Resp: Congenital Anomalies

Front 1

congenital anomalies seen in larynx

Back 1

-absent epiglottis
-bifid epiglottis
-laryngeal atresia (web, LET cleft, larygnomalacia)
-subglottic stenosis

Front 2

presentation of a laryngeal anomaly

Back 2

stridor
absence of larynx in neck
-high pitched wheezing sound resulting from turbulent air flow in upper airway

Front 3

cause of layrngeal atresia

Back 3

failed recanalization of larygneal orifice

Front 4

laryngeal webs

Back 4

-partial failure of recanalization
-thin, incomplete membrane with post, glottis open
-stridor or weak or absent cry, resp distress
-seen with endoscopy

Front 5

larygneomalacia

Back 5

-most common
-squeaky baby
-omega shaped epiglottis
-supraglottic structures prolapse into the airway during inspiration
-high-pitched fluttery
-failure to thrive, cyanotic

Front 6

trachea anomalies presentation

Back 6

-wheezing
-may only be apparent when exertional (crying or BM)
-air flow ceases and cyanosis

Front 7

types of tracheal anomalies

Back 7

-tracheal agenesis
-tracheal stenosis
-tracheal bronchs
-tracheomalacia
-TEF

Front 8

tracheal stenosis

Back 8

-intrinsic narrowing of trachea
-absence of post membrane portion of trachea resulting in tracheal walls completely encircles by cartilaginous rings
-wheezing
-CXR, CT, bronchoscopy

Front 9

tracheal bronchs

Back 9

-bronchus arising from trachea
-asymptomatic found on bronchoscopy
-recurrent infection, atelectasia

-no problem as long as all parts of lungs are supplied

Front 10

tracheomalacia presentation

Back 10

loud coughs
-tracheal lumen collapses during respiration
-misshapen or flattened tracheal rings that don't extend as far around trachea giving membranous portion greater mobility
-collapses when intratrach pressure is less than surrounding
-harsh central expiratory wheeze
-harsh, barking sound

Front 11

TEF

Back 11

trachesophageal fistula
-most common: esophageal atresia
-recurrent aspiration, feeding difficulties, drooling, abdom distension, regurg

Front 12

bronchi anomalies

Back 12

bronchial stenosis
bronchial atresia
bronchiogenic cysts

Front 13

prox bronchial problem use:
distal:

Back 13

bronchoscopy

CT/MRI-help to delineate extent of parenchymal involvement, cysts

Front 14

bronchial stenosis

Back 14

-narrowed
-distal airways and alveoli spared
-wheeze, cough, recurrent pneum.
-hyperinflation and air trapping

Front 15

bronchial atresia

Back 15

-distal airways and alveoli spared
-later ages as an accident

Front 16

bronchiogenic cysts:
locations

Back 16

paratrach
subcarinal*
perihilar*
intrapulm

*most common sites

Front 17

what is a bronchiogenic cyst

Back 17

abnormal budding/detachment of resp tissue from developing respiratory tract
-lined with resp mucosa inc mucous secreting cells
-may cause obstruction
-cough, dyspnea, wheeze, recurr infection
-surgery is curative

Front 18

lung anomalies

Back 18

-congenital lobar emphysema (CCAM)
-lymphangiectasia (sequestration)
-hypoplasia

Front 19

lung aplasia vs agenesis

Back 19

agensis: absent airways, vasculture, lung tissue

aplasia: lacking vasculatre, lung tissue

Front 20

lung agenesis/aplasia on CXR

Back 20

dense normogeneous infiltrate, marked mediastinal shift toward affected side, blurring or loss of heart border

Front 21

CCAM

Back 21

abnormally devo of resp tissue composed mainly of terminal bronchial elements that lack distal alveolar structures

Front 22

types of CCAM

Back 22

type 1: macrocystic
type 2: multiple small
type 3: largely homogenous nearly solid mass

-may be fluid filled
-may limit the growth of rest of the lung

Front 23

dx and tx of CCAM

Back 23

CT
CXR: bubbly look
tx:
-intrauterine aspiration to dec cyst size, fetal surgery to remove, postnatal lobectomy

Front 24

congential lobar emphysema

Back 24

-idiopathic
=hyperinflation of 1 or more lobes with dec vascular markings
-dec breath sounds on affected side
-compress and cause mediastinal shift
-CCT: defines character and extent of lesion
-surgical resection: cure

**74% are R middle and L upper(most common)

Front 25

pulmonary sequestration

Back 25

-lung tissue that loses its communication with central airways and receives its principle vascular supply from systemic rather pulmonic circulation

Front 26

types of pulmonary sequestration

Back 26

-invested by its own pleura (extralobar)
OR
-within other visceral pleura (intralobar)

Front 27

example of an extralobar sequestration

Back 27

sub diaphragmatic vessel from aorta supplying left sided extralobar sequestration

Front 28

if pulmonary sequestration communicates with gut

Back 28

bronchopulmonary foregut malformation

Front 29

extralobar has a higher incidence....

Back 29

of usually Lsided associated abnormalities

Front 30

pulmonary hypoplasia

Back 30

small lung with defective or incomplete disproportionate to gestational or postnatal age

Front 31

causes of pulmonary hypoplasia

Back 31

diaphragmatic hernia
oligohydramnios
multifactorial: w/ downs may devo fewer alveoli

Front 32

tx of pulmonary hypoplasia

Back 32

supportive
surgical removed or mitigated if herniation