. Developmental errors of anterior neuropore
2. Developmental errors of the central midface
3. Developmental errors of the nasobuccal membrane
4. Non-unique mesodermal and germ-line malformations
5. Birth trauma

Question 2

Discuss a differential of nasal obstruction based on the 5 most common etiologies of congenital nasal obstruction.

Answer 2

1. Developmental errors of anterior neuropore
   - Dermoid
   - Encephalocele
   - Glioma
2. Developmental errors of the central midface
   - Arhinia
   - Polyrhinia
   - Accessory Nostril
   - Proboscis Lateralis
   - Craniofacial clefts
   - Cleft lip-associated nasal deformity
   - CNPAS
   - Nasolacrimal duct cysts
3. Developmental errors of the nasobuccal membrane
   - Choanal atresia
   - Choanal stenosis
4. Non-unique mesodermal and germ-line malformations
   - Hemangioma
   - Lipoblastoma
   - Nasopharyngeal Teratoma
5. Birth trauma

Question 3

Describe the embryology of the Anterior neuropore of the nose, and the possible developmental errors that result.

Answer 3

A. ANTERIOR NEUROPORE (Primitive Frontonasal region)
1. 3 weeks - midline neural groove appears along dorsum of embryo –> forms neural tube (precursor of CNS)
2. 4 weeks - neural tube closes (from midpoint to anterior and posterior)
- Caudal and cranial neuropores close last (cranial tip of tube at sphenoid sinus)
– Anterior neuropore forms at optic recess of sphenoid sinus
– Area immediately proximal to the anterior neuropore forms the frontal, nasal, and ethmoid structures

• Neural crest cells from ectoderm of the tube migrates to mesenchyme –> forms bony/cartilaginous structures of the primitive sinonasal complex
  – Anterior neuropore = most distal point of neural crest cell migration
  – Lack of neural crest cells and late tube closure = high risk of developmental defects
  – Foramen cecum: Region between ethmoid & frontal bones, connects with prenasal space, location where cribriform plate condenses
  – Fonticulus Nasofrontalis: Fontanelle between the inferior frontal and nasal bones; later fuses with foramen cecum (sort of an “extranasal” space)
  – Prenasal space: Between nasal bones and nasal capsule/cartilages
  – Nasal capsule: Precursor to nasal cartilages & septum, continuous with ethmoid labyrinth

3. 3-8 weeks: Dura projects through foramen cecum –> traverses prenasal space –> sticks to ectoderm at tip of the nasal bones (site of future rhinion). As foramen closes, dura detaches and retracts back intracranially

POSSIBLE ERRORS:
1. Ectoderm doesn’t unstick –> during retraction the ectoderm gets pulled back along the tract of retreating dura, forming an epithelial line tract to the skull base = Dermoid cyst/fistula/sinus (dura is pulled back into brain, but the ectoderm was stuck to it and elongated within the tract)
2. Foramen closes prematurely, trapping brain tissue in prenasal space = Glioma (dura doesn’t pull back therefore leaves brain behind; whereas for dermoids the dura does pull back and its the ectoderm that is lengthened and left behind)
3. Foramen fails to close completely (persistent intracranial communication) = Meningocele/encephalocele
4. Fonticulus closes abnormally causing an extranasal path to persist = nasofrontal meningoceles/encephalocele/glioma (see photo 5 in Vaccani lecture)

Cummings Chapter 190 for image differences
Vaccani lecture Midline nasal masses of images

Question 4

Describe the embryology of the central midface of the nose, and potential developmental errors that can result

Answer 4

B. CENTRAL MIDFACE

1. 3-4 weeks: Neural crest cells migrate from their origin in the dorsal neural folds to form the first and second branchial arch-derived facial prominences surrounding the Stomodeum. Stomodeum is flanked by the frontonasal prominence superiorly, maxillary prominence laterally, and mandibular process inferiorly
   - Lateral portions of the frontonasal prominences thicken to form nasal placodes –> invaginate to form nasal pits
   - Nasal pits form nasal cavity & sinuses, separated from oral cavity by nasobuccal membrane (aka. oronasal membrane)
   - Ridges of tissue around the pits are the lateral and medial nasal prominences
   - Pits continue to canalize superior to inferior AFTER birth to form nasolacrimal ducts
2. 5 weeks: Nasobuccal membrane is pieirced to form choana
3. 6-12 weeks: Lips and nose form
   - Medial nasal prominences fuse = philtrum and medial upper lip
   - Maxillary prominences = lateral upper lip
   - Lateral nasal prominences = nasal alae

POSSIBLE ERRORS:
1. ARHINIA (Complete agenesis of the nose), which can result from:
- (1) Abnormal migration of neural crest cells
- (2) Failure of fusion of the medial and lateral nasal prominences
- (3) Overgrowth and premature fusion of the medial nasal prominences
- (4) Lack of resorption of the nasal epithelial plugs

2. POLYRHINIA (double nose) and SUPERNUMERARY NOSTRIL (accessory nostril):
   - Incomplete development of the frontonasal process, allowing separation of the developing lateral portions of the nose
   - Medial nasal process and septum continue to develop and are duplicated, forming a double nose
3. PROBOSCIS LATERALIS (Rudimentary nose/appendage off-centre from midline - tubular sleeve of skin attached to the inner canthus of the orbit and heminasal aplasia on the affected side)
   - Secondary to fusion of the maxillary process on the affected side with the contralateral developing nasal process
   - Mesodermal proliferation in the frontonasal and maxillary processes adjacent to the forming nasal pits may lead to fusion of these following epidermal breakdown, leaving the lateral nasal process sequestered as a tube arising in the frontonasal region, and absence of a nasolacrimal duct
4. NASOLACRIMAL DUCT ABSENCE
   - Associated with proboscis lateralis
5. NASOLACRIMAL DUCT CYST
   - Incomplete canalization of the nasal pits
6. CONGENITAL NASAL PYRIFORM APERTURE STENOSIS (CNPAS)
   - Deficient development of the primary palate and bony overgrowth of the nasal process of the maxilla (central megaincisor)
7. MIDLINE CLEFTING
   - Midline & paramedian fusion errors

Errors may be solitary or as part of HOLOPROSENCEPHALY SPECTRUM

Cummings Chapter 190

Question 5

Describe the embryology of the nasobuccal membrane and potential developmental errors that can result

Answer 5

EMBRYOLOGY
- The burrowing nasal pits give rise to the nasal pouches, which lie above the oral/buccal cavity
- The nasobuccal membrane separates the nasal and oral/buccal spaces
- Failure of the rupture and canalization of the nasobuccal membrane underlies choanal atresia

POTENTIAL ERRORS:
- Choanal Atresia
- Choanal stenosis

Dr. Vaccani “Midline nasal masses 2022” lecture

Question 6

Describe the embryology of mesodermal and germ-line malformations of the nose and potential developmental errors that can result

Answer 6

• Proliferations of cell-types distributed throughout the body can cause obstructive tumors in the nose/paranasal sinuses

Examples:
- Hemangiomas
- Lipoblastomas

Question 7

Describe how congenital nasal obstruction can occur from birth trauma?

Answer 7

Nasal septal injury may occur secondary to trauma in the birth canal, producing nasal obstruction

Question 8

Regarding Arhinia, discuss:
1. What is the typical clinical presentation - 4 symptoms
2. 5 exam features
2. What is the management? 5

Answer 8

Symptoms:
1. Respiratory distress and cyanosis with feeding
2. Gulp food between breaths
3. Hypernasal speech (if there is a remnant hole)
4. Hyposmia

Exam Features:
1. Absence of the external nose and nasal airways (septum, sinuses)
2. Hypoplasia of the maxilla
3. Small high-arched palate
4. Hypertelorism (increased distance between body parts)
5. Abnormalities of the eye - anopthalmia, hypoplasia of orbits

MANAGEMENT:
1. Feeding management (G-tube)
2. Prosthetic nose
3. Vertical distraction osteogenesis of midface - increase midfacial height and maximize bone and soft tissue for later reconstruction
4. Reconstruction (create nasal passageway through maxilla, line nasal passage with STSGs and maintain with long term stenting)
5. Dacryocystorhinostomy –> prevent recurrent conjunctivitis from absence of nasolacrimal ducts

Question 9

Regarding Polyrhinia and Supernumerary Nostril, discuss:
1. What is the clinical presentation? 2
2. What are the primary steps in management? 2

Answer 9

POLYRHINIA

Clinical Presentation:
1. Anterior nasal defects (septal duplication, duplicated nasal passageways)
2. Posterior nasal defects (choanal atresia)

Management:
1. Correction of choanal atresia
2. Surgical correctiion - Removing the medial portions of each nasal passage and anastomosing the lateral portions in the midline. This results in broad flat nose with midline depression (can be corrected later by medial in-fracture of nasal bones and rhinoplasty techniques)

Question 10

Supernumerary Nostril, discuss:
1. What is the clinical presentation? 2
2. What are the primary steps in management? 2

Answer 10

SUPERNUMERARY NOSTRIL

Clinical Presentation:
1. External appearance of a small accessory nasal orifice with surrounding redundant soft tissue (orifice may be lateral, medial or superior to nose)
2. True fistulas = discharge from orifice

Treatment:
1. Excision of supernumerary nostril & primary closure or local flaps

Question 11

Regarding Proboscis Lateralis, discuss:
1. What are the associated abnormalities? 2
2. What is the typical management?

Answer 11

Associated abnormalities:
1. CNS abnormalities
2. Congenital ocular lesions - micropthalmia, coloboma, arachnoid cysts

Treatment: Delay until facial growth complete
1. Prosthetic device
2. Reconstruction with bone and cartilage grafts

Question 12

What is the differential for pediatric midline nasal masses?

Answer 12

1. Dermoid (most common) - ectoderm, mesoderm
   - Nasal Dermal Cysts (NDC)
   - Nasal Dermoid Sinus-Cysts (NDSC)
2. Teratoma - ecto+meso+endoderm
   - Sacrococcygeal (not H/ND)
   - Head and Neck: Cervical & Nasopharynx
3. Glioma
   - Extranasal (60%)
   - Intranasal (30%)
   - Combined (10%)
4. Meningocele
5. Encephalocele
   - Occipital (75%)
   - Basal (10%) - further broken down into transethmoidal (most common), sphenoethmoidal, transsphenoidal, sphenoorbital
   - Sincipital (15% upper half of skull) - further broken down into Nasofrontal (40%), nasoethmoidal (40%), and nasoorbital (20%)
6. Neurofibroma
7. Hemangioma

See flow chart from Dr. Vaccani lecture “Midline Nasal Masses 2022”

Question 13

What is the differential for unilateral pediatric nasal obstruction?

Answer 13

A. VASCULAR
1. Juvenile nasal angiofibroma JNA
2. Hemangioma
3. AVM

B. INFLAMMATORY/INFECTIOUS
1. Nasal polyps

C. TRAUMATIC
1. Nasal fracture

D. IATROGENIC
1. Foreign body

E. NEOPLASTIC
1. Lymphoma
2. Rhabdomyosarcoma
3. Teratoma
4. Neurofibroma
5. Hemangiopericytoma

F. CONGENITAL
1. Glioma
2. Encephalocele
3. Meningocele
4. Nasolacrimal duct cyst
5. Congenital nasal pyriform aperture stenosis - CNPAS
6. Choanal atresia

Question 14

What is the most common nasal mass in pediatrics?

Answer 14

Polyps or Foreign body

Question 15

What is the most common cause of infantile nasal obstruction?

Answer 15

Neonatal rhinitis

Question 16

Regarding neonatal rhinitis, discuss:
1. What are the suspected causes? 10
2. What are the possible organisms (when implicated)? 2
3. What is the treatment options? 7

Answer 16

CAUSES: generally considered a non-infectious pathology
1. GERD
2. Allergy (milk)
3. Viral infection
4. Bacterial infection
5. Hormonal (maternal estrogen)
6. Medications (maternal medications)
7. Maternal Cocaine
8. Hypothyroidism
9. Idiopathic
10. Cystic fibrosis

Baby related - GERD, milk allergy, hypothyroid, CF, idiopathic
Mother related - Hormonal, medications, cocaine
Environment related - viral or bacterial infection

ORGANISMS (when implicated - vertical transmission of STIs):
1. Chlamydia
2. Syphillis

TREATMENT:
A. Conservative
1. Nasal saline
2. Nasal suctioning
3. Humidified air
4. Consider nasal stents

B. Medical
1. Nasal decongestants (e.g. Otrivin x 3 days)
2. Nasal steroids (e.g. Decadron 0.1% drops x 2 weeks)
3. Antibiotics if signs of infection

Question 17

Regarding nasal dermoids, discuss:
1. What is it?
2. Clinical presentation - 3. What is the pathognomonic sign?
3. Associations - 6
3. Imaging findings. What are 2 findings suggestive of intracranial extension?
4. Treatment -
5. 4 surgical approaches?
5. Prognosis

Answer 17

Most common pediatric midline nasal mass (1/2-40000)

DEFINITION:
- Frontonasal inclusion cyst or tract formed when nasal ectoderm is retracted towards skull base by retreating dura in 3-8wks GA
- Epithelium lined, Ectodermal & mesodermal components (can contain hair follicles, sebaceous glands, and sweat glands)

CLINICAL PRESENTATION:
1. Can occur anywhere along midline nose - from nasal tip to cranial space (50% dimple present at or near rhinion + wide nasal bridge)
2. Usually show Midline pit/sinus/fiistula: discharge, infection
3. Pathognomic sign - Single big protruding hair (uncommon)
4. Up to 40% extend intracranial - risk meningitis
5. Up to 40% associated with other developmental anomalies

ASSOCIATIONS:
1. Aural atresia, pinne deformity
2. Hydrocephalus
3. MR
4. Cleft L/P
5. Hemifacial microsomia
6. Hypertelorism

IMAGING: CT and MRI
Sensitive signs that tract may continue intracranially:
1. Bifid crista galli
2. Enlarged foramen cecum
MRI: Hyperintense on T1 suggestive of intracranial dermoid (enhances also on T2)

TREATMENT:
1. Aspiration, I&D, Curretage - associated with recurrence
2. Complete surgical excision required to prevent recurrence
2. Consult Neurosurgery if intracranial excision required (craniofacial resection)
~5-10% recurrence

Surgical excision approaches:
1. Open rhinoplasty (most cosmetically acceptable)
2. Lateral Rhinotomy
3. Vertical Rhinotomy
4. Transglabellar subcranial

Kevan Peds Question 81

Question 18

What are the two embryological pathways of spread for dermoid into the brain?

Answer 18

1. Fonticulus Nasofrontalis
2. Foramen Cecum

Vancouver Page 466

Question 19

Regarding Nasal Gliomas, discuss:
1. What are they?
2. What are the different types (3). How do they present differently?
3. Imaging findings
4. Pathology
5. Management - 3

Answer 19

DEFINITION:
- Heterotopic glial tissue (nervous system tissue) that lacks a patent CSF communication to the subarachnoid space
- Do no contain ependymal tissue (lines brain/spinal cord and helps produce CSF), which differentiates them from encephaloceles
- 5-20% connected to brain by a fibrous stalk (this stalk is not brain tissue - if everything is brain tissue then this is an encephalocele)
- NO CSF COMMUNICATION
- High risk meningitis
- M:F 3:2

TYPES:
1. Extranasal (60%): Red-blue non-compressible mass @ glabella
2. Intranasal (30%): Pale, polypoid mass arising from lateral nasal wall near middle turbinate and occasionally from the septum, protrude out from the nostril
3. Combined (10%)

IMAGING: CT/MRI
1. Image before biopsying to r/o encephalocele - don’t want to cause a CSF leak
2. CT to assess bony anatomy of the skull base
3. MRI to assess for presence of soft tissue connections

PATHOLOGY:
1. Dysplastic, neuroglial and fibrovascular tissue
2. No ependymal tissue

TREATMENT: Surgical excision
1. Treat early in life to avoid meningitis & complications
2. Consult Neurosurgery for assistance: manage intracranial portion FIRST (prevent seeding infection from nose side)
3. Approaches similar to dermoid cyst + endoscopic:
- External rhinoplasty
- Transglabellar
- Subcranial
- Bicoronal
- Midline nasal approaches / vertical midline dorsal excision
- Nasal osteotomy when fibrous stalks are present that extend deep to nasal bones toward the base of skull

Recurrent rate 4-10%

Question 20

Discuss the surgical approaches for removing a nasal dermoid.

What are the criteria that must be fulfilled for extracranial removal? 4

Answer 20

Intracranial extension = combined intracranial/extracranial approach

Extracranial access should fulfill four criteria:
1. Excellent access to the midline
2. Access to the base of skull
3. Adequate exposure for reconstruction of the nasal dorsum
4. Acceptable scar

EXTRACRANIAL APPROACHES:
1. External rhinoplasty (most common)
2. Glabellar region with sinus tract:
- Lateral rhinotomy
- Midline vertical incision
3. Glabellar region without sinus tract:
- Transglabellar
- Paracanthal
- Eyebrow
- Bicoronal
4. Lesions extending into the cranial cavity
- Frontal craniotomy

Question 21

How do you differentiate a glioma from an encephalocele on clinical exam and histology? 6 things

Answer 21

Clinical exam: Gliomas has no CSF communication, therefore:
1. Does NOT transilluminate
2. Does NOT pulsate
3. Is NOT compressible
4. Does NOT expand with valsalva
5. NEGATIVE Furstenburg sign (does not expand with compression of ipsilateral IJV)

Histopathology:
1. Glioma does NOT have ependymal tissue (cells that line brain ventricles/lines CSF-filled cavities)

Question 22

What is Furstenburg’s Sign?

Answer 22

Expansion of a nasal mass with compression of both internal jugular veins, associated with encephalocele (CSF filled), but no glioma or dermoid

Question 23

Regarding nasal meningoceles and encephaloceles, discuss:
1. What is it?
2. What are the types? 8

Answer 23

DEFINITION:
- External herniation of brain/meninges
- M = F

TYPES OF MENINGOCELES/ENCEPHALOCELES:
1. Occipital (75%) - back of head (outside the nose)
2. Sincipital (15%) - anterior nose
a. Nasofrontal
b. Nasoethmoidal
c. Nasoorbital
3. Basal (10%) - Posterior nose/sinuses - hypertelorism, wide nose, nasal obstruction
- Transethmoidal
- Transsphenoidal
- Sphenoethmoidal
- Sphenomaxillary

SINCIPITAL MENINGOCELES/ENCEPHALOCELES
1. Nasofrontal
- Course: through bone defect between orbits and forward between nasal and frontal bones to the area superficial to the nasal bones
- Features: Glabellar mass, telecanthus, inferior displacement of nasal bones, wide nasal root
2. Nasoethmoidal
- Course: Through foramen cecum deep to the nasal bones, turning superficially at the cephalic end of the upper lateral cartilage to expand superficial to the upper lateral cartilage
- Features: Mass on the nasal dorsum, superior diisplacement of nasal bones, inferior displacement of alar cartilages
3. Nasoorbital
- Course: Through foramen cecum deep to the nasal and frontal bones through a lateral defect in the medial orbital wall
- Features: Orbital mass, proptosis, visual changes

BASAL MENINGOCELES/ENCEPHALOCELES
1. Transethmoidal (most common)
- Course: Through the cribriform plate into the superior meatus medial to the middle turbinate
- Features: Nasal obstruction, hypertelorism, broad nasal vault, unilateral nasal mass
2. Sphenoethmoidal
- Course: Passes through a bony defect between the posterior ethmoid cells and sphenoid
- Features: Nasal obstruction, hypertelorism, broad nasal vault, unilateral nasal mass
3. Transsphenoidal
- Course: Through a patent carniopharyngeal canal into the nasopharynx
- Features: nasopharyngeal mass, nasal obstruction, associated with cleft palate
4. Spheno-orbital
- Course: Through the superior orbital fissure and out the inferior orbital fissure into the sphenopalatine fossa
- Features: Unilateral exophthalmos, visual changes, diplopia

Question 24

Meningocele/Encephaloceles:
4. Clinical presentation - 6
4. Imaging findings - 3
5. Histopathologic findings - 2
6. Treatment & prognosis
7. Possible risks/complications - 3

Answer 24

CLINICAL PRESENTATION:
Bluish midline nasal mass that is:
1. Compressible
2. Transilluminates
3. Expands with valsalva/straining
4. Expands with compression of bilateral IJVs (Furstenberg’s sign)
5. 40% have associated developmental anomalies
6. High risk meningitis

IMAGING: CT and MRI
1. CT outlines the bony cartilaginous defect, whereas MRI provides complementary information regarding the soft tissue characteristics of the mass
2. Excludes anomalies such as agenesis of the corpus callosum and hydrocephalus
2. MRI differentiates meningoceles from meningoencephaloceles

HISTOPATHOLOGIC:
1. Sincipital and basal encephaloceles have glial component, with astrocytes surrounded by collagen, submucosal glands, and sometimes nasal septal cartilage or calcification
2. Presence of ependymal tissue (ventricle lining - produces CSF)

TREATMENT:
1. Similar to glioma/dermoid (See card)

RISKS/COMPLICATIONS:
1. CSF leak
2. Meningitis
3. Hydrocephalus

Prognosis: 4-10% recurrence

Question 25

Regarding CNPAS (Congenital Nasal Pyriform Aperture Stenosis), discuss:
1. What is it?
2. Associations?
2. What is the main pathophysiological cause?
3. Clinical features & clinical presentation

Answer 25

DEFINITION:
- Congenital Nasal piriform aperture stenosis
- Caused by lateral bony overgrowth of the maxillary process narrowing the pyriform aperture
- May be isolated or as part of the “central megaincisor and holoprosencephaly spectrum” of congenital midline lesions
- Holoprosencephaly is a failure of the embryonic forebrain to cleave sagittally into cerebral hemispheres, transversely into a diencephalon, and horizontally into olfactory and optic bulbs.

FEATURES:
1. Medialized maxillary prominences (aka. megaincisor)
2. Narrow piriform aperture
- Normal ≥ 11mm
- Average = 17mm
- CNPAS ≤ 8mm

CLINICAL PRESENTATION:
- Similar to choanal atresia - nasal obstruction, respiratory distress relieved by crying , snorting, gasping
- Difficulty passing a catheter into the nasopharynx in severe cases

Kevan Peds Question 86
Cummings Chapter 190

Question 26

For CNPAS:
What is the common association (1)
% with this association
Clinical features of this association (7)
Syndromes with this association?

Answer 26

ASSOCIATION: Genetic Syndrome - Solitary Median Maxillary Central Megaincisor Syndrome (34-65% of all cases)
1. Intellectual disability
2. V-shaped palate
3. Arch-shaped appearance of upper lip
3. Prominent maxillary alveolus
4. Absent labial frenulum
5. Narrow nose

“I VAPAN”

Can have: Holoprosencephaly (hypopituitarism), microcephaly, cardiac defects, VACTERL, CHARGE, Velocardiofacial syndrome

Question 27

CNPAS: Imaging and other tests that should be done when the diagnosis is made?

Answer 27

IMAGING:
1. CT to measure width of piriform aperture
2. MRI Brain: Look for Holoprosencephaly and pituitary abnormalities

ADDITIONAL TESTING:
1. Genetic testing
2. Consider hypothalamic-pituitary screening (ACTH, TSH, Luteal Hormone, Follicle Stimulating Hormone, Prolactin, Growth Hormone, Melanocyte stimulating hormone)

Question 28

Treatment cnpas
- 5 conservative
- Surgical approach. What is the indication for surgery?

Answer 28

TREATMENT:
A. Conservative
1. Nasal trumpet, nasal stents
2. Nasal steroids (spray form)
3. McGovern nipple
4. If sprays fail, Dexamethasone Ophthlamic drops: only x3-4 days as drops can induce steroid side effects/adrenal suppression
4. May temporize until child has grown enough that stenosis is no longer symptomatic (may also improve with maxillofacial growth)
5. Intubation

B. Surgical
1. Sublabial approach to drill lateral & inferior margins of aperture ± stents x 1-4 weeks post-op
2. Usually surgery is NOT required unless stenosis < 5.7mm

Question 29

NOT IN VANCOUVERS

What is Holoprosencephaly?
What are the 5 types of facial anomalies associated?

Answer 29

DEFINITION:
- Failure of embryonic forebrain to cleave sagitally into cerebral hemispheres; transversely into a diencephalon, and horizontally into olfactory and optic bulbs

FIVE TYPES OF ASSOCIATED FACIAL ANOMALIES:
1. Cyclopia (single eye and single orbit with arhinia and proboscis)
2. Ethmocephaly (extreme hypertelorism, separate orbits, and arhinia)
3. Cebocephaly (hypertelorism and proboscis-like nose without cleft lip)
4. Median cleft lip (orbital hypertelorism and flat nose)
5. Median philtrum-premaxilla anlage (hypertelorism, bilateral cleft lip, and a median process representing the philtrum-maxilla anlage)

Premaxillary dysgenesis: Hypertelorism, a flat nasal bridge, and a central megaincisor (60%)

Other components of the spectrum:
1. PItuitary disorders
2. Dental and facial anomalies

Note: It is prudent for patients with central maxillary incisor to undergo further investigations, including
- CNS malformation (imaging)
- Chromosomal analysis
- Pituitary function testing

Question 30

Regarding nasal obstruction without choanal atresia (NOWCA), discuss:
1. When does it typically present?
2. What is the pathophysiology?
3. What is the etiology?
4. Management?

Answer 30

ONSET:
1. 3-6 weeks after birth

PATHOPHYSIOLOGY:
1. Growth of anterior and posterior limits of the nasal cavity (piriform aperture and choana) occurs more rapidly than the middle nasal fossa width
2. MImics choanal atresia

ETIOLOGY:
- Functional nasal obstruction secondary to one or more of:
1. Adenoid hypertrophy
2. Choanal stenosis
3. Medial impingement of the lateral nasal wall in the midportion of the nasal cavity
4. Edematous mucosal changes

MANAGEMENT:
1. Non-operative
2. Resolves with facial growth

Question 31

Regarding nasolacrimal duct cyst, discuss:
1. Definition
2. Presentation & exam findings - 4
3. Scope findings - 1
3. Imaging findings - 2
4. Management - 3 conservative
5. What are 3 indications for surgery? Give 3 surgical options

Answer 31

DEFINITION:
- Incomplete canalization of the nasolacrimal duct –> unilateral intranasal mass
- Canalization starts at 4 wks GA but continues up to 1 year after birth
- 30% infacts have incomplete duct at birth (but nearly all resolve)
- 85% resolve by 9 months

CLINICAL PRESENTATION:
1. Nasal obstruction
2. Feeding difficulties
3. Aspiration
4. Breathing difficulties (obligate nose-breathers)

SCOPE:
1. Cystic mass in inferior meatus (at Hasner’s valve - mucosal flap at the distal end of the nasolacrimal duct, prevents air from entering the lacrimal sac when the nose is blown)

IMAGING:
1. CT - dilated lacrimal sac/duct
2. Intranasal cyst

TREATMENT:
A. Conservative
1. Temporizing measures: nasal steroids, nasal decongestants, nasal trumpet, NG tube
2. Usually completes canalization by 9 months

B. Surgical
1. Indicated for infection, respiratory obstruction, or nutrition (feeding difficulties) - “ION”
2. Endoscopic Marsupialization
3. ± CO2 laser
4. ± Nasolacrimal duct stents - ophthalmology consult for intraop nasolacrimal duct probing stenting

Question 32

Regarding choanal atresia, discuss:
1. Epidemiology. What is the official definition of choanal stenosis?
2. Types - 3
3. What are the bony limits of the choana?
4. What are Structural characteristics of choanal atresia - 4
4. Theories on causes 4

Answer 32

EPIDEMIOLOGY:
1. 1/5000 - 8000 live births
2. F>M 2:1
3. Unilateral > Bilateral (2:1)
4. R > L (2:1)
5. 50% unilateral and 75% bilateral associated with syndrome/other anomalies
6. Choanal stenosis < 6mm choana

TYPES OF CHOANAL ATRESIA:
1. Bony (30%)
2. Bony-Membranous (70%)
3. Pure membranous (very rare, < 1% - some feel this doesn’t exist)

BONY LIMITS OF CHOANA:
Lateral - medial pterygoids
Medial - Vomer
Inferior - Horizontal process of the palatine bone
Superior - sphenoid body/vomer/medial pterygoid (where they all meet)

STRUCTURAL CHARACTERISTICS:
1. Narrow nasal cavity
2. Lateral bony obstruction by the pterygoid plates
3. Medial obstruction caused by thickening of the vomer
4. Membranous obstruction

CAUSE (Theories):
1. Persistence of nasobuccal (aka. oronasal) membrane
2. Persistence of buccopharyngeal (aka. oropharyngeal) membrane
3. Incomplete resorption of nasopharyngeal mesoderm
4. Misdirected mesodermal flow during embryological development

Kevan Peds Question 88

Question 33

Choana atresia
- Clinical presentation - 2 unilateral and 1 bilateral sign
- Evaluation methods - 4
- Diagnostic Findings on CT - 3

Answer 33

CLINICAL PRESENTATION:
1. Unilateral: foul rhinorrhea, unilateral obstruction, typically present later in life
2. Bilateral: cyclical cyanosis relieved with crying

EVALUATION:
1. Kleenex test
2. Mirror test (for humidity)
3. Failure to pass 6-French catheter (2mm diameter) at the 32mm distance mark
4. Scope

IMAGING FINDINGS:
1. CT to distinguish from CNPAS, r/o mass, determine type, evaluate height of skull base
2. Diagnostic Features on CT:
- Medialized pterygoid plates
- Wide vomer
- Blocked choana

Question 34

Choana atresia treatment.

When do you do it
What are 5 surgical approaches.

What are indications to stent - 2

Answer 34

TREATMENT:
1. Unilateral: Delay surgery for several months to allow nasal passages to grow (enhances ease of surgery) - at least 1yo
2. Bilateral: Oropharyngeal airway or ETT, G-tube feeds; early surgery, McGovern nipple (inverted nipple stays in the mouth to encourage oral breathing)
3. Intubation not required unless mechanical ventilation required

SURGICAL APPROACHES:
1. Transoral endoscopic with 120 degree scope (preferred)
2. Transpalatal
3. Transseptal
4. Transnasal
5. Transantral

POST-OPERATIVE CARE:
1. ICU monitoring
2. Frequent suctioning
3. Antibiotics
4. PPI

INDICATIONS TO STENT:
1. Stent bilateral
2. Don’t stent unilateral (unless revision surgery)
3. Syndromic tend to stent longer
4. Mitomycin C can also be used (not available in Canada)

Question 35

What are 7 syndromes associated with choanal atresia?

Answer 35

4 C’s and 3 T’s

1. CHARGE
2. Craniosynostoses (e.g. Crouzon, Apert)
3. CATCH-22 (22q11 deletion - Velocardiofacial)
4. Cleft Palate
5. Treacher Collins
6. Trisomy 21
7. Trisomy 18

Question 36

Regarding Nasopharyngeal Teratomas, discuss:
1. What are they?
2. How is it diagnosed? 3
3. How are they managed? 3

Answer 36

DEFINITION
- Benign germ cell tumor of all 3 tissue types: ecto/meso/endoderm
- Most common germ cell tumors of childhood, almost always benign
- Usually midline (embryonal fusion plane), but can also form on organs
- Malignant transformation is possible (but rare)
- Head/neck teratomas < 5% of all teratomas (most common gonads)
- Most common benign neoplasm in the nasopharynx in children

CLINICAL PRESENTATION
- Nasopharyngeal teratomas may be sessile or peduncuated
- May protrude through mouth
- Sometimes associated with anencephaly, hemicrania, and palatal fissures
- Severe acute respiratory distress
- Smaller lesions –> feeding difficulties may be only presenting symptom

DIAGNOSIS:
1. In-Utero U/S or MRI
- Polyhydramnios (from impaired swallowing)
- Large tumors visible
- Elevated maternal alpha-fetoprotein (AFP) levels

2. Post-natal
   - CT/MRI: Multi-density, may have tooth/bone, well encapsulated and no intracranial connections
   - Fine needle aspiration for cytology

MANAGEMENT:
1. Large teratomas may require EXIT procedure to secure airway (Ex-utero intrapartum treatment)
2. Surgical excision
- Transoral approach: split soft palate or palate possibly needed
- Endoscopic approaches
- Lateral rhinotomy or transcervical incision approaches
- Craniofacial approach if intracranial component
3. Follow AFP levels to monitor for recurrence

Cummings Chapter 190

Question 37

What are the pathophysiologic factors that contribute to pediatric chronic rhinosinusitis?

Answer 37

A. Genetic Factors
1. Familiial risk
2. Potassium channel epithelial physiology and development of CRS
3. CFTR mutations (even in absence of CF phenotype)

B. Bacteriology
1. Alpha-hemolytic streptococci
2. S. aureus
3. S. Pneumoniae
4. H. Influenzae
5. M. Catarrhalis
Anaerobes uncommon

C. Biofilm formation

D. Adenoid contribution
1. Larger surface area covered by biofilm

E. Inflammatory Changes
1. Eosinophils and CD4+ cells prominent in order children with CRS
2. Lymphocytes and neutrophils prominent in younger children
3. Less disruption of epithelial morphology compared to adults
4. Higher levels of cytokines
5. Submucosal glandular hyperplasia; MUC5B is the predominant glandular mucin

Cummings Chapter 200 Box 200.2

Question 38

List risk factors for pediatric rhinosinusitis 8

Answer 38

1. URTI (kids get 6-8/year - mostly viral) - 10% complicated by sinusitis
2. Allergy (perennial, especially mold)
3. Asthma
4. GERD
5. Dental infections (most likely in adults - rare cause in kids)

Other comorbidities:
1. Immunodeficiency
2. Primary ciliary dyskinesia (PCD)
3. Cystic fibrosis

Question 39

How does URTIs lead to sinusitis?

Answer 39

1. Viral URTI –> inflammation, edema, ciliary dysfunction –> mucous/inflamed tissue obstruct sinus ostia –> mucous retention in sinuses
2. Secondary bacterial superinfection possible (e.g. Strep pneumo, Staph aureus, M Catarrhalis, H. Flu)
3. Ciliary function returns as viral infection passes (80% resolve without treatment)

Question 40

Which viruses are most commonly associated with acute rhinosinusitis?

Answer 40

1. Rhinovirus
2. Influenzae
3. Parainfluenzae
4. Adenovirus
5. Coronavirus
6. Respiratory Syncytial virus

Question 41

Define acute, subacute, and chronic sinusitis and recurrent acute

Answer 41

Acute = symptoms lasting 1-4 weeks

Subacute = symptoms lasting 4-8 weeks

Chronic = Symptoms lasting > 8-12 weeks without symptom free periods

Recurrent acute = 4 or more episodes/year

Question 42

Describe the clinical presentation diagnostic criteria of pediatric chronic sinusitis.

Answer 42

CLINICAL DIAGNOSIS OF CRS IN CHILDREN

A. Symptom Complex: 2 or more of the following symptoms, with one of the first two symptoms must be present
1. Nasal discharge (anterior or posterior)
2. Nasal congestion/blockage
3. Cough
4. Facial pain/pressure

B. Objective findings: At least one
1. Abnormal nasal endoscopy
2. Abnormal CT scan of the paranasal sinuses

C. Duration of Disease: ≥12 weeks

Question 43

For pediatric sinusitis,
1. What features suggest viral cause - 7
2. What features suggest bacterial cause? - 4
2. What features suggest severe infection? - 2

Answer 43

FEATURES SUGGESTING VIRAL:
1. Rhinorrhea
2. Post-nasal drip
3. Cough
4. Nasal obstruction/congestion
5. Halitosis
6. Headache
7. Behavioural/irritability

FEATURES SUGGESTING BACTERIAL:
1. Fever
2. Purulent discharge
3. Persistence > 10-14 days
4. No improvement with nasal steroids

FEATURES SUGGESTING SEVERE ABRS:
1. High fever >39 degrees
2. Complications of ARS

Question 44

Describe the management strategy for pediatric Acute Rhinosinusitis

Answer 44

80% viral cause and will self-resolve in 1-2 weeks, therefore conservative management if:
1. Non-severe symptoms
2. ABRS not suspected
3. No complications on presentation

CONSERVATIVE:
1. Symptomatic (nasal saline, humidification, hydration)
2. Nasal steroids
3. Nasal decongestants (x3-4 days max)

ANTIBIOTICS:
1. Indicated if suspect ABRS (fever, purulent discharge, severe symptoms, no improvement after 7-14 days)
- First line: Amoxil
- First line alternate: Macrolide (Azithro, Clarithro), Cefuroxime

2. If no improvement after 72 hours OR previous antibiotics in last 30 days
   - 2nd line: Clavulin, Quinolone (Levoflox, Moxiflox)
   - Duration: 10-14 days
3. No resolution on PO or severe complications (3rd line)
   - IV Clinda/Cefuroxime

Question 45

Describe the management strategy for pediatric CRS:

1. Name 4 medical options
2. Name 4 probable efficacy surgical treatments
3. Name 3 questionable efficacy treatments

Answer 45

1. Saline rinses
2. Topical nasal steroids
3. PPI
4. Long term low dose Erythromycin (Debated)
5. IVIIg (debated)

SURGICAL:
A. Questionable efficacy:
1. Tonsillectomy
2. Antral lavage
3. Inferior meatal antrostomy

B. Probable efficacy:
1. Adenoidectomy (as a first treatment) - 50-70% successful
2. Middle meatal antrostomy/possible ballon sinuplasty
3. Anterior or anterior and posterior ethmoidectomy (note: Frontal sinuses are not developed and sphenoid is rarely diseased in children)
4. FESS

Utility of Adenoidectomy:
1. Eliminates reservoir/biofilm
2. Eliminates obstruction
3. 50% successful in curing CRS

Question 46

What organisms are commonly associated with pediatric acute rhinosinusitis (both viral and bacterial)? Name 3 viral. Name 3 bacterial

Answer 46

VIRAL (most common)
1. Rhinovirus (most common)
2. RSV
3. Adenovirus
4. Influenza
5. Parainfluenza

BACTERIAL
1. Strep pneumo
2. H flu
3. M Catarrhalis
4. Staph aureus

Question 47

What organisms are commonly associated with pediatric chronic rhinosinusitis? name 3

Answer 47

Bacteria:
1. Same bugs as ABRS possible, but less common
2. Coag-negative staph
3. Anaerobes (Peptococcus, Peptostreptococcus, Bacteroides)
4. Gram negatives (pseudomonas)

Aerobic list:
1. Strep pneumoniae
2. M Catarrhalis
3. H flu
4. Staph A, increasing incidence of MRSA
5. alpha hemolytic streptococci
6. Pseudomonas

Anaerobic list:
1. Peptococci
2. Peptostreptococci
3. Bacteroides

Question 48

List 4 indications for CT in pediatric sinusitis

Answer 48

1. Severe or toxic symptoms
2. Evidence of complication (e.g. eye, brain)
3. Failure to improve on maximum medical management x 72 hours
4. Immunocompromised

Question 49

List 4 indications for maxillary sinus culture in pediatric sinusitis

Answer 49

Same as CT indications for pediatric sinusitis

1. Severe or toxic symptoms
2. Evidence of complication (e.g. eye, brain)
3. Failure to improve on maximum medical management x 72 hours
4. Immunocompromised

Question 50

What are the complications of pediatric acute rhinosinusitis? List 13

Answer 50

A. Orbital complications
1. Orbital inflammation (preseptal)
2. Orbital cellulitis
3. Subperiosteal abscess
4. Orbital abscess
5. Cavernous sinus thrombosis
6. Blindness

B. Intracranial complications
1. Meningitis (most common)
2. Epidural abscess
3. Subdural abscess
4. Acute and chronic brain abscess
5. Osteomyelitis
6. Superior Sagittal sinus thrombosis

C. Extracranial complications
1. Pott’s Puffy Tumor (only in kids > 7 years; younger don’t have frontal sinus so uncommon)

Question 51

What are the criteria for medical management of medial subperiosteal abscessees? 5

Answer 51

1. Normal vision, pupil and retina
2. No ophthalmoplegia
3. Intraocular pressure of < 20mmHg
4. Proptosis of ≤5mm
5. Abscess width of ≤4mm

Question 52

What are the Garcia & Harris indications for draining a subperiosteal abscess? 10

Answer 52

Patients may be treated with IV antibiotics & observation, UNLESS:
1. Age > 9 years old
2. Abscess size > 1 cm
3. Abscess in non-medial location
4. Dental source
5. Frontal sinus source / frontal sinusitis
6. Acute vision compromise (optic nerve compromise, retina)
7. Gas in abscess; suspicion of anaerobic subperiosteal infection
8. Non-resolving CRS
9. Previous/recurrent I&D
10. Worsening or not resolving despite max medical treatment for 48-72 hours

https://www.sciencedirect.com/science/article/abs/pii/S0165587623001969

Question 53

According to the Chadha Laryngoscope Review in 2012:
1. Is pre-septal cellulitis related to sinusitis?
2. What are the risk factors for collections in sinusitis?
3. What are the risk factors for failure of medical therapy
4. What is the Chadha classification of subperiosteal absceess?

Answer 53

Pre-septal cellulitis is not related to sinusitis

Risk factors for collections:
1. Proptosis
2. Pain with EOM
3. Gaze restriction
4. WBC > 10 or edema posterior to the lids –> Image with CT (best), MRI also reasonable

Risk factors for failure of medical management:
1. Age > 9
2. Non-medial collections
3. Proptosis > 2mm

Classification:
1. Stage 1: Pre-Septal cellulitis
2. Stage 2a: Medial collection, small < 1cm or < 1.25 cm^3
3. Stage 2b: Medial collection, >1cm, >1.25cm^3
4. Stage 2c: Collection lateral or superior
5. Stage 3: Orbital abscess

Question 54

What are the indications for pediatric maxillary sinus aspirate? 5

Answer 54

1. Severe toxic child
2. Immunocompromised
3. Unresolving symptoms after >10 days
4. Suupurative complications
5. Work up for fever of unknown origin

Question 55

What is the pathway of treatment for Pediatric CRS and pathway to FESS?

What are the indications for pediatric FESS? 11

Answer 55

See flow chart Kevan Pediatrics Question 102

If clinical symptoms for ≥ 12 weeks and objective evidence of CRS –> treat with maximal medical treatment (ie. abx, INCS, saline irrigation, additional treatment)
a. If resolution/improvement of symptoms - continue
b. If persistent symptoms or recurrence/lack of resolution of symptoms then perform CT (and treat comorbidities such as AR, asthma, PCD, immune deficiency, CF, AFS)
- If CT shows low LM score –> adenoidectomy –> FESS if adenoidectomy fails
- If CT shows high LM score –> adenoidectomy + irrigation OR FESS

ABSOLUTE INDICATIONS:
1. CF patient with complete nasal airway obstruction secondary to massive polyposis
2. Antrochoanal polyp
3. Mucoceles and mucopyoceles
4. Intracranial complications
5. Orbital complications
6. Traumatic injury to the optic canal
7. Dacryocystorhinitis (lacrimal sac infection) secondary to sinusitis, resistance to medications
8. Fungal sinusitis
9. Meningoencephaloceles
10. Neoplasms

RELATIVE INDICATIONS:
1. CRS that fails to improve after 4-6 weeks maximal medical management, other causes ruled out

Question 56

What are 2 ways that pediatric allergic fungal sinusitis is different from adults?

Answer 56

1. More likely to have facial skeleton abnormalities
2. More likely unilateral

Question 57

What should you rule out if you find nasal polyps in a child?

How do you work this up?

Answer 57

Cystic Fibrosis - abnormalities in ion transport and water absorption, resulting in dehydration of mucous composition

IMAGING FEATURES:
1. Hypoplastic maxillary sinuses
2. Complete opacification of maxillary sinuses with medial wall bulging in toward nasal cavity (secondary to chronic pressure from contained maxillary secretions)
3. Aggressive polyposis

DIAGNOSTIC TESTS:
1. Sweat chloride test (40mmol/L = normal, >60 = CF, 40-60 equivocal and must retest)
2. Nasal potential difference (measures voltage difference - ie. Na ion transport across nasal mucosa) - rarely done
3. Genetic testing (CFTR gene, chromosome 7)

Question 58

What are indications for FESS in CF patients? 2

Answer 58

1. Severe nasal obstruction
2. Respirologist feels nasal polyposis is exacerbating pulmonary symptoms

Question 59

Why do patients with CF had increased bleeding risk?

Answer 59

Pancreatic insufficiency –> Impaired absorption of Vitamins ADEK –> Vitamin K deficient

Question 60

What are 7 potential causes of false-positive sweat-chloride tests (ie. diseases that alter results but are not cystic fibrosis)

Answer 60

1. Malnutrition
2. Dermatitis
3. Mucopolysaccharidosis
4. Glycogen storage disease
5. Untreated adrenal dysfunction
6. Hypothyroidism
7. Pituitary dysfunction

MD MP HUG

Question 61

What investigations should you conduct for a kid with recurrent ARS or CRS without polyps? 2

Answer 61

1. Primary Ciliary Dyskinesia
   - Nasal brushings - send in glutaraldehyde for electron microscopy
2. Immunodeficiency
   - Measure Ig levels (GMAE)