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

From the Guidelines

The correct statement is (b) foramen of Bochdalek hernia is the most common type, as it accounts for approximately 95% of all congenital diaphragmatic hernia (CDH) cases, occurring more commonly on the posterior left side of the diaphragm. Congenital diaphragmatic hernia (CDH) occurs when there is incomplete formation of the diaphragm during fetal development, allowing abdominal organs to herniate into the chest cavity.

Key Points

• The Bochdalek hernia is the most common type of CDH, with an incidence of 0.17% presenting at an average age of 40 years, as stated in the study by 1.
• While statement (a) is partially correct in that respiratory distress often requires intervention, immediate emergency surgery is not always indicated as initial stabilization of respiratory status is the priority, according to the management guidelines for complicated diaphragmatic hernia in the acute setting: a WSES position paper by 1.
• Statement (c) is incorrect because Morgagni hernias, which occur anteriorly, are typically less symptomatic and often discovered incidentally later in life.
• 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 the condition, as mentioned in the study by 1.

Management of CDH

• Management of CDH typically involves respiratory support, treatment of pulmonary hypertension, and surgical repair once the patient is stabilized, with surgery being the treatment of choice for this condition, as recommended by 1.
• Primary repair for the diaphragmatic defects with non-absorbable sutures should always be attempted when possible, and biologic or biosynthetic meshes can be safely used in clean-contaminated and contaminated diaphragmatic hernia repair, as suggested by 1.

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, with the main focus of management including 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, 5.
• 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 when it does present with symptoms, it can cause acute respiratory distress in the neonatal period 2, 4.
• Is rarely associated with underlying lung pathology:
  • Congenital diaphragmatic hernia is associated with underlying lung pathology, including lung hypoplasia and persistent pulmonary hypertension of the newborn (PPHN), which can affect the survival of an infant with CDH 2, 5, 3.