HELMI LUBIS RIDWAN M. DAULAY WISMAN DALIMUNTHE RINI SAVITRI DAULAY HYPOPLASIA OF THE LUNG

Definition  Pulmonary hypoplasia = pulmonary agenesis = small lung  incompletely developed lung parenchyma connected to brochi that may also be underdeveloped depending on when the presumed causal insult took effect in embryogenesis.

Types Bilateral pulmonary agenesis  Rare  Associated with anencephaly, diaphragmatic hernias, urinary tract abnormalities, right sided heart malformations. Unilateral pulmonary agenesis  More common  Absence of the carina and the trachea running directly into a single bronchus.

Pathology The sole lung is larger than normal and this enlargement is true hypertrophy and not emphysema. Pulmonary hypoplasia involves a decrease in both the number of alveoli and the number of airway generations.

Causes System FaultExamples Lack of spaceAbnormal thoracic, abdominal, or amniotic cavity contents Abnormal vascular supplyPulmonary valve or artery stenosis Tetralogy of Fallot Neuromuscular diseaseCNS, anterior horn cell, peripheral nerve, or muscle disease (particularly severe spinal muscular atrophy and myotonic dystrophy inherited from mother) reducing fetal breathing movements. Table 1. Causes of bilateral congenital small lungs

Table 2. Congenital small lungs due to extrapulmonary mechanical factors Extrapulmonary mechanical factors Examples Abnormal thoracic contentsDiaphragmatic hernia Pleural effusion Large congenital thoracic malformation Thoracic compression from belowAbdominal tumors Ascites Thoracic compression from the sideAmniotic bands Oligohydramnions Asphyxiating dystrophy/scoliosis or other chest wall deformity

Clinical finding Best considered to be present in term babies Bilateral pulmonary agenesis  Severe respiratory distress and failure  Usually associated with other abnormalities Unilateral pulmonary agenesis  Few symptoms and non spesific finding. Persistent pulmonary hypertension is often present when pulmonary hypoplasia presents in the newborn period.

Treatment No specific treatment Supportive measures :  Mechanical ventilation  Supplemental oxygen Antibiotics and annual influenza vaccine for older patient.

Prognosis The prognosis is worst for right sided agenesis owing to a higher incidence of associated life threatening malformations and a higher rate of infection due to spillage into the collateral lung. Death may also result from complications of pulmonary hypertension associated with congenital heart disease.

HELMI LUBIS RIDWAN M. DAULAY WISMAN DALIMUNTHE RINI SAVITRI DAULAY DIAPHRAGMATIC HERNIA

Herniation of abdominal content into the thoracic cavity  congenital or traumatic defect in the diaphragm Defect:  Esophageal hiatus (hiatal)  Adjacent to the hiatus (paraesophageal)  Retrosternal (Morgagni)  Posterolateral (Bochladeck)

Epidemiology Incidence: 1 in 5000 lives birth Defect:  Common: left (70-85%)  Bilateral (5%)

Etiology Separation of developing thoracic and abdominal cavities  closure of the posterolateral pleuroperitoneal canal  8 th week of gestation Failure of closure  congenital posterolateral diaphragmatic hernia Diaphragm defect:  Small  Entire diaphragm

Clinical Manifestation Majority:  Severe respiratory distress (first hour of life) Delayed presentation:  Vomiting  intestinal obstuction  Mild respiratory simptom Occasionally:  Ischemia  incarceration of the intestine  Sepsis  Cardiorespiratory collapse Unrecognized:  Sudden death

Diagnosis Prenatal :  USG  Echocardiography  Amniocentesis After birth:  Clinical: severe respiratory collapse (first 24 hours), absence of breath sound, shift of heart sound, scaphoid abdomen  Radiology:  CXR  lateral: intestine passing the through posterior portion of diaphragm  USG & Fluoroscopy  distuingish true hernia and evantratio  CT Scan  exclude pneumatocele

Treatment ECMO (Extracorporeal membrane oxygenation) Preoperative stabilization:  Sedation  Paralysis  Modest hyperventilation Operative

Prognosis Mortality rate after birth: 7-10% ECMO: 40-60% survive Poor prognosis:  Large anomaly  Symptoms occur in first 24 hours  Severe respiratory distress Recurency: 20-40% in first year

HELMI LUBIS RIDWAN M. DAULAY WISMAN DALIMUNTHE RINI SAVITRI DAULAY EVENTRATION DIAPRAGM

Etiology:  Thinned diaphragm muscle  Traumatic Classification:  Congenital or acquired  Complete or partial  Unilateral or bilateral  Infant or adult

Clinical manifestation:  Small  asymptomatic  Large:  Dispnoe, tachypnoe, cyanosis  Shift of trachea and heart  Scaphoid abdomen  Vomit  Recurrent respiratory tract infection

Diagnosis:  Radiology:  CXR: lift of diaphragm  Fluoroscopy: paradoxal esophageal reflux Treatment:  Surgical plication  may be indicated Prognosis: unpredictable

HELMI LUBIS RIDWAN M. DAULAY WISMAN DALIMUNTHE RINI SAVITRI DAULAY DIAPHRAGMATIC PARALYSIS

Injury of phrenic nerve  usually post operative Diaphragm function usually return in 1-2 mo, unless the nerve completely diveded Surgical plication  may be indicated