Kelainan kongenital 2010.ppt

7/27/2019 Kelainan kongenital 2010.ppt

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H E L M I L U B I S

RIDWAN M. DAULAY

W I S M A N D A L I M U N T H E

RIN I S AV IT R I DAUL AY

HYPOPLASIA OF THE

LUNG


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Pulmonary hypoplasia (small lung) defined aslung weight more than 2 SD below the normal forage (or gestational age)

Almost always accompanied by hypoplasia of thecorresponding pulmonary vessel

Hypoplasia as an isolated phenomenon is rare


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Causes

Condition leading to an egress of lungfluid

Severe oligohydramnion (frompremature rupture of membranes,bilateral renal agenesis, urinary tractobstruction)

Compression of thoracic cage andabdominal contents by the uteruslimitation of breathing movement

SOL Congenital diaphragmatic herniaLung malformationThoracic tumorPleural effusionAbdominal condition pressing on thediaphragma (massive ascites)

Thoracic cage anomalies AchondroplasiaScoliosis

Table 1. Most common causes of Pulmonary Hypoplasia


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Conditions preventing normal fetalbreathing movements

AnencephalyPhrenic nerve agenesis

Thoracic compression from below Abdominal tumorsAscites

Thoracic compression from the side Amniotic bandsOligohydramnions

Asphyxiating dystrophy/scoliosis orother chest wall deformity


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Clinical Finding

Early infancy respiratory distress ( mild tosevere depending on the degree of hypoplasia)

Severe bilateral hypoplasia

thoracic cagereduced in size bell shaped


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Diagnosis

Chest X ray:

Ribs may appear crowded

Low thoracic to abdominal ratio

Isotope scanning

perfusion>ventilation onthe side of the lesion


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Treatment

No specific treatment

Supportive measures :

Mechanical ventilation Supplemental oxygen


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Prognosis

Infants who remain on high pressure ventilation andhigh inspired oxygen concentration at the end of the

first week extremely bad prognostic


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H E L M I L U B I S

RIDWAN M. DAULAYW I S M A N D A L I M U N T H E


CONGENITAL

DIAPHRAGMATIC HERNIA


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Definition: developmental abnormality of thediaphragm that allow abdominal viscera to enter thethoracic cavity

Defect: Most common: posterolateral (Bochladeck) 90% on the left

side, 10% on the right side and 1% bilateral

Retrosternal (Morgagni)


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Epidemiology

Incidence:

1 in 2000-4000 lives birth

: = 1,5:1


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Pathogenesis

Premature migration of the gut into the abdominalcavity after the periode of extracoelomic growth(compression theory)

Abnormal lung development/hypoplasia whichpermits the herniation of the gut into the chest

Problem with phrenic nerve development leading toincomplete formation of the diaphragm

Delayed closure of the pleuroperitoneal fold


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Prenatal Diagnosis

USG

MRI


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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


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Initial Management

Avoiding bag and mask ventilationminimizegaseous 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


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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


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Treatment

Preoperative stabilization: Intubated

Mechanical ventilation : Peak inspiratory pressure


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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


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Congenital Lobar

Emphysema

H E L M I L U B I S

RIDWAN M. DAULAYW I S M A N D A L I M U N T H E



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Congenital Lobar Emphysema

Pulmonary emphysema

distention of airspaces with irreversibledisruption of the alveolar septa

Rare condition


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Etiology

Some cases are caused by easily identifiable partialobstruction such as mucosal flaps

In many cases deficiency of bronchial cartilage is

thought to be the cause


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Histology

Majority normal radial alveolar counts but with noapparent maturation with age when compared to age-matched control


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Affected lobe:

Left upper (42%)

Right middle (35%)

Right upper (21%)

Lower lobes (2%).

The affected lobe cannot deflate, but displaceadjacent lobes and subsequently the mediastinalstructure


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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


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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 maybe collapsed

Occasionally, initial chest radiograph may demonstrate an

opaque lung field then clears and the affected lung becomes

overinflated and hyperlucent


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....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 thecontralateral hemithorax


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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


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Treatment

Conservatively children who do not sufferrespiratory compromise

Lobectomy is indicated in the event of respiratorydistress


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Kelainan kongenital 2010.ppt