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HELMI LUBIS RIDWAN M. DAULAY WISMAN DALIMUNTHE RINI SAVITRI DAULAY HYPOPLASIA OF THE LUNG
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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.
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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.
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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.
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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
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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
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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.
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Treatment No specific treatment Supportive measures : Mechanical ventilation Supplemental oxygen Antibiotics and annual influenza vaccine for older patient.
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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.
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HELMI LUBIS RIDWAN M. DAULAY WISMAN DALIMUNTHE RINI SAVITRI DAULAY DIAPHRAGMATIC HERNIA
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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)
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Epidemiology Incidence: 1 in 5000 lives birth Defect: Common: left (70-85%) Bilateral (5%)
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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
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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
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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
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Treatment ECMO (Extracorporeal membrane oxygenation) Preoperative stabilization: Sedation Paralysis Modest hyperventilation Operative
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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
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HELMI LUBIS RIDWAN M. DAULAY WISMAN DALIMUNTHE RINI SAVITRI DAULAY EVENTRATION DIAPRAGM
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Etiology: Thinned diaphragm muscle Traumatic Classification: Congenital or acquired Complete or partial Unilateral or bilateral Infant or adult
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Clinical manifestation: Small asymptomatic Large: Dispnoe, tachypnoe, cyanosis Shift of trachea and heart Scaphoid abdomen Vomit Recurrent respiratory tract infection
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Diagnosis: Radiology: CXR: lift of diaphragm Fluoroscopy: paradoxal esophageal reflux Treatment: Surgical plication may be indicated Prognosis: unpredictable
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HELMI LUBIS RIDWAN M. DAULAY WISMAN DALIMUNTHE RINI SAVITRI DAULAY DIAPHRAGMATIC PARALYSIS
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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
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