Congenital diaphragmatic hernia
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Congenital Diaphragmatic Hernia. R1 謝佩芳. 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.

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History l.jpg
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


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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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  • Cardiac Sonography

    • PFO

    • PDA

    • mild to moderate TR

    • pulmonary hypertension

  • Brain Sonography

    • Within normal limit


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Initial Management

  • Intubation

  • Ventilator support with permissive hypercapnia

  • Monitor preductal SpO2 and keep > 90%

  • Right side down

  • Continuous OG decompression



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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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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


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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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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


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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


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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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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


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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 cmH2O

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


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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



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Management 2002; 166: 911-915

  • ECMO

  • Inhaled Nitric Oxide

  • Exogenous Surfactant Therapy

Paediatric Respiratory Reviews 2002; 3: 339-348


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Delayed Surgery and 2002; 166: 911-915Preoperative 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 and 2002; 166: 911-915Preoperative 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 2002; 166: 911-915

  • 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 2002; 166: 911-915

  • 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 distention

Miller's Anesthesia, 5th ed.


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Reference 2002; 166: 911-915

  • Nelson Textbook of Pediatrics, 17th ed., 2004

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

  • Paediatric Respiratory Reviews 2002; 3: 339-348

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

  • Paediatric Anaesthesia 2000; 10:377-379

  • Miller's Anesthesia, 5th ed.


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