Pulmonology

Front 1

Choanal atresia

Back 1

-- MC congenital anomaly of the nose

-- If bilateral - upper airway obstruction, cyanosis that worsens with feeds and improves with crying

-- If unilateral -- -presents later in life

Attempt to pass a feeding catheter. If does not passive, highly suggestive. Obtain CT with intranasal contrast

-- Can be isolated or part of syndrome

(CHARGE, Treacher-Collins) Coloboma, Heart disease, Atresia choanae, Retarded growth/development, Genital anomalies, Ear anomalies

Front 2

Pyriform Aperture Stenosis

Back 2

-- presents similar to chonal atresia but less common

-- bony overgrowth an the anterior body opening (nasal nasal aperture)

CT -- small nasal opening

may require tracheosomy before surgical correction

Front 3

Foreign Body in Nose

Back 3

-- Suspect when see child with foul smelling unilateral nasal discharge

Front 4

Epistaxis

Back 4

-- nose picking

-- trauma foreign bodies

-- MC in dry winter months

-- drug use in adolescents

Front 5

Ankyloglossia

Back 5

-- lingual frenulum limits movement of anterior tongue tip

-- 3-5% with male predominance

-- Rx frenulectomy

Front 6

Thyroglossal Duct Cysts

Back 6

-- MIDLINE cystic mass in neck

-- asymptomatic if not infected. Then they rapidly increase in size and cause respiratory compromise

-- Rx: surgically remove thyroglossal duct cysts

Front 7

Laryngomalacia

Back 7

-- MC congenital anomaly of larynx, MC cause of stridor in infants

-- Usually self limiting

-- Stridor typically develops within the first few weeks of life and progresses in severity over several months. Due to laryngeal cartilage not stiff enough, inspiration causes significant luminal narrowing, resulting in inspiratory stridor.

-- exacerbated by SUPINE positioning, exertion, crying, feeding

-- prone position or lying on side and extending the next may relieve the stridor

-- resolution usually by 18-24 months

-- diagnosis made by flexible laryngoscopy -- patient needs to be awake to be seen

Front 8

Laryngeal Atresia/Web

Back 8

-- failure of larynx to recanalize; can lead to laryngeal web

-- usually incompatible with life

-- Rx: incision or dilation. Tracheostomy when severe.

-- Order genetic testing for 22q11 gene deletion

Front 9

Tracheal Agenesis

Back 9

-- rare, 3 types

-- severe respiratory distress when born

-- suspect if trachea cannot be intubated despite larynx being visualized

Front 10

Tracheal Stenosis

Back 10

-- Severe retractions and dyspnea and have expiratory stridor.

-- Tracheomalacia and Bronchomalacia --> airway cartilage does not have adequate tone and collapses with breathing --> expiratory stidor

Front 11

Lobar emphysema

Back 11

-- one or more lobes are markedly enlarged with fluid or air. Left upper lobe MC.

Rx: Oxygen; lobectomy if severe.

Front 12

Pulmonary Hypoplasia

Back 12

-- common cause of neonatal death

-- associated with oligohydramnios and premature rupture of membranes

-- prognosis depends on severity. Severe cases baby dies. Can use mechanical ventilation or ECMO (extracorporeal membrane oxygenation)

Front 13

Congential Pulmonary Venolobar Syndrome ("Scimitar Syndrome")

Back 13

-- rare disorder in which pulmonary venous (oxygenated) blood from right lung returns to inferior vena cava (IVC) just above or below the diaphragm.

-- Left (oxygenated) to right (deoxygenated) shunt.

-- X ray show shadow of veins "scimitar like" (Turkish sword).

-- Rx: Occlude aortopulmonary collaterals. High mortality

Front 14

Pulmonary AV Malformations

Back 14

-- dyspnea, bleeding with hemoptysis, exercise intolerance

Front 15

Pulmonary sequestrations

Back 15

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

-- can be intralobar or extralobar

-- intralobar is usually in lower lobes of each lung; anomalous vessels off aorta supply intralobar sequestrations with drainage through pulmonary veins. Usually isolated and typically identified during childhood.

- Exralobar. MC in males, on left side. Supplied by pulmonary or systemic artery branches. Often communicates with foregut. Most diagnosed in infancy.

--> bronchoscopy is not helpful because sequestration not connected to normal airway.

- diagnose with CXR/CT, Doppler

Front 16

Bronchogenic cysts

Back 16

-- abnormal budding of tracheal diverticulum

-- MC cyst of infancy

-- prone to infection

Front 17

Nasal Polyp

Back 17

-- suspect CF in anyone under 12 with one

-- Nasal steroids (NOT decongestants) have been shown to be effective

Front 18

With scoliosis, at what angle may you start to find pulmonary function abnormalities

Back 18

50%

Front 19

Pectus Carinatum

Back 19

pigeon breast, sticks out

--- usually asymptomatic

Front 20

Asphyxiating Thoracic Dystrophy (Jeune Syndrome)

Back 20

-- AR

-- short ribs, small rib cage, renal disease

Front 21

Spinal Muscular Atrophy (SMA)

Back 21

-- 2nd most common lethal AR disorder

-- chromosome 5q

-- hyptonia, muscle atrophy, fasciculations

-- degeneration of anterior horn cell, sometimes bulbar nuclei

-- symmetric muscle weakness, proximal muscles affected > distal ones.

-- Because only motor anterior horn cell affected, sensory and intellectually they are fine

-- 4 types:

1) Type 1 Werdnig-Hoffman (severe infantile SMA), presents before 6 months of age; often die before 2, need trach

2) Type 2,3,4

-- 95% of cases can be diagnosed with gene mutation screening. Defect is SMN1 gene.

-- CPK is normal.

Front 22

Duchenne Muscular Dystrophy

Back 22

-- x-linked recessive

-- mutation in dystrophin gene

-- 1/3,000 male births

-- presents at 2-6 years old with frequent falling, waddling, toe walking

-- calf muscle pseudohypertrophy and Gowers sign

-- CPK elevated

-- Cardiopmyopathy

-- Diagnose with muscle biopsy (atrophic muscle fibers) and immunochemical staining. Genetic testing also available

Front 23

Myasthenia gravis

Back 23

-- rare in children but still the MC primary disorder of neuromuscular transmission

-- Neonatal myasthenia gravis occurs when newborn is exposed transplacentally to maternal acetylcholine receptor antibodies. Resolves 2-12 weeks after maternal antibodies cleared

-- congenital myasthenia gravis is AR. Do NOT have circulating antibodies to acetylcholine receptor

-- Juvenile myasthenia gravis - acquired AI disorder, girls > boys, after 10 years of life. Circulating autoantibodies to acetylcholine receptors are in 80-90% . Worse with repetitive movement.

-- Clasically diagnose MG by testing for specific autoantibodies directed at acetylcholine receptor (AChR-Ab) or against a receptor associated protein, muscle specific tyrosine kinase.

Rx: oral anticholinesterase, most commonly pyridostigmine

--> thymectomy induces remission in 50-60% cases. Plasmapheresis is beneficial for short term amelioration

Front 24

Why should you not use antihistamines in small children with URI

Back 24

they decrease cilia movement and can delay mucus clearing

Front 25

When should you suspect bacterial sinusitis

Back 25

suspect if sinus symptoms last longer than 10 days or when sinus symptoms worsen during the course of a resolving URI

Front 26

Potts puffy tumor

Back 26

Osteomyelitis of the frontal bone associated with a subperiosteal abscess: forehead or scalp swelling and tenderness, headache, photophobia, fever, vomiting, lethargy

Front 27

Mucormycosis

Back 27

fungal disease that can be life threatening. Occurs in poorly controlled diabetics and can present as black eschar on nasal turbinate.