Pulmonology

Question 1

How does Choanal Atresia present

Answer 1

unilateral or bilateral boney or membranous septum between nose and pharynx

can breath through their mouth but no their nose
have difficulty feeding
have respiratory distress and improve with crying

infant with unilateral atresia are asymptomatic until secretions block the one working nare

Question 2

How is choanal atresia diagnosed and what is the treatment?

Answer 2

can’t pass a firm catheter through each nostril to a depth of 3-4 cm
confirm with CT scan
give adequate oral airway that also allows feeding (OG tube) if severe difficulty breathing, tracheostomy
repair is surgical

Question 3

What are the findings in CHARGE syndrome

Answer 3

Colomboma
Heart defect
Atresia (choanal)
Retardation of Growth
Genital anomalies
Ear anomalies

Question 4

What are some other rare causes of congenital nasal disorders

Answer 4

nasal masses - dermoid, encephaloceles, gliomas
pyriform aperture stenosis which mimics choanal atresia
If these have intracranial extension, resection is required to prevent intracranial infection

Question 5

What is lingual ankyloglossia and what is the management

Answer 5

lingual frenulum limits anterior movement of the tongue tip
usually infants can adjust and feed well though some require frenulectomy if unable to nurse (usually not the case)
can cause speech difficulties and this would be another indication for frenulectomy
some times older children elect for frenulectomy if interferes with activities like ice cream cone licking :)

Question 6

How does lingual thyroid present

Answer 6

thyroid fails to descend
raised violaceous mass at base of tongue

may be only functioning thyroid tissue,
can use thyroid hormone to make smaller as infection, puberty and pregnancy can cause enlargement with dysphagia and airway obstruction

Question 7

How does a thyroglossal duct cyst present and what is the treatment?

Answer 7

cystic masses in the midline of the neck
asymptomatic unless they become infected
if infected can quickly get bigger an cause airway compromise
must be surgically removed

Question 8

What is the most common cause of stridor in the newborn

Answer 8

laryngomalacia
laryngeal cartilage is not stiff enough resulting in stridor
most commonly heard at 2 weeks of age and worse with agitation
most outgrow by 12 to 18 months
in severe cases may affect feeding or cause night time hypoxia

Question 9

What genetic syndrome should be tested for in those with a glottic web? How do glottic webs present?

Answer 9

22q11, DiGeorge
can be partial or complete
present with stridor, abnormal voice or respiratory distress
if severe can require tracheostomy

Question 10

What are some other causes of stridor in a newborn?

Answer 10

Subglottic stenosis: stridor and respiratory distress, diagnosed with endoscopy, surgical correction in those who are symptomatic

Laryngeal Cysts: from mucus secreting epithelium stridor and hoarseness, require removal (occur usually with prolonged intubation than congenital)

Laryngoceles: epithelium lined diverticula from laryngeal ventricle, can present with obstruction or a mass, treat with excision

Question 11

How does tracheal stenosis present and what is the treatment?

Answer 11

segmental stenosis that can occur anywhere along the trachea
retractions with dyspnea
EXPIRATORY stridor
also consider in those with prolonged or recurrent croup
bronchoscopy

Question 12

What is seen in lobar emphysema and over inflation

Answer 12

causes severe respiratory distress in the neonatal period with increased resonance, even mediastinal shift
one or more lobes is markedly enlarged with air (often left upper lobe) due to an obstruction intrinsic to the airway
severe cases require lobectomy

Question 13

What is pulmonary hypoplasia

Answer 13

decrease in number of alveoli resulting in severe respiratory distress with hypoxemia and hypercarbia
in utero there is often oligohydramnios often premature rupture of membranes with premature delivery
if less severe can be placed on mechanical ventilation or even ECMO till lungs grow

Question 14

What is scimitar syndrome

Answer 14

pulmonary venous blood from all or part of the right lung returns to the IVC

chest XR show the shadow of these veins and it looks like a Turkish sword

can present as heart failure or pulmonary hypertension
treatment is with surgery

Question 15

What is the most common disorder associated with pulmonary arteriovenous malformations and what are the symptoms?

Answer 15

hereditary hemorrhagic telangiectasia or Rendu-Osler-Weber Syndrome
many have recurrent nose bleeds
PAVM increases with age
causes right to left shunting of systemic blood into pulmonary veins to the left heart leading to hypoxemia
large shunts lead to dyspnea, hemoptysis and exercise intolerance
at risk for stroke, TIA and brain abscesses
if symptomatic treat with procedural angiography

Question 16

What is a pulmonary sequestration and how does it present?

Answer 16

mass of abnormal non-functioning lung tissue, isolated from the rest of the lung and fed by systemic arteries

may result in recurrent pneumonia, hemoptysis or infection. some older children have pleuritic chest pain

evaluate with CT/MRI, US can show blood flow
surgically remove

Question 17

What is a bronchogenic cyst, how does it present and what is the treatment

Answer 17

abnormal budding of the tracheal diverticulum
can cause symptoms due to compression on near by structures
can cause fever, chest pain and productive cough
if they rupture can cause pneumothorax or hemoptysis
excise these lesions even if asymptomatic as risk for future symptoms is high

Question 18

How does a nasal foreign body usually present

Answer 18

unilateral nasal drainage that is often foul smelling

Question 19

What are some causes of epistaxis and how is it treated

Answer 19

nose picking is the most common cause
trauma, foreign bodies and neoplasms
cocaine
hereditary hemorrhagic telangiectasia
apply pressure for 5-10 minutes
vasoconstrictor nose sprays
cautery with silver nitrate
nasal packing
further workup is warranted if epistaxis is difficult to correct

Question 20

What are the most common causes of nasal polyps in children and how are they treated

Answer 20

Cystic Fibrosis
Aspirin triad: asthma, nasal polyps and aspirin sensitivity

mouth breathing with nasal sounding voice
treat with nasal steroids or surgery if do not respond, cause obstruction, recurrent infection or nasal deformity

Question 21

What are some causes of scoliosis and when does it cause cardiopulmonary compromise?

Answer 21

idiopathic
neuromuscular disease
congenital rib/vertebral anomalies

cardiopulmonary compromise: exceeds 90 degree
pulmonary abnormalities when thoracic curve exceeds 50 degrees

Question 22

How is scoliosis treated

Answer 22

if found early, bracing corrects or limits progression

surgical correction is often needed if the cause is neuromuscular weakness

Question 23

What is pectus excavatum, what symptoms does it cause and when is it corrected

Answer 23

depression of the midsternum
rarely causes respiratory or cardiac problems but can cause exercise intolerance
severe pectus can shift the heart leftward
CT scan can show severity and exercise testing can show impairment
surgery for those with associated scoliosis or significant pulmonary cardio limitation

Question 24

What is pectus carinatum

Answer 24

sticking out of the sternum with lateral depression of the costal cartilages
rarely symptomatic

Question 25

What is Jeune Syndrome or asphyxiating thoracic dystrophy

Answer 25

AR short ribs and small rib cage and renal disease some also have short limb dwarfism. pelvic anomalies polydactyly and hepatic involvement

Question 26

What is spinal muscular atrophy and what are the symptoms

Answer 26

Defect is the SMN1 gene CPK is normal AR hypotonia, muscular weakness, muscular atrophy, fasciculations, proximal muscles weaker than distal degeneration of the anterior horn cell and sometimes bulbar nuclei NO sensory or intellectual disability

Question 27

What are the 4 type of SMA

Answer 27

Type 1: Werdnig-Hoffmann or severe infantile form, presents before 6 months with hypotonia, weakness, difficulty feeding and tongue fasciculations, most die by 2 Type 2: normal at birth and lose milestones by age 2. Majority die by 12. will have static periods of weakness that progress during illness Type 3: Kugelberg-Welander, mild SMA presents between 2 and 17, become unable to walk but have normal life span Type 4: like type 3 but presents in 2nd and 3rd decade

Question 28

What is seen with Duchenne muscular dystrophy

Answer 28

x linked recessive mutation in dystrophin gene that makes dystrophin protein boy present 2-6 years old with frequent falling, waddling gait and toe walking calf pseudohypertrophy Gower's sign-climbing up the legs, uses hands to get off of the floor due to muscle weakness CPK is elevated cardiomyopathy diagnose with muscle biopsy or gene testing

Question 29

What is seen in neonatal myasthenia gravis

Answer 29

newborn exposed to transplacental passage of mom's acetylcholine receptor antibodies hypotonia, weak cry, difficulty feeding, facial weakness and palpebral ptosis respiratory compromise occurs resolves in 2-12 weeks when maternal antibodies have cleared

Question 30

What is congenital myasthenia gravis

Answer 30

AR | those affected do not have a circulating antibody

Question 31

What is seen in Juvenile myasthenia gravis

Answer 31

acquired autoimmune disorder | gradual progression of muscle weakness, worse with repeated movements. eye muscles are involved

Question 32

How is myasthenia gravis diagnosed and treated

Answer 32

Give an acetylcholinesterase medication like neostigmine or edrophonium and look for improvement check for antibodies against the acetylcholine receptor or muscle specific tyrosine kinase treat with oral anticholinesterase like pyridostigmine thymectomy may reduce remission in 50-60% of cases plasmapheresis as short term amelioration

Question 33

What is the usual number of respiratory infections for a child to have in a year and when do they usually occur

Answer 33

3-8 infections a year | usually fall and winter

Question 34

What are risk factors for viral URI and what viruses are most often the cause

Answer 34

day care attendance smoke exposure lower SES over crowding rhinovirus, coronavirus, adenovirus and coxsackievirus

Question 35

What is the management of viral URI and what are some common complications

Answer 35

Do not order laboratory tests antitussives and decongestants should not be used in those < 2 years old saline nose drops and bulb syringe no antihistamines-decrease cilia movement and delay mucous clearance nothing shortens the duration complications: otitis media, asthma exacerbation, pneumonia green discharge does not mean there is sinusitis

Question 36

How does bacterial sinusitis present and what are the most common organisms?

Answer 36

strep pneumonia, Moraxella and non-typeable H. flu cough, nasal discharge and halitosis, cough worsens when supine, sore throat from drainage and occasionally fever adolescents can have facial pain, tenderness and facial swelling clue: symptoms do not improve in 7-10 days, suspect if symptoms last longer than 10 days or sinus symptoms worsen as URI resolves

Question 37

What testing can be done for bacterial sinusitis

Answer 37

CT scan in not recommended for those with uncomplicated acute infection to get bacterial cultures have to aspirate the sinus by direct antral puncture, this is only for the immunocompromised, life threatening illness or those who have been unresponsive to therapy

Question 38

What is preseptal or periorbital cellulitis

Answer 38

complication of sinusitis: swelling and erythema of the eyelids and periorbital area, no ptosis or limitation to eye movement

Question 39

What is orbital cellulitis

Answer 39

pain with eye movement, conjunctival swelling, proptosis, limitation of eye movements, diplopia and vision loss

Question 40

What is potts puffy tumor

Answer 40

osteomyelitis of the frontal bone associated with sub periosteal abscess forehead swelling and tenderness, headache, photophobia, fever, vomiting and lethargy complication of sinusitis

Question 41

How does septic cavernous sinus thrombosis present

Answer 41

bilateral ptosis, proptosis, ophthalmoplegia, periorbital edema, headache and change in mental status complication of sinusitis

Question 42

What is the most common cause of acute sore throat in children?

Answer 42

viral infection | often with other symptoms like conjunctivitis, coryza, cough, stomatitis, diarrhea

Question 43

How does group A strep pharyngitis present

Answer 43

begins with non-specific symptoms of headache, abdominal pain or vomiting with high fever. Children then develop sore throat with few other symptoms, no URI symptoms tonsils are erythematous, palatal petechiae

Question 44

How is group A strep pharyngitis diagnosed?

Answer 44

Rapid step test with follow up culture if the rapid testing is negative

Question 45

What are the concerning complications of group A strep pharyngitis and how is group A infection treated

Answer 45

Rheumatic fever which can be prevented if strep is treated within 9 days of symptoms (only occurs after throat infection) Post strep glomerulonephritis- happens regardless of therapy penicillin shortens disease course use azithromycin, erythromycin for those who are penicillin allergic can return to school next day after taking antibiotic

Question 46

How should chronic carriers of Group A strep be managed?

Answer 46

antibiotic therapy is not indicated except: in setting of outbreak of rheumatic fever or post strep glomerulophritis outbreak in a semi-closed community (dorm, jail) family history or rheumatic fever multiple episodes of symptoms of GAS in a family despite appropriate therapy use clindamycin TID to treat for 10 days

Question 47

What is streptococcosis

Answer 47

in children < 2 years of age coryza with postnasal discharge, fever (can last up to 8 weeks), pharyngitis, poor appetite and tender cervical lymphadenitis

Question 48

How does a retropharyngeal abscess present?

Answer 48

abrupt onset of high fever and difficulty swallowing usually occurs during acute pharyngitis with refusal to eat, severe throat pain, hypertension of the head and gurgling respirations. Drooling soon develops

Question 49

What is seen on XR in retropharyngeal abscesses?

Answer 49

lateral XR of nasopharynx and neck: mass, will be more than 50% of the width of the adjacent vertebral body

Question 50

What is the treatment of retropharyngeal abscess?

Answer 50

Medical emergency due to chance of aspiration on the pus or extension into fascial planes treat with nafcillin and clindamycin or Amp-sulbactam can do drainage if necessary

Question 51

How does a peritonsillar abscess present?

Answer 51

Fever, often high to 105, severe pain, trismus, difficulty speaking or swallowing, hot potato voice, displaced uvula

Question 52

What are the indications for tonsillectomy

Answer 52

recurrent pharyngitis (7 in 1 year, 5 in each of the last two or 3 in each of the last three) sleep apnea marked/severe adenotonsillar hypertrophy Does not help otitis media or chronic sinusitis

Question 53

What are the indications for Adenoidectomy

Answer 53

``` persistent mouth breathing chronic otitis media with effusion hyponasal speech adenoid facies persistent or recurrent nasopharyngitis that seems temporally related to hypertrophied adenoid tissue ```