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