1. Congenital Diaphragmatic Hernia R1 謝佩芳

2. History Maternal history: 28 y/o mother, G1P1, no systemic disease, no drug or radiation exposure during pregnancy No congenital anomaly family history GA: 37+6 weeks, C/S

3. History BBW: 2862 gm ( 50-75th percentile), BBL: 49.5cm ( 50-75th percentile ) Apgar score 5(1’)-->7(5 ’ ) PROM(-), DOIC(-), Perinatal insult(- ), meconium stain(-)

4. History Prenatal examination at GA 32 wk: fetal diaphragmatic hernia with right lung agenesis, liver herniation After birth: General cyanosis, scaphoid abdomen

5. CXR at Birth

6. Cardiac Sonography PFO PDA mild to moderate TR pulmonary hypertension Brain Sonography Within normal limit

7. Initial Management Intubation Ventilator support with permissive hypercapnia Monitor preductal SpO2 and keep > 90% Right side down Continuous OG decompression

8. Ventilator Setting Date Mod e RRFiO2 PEEP PIPMAPpH PaCO 2 PaO2 HCO 3 SpO2 9/6IMV40 100 - ->45 5219.47.26446.371.421.192 9/7IMV40555219.67.34845.681.725.289 9/8IMV403052210.399

9. Surgery Supine postion Skin incision at sobcostal area Pull the liver and intestine back to abdominal cavity Use mesh repair

10. Post-OP Course DateModeR FiO2 PEEP PIPMAPpH PaCO 2 PaO2HCO3SpO2 9/9IMV400.355188.87.443377.422.598 9/10~ 16 IMV40 0.35 --> 0.21 5 15~198~992~98 9/16 Extubatio n --> NCPAP 0.2152210.399 9/29 O2 cannul a 0.21 98 ~ 100 9/30 Room air 0.21

11. Congenital Diaphragmatic Hernia

12. 1:2500 births The majority are sporadic 70-85% on the left side Malrotation of the intestine Some degree of pulmonary hypoplasia Epidemiology Nelson Textbook of Pediatrics, 17th ed., 2004

13. Associated Anomalies CNS lesions Esophageal atresia Omphalocele CVS lesions Syndromes: trisomy 21, 13, 18, Fryn, Brachmann-de Lange, Pallister-Killian Nelson Textbook of Pediatrics, 17th ed., 2004

14. Pathology Not limited to the diaphragm Hypoplastic lungs: decrease in the number of alveoli and bronchial generation abnormal pulmonary vasculature (decrease in volume and increase in muscular mass) Pulmonary hypertension Nelson Textbook of Pediatrics, 17th ed., 2004

15. Diagnosis Prenatal diagnosis ultrasonography Evaluation of other anomalies: echocardiography, amniocentesis Postnatal diagnosis Severe respiratory distress Scaphoid abdomen Mediastinal shift away from the side of the lesion X-ray

17. Management “Gentle handling” Avoid iatrogenic lung damage Delayed surgery following stabilization of the patient Paediatric respiratory Reviews 2004;5(Suppl A): S277–S282

18. Management Intubation Gentle ventilation No attempt should be made to expand the hypoplastic lung NG tube, A-line, CVC American Journal of Respiratory and Critical Care Medicine 2002; 166: 911-915

19. Management Objective of Positive-pressure Ventilation PIP ≦ 25 cm H2O Preductal SaO2 ≧ 85% Tolerating hypercapnia (PaCO2 45-55 mmHg) if necessary as long as there is a compensated pH (> 7.35) American Journal of Respiratory and Critical Care Medicine 2002; 166: 911-915

20. Management Permissive Hypercapnia Avoid the use of sedation and allow the infant to breathe spontaneously on the ventilator PIP is strictly limited (<30 ch H2O) to avoid barotrauma PEEP is used to to maximize alveolar recruitment Paediatric Respiratory Reviews 2002; 3: 339-348

21. Management High frequency oscillatory ventilation (HFOV) To limit lung injury when PIP ≧ 25 cm H2O using conventional ventilation Avoiding barotrauma Improved survival with deferred surgery MAP ≦ 14-16 cmH2O American Journal of Respiratory and Critical Care Medicine 2002; 166: 911-915

22. Management High frequency oscillatory ventilation (HFOV) Infants born with CDH have pulmonary hypoplasia with increased pulmonary vascular resistance and surfactant deficiency. They are very susceptible to barotrauma from the ventilator pressures required to achieve adequate oxygenation and CO2 elimination with CMV and require high FiO2. A safe form of mechanical ventilation during CDH repair Paediatric Anaesthesia 2000; 10:377-379

23. American Journal of Respiratory and Critical Care Medicine 2002; 166: 911-915

24. Management ECMO Inhaled Nitric Oxide Exogenous Surfactant Therapy Paediatric Respiratory Reviews 2002; 3: 339-348

25. Delayed Surgery and Preoperative Stabilization No conformity about the optimal timing May range from several hours in patients with very modest ventilatory needs to several weeks in patients who had been subjected to ECMO therapy and weaned off successfully Paediatric Respiratory Reviews 2004; 5(Suppl A): S277–S282

26. Delayed Surgery and Preoperative Stabilization Stable pulmonary vascular resistance without significant right-to-left shunt If stabilization is not possible, most infants will require ECMO support. Timing for repair of the diaphragm on ECMO is controversial. Nelson Textbook of Pediatrics, 17th ed., 2004

27. Anesthetic Consideration Awake intubation without bag-and- mask-assisted ventilation A-line Blunting the stress response: analgesia with narcotics and by controlling respiration with muscle relaxant Miller's Anesthesia, 5th ed.

28. Anesthetic Consideration Careful control of ventilation and oxygenation prevents sudden increases in pulmonary artery pressure (PaCO2 100 mm Hg) Avoid hypothermia in order to decrease the oxygen consumption needed for thermogenesis Anesthetic agents that could depress the myocardium are avoided until the chest is decompressed. Avoid nitrous oxide to prevent bowel distention Miller's Anesthesia, 5th ed.

29. Reference 1. Nelson Textbook of Pediatrics, 17th ed., 2004 2. Paediatric Respiratory Reviews 2004; 5(Suppl A): S277–S282 3. Paediatric Respiratory Reviews 2002; 3: 339-348 4. American Journal of Respiratory and Critical Care Medicine 2002; 166: 911- 915 5. Paediatric Anaesthesia 2000; 10:377- 379 6. Miller's Anesthesia, 5th ed.