congenital diaphragmatic hernia r1 謝佩芳. history maternal history: 28 y/o mother, g1p1, no...

Congenital Diaphragmatic

Hernia

Congenital Diaphragmatic

HerniaR1 謝佩芳R1 謝佩芳

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

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

PFOPDAmild to moderate TRpulmonary hypertension

Brain Sonography

Within normal limit

Initial ManagementIntubation

Ventilator support with permissive hypercapnia

Monitor preductal SpO2 and keep > 90%

Right side down

Continuous OG decompression

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

Surgery

Supine postion

Skin incision at sobcostal area

Pull the liver and intestine back to abdominal cavity

Use mesh repair

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

Congenital Diaphragmatic

Hernia

Congenital Diaphragmatic

Hernia

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

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

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

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

Management

“Gentle handling”

Avoid iatrogenic lung damage

Delayed surgery following stabilization of the patient

Paediatric respiratory Reviews 2004;5(Suppl A): S277–S282

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 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

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

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

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

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 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

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

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.

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.

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.