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HYPOPLASIA OF THE

LUNG

HELMI LUBIS
R I D W A N M . D A U L AY
WISMAN DALIMUNTHE
R I N I S AV I T R I D A U L AY
 Pulmonary hypoplasia (small lung)  defined as
lung weight more than 2 SD below the normal for
age (or gestational age)

 Almost always accompanied by hypoplasia of the


corresponding pulmonary vessel

 Hypoplasia as an isolated phenomenon is rare


Causes

Table 1. Most common causes of Pulmonary Hypoplasia


Condition leading to an egress of lung •Severe oligohydramnion (from
fluid premature rupture of membranes,
bilateral renal agenesis, urinary tract
obstruction)
•Compression of thoracic cage and
abdominal contents by the uterus 
limitation of breathing movement
SOL •Congenital diaphragmatic hernia
•Lung malformation
•Thoracic tumor
•Pleural effusion
•Abdominal condition pressing on the
diaphragma (massive ascites)
Thoracic cage anomalies •Achondroplasia
•Scoliosis
Conditions preventing normal fetal •Anencephaly
breathing movements •Phrenic nerve agenesis

Thoracic compression from below Abdominal tumors


Ascites

Thoracic compression from the side Amniotic bands


Oligohydramnions
Asphyxiating dystrophy/scoliosis or
other chest wall deformity
Clinical Finding

 Early infancy  respiratory distress ( mild to


severe depending on the degree of hypoplasia)
 Severe bilateral hypoplasia  thoracic cage
reduced in size  bell shaped
Diagnosis

 Chest X ray:
 Ribs may appear crowded

 Low thoracic to abdominal ratio

 Isotope scanning  perfusion>ventilation on


the side of the lesion
Treatment

 No specific treatment
 Supportive measures :
 Mechanical ventilation

 Supplemental oxygen
Prognosis

 Infants who remain on high pressure ventilation and


high inspired oxygen concentration at the end of the
first week  extremely bad prognostic
CONGENITAL
DIAPHRAGMATIC HERNIA

HELMI LUBIS
R I D W A N M . D A U L AY
WISMAN DALIMUNTHE
R I N I S AV I T R I D A U L AY
 Definition: developmental abnormality of the
diaphragm that allow abdominal viscera to enter the
thoracic cavity

 Defect:
 Most common: posterolateral (Bochladeck)  90% on the left
side, 10% on the right side and 1% bilateral
 Retrosternal (Morgagni)
Epidemiology

 Incidence:
 1 in 2000-4000 lives birth

 ♂:♀ = 1,5:1
Pathogenesis

 Premature migration of the gut into the abdominal


cavity after the periode of extracoelomic growth
(compression theory)

 Abnormal lung development/hypoplasia which


permits the herniation of the gut into the chest

 Problem with phrenic nerve development leading to


incomplete formation of the diaphragm

 Delayed closure of the pleuroperitoneal fold


Prenatal Diagnosis

 USG
 MRI
Clinical Manifestation

 Majority:
 Severe respiratory distress (first hour of life)
 Scaphoid abdomen
 Apparent dextrocardia (since 90% CHD are on the left)
 Decreased breath sound over the involve chest

 Delayed presentation:
 Vomiting  intestinal obstuction, gastric volvulus
 Mild respiratory simptom

 Occasionally:
 Ischemia  incarceration of the intestine
 Sepsis
 Cardiorespiratory collapse

 Unrecognized:
 Sudden death
Initial Management

 Avoiding bag and mask ventilation  minimize


gaseous distention of the stomach and intestines,
which would further compromise lung function
 Prompt endotracheal intubation
 Nasogastric tube passed and placed then chest x ray
was done
Diagnosis post natal

 Radiology:
 CXR  lateral: intestine passing the through posterior portion of
diaphragm
 USG & Fluoroscopy  distuingish true hernia and evantratio
 Barrium follow through
 CT Scan
 Echocardiography  pulmonal hypertension
Treatment

 Preoperative stabilization:
 Intubated

 Mechanical ventilation :
 Peak inspiratory pressure <25mmHg
 Sedation  allow coordination of the patient with the ventilator

 Operative:
 Generally shift from emergency repair to a delayed approach
after stabilization of the infant
Prognosis

 Mortality rate after birth: 7-10%


 Poor prognosis:
 Large anomaly

 Symptoms occur in first 24 hours

 Severe respiratory distress

 Recurency: 20-40% in first year


Congenital Lobar
Emphysema

HELMI LUBIS
R I D W A N M . D A U L AY
WISMAN DALIMUNTHE
R I N I S AV I T R I D A U L AY
Congenital Lobar Emphysema

 Pulmonary emphysema
 distention of airspaces with irreversible
disruption of the alveolar septa
 Rare condition
Etiology

 Some cases are caused by easily identifiable partial


obstruction such as mucosal flaps
 In many cases deficiency of bronchial cartilage is
thought to be the cause
Histology

 Majority  normal radial alveolar counts but with no


apparent maturation with age when compared to age-
matched control
 Affected lobe:
 Left upper (42%)

 Right middle (35%)

 Right upper (21%)

 Lower lobes (2%).

 The affected lobe cannot deflate, but displace


adjacent lobes and subsequently the mediastinal
structure
Clinical feature

 Respiratory distress
 Suggestive of a tension pneymothorax:
 Hyperresonance of the affected hemithorax

 Diminishes breath sounds

 Deviation of mediastinal structures to the contralateral side


Diagnostic Tools

 Chest radiograph
 Hyperlucent lobe with features of compression and collapse of
adjacent lung and depression of ipsilateral diaphragm
 The mediastinum is deviated, and the contralateral lung may
be collapsed
 Occasionally, initial chest radiograph may demonstrate an
opaque lung field then clears and the affected lung becomes
overinflated and hyperlucent
....diagnostic tools

 Ventilation-perfusion scanning
› May demonstrate delayed uptake and clearance of isotope
and reduce blood flow in the affected lobe
 Bronchoscopy
› Reveal causes of intrinsic obstruction
› Permit the removal of the foreign body or inspissated
secretions
 Echocardiogram
› Evaluating the heart and the great vessels

 CT scan
› Evaluating the anatomy of the emphysematous lobe
› Size and relations, wheter it has herniated into the
contralateral hemithorax
Differential diagnosis

 Failure of airspace emptying


 Partial obstruction due to inspissated mucus

 Endobronchial granulomas

 Bronchiale atresia

 Congenital heart disease

 Bronchogenic cyst

 Loss of lung volume on the contralateral side


 Lobar or lung collapse

 Chylothorax
Treatment

 Conservatively  children who do not suffer


respiratory compromise
 Lobectomy is indicated in the event of respiratory
distress