Pediatric thoracic surgery Flashcards
Formation of trachea
Week 4 – laryngo-tracheal bud, rising from the anterior
foregut (primitive esohagus)
= future trachea – separating from the proenteron by tracheo-esophageal septum
– and bifurcation into -> primitive bronchi
End of process – more than (10)7 airways
Broncho-pulmonary malformations (BPM)
PRENATAL DIAGNOSIS
• ULTRASOUND (W18-W24)
• Differential diagnosiS for other BPM (+/- fetal MRI)
• Differential diagnosis with other congenital anomalies
(dyaphragmatic hernias, cardiac malformations etc.)
• Perinatal management planning
• Associated anomalies diagnosis
!!• Fetal distress
• Fetal hydrops
• Fetal death in utero
BPM POSTNATAL DIAGNOSIS
• 25% symptomatic at birth/neonatal period
• ACUTRE RESPIRATORY DISTRESS SYNDROME
(ARDS) of the newborn
• Cough, tachypnea, cyanosis, respiratory effort
(thoraco-abdominal balancement – paradoxal
breathing, intercostal retractions etc.)
• 75% - incidental finding or following a complication
• Pulmonary infection
• Pneumothorax
• Chronic cough, shortness of breath – dyspnea,
cyanosis
• No lung sounds, thoracic asymmetry, percussion
changes (hyperresonance or dullness), respiratory
failure signs
BPM MANAGEMENT
• PRENATAL
• ultrasound follow-up (+/- MRI), if there are no fetal distress / hydrops signs • thoraco-aminiotic shunt • thoracocentesis (if possible – macrocysts) • premature birth indication • steroids therapy • fetal surgery (laser ablation) • EXIT (ex-utero intrapartum therapy)
BPM MANAGEMENT
• POSTNATAL
• classical / minimally-invasive approach
• elective indication – if there are no
complications
• emergency in case of ARDS
• atypical, segmentary orlobar resections
• pleural drainage
Congenital adenomatoid cystic malformations of the
lung
• heterogenous group of cystic/non-cystic lung malformation – excessive proliferation of the immature terminal bronchioles in a pulmonary segment
(pseudoglandulary stage ☝)
• 0,66-1,5 : 10000 births – the most frequent BPM (50-70% of all BPMs)
• Vascular intake from the pulmonary system
• Communication with the bronchi tree
• Hybrid lesions are well known(systemic vascular intake – most frequently, aorta)
Congenital adenomatoid cystic malformations of the
lung
CLINICAL FEATURES:
- uniqe, unilateral, unilobar mass
- more frequently on the right, and upper lobes
- asymptomatic in most of the cases
- identified following a pulmonary infection or pneumothorax
- most of the cases- no associated congenital malformations
Congenital adenomatoid cystic malformations of the
lung
DIFFERENTIAL DIAGNOSIS
- Other BPMs (bronchogenic cysts, congenital lobar emphysema, pulmonary sequestration)
- Congenital diaphragmatic hernia
- Pneumatocele
Congenital adenomatoid cystic malformations of the
lung
COMPLICATIONS
- prenatal - like in all BPMs
- postnatal – specific to all BPMs, but more likely – infection, pneumothorax
- specific – malignant potential – rabdomyosarcoma, pulmonary blastoma, bronho-alveolary carcinoma
Congenital adenomatoid cystic malformations of the
lung
TREATMENT
- Elective – considering the possible complications (malignant potential!)
- Segmentectomy
- Lobectomy
Broncho-pulmonary sequestration (BPS)
• rare congenital malformation, unfunctional lung tissue (not air-filled, without any
communication to the normal tracheo-bronchial tree) whose blood intake comes from
aberrant vessels originating from the system circulation
• 0,15-6,4% of all BPMs
• Systemic vascular intake
BPS CLINICAL FEATURES
• feeding problems, breathing
difficulties, cardiac failure signs – VASCULAR STEAL
• infection – despite no communication with the respiratory tree
BPS
IMAGISTIC EVALUATION – highly
suggestive
• Homogeneity,
good delineation,
triangular aspect (lateral base),
identification of an aberrant feeding vessel (75% - an aortic branch)
Congenital lobar emphysema
lower airways malformation – hyperinflation of one or more pulmonary lobes determining variable grades of secondary compression over the the adjacent pulmonary parenchima or mediastinum
• 1:20000-30000 births
• M:F 3:1
Congenital lobar emphysema
IMAGISTIC EVALUATION
- High lung translucency and vascular markings paucity
* Mediastinum shift / heart shift / diaphragmatic flattening
Congenital lobar emphysema
TREATMENT
- Conservatory
- Lobectomy
- Segmentectomy
- Specific – may constitute an emergency