CONGENITAL%20DIAPHRAGMATIC%20HERNIA%20%20Maj%20Asrar%20Ahmad%20MBBS,%20FCPS

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CONGENITAL DIAPHRAGMATIC
HERNIAMaj Asrar AhmadMBBS, FCPS
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Anatomy
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Embryology
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Pathophysiology
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Pathophysiology

• Pulmonary hypoplasia
• compression theory
• - modeled in fetal lambs
• - rationale for early surgery
• global embryopathy
• - modeled in newborn rats
• - rationale for new therapeutic ideas
• Pulmonary hypertension
• - causes persistent fetal circulation

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Incidence

• 12500-5000 live births
• 1100 cases in the U.S. annually
• 80 Left side
• survival remains around 65

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Diagnosis

• Antenatal
• U/S at 20 weeks gestation
• 60
• Polyhydramnios intrathoracic stomach or liver
  abdominal circumference lung-to-head ratio

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Presentation

• Shortness of breath
• Scaphoid abdomen
• Three general presentations
• Severe respiratory distress at the time of birth.
• Respiratory deterioration hours after delivery
• Benefit from correction of hypoxemia and
  pulmonary hypertension
• Feeding difficulties, chronic respiratory
  disease, pneumonia
• 10-20 intestinal obstruction

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Initial Management

• Oxygenate but avoid barotrauma
• Intubate
• Sedate
• NGT for decompression

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Medical Management

• Medical emergency not surgical
• Pulmonary vasodilators
• Inotropes
• High frequency oscillatory ventilation
• ECMO
• Surfactant
• Antenatal steroids?

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ECMO
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ECMO
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Surgical Management
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Surgical Management
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Surgical Management
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Surgical Management
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Developing Therapies

• Fetal surgery
• PLUG fetal surgery
• Growth factors

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