Pulmonology Flashcards
How does Choanal Atresia present
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
How is choanal atresia diagnosed and what is the treatment?
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
What are the findings in CHARGE syndrome
Colomboma Heart defect Atresia (choanal) Retardation of Growth Genital anomalies Ear anomalies
What are some other rare causes of congenital nasal disorders
nasal masses - dermoid, encephaloceles, gliomas
pyriform aperture stenosis which mimics choanal atresia
If these have intracranial extension, resection is required to prevent intracranial infection
What is lingual ankyloglossia and what is the management
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 :)
How does lingual thyroid present
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
How does a thyroglossal duct cyst present and what is the treatment?
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
What is the most common cause of stridor in the newborn
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
What genetic syndrome should be tested for in those with a glottic web? How do glottic webs present?
22q11, DiGeorge
can be partial or complete
present with stridor, abnormal voice or respiratory distress
if severe can require tracheostomy
What are some other causes of stridor in a newborn?
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
How does tracheal stenosis present and what is the treatment?
segmental stenosis that can occur anywhere along the trachea
retractions with dyspnea
EXPIRATORY stridor
also consider in those with prolonged or recurrent croup
bronchoscopy
What is seen in lobar emphysema and over inflation
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
What is pulmonary hypoplasia
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
What is scimitar syndrome
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
What is the most common disorder associated with pulmonary arteriovenous malformations and what are the symptoms?
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
What is a pulmonary sequestration and how does it present?
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
What is a bronchogenic cyst, how does it present and what is the treatment
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
How does a nasal foreign body usually present
unilateral nasal drainage that is often foul smelling
What are some causes of epistaxis and how is it treated
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
What are the most common causes of nasal polyps in children and how are they treated
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
What are some causes of scoliosis and when does it cause cardiopulmonary compromise?
idiopathic
neuromuscular disease
congenital rib/vertebral anomalies
cardiopulmonary compromise: exceeds 90 degree
pulmonary abnormalities when thoracic curve exceeds 50 degrees
How is scoliosis treated
if found early, bracing corrects or limits progression
surgical correction is often needed if the cause is neuromuscular weakness
What is pectus excavatum, what symptoms does it cause and when is it corrected
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
What is pectus carinatum
sticking out of the sternum with lateral depression of the costal cartilages
rarely symptomatic
What is Jeune Syndrome or asphyxiating thoracic dystrophy
AR
short ribs and small rib cage and renal disease
some also have short limb dwarfism. pelvic anomalies polydactyly and hepatic involvement
What is spinal muscular atrophy and what are the symptoms
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
What are the 4 type of SMA
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
What is seen with Duchenne muscular dystrophy
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
What is seen in neonatal myasthenia gravis
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
What is congenital myasthenia gravis
AR
those affected do not have a circulating antibody
What is seen in Juvenile myasthenia gravis
acquired autoimmune disorder
gradual progression of muscle weakness, worse with repeated movements. eye muscles are involved
How is myasthenia gravis diagnosed and treated
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
What is the usual number of respiratory infections for a child to have in a year and when do they usually occur
3-8 infections a year
usually fall and winter
What are risk factors for viral URI and what viruses are most often the cause
day care attendance
smoke exposure
lower SES
over crowding
rhinovirus, coronavirus, adenovirus and coxsackievirus
What is the management of viral URI and what are some common complications
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
How does bacterial sinusitis present and what are the most common organisms?
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
What testing can be done for bacterial sinusitis
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
What is preseptal or periorbital cellulitis
complication of sinusitis: swelling and erythema of the eyelids and periorbital area, no ptosis or limitation to eye movement
What is orbital cellulitis
pain with eye movement, conjunctival swelling, proptosis, limitation of eye movements, diplopia and vision loss
What is potts puffy tumor
osteomyelitis of the frontal bone associated with sub periosteal abscess
forehead swelling and tenderness, headache, photophobia, fever, vomiting and lethargy
complication of sinusitis
How does septic cavernous sinus thrombosis present
bilateral ptosis, proptosis, ophthalmoplegia, periorbital edema, headache and change in mental status
complication of sinusitis
What is the most common cause of acute sore throat in children?
viral infection
often with other symptoms like conjunctivitis, coryza, cough, stomatitis, diarrhea
How does group A strep pharyngitis present
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
How is group A strep pharyngitis diagnosed?
Rapid step test with follow up culture if the rapid testing is negative
What are the concerning complications of group A strep pharyngitis and how is group A infection treated
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
How should chronic carriers of Group A strep be managed?
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
What is streptococcosis
in children < 2 years of age
coryza with postnasal discharge, fever (can last up to 8 weeks), pharyngitis, poor appetite and tender cervical lymphadenitis
How does a retropharyngeal abscess present?
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
What is seen on XR in retropharyngeal abscesses?
lateral XR of nasopharynx and neck: mass, will be more than 50% of the width of the adjacent vertebral body
What is the treatment of retropharyngeal abscess?
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
How does a peritonsillar abscess present?
Fever, often high to 105, severe pain, trismus, difficulty speaking or swallowing, hot potato voice, displaced uvula
What are the indications for tonsillectomy
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
What are the indications for Adenoidectomy
persistent mouth breathing chronic otitis media with effusion hyponasal speech adenoid facies persistent or recurrent nasopharyngitis that seems temporally related to hypertrophied adenoid tissue
