1. DIAPHRAGMATIC HERNIA Dr Ho Huyen Emergency Department Children’s Hospital 2

2. 1. OVERVIEW 2. CLINICAL MANIFESTATIONS 3. DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS 4. PROGNOSTIC FACTORS 5. OBSTETRICAL MANAGEMENT 6. NEONATAL MANAGEMENT 7. SUMMARY AND RECOMMENDATIONS

3. OVERVIEW

4. OVERVIEW • 1:2200 births • Almost occurs on the left (Right-sided: 11%, bilateral herniation: 2%) • Advent of antenatal diagnosis and improvement of neonatal care: survival has improved

5. CLINICAL MANIFESTATIONS • Respiratory distress • Barrel-shaped chest • Absence of breath sounds on the ipsilateral side • Heartbeat is displaced to the right • Developing persistent pulmonary hypertension

6. barrel-shaped chest

7. Left-sided diaphragmatic hernia

8. DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS • Diagnosis: • Suspected in any full term infants with respiratory distress especially in the absence of breath sounds • Antenatal ultrasound • Natal chest radiography • Differential diagnosis: includes other causes of pulmonary hypoplasia (primary or secondary)

9. PROGNOSTIC FACTORS • Abnormal karyotype • Severe associated anomalies • Right-sided defect • Liver herniation • Fetal lung volume: Lung area to head circumference ratio

10. OBSTETRICAL MANAGEMENT Suspected CDH on prenatal ultrasound, suggest: • Ultrafast fetal MRI: find abnormalities, liver herniation, estimate lung volumes • Fetal echocardiography • Fetal karyotype

11. NEONATAL MANAGEMENT Initial treatment: •  In the delivery room: • immediately intubated • blow-by oxygen and/or bag-masking are contraindicated • ventilated with low peak pressure (goal<25 cm H2O) to minimize lung injury • nasogastric tube  Any delay in obtaining an airway can intensify the resultant acidosis and hypoxia, which can increase the risk of pulmonary hypertension.

12. NEONATAL MANAGEMENT • Ventilation: • PIP: 20 to 25 cm H2O • PEEP: should be maintained at physiologic levels (3 to 5 cm H2O) • Preductal SaO2: > 80% • PaO2 : > 60 mmHg • Treatment: surgery Retrospective review: the survival rate improved in patients whose delay surgery compared to immediate surgical repair

13. SUMMARY • CDH is a developmental defect in the diaphragm • The morbidity and mortality of CDH is related to the severity of lung hypoplasia, pulmonary hypertension and associated anomalies • Most case often develop respiratory distress in the first few hours or days of life • Diagnosis: prenatal ultrasound, natal X-ray

14. RECOMMENDATIONS • Immediate endotracheal intubation(Grade 2C). NOT using blow-by oxygen and/or bag-masking (Grade 1C) • Ventilatory support with minimal airway pressure to maintain preductal SaO2 > 80% and PaO2 > 60 mm Hg (Grade 2C). • Other supportive: nasogastric tube, maintenance of the mean arterial blood pressure >50 mm Hg. • Timing of surgical: unclear, dependent on the severity of the respiratory distress

15. Thank you!