A congenital diaphragmatic hernia (CDH) occurs through an embryologic defects in the diaphragm.
Various types of CDH -
• Bochdalek hernia
• Morgagni hernia
• Diaphragm eventration and
• Central tendon defects of the diaphragm
Pathogenesis -
There are 4 basic embryologic components responsible for formation of the diaphragm -
• Septum transversum
• Two pleuroperitoneal folds
• Cervical myotomes
• Dorsal mesentery
Failure of the development of the pleuroperitoneal folds and subsequent muscle migration is the main cause of CDH.
Malformation of the diaphragm allows the abdominal organs to herniate into the defect and hinders ipsilateral lung development.
Bochdalek hernia - It is posterolateral defects of the diaphragm, resulting in either failure in the development of the pleuroperitoneal folds or absent migration of the diaphragmatic musculature.
About 90% patients present in the neonatal period or within the first year of life. These cases have a mortality of nearly 50%. Most of the morbidity and mortality of CDH is due to hypoplasia of the lung and pulmonary hypertension on the affected side. Hence, early diagnosis and management is important for survival.
Most of Bochdalek hernias (80–85%) occur on the left side of the diaphragm.
Presentation -
Right-sided heart; decreased breath sounds on the affected side; scaphoid abdomen; bowel sounds in the thorax, respiratory distress, and/or cyanosis on auscultation.
Classical triad - Respiratory distress, Dexrocardia, Scaphoid abdomen.
Main cause of morbidity and mortality - Pulmonary hypoplasia and pulmonary hyerptension.
Diagnosis -
• Low levels of maternal serum alpha-fetoprotein (AFP)
• X-ray - bowel and stomach in the chest cavity and shift of the mediastinum (usually to the right)
• USG criteria -
1. Polyhydramnios
2. Absent or intrathoracic stomach bubble
3. Mediastinal and cardiac shift away from the side of herniation.
4. Fetal hydrops (rare)
Treatment -
• High frequency oscillatory ventilation
• ECMO (reserved for individuals whose condition fails to improve with both HFOV and conventional mechanical ventilation)
• Early repair is not indicated and should be delayed until the infant is better prepared to survive the operation.
• For treatment of CDH in the neonatal period, a subcostal incision is used. In primary repair of the hernia. The abdominal contents are reduced, and the edges of the diaphragm are then approximated with nonabsorbable suture.
• For large defects prosthetic material such as expanded polytetrafluoroethylene (ePTFE) or polypropylene is used.
Various types of CDH -
• Bochdalek hernia
• Morgagni hernia
• Diaphragm eventration and
• Central tendon defects of the diaphragm
Pathogenesis -
There are 4 basic embryologic components responsible for formation of the diaphragm -
• Septum transversum
• Two pleuroperitoneal folds
• Cervical myotomes
• Dorsal mesentery
Failure of the development of the pleuroperitoneal folds and subsequent muscle migration is the main cause of CDH.
Malformation of the diaphragm allows the abdominal organs to herniate into the defect and hinders ipsilateral lung development.
Bochdalek hernia - It is posterolateral defects of the diaphragm, resulting in either failure in the development of the pleuroperitoneal folds or absent migration of the diaphragmatic musculature.
About 90% patients present in the neonatal period or within the first year of life. These cases have a mortality of nearly 50%. Most of the morbidity and mortality of CDH is due to hypoplasia of the lung and pulmonary hypertension on the affected side. Hence, early diagnosis and management is important for survival.
Most of Bochdalek hernias (80–85%) occur on the left side of the diaphragm.
Presentation -
Right-sided heart; decreased breath sounds on the affected side; scaphoid abdomen; bowel sounds in the thorax, respiratory distress, and/or cyanosis on auscultation.
Classical triad - Respiratory distress, Dexrocardia, Scaphoid abdomen.
Main cause of morbidity and mortality - Pulmonary hypoplasia and pulmonary hyerptension.
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• Low levels of maternal serum alpha-fetoprotein (AFP)
• X-ray - bowel and stomach in the chest cavity and shift of the mediastinum (usually to the right)
• USG criteria -
1. Polyhydramnios
2. Absent or intrathoracic stomach bubble
3. Mediastinal and cardiac shift away from the side of herniation.
4. Fetal hydrops (rare)
Treatment -
• High frequency oscillatory ventilation
• ECMO (reserved for individuals whose condition fails to improve with both HFOV and conventional mechanical ventilation)
• Early repair is not indicated and should be delayed until the infant is better prepared to survive the operation.
• For treatment of CDH in the neonatal period, a subcostal incision is used. In primary repair of the hernia. The abdominal contents are reduced, and the edges of the diaphragm are then approximated with nonabsorbable suture.
• For large defects prosthetic material such as expanded polytetrafluoroethylene (ePTFE) or polypropylene is used.
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