Pedi Nose and Sinus

Question 1

What are the two most common maxillofacial

fractures in children?

Answer 1

Nasal bone and mandibular fractures

Question 2

If a child develops a septal hematoma after
sustaining a nasal fracture and subsequently
develops a septal abscess, what nasal deformity
might the child develop later in life?

Answer 2

Saddle nose deformity, deformed columella or nasal base

Question 3

What must be considered in the performance of
a complete examination of a child who has
sustained a significant nasal injury?

Answer 3

Sedation

Question 4

What is the key factor that differentiates the
treatment of nasal trauma in pediatric patients
compared with adult patients?

Answer 4

Ongoing facial growth

Question 5

Why do infants diagnosed with an obstructive
septal deviation after nasal trauma require
urgent evaluation?

Answer 5

They are obligate nasal breathers.

Question 6

When should pediatric nasal fractures be reduced?

Answer 6

Reduction should be done either within 3 to 6 hours of
injury, before the onset of swelling, or 3 to 10 days after the
injury. If immediate reduction cannot be performed, the
fractures should be evaluated 3 to 7 days after the injury,
when edema has subsided.

Question 7

What is the differential diagnosis for a congenital

midline nasal mass?

Answer 7

● Dermoid (most common)
● Glioma
● Encephalocele
● Epidermoid cysts
● Hemangiomas
● Teratomas
● Neurofibromas
● Lipomas
● Lymphangiomas

Question 8

Name the midline nasal epithelial-lined cyst, sinus,
or tract that forms as a result of regression of the
embryologic neuroectodermal tract, pulling skin
elements into the prenasal space.

Answer 8

Nasal dermoid forms and contains keratin debris, hair

follicles, sebaceous glands, and sweat glands.

Question 9

What are the clinical findings in a patient with a

nasal dermoid cyst?

Answer 9

● It occurs more often in male than in female patients.
● Noncompressible mass.
● Furstenberg sign is negative (i.e., no enlargement with
compression of the jugular veins); it is firm and nontender.
● It does not transilluminate.
● The cyst is located in the midline, most commonly along
the dorsum, resulting in a widened dorsum.
● Cyst can be intranasal, extranasal, or intracranial.
● It may have a sinus opening with intermittent discharge
of sebaceous material.
● Hair protrudes through a punctum, is pathognomonic,
but not commonly seen.

Question 10

How often do nasal dermoids extend intracranially?

Answer 10

In ~ 25% of cases, they most often communicate through
the foramen cecum or the cribriform plate to the base of
the frontal fossa with extradural adherence to the falx
cerebri and are associated with an increased risk of
meningitis.

Question 11

What radiographic findings suggest a nasal

dermoid with intracranial extension?

Answer 11

● Bifid crista galli

● Enlarged foramen cecum

Question 12

Discuss the treatment of nasal dermoids.

Answer 12

● Treatment involves complete surgical excision. Ap-
proaches vary depending on presence of intracranial

extension, and recurrence is common.

● Extracranial approach: Vertical midline incision, trans-
verse incision, lateral rhinotomy, external rhinoplasty,

inverted-U incision, and degloving procedures
● Combined approach: Extracranial approach combined
with a frontal craniotomy

Question 13

What congenital nasal anomaly consists of ectopic
glial tissue that lacks a patent cerebrospinal fluid
(CSF) communication to the subarachnoid space
but in 15 to 20% maintains a fibrous affiliation?

Answer 13

Nasal gliomas

Question 14

How do nasal gliomas form?

Answer 14

Theories about formation:

● Abnormal closure of fronticulus frontalis, isolating the
brain tissue from the intracranial cavity
● Nidus of ectopic neuroepithelia
● An outgrowth of olfactory tissue through the cribriform
plate

Question 15

What is the clinical presentation of nasal gliomas?

Answer 15

Extranasal gliomas (60%) are smooth, firm, noncompressible
masses that occur most commonly at the glabella, although
they may arise along the side of the nose or the
nasomaxillary suture line.
Intranasal gliomas (30%) are polypoid pale masses that arise
from the lateral nasal wall near the middle turbinate and
occasionally from the nasal septum and can protrude from
the nostril.
Combined (10%)
Intracranial extension (15%)

Question 16

What congenital nasal anomaly consists of an
extracranial herniation of the cranial contents
through a defect in the skull? Meninges only?
Brain matter and meninges?

Answer 16

Encephalocele, meningocele, and meningoencephalocele,

respectively

Question 17

How are nasal encephaloceles classified?

Answer 17

Location of the skull base defect:

● Sincipital (60%): Arise between the frontal and ethmoid
bones at the foramen cecum, immediately anterior to the
cribriform plate.
● Basal (40%): Arise between the cribriform plate and the
superior orbital fissure or posterior clinoid fissure.
Note: The most common congenital encephaloceles are
occipital (75%).

Question 18

What are the subtypes of sincipital encephaloceles?

Answer 18

● Nasofrontal: Defect is located at the glabella between the

nasal and frontal bones.
● Nasoethmoidal: Sac exits through the foramen cecum,
passing under the nasal bones and above the upper
lateral cartilages, creating a lateral nasal mass.
● Nasoorbital: Sac transverses the foramen cecum before
extending into the orbit via a defect in its medial wall.

Question 19

What are the subtypes of basal encephaloceles?

Answer 19

● Transethmoidal: Sac extends medial to the superior
turbinate via a cribriform plate defect.
● Sphenoethmoidal: Sac protrudes into the nasopharynx via
a defect between the posterior ethmoid and the anterior
sphenoid wall.
● Transsphenoidal: Sac also is seen in the nasopharynx,
exiting intracranially through an open craniopharyngeal
canal.
● Sphenoorbital: It protrudes through the superior orbital
fissure and out the inferior orbital fissure into the
sphenopalatine fossa.

Question 20

What are the common clinical findings associated

with encephaloceles?

Answer 20

● Bluish/red mass that is soft, compressible
● (Positive) Furstenburg test (expands with compression of
internal jugular vein)
● Pulsatile
● Does transilluminate

Question 21

What imaging is best for the diagnosis and surgical

planning for an encephalocele?

Answer 21

CT and MRI

Question 22

Why is surgical repair the treatment of choice for

encephaloceles?

Answer 22

To prevent CSF leak, meningitis, and brain herniation

Question 23

Describe the four hypotheses that have been
offered to explain the development of choanal
atresia.

Answer 23

● Buccopharyngeal membrane persistence
● Abnormal neural crest cell migration
● Bucconasal membrane persistence
● Adhesion formation in the nasochoanal region as a result
of abnormal mesoderm

Question 24

What are the clinical features of choanal atresia?

Answer 24

● 1:5,000 to 8,000 live births
● 75% unilateral, right > left
● Female-to-male ratio: 2:1
● 50% of patients with unilateral atresia and up to 75% of
patients with bilateral atresia have other associated
congenital anomalies.
● Bony 30%, membranous 70%

Question 25

What are the anatomic features of choanal | atresia?

Answer 25

● A narrow nasal cavity ● Lateral bony obstruction by the pterygoid plates ● Medial obstruction caused by thickening of the vomer ● Membranous or bony obstruction

Question 26

What syndromes are associated with choanal | atresia?

Answer 26

● CHARGE association ● FGFR-related craniosynostosis syndromes (e.g., Crouzon syndrome, Pfeiffer syndrome, Apert syndrome, Jackson-Weiss syndrome, Muenke syndrome, Antley-Bixler syndrome) ● Down syndrome ● Treacher-Collins syndrome ● Solitary medianmaxillary central incisor syndrome

Question 27

At what age are most infants no longer obligate | nasal breathers?

Answer 27

6 to 9 months

Question 28

You are called emergently to evaluate a newborn in respiratory distress. On evaluation, you note cyanotic episodes relieved by crying. Examination is otherwise benign, and the medical history shows no complications. What is the most likely diagnosis?

Answer 28

Bilateral choanal atresia

Question 29

How does unilateral choanal atresia commonly | manifest?

Answer 29

Typically it is not seen until the patient is aged 5 to 24 months or a young adult as a result of unilateral nasal obstruction or rhinorrhea.

Question 30

You suspect possible choanal atresia in a newborn with respiratory distress. What physical examination maneuver can be used to help determine whether choanal atresia is present?

Answer 30

One can attempt to pass a nasogastric tube or small catheter (< 8 French, generally a 5/6 French is used) through the nose. If it does not pass, the presence of choanal atresia is suspected and further workup is required.

Question 31

What is the definitive diagnostic test for choanal | atresia?

Answer 31

CT scan

Question 32

What three surgical approaches can be considered | for the treatment of choanal atresia?

Answer 32

● Transpalatal ● Transnasal: puncture (Fearon) ● Endoscopic endonasal (transnasal, transoral, combined); puncture, drill, dilation

Question 33

What symptoms might suggest congenital nasal | pyriform aperture stenosis (CNPAS)?

Answer 33

Respiratory distress, poor feeding, failure to thrive, and | recurrent cycles of cyanosis and apnea

Question 34

What causes CNPAS?

Answer 34

CNPAS occurs secondary to bony overgrowth of the medial nasal process of the maxilla into the nasal aperture resulting in a pyriform aperture smaller than 11 mm.

Question 35

What congenital anomalies are associated with | CNPAS?

Answer 35

● Holoprosencephaly (HPE): Clinical features include facial dysmorphisms such as ocular hypotelorism, midline cleft lip and/or flat nose, cerebral malformations, learning disabilities, arrhinencephaly, agenesis of the corpus callosum, hypopituitarism, single maxillary central incisor. ● Solitary median maxillary central incisor syndrome (SMMCI): Clinical features include severe to mild intellectual disability, congenital heart disease, cleft lip and/or palate and less frequently, microcephaly, hypopituitarism, hy- potelorism, convergent strabismus, esophageal and duodenal atresia, cervical hemivertebrae, cervical der- moid, hypothyroidism, scoliosis, absent kidney, micro- penis, and ambiguous genitalia.

Question 36

What are the treatment options for CNPAS?

Answer 36

● Nonoperative management: Nasal trumpets, topical steroids, and vasoconstrictive drops may be attempted until growth results in increased nasal airway size. ● Operative management: A sublabial approach is done to expose the inferior and lateral pyriform aperture. A small diamond burr is then used to widen the bony lateral and inferior margins.

Question 37

What are the features of complete agenesis of the | nose (arrhinia)?

Answer 37

``` ● Absence of the external nose, nasal airways, and olfactory apparatus ● Hypoplasia of the maxilla ● A small high-arched palate ● Hypertelorism ```

Question 38

What are the possible causes of congenital | anosmia?

Answer 38

● Most commonly autosomal dominant ● Defective transportation of odorants to the olfactory neuroepithelium as a result of congenital malformations in the nasal cavity ● Disrupted signal transduction or signal propagation ● Malformation of regions in the brain essential for olfaction

Question 39

What syndromes are associated with congenital | anosmia?

Answer 39

● Kallmann syndrome ● Congenital insensitivity to pain ● Ciliopathies including Bardet-Biedl syndrome and Leber congenital amaurosis

Question 40

Where is the most common site of obstruction | causing nasolacrimal duct cysts (dacrocystoceles)?

Answer 40

Inferior meatus (membrane of Hasner). Note: Recanalization of the nasolacrimal duct occurs from the lacrimal system inferiorly.

Question 41

``` What symptoms are associated with nasolacrimal duct cysts (dacrocystoceles)? ```

Answer 41

Epiphora (tearing), nasal obstruction, respiratory distress in neonates (obligate nasal breathers), aspiration, feeding difficulty

Question 42

What are common physical examination findings that suggest nasolacrimal duct cyst (dacrocystocele)?

Answer 42

Bluish swelling of the skin overlying the nasolacrimal duct, cyst in the inferior meatus, superior displacement of the medial canthal tendon, and epiphora

Question 43

What is the first-line therapy for a nasolacrimal | duct cyst?

Answer 43

Massage

Question 44

When should you offer surgical intervention for a | nasolacrimal duct cyst (dacrocystocele)?

Answer 44

Infant with significant symptoms (i.e., feeding difficulty, | infection, or respiratory difficulty)

Question 45

``` What is the surgical treatment of nasolacrimal duct cysts (dacrocystoceles)? ```

Answer 45

Endoscopic marsupialization (opening the cyst into the inferior meatus). Ophthalmologist may need to probe duct and possibly place stents to ensure patency.

Question 46

During week 4 of development, a pouch forms along the dorsal stomodeum. During the week 5, the infundibular stalk and this pouch come into contact and the opening of the pouch is occluded at the buccopharyngeal junction and is separated from the oral cavity by week 6. The pituitary gland then develops from the anterior wall of the pouch (pars distalis) and a small portion of the posterior wall of the pouch (pars intermedia). Normally, the remnant pouch lumen is obliterated; if not, what is this condition called?

Answer 46

Rathke cleft/pouch cyst

Question 47

Describe the characteristics of a Rathke pouch | cyst.

Answer 47

Non-neoplastic, sellar/suprasellar epithelial lined cyst (sella turcica). Most often they are small and asymptomatic.

Question 48

How do Rathke pouch cysts most commonly | manifest?

Answer 48

During the fifth to sixth decade of life, female predom- inance. They are usually asymptomatic, but large lesions may cause visual disturbance, pituitary dysfunction, and/or headaches. They can be seen on MRI.

Question 49

A tumor derived from a Rathke pouch is called | what?

Answer 49

Craniopharyngioma

Question 50

What benign cyst/bursa can form in the cleavage plane between the nasal cavity and pharynx (Rathke pouch, notochord remnant) as a result of obstruction, inflammation, or infection of the pharyngeal bursa?

Answer 50

Thornwaldt cyst

Question 51

What symptoms are most commonly associated | with a Thornwaldt cyst?

Answer 51

None. Occasionally patients complain of postnasal drip with intermittent drainage of the cyst or halitosis. If the cyst enlarges or becomes infected, nasal obstruction or eusta- chian ET dysfunction can result.

Question 52

How are Thornwaldt cysts treated?

Answer 52

If the diagnosis is clear, observation and reassurance are recommended. If cysts are dark-colored from hemorrhage or hemosiderin, consider biopsy after obtaining an MRI to rule out intracranial communication. If symptomatic, they can be surgically removed, taking care to remove the entire cysts, which can extend to the prevertebral fascia.

Question 53

During a routine examination, you note a smooth, mucus-covered mass within the adenoid pad. Imaging reveals a rhomboid-shaped cyst with no bony or intracranial communication. What is the likely cause?

Answer 53

Intra-adenoidal cyst

Question 54

What causes are associated with pediatric | sinusitis?

Answer 54

``` ● Immature immune system ● Small developing sinuses ● Viral upper respiratory infections ● Allergy/allergic rhinitis ● Immunodeficiency ● Gastroesophageal reflux disease ● Cystic fibrosis ```

Question 55

What are the diagnostic criteria for pediatric | acute bacterial sinusitis?

Answer 55

Clinical diagnosis can be made when a child has an acute upper respiratory infection (URI) and the following (Amer- ican Academy of Pediatrics [AAP] clinical guidelines, 2013) symptoms: ● Persistent illness (i.e., nasal discharge of any quality or daytime cough or both lasting more than 10 days without improvement) or ● Worsening course (i.e. new onset of nasal discharge, daytime cough, or fever after initial improvement) or ● Severe onset (i.e., concurrent fever with temperature ≥ 39°C or 102.2°F) and purulent nasal discharge for at least 3 consecutive days (Evidence Quality: B, Recommendation)

Question 56

What are the predominant pathogens in pediatric | acute sinusitis?

Answer 56

``` Streptococcus pneumoniae (25 to 30%) Haemophilus influenzae (15 to 20%) Moraxella catarrhalis (15 to 20%) ```

Question 57

When should imaging be obtained in the evaluation of child in whom acute bacterial sinusitis is suspected?

Answer 57

Only if there is concern for orbital or intracranial | involvement

Question 58

When should a clinician recommend antibiotic therapy instead of supportive care (nasal irrigation, intranasal corticosteroids, topical or oral decongestants, mucolytics, and/or topical or oral antihistamines) and close observation for a child with presumed acute bacterial sinusitis?

Answer 58

AAP clinical practice guidelines, 2013: ● Severe onset and worsening course (signs, symptoms, or both) (Quality of Evidence B, Strong Recommendation) or ● No improvement after a 3-day course of observation with persistent illness (nasal discharge of any quality or ● Cough or both for at least 10 days without improvement) (Quality of Evidence: B, Recommendation)

Question 59

What three risk factors are likely to increase the resistance of organisms to amoxicillin in both acute bacterial sinusitis and acute otitis media?

Answer 59

AAP clinical practice guidelines, 2013 ● Day care or child care attendance ● Antibiotic treatment within the previous 30 days ● Age < 2 years

Question 60

``` What antibiotic(s) should be considered for children with acute bacterial sinusitis? ```

Answer 60

● Amoxicillin ± clavulanate. Duration varies: continue 7 days after resolution of symptoms. ● Standard-dose amoxicillin (45 mg/kg daiy divided into two doses): Mild to moderate severity illness in a child who does not attend day care, has not been treated with antibiotics in the previous 30 days, and whose age is > 2 years. ● High-dose amoxicillin (80 to 90 mg/kg daily divided in two doses, maximum 2 g/day): In communities with a > 10% incidence of S. pneumoniae resistance ● Amoxicillin-clavulanate (amoxicillin 80 to 90 mg/kg daily divided into two doses, maximum 2 g/day): Moderate to severe illness or who attend day care, have received antibiotics within 30 days, or are < 2 years of age. ● Ceftriaxone (50 mg/kg dose given intramuscularly (IM) or IV) if unable to tolerate oral administration, followed by an oral antibiotic to complete therapy if improvement is noted within 24 hours. ● Clindamycin = cefexime or linezolid and cefexamine or levofloxacin: If the child’s condition worsens after 72 hours on high-dose augmentin ● Options to consider if the child is allergic to penicillin: cefdinir, cefuroxime, cefpodoxime, clindamycin and ce- fexime, linezolid, or a flouroquinolone. ● Inpatient IV antibiotics should be considered in pediatric patients with complicated bacterial sinusitis (AAP, 2013).

Question 61

Describe the major complications of pediatric | sinusitis.

Answer 61

● Orbital/periorbital inflammation from ethmoid sinuses: Preseptal cellulitis, orbital cellulitis, subperiosteal abscess, and orbital abscess ● Intracranial spread from the frontal and sphenoid sinuses: Meningitis, epidural abscess, subdural empyema, intra- cerebral abscess, and cavernous or sagittal sinus thrombosis

Question 62

How are orbital complications from sinusitis | classified?

Answer 62

The Chandler Classification ● Group 1: Inflammatory (preseptal) edema of eyelids without tenderness; obstruction of venous drainage; no associated visual loss or limitation of ocular movements ● Group 2: Orbital cellulitis with diffuse edema of the adipose tissue in the orbital contents secondary to inflammation and bacterial infections; no abscess formation ● Group 3: Subperiosteal abscess, abscess formation between the orbital periosteum and the bony orbital wall. The mass displaces the globe in the opposite direction (usually down and lateral); the proptosis may be severe with decreased ocular mobility and visual acuity. The abscess may rupture into the orbit through the orbital septum. ● Group 4: Orbital abscess, a discrete abscess within the orbit. Proptosis is usually severe but is symmetrical and not displaced as in the subperiosteal abscess. Complete ophthalmoplegia results, and visual loss occurs in 13%. ● Group 5: Cavernous sinus thrombosis; progression of the phlebitis into the cavernous sinus and to the opposite side, resulting in bilateral symptoms

Question 63

What are the diagnostic criteria for pediatric | chronic sinusitis?

Answer 63

American Academy of Otolaryngology (2013) ● One or more symptoms of sinusitis > 12 weeks ● Six or more episodes of acute sinusitis/year ● Acute exacerbations without complete resolution between episodes

Question 64

What is the role of intravenous immune serum globulin (IVIG) therapy in pediatric chronic rhinosinusitis?

Answer 64

IVIG may have a role in the treatment of chronic or recurrent acute sinusitis in a select group of patients whose disease is recalcitrant despite maximizing conventional medical therapy. Many believe that IVIG may not be acting as replacement therapy for a humoral immune deficiency but more so as an anti-inflammatory or immune-modulat- ing agent that interrupts the chronic inflammatory process in patients with chronic sinusitis.

Question 65

What conventional management options are | available for chronic pediatric sinusitis?

Answer 65

Nasal irrigation, intranasal corticosteroids, topical/oral decongestants, mucolytics, and/or topical/oral antihis- tamines. Patients should also undergo a workup for allergies, reactive airway disease, headache, immuno- deficiency, and cystic fibrosis as indicated.

Question 66

Discuss the surgical options for treatment of | pediatric chronic sinusitis.

Answer 66

● Adenoidectomy ● Maxillary antral lavage ● Functional endoscopic sinus surgery: middle meatal antrostomy, anterior or total ethmoidectomy

Question 67

What are the indications for sinus surgery for | pediatric sinusitis?

Answer 67

Absolute indications: ● Complete nasal obstruction in cystic fibrosis attributable to massive polyposis or closure of the nose by medial- ization of the lateral nasal wall ● Antrochoanal polyp ● Intracranial complications ● Mucoceles and mucopyoceles ● Orbital abscess ● Traumatic injury in the optic canal (decompression) ● Dacryocystorhinitis resulting from sinusitis and resistant to appropriate medical treatment ● Fungal sinusitis ● Some meningoencephaloceles ● Some neoplasms Relative indications: ● Chronic rhinosinusitis that persists despite optimal medical management and after exclusion of any systemic disease ● Optimal medical management includes 2 to 6 weeks of adequate antibiotics (IV or oral) and treatment of concomitant diseases.

Question 68

What primary immunodeficiencies are associated | with chronic sinusitis?

Answer 68

● Common variable immunodeficiency ● Immunoglobulin (Ig)A deficiency ``` ● IgG subclass deficiencies, most commonly IgG3 defi- ciency (important for defense against Moraxella catarrhalis ``` and Streptococcus pyogenes)

Question 69

Discuss the anatomical abnormalities of the | sinuses in patients with cystic fibrosis.

Answer 69

The chronic inflammatory disease and decreased ventilation of the sinuses prevent pneumatization, resulting in dimin- ished postnatal growth of the sinus systems already present at birth, the maxillary and ethmoid sinuses. In addition, there is a lack of development, or hypoplasia, of the frontal and sphenoid sinuses. Incidence of nasal polyposis in cystic fibrosis varies from 6 to 48% and does not usually occur before 5 years of age or after age 20.