What are the characteristics and clinical presentations of congenital diaphragmatic hernia (CDH)?

From the Guidelines

Regarding congenital diaphragmatic hernia, the correct answer is (b) foramen of Bochdalek hernia is the most common type. Congenital diaphragmatic hernia (CDH) occurs when there is a defect in the diaphragm that allows abdominal organs to herniate into the chest cavity. The Bochdalek hernia, which occurs at the posterolateral aspect of the diaphragm, accounts for approximately 70-90% of all CDH cases 1. While statement (a) is partially true in that severe respiratory distress may require urgent intervention, not all cases require emergency surgery immediately after birth, as some infants benefit from preoperative stabilization. Statement (c) is incorrect because Morgagni hernias, which occur anteriorly, are typically less symptomatic and often present later in life or are found incidentally. Statement (d) is false because CDH is commonly associated with pulmonary hypoplasia and pulmonary hypertension, which are significant underlying lung pathologies that contribute to the morbidity and mortality of affected infants, as reported in a study by the American Heart Association and American Thoracic Society 1.

Some key points to consider in the management of CDH include:

• The use of biological and bioabsorbable meshes to reduce recurrence 1
• The importance of preoperative assessment and prompt surgical intervention 1
• The role of laparoscopic repair in reducing postoperative complications 1
• The high incidence of gastroesophageal reflux after repair of CDH, which may require fundoplication 1
• The potential for complications such as respiratory failure, ileus, and cardiac injury 1
• The importance of proper fixation of the prosthetic material to the defect edges to prevent recurrence 1

Overall, the management of CDH requires a multidisciplinary approach and careful consideration of the individual patient's needs and circumstances.

From the Research

Congenital Diaphragmatic Hernia

• Requires emergency operation if respiratory distress is present:
  • Infants with congenital diaphragmatic hernia may experience severe respiratory distress within minutes of birth and may require resuscitation and stabilization of cardio-pulmonary function prior to surgery 2.
  • Immediate management at birth includes bowel decompression, avoidance of mask ventilation and endotracheal tube placement if required, and the main focus of management includes gentle ventilation, hemodynamic monitoring and treatment of pulmonary hypertension followed by surgery 3.
• Foramen of Bochdalek hernia is the most common type:
  • The foramen of Bochdalek hernia is the most common type of congenital diaphragmatic hernia, where abdominal contents pass into the thoracic cavity through a diaphragmatic defect, limiting the space available for the developing lungs 2, 4.
• Foramen of Morgagni hernia presents with respiratory distress:
  • The Morgagni hernia is rare and often incidentally diagnosed on routine chest x-ray in asymptomatic patients, but it can present with respiratory distress in some cases, especially in the neonatal period 2, 5.
• Is rarely associated with underlying lung pathology:
  • Congenital diaphragmatic hernia is associated with underlying lung pathology, including pulmonary hypertension and pulmonary hypoplasia, which are recognized as the two most important factors in the pathophysiology of congenital diaphragmatic hernia 4, 3.