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Congenital Diaphragmatic
Hernia
Congenital Diaphragmatic
HerniaR1 謝佩芳R1 謝佩芳
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HistoryMaternal 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
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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(-)
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History
Prenatal examination at GA 32 wk: fetal diaphragmatic hernia with right lung agenesis, liver herniation
After birth: General cyanosis, scaphoid abdomen
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CXR at Birth
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Cardiac Sonography
PFOPDAmild to moderate TRpulmonary hypertension
Brain Sonography
Within normal limit
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Initial ManagementIntubation
Ventilator support with permissive hypercapnia
Monitor preductal SpO2 and keep > 90%
Right side down
Continuous OG decompression
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Ventilator Setting
DateMod
eRR FiO2 PEEP PIP MAP pH PaCO
2 PaO2HCO
3SpO2
9/6 IMV 40100
-->455 21 9.4 7.264 46.3 71.4 21.1 92
9/7 IMV 40 55 5 21 9.6 7.348 45.6 81.7 25.2 89
9/8 IMV 40 30 5 22 10.3 99
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Surgery
Supine postion
Skin incision at sobcostal area
Pull the liver and intestine back to abdominal cavity
Use mesh repair
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Post-OP CourseDate Mode
RR
FiO2 PEEP PIP MAP pH PaCO2 PaO2 HCO3 SpO2
9/9 IMV 40 0.35 5 18 8.8 7.44 33 77.4 22.5 98
9/10~16 IMV 40
0.35-->
0.215 15~19 8~9 92~98
9/16
Extubation
-->NCPAP
0.21 5 22 10.3 99
9/29O2
cannula
0.21 98~
1009/30
Room air
0.21
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Congenital Diaphragmatic
Hernia
Congenital Diaphragmatic
Hernia
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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
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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
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PathologyNot 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 hypertensionNelson Textbook of Pediatrics, 17th ed., 2004
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DiagnosisPrenatal 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
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Management
“Gentle handling”
Avoid iatrogenic lung damage
Delayed surgery following stabilization of the patient
Paediatric respiratory Reviews 2004;5(Suppl A): S277–S282
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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
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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
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ManagementPermissive 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
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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 cmH2OAmerican Journal of Respiratory and Critical Care Medicine 2002; 166: 911-915
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ManagementHigh 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
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American Journal of Respiratory and Critical Care Medicine 2002; 166: 911-915
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Management
ECMO
Inhaled Nitric Oxide
Exogenous Surfactant Therapy
Paediatric Respiratory Reviews 2002; 3: 339-348
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Delayed Surgery andPreoperative 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
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Delayed Surgery andPreoperative 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
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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.
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Anesthetic Consideration
Careful control of ventilation and oxygenation prevents sudden increases in pulmonary artery pressure (PaCO2 < 40 mmHg and PaO2 > 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 distentionMiller's Anesthesia, 5th ed.
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Reference1. 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.