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 fluid Severe oligohydramnion (from 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)
Achondroplasia Scoliosis

Thoracic cage anomalies

Conditions preventing normal fetal breathing movements

Anencephaly Phrenic nerve agenesis

Thoracic compression from below Thoracic compression from the side

Abdominal tumors Ascites 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 agematched 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