Congenital Diaphragmatic Hernia

R1

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

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(-)

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

CXR at Birth

Cardiac Sonography
PFO PDA mild to moderate TR pulmonary hypertension

Brain Sonography
Within normal limit

Initial Management
Intubation
Ventilator support with permissive hypercapnia Monitor preductal SpO2 and keep > 90%

Right side down

Continuous OG decompression

Ventilator Setting
Date Mod e RR FiO2 PEEP PIP MAP pH
PaCO 2

PaO2

HCO 3

SpO2

9/6

IMV

40

100 ->45

21

9.4

7.264 46.3

71.4

21.1

92

9/7

IMV

40

55

21

9.6

7.348 45.6

81.7

25.2

89

9/8

IMV

40

30

22

10.3

99

Surgery
Supine postion
Skin incision at sobcostal area

Pull the liver and intestine back to abdominal cavity

Use mesh repair

Post-OP Course
Date 9/9
9/10~ 16

Mode IMV
IMV
Extubatio n --> NCPAP

R R 40
40

FiO2 PEEP 0.35

0.35 --> 0.21

PIP 18
15~19

MAP 8.8
8~9

pH 7.44

PaCO 2

PaO2 HCO3 SpO2

5
5

33

77.4

22.5

98
92~98

9/16

0.21

22

10.3

99

9/29
9/30

O2 cannul a Room air

0.21
0.21

98 ~ 100

Congenital Diaphragmatic Hernia

Epidemiology
1:2500 births

The majority are sporadic

70-85% on the left side

Malrotation of the intestine

Some degree of pulmonary hypoplasia
Nelson Textbook of Pediatrics, 17th ed., 2004

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

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

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

Management
Gentle handling

Avoid iatrogenic lung damage

Delayed surgery following stabilization of the patient
Paediatric respiratory Reviews 2004;5(Suppl A): S277S282

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

Management
Objective of Positive-pressure Ventilation

PIP 25 cm H2O

Preductal SaO285%
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

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

Management
High frequency oscillatory ventilation (HFOV)

To limit lung injury when PIP25 cm H2O using conventional ventilation

Avoiding barotrauma
Improved survival with deferred surgery MAP14-16 cmH2O
American Journal of Respiratory and Critical Care Medicine 2002; 166: 911-915

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

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

Management

ECMO

Inhaled Nitric Oxide

Exogenous Surfactant Therapy
Paediatric Respiratory Reviews 2002; 3: 339-348

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): S277S282

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

Anesthetic Consideration
Awake intubation without bag-andmask-assisted ventilation

A-line
Blunting the stress response: analgesia with narcotics and by controlling respiration with muscle relaxant
Miller's Anesthesia, 5th ed.

Careful control of ventilation and Anesthetic Consideration 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
Miller's Anesthesia, 5th ed.

1. Nelson Textbook of Pediatrics, 17th ed., Reference 2004 2. Paediatric Respiratory Reviews 2004; 5(Suppl A): S277S282

3. Paediatric Respiratory Reviews 2002; 3: 339-348

4. American Journal of Respiratory and Critical Care Medicine 2002; 166: 911915 5. Paediatric Anaesthesia 2000; 10:377379